An Introduction and Practical Guide
Dizziness and vertigo are common symptoms. Patients may present to general practitioners,
ENT surgeons, neurologists or general medicine specialists but are often poorly managed.
Dizziness and Vertigo: An Introduction and Practical Guide is an essential text which
contains all the basic knowledge and practical skills necessary for managing patients
with these symptoms. It provides a comprehensive overview of dizziness and vertigo,
how to accurately diagnose patients and how to treat them.
Dizziness and Vertigo
Dizziness
and
Vertigo
Dizziness
and
Vertigo
An Introduction
and Practical Guide
An Introduction and Practical Guide
Key features
• Concise, practical and easy to read
• Highly illustrated throughout to aid understanding
• Written by experts in the field
• Companion volume to the successful ENT: An Introduction
and Practical Guide, from the same editors
Rahul G Kanegaonkar FRCS(ORL-HNS) is a Consultant in Otolaryngology at
Medway Maritime Hospital, Kent, UK, and an Honorary Senior Lecturer in Otorhinolaryngology at the Anglia Ruskin University.
James R Tysome MA, PhD, FRCS(ORL-HNS) is a Consultant in Otolaryngology
and Skull Base Surgery at Addenbrooke’s Hospital, Cambridge, UK.
Kanegaonkar
Tysome
K17350
Edited by
Rahul G. Kanegaonkar
James R. Tysome
ISBN: 978-1-4441-8268-2
90000
9 781444 182682
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Dizziness
anD
Vertigo
An IntroductIon
And PrActIcAl GuIde
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Dizziness
anD
Vertigo
An IntroductIon
And PrActIcAl GuIde
Edited by
rahul G Kanegaonkar, FrcS(orl-HnS)
Consultant in otolaryngology, Medway Maritime Hospital, Kent, UK
Honorary senior Lecturer in otorhinolaryngology, anglia ruskin University, Chelmsford, UK
James r tysome MA, Phd, FrcS(orl-HnS)
Consultant in otolaryngology and skull Base surgery, addenbrooke’s Hospital, Cambridge, UK
Boca Raton London New York
CRC Press is an imprint of the
Taylor & Francis Group, an informa business
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CRC Press
Taylor & Francis Group
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© 2014 by Taylor & Francis Group, LLC
CRC Press is an imprint of Taylor & Francis Group, an Informa business
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Version Date: 20140121
International Standard Book Number-13: 978-1-4441-8269-9 (eBook - PDF)
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To,
Dipalee, Amee and Deven
and
Laura, George, Henry and Max
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Contents
Preface
List of contributors
General Introduction
James Tysome and Rahul Kanegaonkar
1 Anatomy and physiology of the peripheral vestibular system
Rahul Kanegaonkar
ix
xi
xv
1
2 Clinical assessment of vertigo
Mudit Jindal and Aanand Acharya
11
3 Imaging in dizziness and vertigo
Neshe Sriskandan and Steve Connor
19
4 Special investigations used in the assessment of the dizzy patient 33
Presanna Premachandra
5 Differential diagnosis
Rahul Kanegaonkar
47
6 Benign paroxysmal positional vertigo
Nitesh Patel
49
7 Acute peripheral vestibular loss
Ambrose Lee
61
8 Vestibular migraine
Nitesh Patel
73
9 Multilevel vestibulopathy
Mudit Jindal
79
10 Cholesteatoma
Attila Dezso
83
11 Ménière’s disease
Neil Donnelly
89
12 Superior semicircular canal dehiscence
James Rainsbury and Richard Irving
101
13 Vestibular schwannoma
James Tysome
109
vii
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14 Perilymph fistula
Richard Gurgel
113
15 Central pathology causing dizziness
C. Eduardo Corrales
117
16 Systemic conditions affecting balance
Stephen Broomfield
125
17 Vestibular rehabilitation – principles and practice
Rachel Ritchie
135
18 Psychological aspects of dizziness
Raj Attavar and Amalsha Vithanaarachichi
145
Index
153
viii Contents
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Preface
It is with great sadness that many patients with dizziness and vertigo have
been told that there is little that can be done for them, and that they simply
have to live with their symptoms.
This book hopes to challenge these misconceptions.
The majority of dizzy patients can be cured.
The emotional burden associated with a balance disorder should never
be underestimated and symptoms of anxiety or depression must also be
addressed in order to achieve a cure.
We hope that this book will inspire doctors, and change perspectives such
that medical practitioners look kindly and sympathetically upon dizzy
patients. Arriving at a diagnosis may be challenging, but initiating appropriate treatment will transform the quality of life of many patients and
their families.
Rahul Kanegaonkar and James Tysome
ix
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List of contributors
Editors
Rahul Kanegaonkar FRCS (ORL-HNS)
Consultant Ear, Nose and Throat Surgeon
Medway NHS Foundation Trust
Kent, UK
Honorary Senior Lecturer
Postgraduate Medical Institute
Chelmsford Campus
Anglia Ruskin University
Chelmsford, UK
James R. Tysome PhD FRCS (ORL-HNS)
Consultant Ear, Nose and Throat and Skull Base Surgeon
Cambridge University Hospitals NHS Foundation Trust
Cambridge, UK
Contributors
Aanand Acharya FRCS (ORL-HNS)
Specialist Registrar in Otorhinolaryngology
West Midlands Rotation, UK
Raj Attavar MRCPsych
Consultant Psychiatrist
Southern Health NHS Trust
Buckinghamshire, UK
Stephen Broomfield FRCS (ORL-HNS)
Consultant ENT Surgeon
North Bristol NHS Trust
Bristol, UK
Steve Connor FRCR
Consultant Radiologist
Guy’s and St Thomas’ NHS Foundation Trust
London, UK
xi
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C. Eduardo Corrales MD
Neurotology Fellow
Otolaryngology – Head and Neck Surgery
Stanford University School of Medicine
California, USA
Attila Deszo FRCS (ORL-HNS)
Consultant Otologist and Implant Surgeon
Walsall Healthcare NHS Trust
West Midlands, UK
Neil Donnelly MSc FRCS (ORL-HNS)
Consultant Otoneurological and Skull Base Surgeon
Cambridge University Hospitals NHS Foundation Trust
Cambridge, UK
Richard Gurgel MD
Assistant Professor
Division of Otolaryngology – Head and Neck Surgery
University of Utah
Utah, USA
Richard Irving MD FRCS
Consultant in Otology, Neurotology and Skull Base Surgery
Queen Elizabeth Hospital Birmingham
Birmingham Children’s Hospital
Birmingham, UK
Mudit Jindal FRCS (ORL-HNS)
Consultant ENT Surgeon
Russell Hall Hospital
Dudley
West Midlands, UK
Ambrose Lee MD MSc CCFP(EM) FCFP FRCS(C)
Consultant Otologist
Toronto General Hospital
Toronto, Canada
Nitesh Patel FRCS (ORL-HNS)
Consultant ENT Surgeon and Clinical Lead
Whipps Cross and Newham University Hospitals, UK
Presanna Premachandra MSc
Principal Audiologist and Lead for Adult Rehabilitation and Diagnostics
Guy’s and St Thomas’ NHS Foundation Trust
London, UK
xii List of contributors
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James Rainsbury FRCS (ORL-HNS)
Consultant Ear, Nose and Throat Surgeon
Derriford Hospital
Plymouth, UK
Rachel Ritchie MSc MCSP
Senior Vestibular Physiotherapist
Guy’s and St Thomas’ NHS Foundation Trust
London, UK
Neshe Sriskandan FRCR
Cross-sectional Imaging Fellow in Radiology
Guy’s and St Thomas’ NHS Foundation Trust
London, UK
Amalsha Vithanaarachichi MRCPsych
Specialist Registrar in Psychiatry
Southern Health NHS Trust
Buckinghamshire, UK
List of contributors xiii
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General
introduction
James Tysome and Rahul Kanegaonkar
Dizziness and vertigo are common symptoms. Epidemiological studies
have shown that vertigo and balance disorders affect 30% of the general
population before the age of 65 years. Annually, five out of every thousand
patients present to their general practitioner complaining of symptoms
classified as vertigo, with another ten per thousand with symptoms of
­dizziness or giddiness.
The subject of balance disorders in the elderly becomes critical when
­considering that falls, and the subsequent injuries sustained, are the leading
cause of death in this age group. A survey of patients between the ages of
65 and 75 years, with no major health or balance disturbance history, found
one third reported a significant fall annually, rising to 40% over the age of
75 years. In those, however, with an acute or chronic vestibular ­deficit the
relative risk of a fall was greater still.
Irrespective of age, a separate but relevant issue is that of the adverse
­psychological impact on those with a balance disorder. Two thirds of
patients develop psychiatric disturbances, including depression and
­a nxiety. These clearly limit social and occupational activities which in
turn may lead to a worsening of the vertiginous symptoms experienced.
Patients are often seen by multiple clinicians in different specialties, for
example general practitioners, otolaryngologists, general physicians, and
neurologists. As a result, they may undergo multiple consultations and
investigations before a definitive diagnosis is made and treatment initiated. This delay may have a severe adverse impact on their work, family and
hence quality of life.
General overview
Normal balance function relies on sensory information from the visual,
auditory, peripheral vestibular and somatosensory systems, as well as hearing. This sensory information is integrated, modulated and ‘interpreted’
within the central nervous system to enable gaze and postural stabilization and provide information regarding self and environmental movement
xv
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Input
Integration and
interpretation
Output
Gaze stabilization
Vision
Peripheral vestibular system
Templates
Postural control
Proprioception
Hearing
Spatial
awareness
Figure 0.1. An overview of normal human balance.
(see Figure 0.1). ‘Interpretation’ requires comparing relayed sensory
information with preformed templates within the central nervous system.
Absence of a suitable template, or a mismatch between the two, results in
symptoms of dizziness, vertigo or unsteadiness.
The relative importance of the sensory information used is likely to vary
depending on one’s environment and position. Over reliance on one
particular sensory input may result in disequilibrium and dizziness even
in normal subjects (e.g. vision in subjects with motion sickness). In general, unilateral pathology affecting one sensory input may through central
compensation result in little or no functional deficit, but abnormalities in
more than one sensory input or concomitant central pathology may result
in profound and debilitating vertigo and dizziness.
Hence, the assessment of the vertiginous patient requires a thorough general clinical assessment. As symptoms are often due to peripheral vestibular
pathology, particular emphasis should be placed on the assessment of this
sensory pathway.
xvi General introduction
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1
Anatomy and
physiology of
the peripheral
vestibular system
Rahul Kanegaonkar
Contents
Introduction
Inner ear
Physiology of the vestibular system
Signal transduction
Neural pathways
Vestibular reflexes
References
1
1
4
4
6
6
8
Introduction
The ear is conventionally divided into three
­separate but related anatomical subunits. The
external ear consists of the pinna, external a­ uditory
canal and lateral aspect of the tympanic membrane. The middle ear is an air filled space bounded
­laterally by the tympanic membrane and medially
by the promontory, oval and round windows, and
the horizontal portion of the facial nerve. This cleft
houses the ossicular chain and functions as a transformer mechanism to overcome the impedance
mismatch that occurs when transferring sound
energy from air to fluid.1 The external and middle
ear function to deliver sound energy to the inner
ear (Figure 1.1).
Inner ear
The inner ear is contained within a dense portion of
bone within the petromastoid part of the temporal
bone referred to as the bony labyrinth. Derived from
the otic capsule during early e­ mbryonal development, this structurally complex organ is separated
into two functionally distinct parts; the cochlea
responsible for detecting sound, and the peripheral
vestibular system responsible for detecting static,
linear and angular head movement (Figure 1.2).
The bony labyrinth is filled with perilymph, and
communicates with the cerebrospinal fluid of the
intracranial cavity. Contained within the bony
labyrinth and supported by connective tissue, it is
an anatomically and biochemically distinct closed
structure, the membranous labyrinth. This structure is filled with endolymph and consists of five
confluent but functionally different membranous
segments involved in the detection of movement.
Anatomy and physiology of the peripheral vestibular system 1
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External ear
Middle ear
Internal ear
Pinna
Vestibular nerve
Cochlear nerve
External
auditory
canal
Cochlea
Round window
Eustachian tube
Middle ear ossicles
Figure 1.1. Cross section of the ear.
Oval window
Vestibular apparatus
Spiral lamina
Cochlea
Round window
Figure 1.2. The bony labyrinth.
The saccule and utricle are responsible for detecting
static and linear head movement, while the semicircular canals function to detect head rotation (see
Figure 1.3).
The semicircular canals are orientated in approximately orthogonal planes to each other2 and
organised into three functional pairs: The two
lateral semicircular canals; the superior canal and
the contralateral posterior canal; and the posterior canal and the contralateral superior canal
(note Figure 1.4).
The sensory neuroepithelium responsible for
detecting linear acceleration is limited to specific
regions, the maculae. Whilst the macula of the
saccule is orientated to principally detect linear
acceleration and head tilt in the vertical plane, the
macule of the utricle detects linear acceleration
and head tilt in the horizontal plane.3 The hair
cells of the maculae are arranged in an elaborate
manner and project into a fibro-calcareous sheet,
the otoconial membrane. As this membrane has
a specific gravity greater than the surrounding
endolymph, head tilt and linear movement result
2 Dizziness and Vertigo: An Introduction and Practical Guide
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Vestibular ganglion
Superior semicircular canal
Vestibular nerve
Utricle
Facial nerve
Saccule
Posterior semicircular canal
Lateral semicircular canal
Cochlear nerve
Ampulla
Orientation of hair cells in the utricle
Cochlea
Striola
Orientation of hair cells in the saccule
Figure 1.3. The membranous labyrinth. The maculae of the saccule and utricle are orientated at 90 degrees
to each other in order to detect vertical and horizontal movement. In contrast to the ampullae, hair cells are
arranged around a curvilinear depression of the otoconial membrane, the striola. Arrows indicate the direction of maximal stimulation for both neuroepithelial regions.
A
Superior
semicircular
canal
Utricle
R
L
Lateral
semicircular
canal
Posterior
semicircular
canal
P
Figure 1.4. The orthogonal relationship of the semicircular canals.
Anatomy and physiology of the peripheral vestibular system 3
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Striola
Otoconia
Stereocilia
Gel layer
Reticular membrane
Hair cell
Supporting cells
Gravitational force
Figure 1.5. The otoconial membrane. Static head position and movement results in a relative movement of
the relatively more dense otoconial membrane.4
in the otoconial membrane moving relative to the
underlying hair cells. The shearing force produced
causes depolarisation of the underlying hair cells
(Figure 1.5).
The sensory neuroepithelium of the semicircular canals is limited to a dilated segment of each
bony and membranous labyrinth, the ampulla.
A crest perpendicular to the long axis of each
canal bears a mound of connective tissue within
this region from which project a layer of hair cells.
Their cilia protrude into a gelatinous mass, the
cupula that may be deflected during angular head
movements.
Physiology of the vestibular system
Signal transduction
The neuroepithelium of the ampullae and maculae are sensitive to movement by virtue of an
arrangement of hair cells that constitute the
transduction mechanism for the peripheral vestibular organs.4 Of a total of approximately 63,000
hair cells in each peripheral vestibular organ,
4 Dizziness and Vertigo: An Introduction and Practical Guide
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23,000 are located in the cristae of the semicircular canals and 40,000 in the maculae of the saccule
and utricle.
The apical surface of each hair cell bears an asymmetrically arranged bundle of 50–100 nonmotile
cilia. These are arranged in a stepwise fashion,
becoming progressively taller towards the longest
cilium, the kinocilium.5 The distal tips of the cilia
are connected by extracellular bridges or tip-links.
A shearing force, such as the relative endolymph
flow during head rotation, causes the cilia to bend,
with movement towards the kinocilium resulting in depolarisation, whilst movement in the
opposite direction results in hyperpolarisation
(Figure 1.6).6 The hair cells on the cristae of the
lateral semicircular canals are arranged such that
endolymphatic flow towards the utricle (ampullofugal flow) results in d
­ epolarisation with flow away
from the utricle (ampullopetal flow) result in
hyperpolarisation. The reverse is the case for the
superior and posterior semicircular canals with
ampullopetal flow excitatory and ampullofugal
flow inhibitory.
Neural firing rate
a.
b.
c.
Figure 1.6. (a–c) Cupula movement. Endolymph flow towards the kinocilium results in an increase above
the resting firing rate (b). Flow in the opposite direction results in a fall below the resting firing rate (c).
Anatomy and physiology of the peripheral vestibular system 5
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An electrical gradient exists between the potassium
rich endolymph and the sodium rich cellular cytoplasm.7 Deflection of cilia towards the kinocilium
and subsequent opening of associated pores results
in a transduction current that induces ­permeability
changes on the basolateral membrane, leading
to depolarisation of the hair cell and subsequent
­neurotransmitter release. The basal surface of each
cell is in contact with both afferent and efferent
nerve fibres that are ­collectively held in place by
supporting cells. There is a continuous discharge
from the afferent nerves of the hair cells that is at
rest symmetrical for each labyrinth.8
Neural pathways
The vestibular labyrinth provides the major
­sensory input to the vestibular nuclear complex that is located on the floor of the fourth
­ventricle.9 In man approximately 15,000 primary
afferent nerve fibres of the vestibulocochlear
nerve relay signals centrally to second order
­neurones in four morphologically and anatomically ­d istinct regions within the vestibular nucleus (Figure 1.7).
The main vestibular nuclear subgroups are
the superior (Bechterew’s), lateral (Deiter’s),
medial (triangular nucleus of Schwabe), and
inferior. However, inputs from the labyrinth
are not equally distributed to all four regions of
the vestibular nucleus. There are clear separations of afferent fibres such that specific areas
­preferentially receive afferents from specific
receptors.
The principal peripheral labyrinthine input
to the superior vestibular nucleus comes
from the cristae of the semicircular canals.
Efferent fibres mainly run in the ipsilateral and
contralateral medial longitudinal fasciculus
to innervate the motor nuclei of the extrinsic
muscles of the eye, and hence this vestibular
nucleus provides a major relay centre for semicircular canal mediated ocular reflexes. In contrast,
the medial vestibular nucleus receives input
from the utricle in addition to the semicircular
canals. Efferent output runs in the descending
medial longitudinal fasciculus to cervical and
thoracic levels via the medial vestibulospinal
tract. Subsequently fibres pass bilaterally to the
nuclei of the oculomotor nerves, the cerebellum, the reticular formation and the contralateral ­vestibular nuclei. The medial vestibular
nucleus appears to be important in not only
controlling eye, head and neck movements but
via commissural connections important in the
compensatory processes that follow a peripheral
vestibular deficit.
The lateral vestibular nucleus is an important
station for the control of vestibulospinal reflexes.
Afferent input is received from both the labyrinth
(­otoliths and semicircular canals) and cerebellum,
with somatotopically arranged efferent projection to the spinal cord as the vestibulospinal
tract. Efferent fibres also pass bilaterally via the
medial longitudinal fasciculus to the oculomotor
nuclei and hence participate in the oculomotor
reflexes.
The inferior, or descending, vestibular
nucleus receives afferent projections from the
­labyrinth and cerebellum, and minimal monosynaptic input from the spinal cord. Efferent
fibres pass to the cerebellum and reticular
formation.
Vestibular reflexes
The principle functions of the vestibular ­s ystem
are those of gaze stabilisation and postural
control. This is achieved by means of a number
of ref lexes such as the vestibulospinal ref lex
which allows rapid correction of posture in
response to head acceleration (extension of
the ipsilateral limbs and contraction of the
contralateral limbs).9 This pathway is mediated
through the superior semicircular canals and
otolithic organs via the lateral vestibulospinal
tract. In contrast, the r­ ighting ref lex maintains
head position in a h
­ orizontal plane irrespective
of trunk position and is mediated via the medial
vestibulospinal tract.
6 Dizziness and Vertigo: An Introduction and Practical Guide
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1
6
2
3
Brainstem
Peripheral
vestibular system
4
5
7
8
16
13
10
12 9
11
14
Spinal cord
15
Figure 1.7. Central connections of the central vestibular system. 1 – Vestibular cortical area of the ­pariental
lobe, 2 – Ventral intermediate nucleus of the thalamus, 3 – Oculomotor nucleus, 4 – Trochlear nucleus,
5 – Vestibulothalamic tract, 6 – Cerebellum, 7 – Medial longitudinal fasciculus, 8 – Superior v­ estibular
nucleus, 9 – Abducens nerve, 10 – Vestibular nerve, 11 – Inferior vestibular nucleus, 12 – Medial v­ estibular
nucleus, 13 – Lateral vestibular nucleus (of Deiters), 14 – Lateral vestibulospinal tract, 15 – Medial
­vestibulospinal tract, 16 – Membranous labyrinth.
The vestibulo-ocular reflex provides image
­stabilisation during head rotation and is
­i llustrated in Figure 1.8. This reflex forms the basis
for a number of important clinical i­ nvestigations
of peripheral vestibular function including the
caloric and head thrust test.
An abnormality of the labyrinth leading to an
absent signal from one lateral semicircular canal
in the presence of normal function on the other
side would therefore be misinterpreted as head
rotation. This would conflict with visual and
somatosensory information resulting in vertigo.
Anatomy and physiology of the peripheral vestibular system 7
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Lateral rectus
Medial rectus
Oculomotor nucleus
Abducens nucleus
Vestibular nucleus
Neural firing rate
Head turning
Figure 1.8. The excitatory pathways of the vestibulo-ocular reflex. As a result of head rotation, endolymph
flow within the semicircular canals causes movement of the cupulae within the ampullae of the lateral semicircular canals and relative shearing of the underlying stereocilia. Neural impulses increase on the right and
decrease on the left. Neural connections to the IIIrd and VIth cranial nuclei result in contraction of the left
lateral rectus and right medial rectus stabilising gaze [arrow = start of head rotation].
References
1 Gelfand SA. Essentials of Audiology. 2nd ed.
New York, NY: Thieme Medical Publishers,
Inc; 2001.
2 Blanks RH, Curthoys IS, Markham CH.
Planar relationships of the ­semicircular
canals in the cat. Acta Otolaryngol.
1975;80:185.
3 Baloh RW, Honrubia V. Vestibular function:
an overview. Clinical Neurophysiology of the
Vestibular System. Oxford: Oxford University
Press; 2001:3–22.
4 Savundra P, Luxon LM. The anatomy and physiology of vertigo and balance. In: Luxon LM, Davies
RA, eds. Handbook of Vestibular Rehabilitation.
London: Whurr Publishers Limited; 1997.
8 Dizziness and Vertigo: An Introduction and Practical Guide
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5 Kikuchi T, Takasaka T, Tonosaki A, Watanabe H.
Fine structure of guinea pig vestibular
­kilocilium. Acta Otolaryngol. 1989;108:26–30.
6 Hudspeth AJ, Corey DP. Sensitivity, polarity,
and conductance change in the response of vertebrate hair cells to controlled mechanical stimuli. Proc Natl Acad Sci USA. 1977;74:2407–2411.
7 Savundra P, Luxon LM. The physiology of
vertigo and its application to the dizzy patient.
In: Kerr A, ed. Scott-Brown’s Otolaryngology.
London: Butterworths; 1997.
8 Harada Y. The Vestibular Organs: SEM Atlas of
the Inner Ear. Amsterdam/Berkeley/Milano:
Kugler & Ghedini Publications; 1988.
9 Shepard NT, Telian SA. Basic anatomy and
physiology review. Practical Management of the
Balance Disorder Patient. San Diego: Singular
Publishing Group, Inc; 1996:1–16.
Anatomy and physiology of the peripheral vestibular system 9
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2
Clinical assessment
of vertigo
Mudit Jindal and Aanand Acharya
Contents
History
Examination
Otological examination
Neurological examination
Eye examination
Cerebellum examination
Other neurological tests
Stepping test
Cardiovascular examination
Conclusion
References
11
12
13
13
13
15
16
16
16
16
17
History
A detailed and accurate history is essential in
making a diagnosis in a patient with ­dizziness.
It is i­ mportant to understand exactly what
­sensation the patient experienced. One must
­distinguish true rotatory vertigo, the illusion of
self or ­environmental rotation, from unsteadiness,
­light-headedness and dizziness.
Understanding the events surrounding the first
episode is key. Specific points that require exploring
include:
1 The circumstances under which the epi-
sode occurred: What exactly was the patient
doing when they first experienced their
­symptoms? Were there positional, visual or
acoustic triggers to the episode? Was the onset
of symptoms sudden or gradual in onset, or
did they simply wake with their dizziness.
2 The longevity and frequency of the episodes:
Did the dizziness last for seconds, minutes,
hours, or days? Was this a single, recurrent
or continued sensation? Were the symptoms
recurrent beyond the initial episode and did
they resolve between the episodes? If so, the
above ­characteristics of each episode should be
evaluated. In conditions such as a peripheral
vestibular deficit, labyrinthitis or brainstem
stroke, patients usually experience an acute,
single episode of vertigo, which improves
over days to weeks. In patients with recurrent
vertigo or dizziness the presence of triggers
Clinical assessment of vertigo 11
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becomes critically important if an accurate
diagnosis is to be made.
3 Were there any predisposing factors?
A ­history of head injury may suggest benign
­paroxysmal positional vertigo (BPPV), or
barotrauma s­ udden labyrinthine failure. The
­presence of a preceding viral upper respiratory
tract ­infection or use of potentially ototoxic
­medication may be significant.
4 Were there any associated features? For
example:
a Otological symptoms: These include
­hearing loss, tinnitus, aural fullness,
otalgia and otorrhoea. The character of
any hearing loss (fluctuating, sudden
or p
­ rogressive) and its association with
­tinnitus may suggest inner ear pathology
such as Ménière’s disease, labyrinthitis,
or a vestibular schwannoma.
b Neurological symptoms: The presence of
any neurological features including headache, weakness or cranial nerve involvement may indicate central nervous system
(CNS) pathology such as a cerebrovascular
accident, tumour, or perhaps multiple
sclerosis (MS). Associated photophobia,
phonophobia or alteration of smell or taste
may suggest vestibular migraine.
c Cardiovascular symptoms: Postural
hypotension and cardiac arrhythmia are
recognised causes of dizziness. The onset
of unsteadiness on changing ­posture
may indicate a diagnosis of postural
­hypotension while symptoms on flexion or extension of the neck may suggest ­vascular occlusion of the posterior
­cerebral ­circulation. Brandt et al.1 eloquently summarised the findings in rotational vertebral artery occlusion (RVAO)
syndrome: contralateral head rotation
compressing a dominant vertebral artery
which provides the major component of
the vertebrobasilar blood supply results
in rotational vertigo with mixed torsional
downbeat horizontal nystagmus toward
the compressed artery. This occurs as a
result of labyrinthine rather than central
brainstem ischaemia. Although rare,
this is a syndrome that is important to
recognise as surgical ­decompression is
curative.
d Visual symptoms: Oscillopsia, visual
instability with head movement, may
suggest bilateral vestibular hypofunction. This may occur after meningitis,
­gentamicin ototoxicity or idiopathic.
Oscillopsia unrelated to head movement
may r­ epresent an acquired CNS nystagmus
(acquired p
­ endular, downbeat or torsional
nystagmus) or may simply be paroxysmal.
Triggered p
­ aroxysmal ­oscillopsia is rare,
the most common being Tulio phenomenon in superior semi-circular canal
dehiscence syndrome.2
It is useful to enquire about any recent
visit to an optician as patients can experience transient dizziness following a slight
change in prescription or type of spectacles
such as switching to vari- or bi-focal lenses.
It is also not uncommon to experience
disequilibrium following an alteration in
axis of the lens.
5 Past medical history: This should focus in
particular on the presence of cardiovascular
disease, metabolic disorders (such as thyroid
dysfunction or diabetes), musculoskeletal disorders, previous history of migraine and ocular
disorders.
6 Drug history: Many commonly prescribed
medications can cause dizziness and it is
important to enquire if symptoms coincided
with a change in medication.
Examination
A neuro-otological examination should be
­performed in every case, supplemented
where necessary with a cardiovascular
assessment.
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Otological examination
Otoscopy must be performed in every patient. If the
ear is occluded with wax this should be removed
in order to exclude the presence of an underlying
cholesteatoma. A cholesteatoma may erode into
the inner ear resulting in a fistula, and pneumatic
otoscopy or digital pressure over the tragus may
provoke nystagmus (Hennebert’s sign).
A modified Valsalva manoeuvre or exposure to
a sudden loud sound may also provoke transient
nystagmus in those with a superior semicircular
canal dehiscence (Tullio phenomenon). The latter
has been suggested to arise secondary to movement
of the stapes footplate secondary to the stapedius
reflex, with a pressure wave travelling through the
inner ear. This diagnosis is supported by the lateralisation of Weber’s tuning fork test to the diseased
ear and the presence of a low frequency air bone gap
at two or more frequencies on pure tone audiometry.
Neurological examination
Whilst a cranial nerve examination should be performed in the assessment of the dizzy patient, particular attention should be paid to specific aspects
of this part of the examination.
Eye examination
Clinical examination of eye movements may help
to differentiate between peripheral and central
vestibular disorders. Examination begins with
an assessment of the range of eye movement. The
presence of diplopia or disconjugate eye movements
at this stage should prompt a formal independent
examination of cranial nerves III, IV and VI.
Often the examination for abnormal eye movements can only be accurately performed with the
assistance of Frenzel lenses, infrared occulography
or other such modalities that allow identification
of subtle abnormalities of ocular movement. In a
patient with a suspected peripheral vestibular disorder, the examination has two broad aims: the first
is to search for ocular signs indicative of a peripheral vestibular disorder, and the second is to
exclude other central nervous system (CNS) lesions
by making sure that eye movements which are
mediated by a non-vestibular pathway are indeed
normal. The examination should include:
1 Spontaneous and gaze-evoked nystagmus:
In the presence of nystagmus the waveform
(saw-tooth or pendular) and direction of the
beat (fast phase) must be documented. Usually
the fast phase is away from the peripheral
vestibular lesion, however in the acute irritative phase of a peripheral vestibular insult,
it may be toward the side of the peripheral
vestibular lesion. The presence of spontaneous
nystagmus in primary gaze in a patient who is
otherwise well (not experiencing an episode
of vertigo) is suggestive of a central pathology. Similarly, pendular, vertical or torsional
nystagmus is almost certainly of central origin.
Alexander’s law states that a second or third
degree nystagmus will enhance on gaze deviation in the direction of the fast phase, but it
must be remembered that the scale of severity
of nystagmus (first, second or third degree)
and Alexander’s law apply mostly to peripheral vestibular nystagmus rather than central
nystagmus. Gaze paretic nystagmus (whereby
the patient has difficulty in holding gaze in an
eccentric position in the orbit) is due to damage of the gaze-holding mechanisms mediated
by ipsilateral brainstem and cerebellar structures. It is also important to perform the cover
test to exclude latent nystagmus.
2 Convergence: This often enhances spontaneous
nystagmus so these two parts of the examination
are often performed at the same time. Absence of
convergence occurs in midbrain lesions.
3 Smooth pursuit: In principle, the presence of
normal pursuit rules out a central vestibular disorder. Equally a patient with balance
symptoms and broken or saccadic pursuit
movements suggest neurological rather than
labyrinthine disorder.
4 Saccades: Three properties of saccades that need
to be assessed are velocity, accuracy and binocular conjugacy (conjugate or dysconjugate):
a Velocity: This can be normal, slow
or saccades can be absent altogether.
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Neurodegenerative disorders involving the
saccadic system reduce saccade velocity,
and result in slow, single movements rather
than a fragmented movement.
b Accuracy: One or two small corrective
saccades can occur in normal subjects. The
presence of three or more corrective saccades
is considered saccadic hypometria and can
point to pathology in one of a number of
areas, including the cortex, basal ganglia,
brainstem, cerebellum, oculomotor nucleus,
oculomotor nerve and muscles. Conversely,
saccadic hypermetria, where the initial saccade is too large and overshoots the target
(requiring corrective saccades in the opposite direction) is suggestive for pathology of
the anterior lobe of the cerebellum.
c Conjugacy: Internuclear ophthalmoplegia (INO) occurs when saccades in the
abducting eye are fast and large whereas
saccades in the adducting eye are small and
slow. For example, a right eye INO means
that right eye adduction during gaze to
the left is slow or incomplete due to a right
medial longitudinal fasciculus lesion. Since
innervation to eye muscles is functionally
linked, the attempt of the CNS to overcome
this limitation in adduction results in
hypermetric saccades of the contralateral,
abducting eye. The presence of INO is
highly suggestive of MS.
5 Vestibulo-Ocular Reflex (VOR): The VOR
­stabilises gaze during head movements, thereby
allowing clear vision during activities such
as walking, running or head turning. This is
achieved by matching head movement with
a slow phase eye movement that is of equal
velocity but in an opposite direction to the head
movement. The neuroanatomical pathway is
a 3-neuron reflex involving a vestibular nerve
ganglion (Scarpa ganglion), a vestibular nucleus
neuron and an oculomotor nuclear neuron
(III, IV or VI). Assessment of the VOR in clinic
facilitates the identification of a unilateral or
bilateral vestibular loss, with a more severe and
more acute lesion being easier to identify.
The clinical manoeuvres employed in the
assessment of the VOR include:
a A slow doll’s head manoeuvre. This is
assessed by:
i Direct observation of the eyes – the
presence of catch-up saccades towards a
prescribed target indicates failure of the
lateral semicircular canal on the side in
the direction of the head turn.
ii Measurements of visual acuity – visual
acuity is measured with the head
stationary and again with the head
oscillating at approximately 1 Hz.
A reduction in visual acuity of more
than two lines indicates a bilateral
abnormality of the VOR. This test
cannot be applied to unilateral lesions,
which are more effectively identified
using the head thrust test (see later).
iii Ophthalmoscopy – the head is turned
during ophthalmoscopy whilst the
viewing eye maintains fixation on a
distant object. If the optic disc remains
absolutely steady the VOR is functioning well. As ophthalmoscopy is not
routinely performed in the ear nose
and throat (ENT) clinic this test is not
usually performed.
b A fast doll’s head manoeuvre – the head
thrust test. This test is more efficient
than the slower doll’s head manoeuvre in
identifying unilateral lesions. The VOR is
only really irreplaceable at high velocities
and accelerations of the head when neither
pursuit nor optokinetic or cervical mechanisms can fully take over from the VOR. For
this reason Halmagyi and Curthoys have
popularised the head thrust manoeuvre.3,4
A fast right head turn will induce a patient
with right-sided vestibular loss to introduce
one or more catch-up saccades towards
the target, that is, towards the left. The test
is therefore useful for identifying acute
peripheral vestibular disorders. It has a low
sensitivity but a high specificity for a unilateral PVD. In the case of chronic, incomplete
or compensated lesions the test is often
negative or inconclusive. It has been suggested that more than 50% canal paresis is
required for a head thrust test to be positive.
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c Head shake test: This is performed by
t­ aking hold of a patients head and rapidly (2 Hz) shaking it from side to side
for 20 ­seconds with eyes closed and open.
Horizontal nystagmus is suggestive of a
peripheral vestibular deficit, induced ­vertical
nystagmus central pathology.
d The dynamic visual acuity test: Involves
asking a subject to read a Snellen chart at
rest and then with their head shaken (at
0.2 Hz). A greater than 3 line drop is suggestive of a peripheral vestibular deficit.
It is important to appreciate that a head thrust
and head shake test are often unreliable and
therefore a caloric test may still be required to
confirm clinical suspicion of peripheral vestibular failure.
6 Positional manoeuvres: Positional manoeuvres
(e.g. Dix-Hallpike test) are a vital component of
the examination in all patients with a balance
disorder as benign paroxysmal positional vertigo (BPPV) is one of the most common causes
of vertigo. For detailed explanation of DixHallpike test see Chapter 6. The purpose of the
examination is to elicit vertigo and the patient
should be warned of this. The eyes must be
carefully observed during the examination for
the presence of nystagmus. The patient must be
instructed to look straight ahead at a point on
the examiner’s head (e.g. bridge of nose) and
the head down position must be maintained
for a minimum of 30 seconds to account for
long latencies. Typical posterior canal BPPV
is characterised by torsional geotropic nystagmus which demonstrates a latency, adaptation
(decline and disappearance of the nystagmus
within a minute or two) and fatigability.
Other types of nystagmus may be observed during
the Dix-Hallpike manoeuvre, which do not represent
posterior canal BPPV. Seventy-five percent of induced
vertical nystagmus is secondary to central ­pathology5
with the remaining 25% most likely to be due to
superior canal BPPV. In the case of superior canal
BPPV ideally one would see a torsional component
to the down beating nystagmus although the right/
left specificity to trigger superior canal BPPV seems
less than for posterior canal BPPV. For superior canal
BPPV the left head hanging Dix-Hallpike position
should provoke a right superior canal BPPV, and viceversa, due to the co-planar arrangement of the left
posterior and right superior canals (and vice-versa).
An important factor in provoking superior canal
BPPV seems to be placing the head as low as possible
and this may be best achieved by taking the patient
from the sitting upright position to the straight back,
head hanging position in one movement.6
A purely horizontal nystagmus observed during
a Dix-Hallpike manoeuvre most likely represents
lateral (horizontal) canal BPPV. This produces an
intense high frequency nystagmus and vertigo when
the head is turned in either direction. The DixHallpike manoeuvre may not identify 20% of lateral
canal BPPV cases so in those cases with a good history for BPPV but negative Dix-Hallpike manoeuvre
adjustments should be made to optimise the head
position to assess for lateral canal BPPV. The optimum head position is with the head end of the couch
raised 20–30 degrees above horizontal, followed by a
full head turn in the axis of the body in each direction. A persistent horizontal nystagmus, however, is
likely to represent significant central pathology.
Cerebellum examination
Cerebellar function should be assessed by:
pointing
•• finger-nose
dysdiadochiokinesis
test. While Romberg initially
• Romberg’s
described this test in 1846 in relation to
tabes dorsalis, it was Barany in 1910 who suggested that patients with a unilateral peripheral vestibular disorder might fall towards the
side of the disorder. Those presenting with a
unilateral peripheral vestibular or cerebellar
disorder might sway towards the ipsilateral
side, whereas sway which is predominantly
posterior might be inorganic in nature.
Patients are asked to stand with their feet together,
eyes closed for 30 seconds. This may be ‘sharpened’,
or made more challenging, by asking the subject
to stand with one foot in front of the other (tandem stance) or on foam. The extent of sway may be
Clinical assessment of vertigo 15
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recorded using dynamic posturography or using
the ‘D+R Balance’ application.
Other neurological tests
Stepping test
Fukuda’s modification of Unterberger’s ‘Tretversuch’
test8 has been suggested to be indicative of a peripheral vestibular pathology. Patients are asked to march
on the spot arms outstretched with their eyes closed.
Rotation of 30 degrees or more following 50 steps
is considered to be significant and suggestive of a
peripheral vestibular hypofunction. Further studies
however have suggested that an apparently abnormal
stepping test is a poor indicator of peripheral vestibular dysfunction and should be considered with
gross unsteadiness and a positive Romberg’s test as
a prompt for further, more detailed, investigation
of vestibular function. Conversely an apparently
normal stepping test does not exclude the presence
of a peripheral vestibular defect, but may represent
good compensation for such a lesion and should not
preclude more detailed investigation of a clinically
suspect patient.7 In a study involving 736 participants Honaker et al.8 found that the Fukuda stepping
test had a 50% sensitivity and 61% specificity for
turning towards the weaker labyrinth.
Gait and stance should also be examined by asking
the patient to walk at their own pace down a corridor.
Cardiovascular examination
This should include blood pressure measurements
(lying and standing) and listening for the presence
of bruits.
Conclusion
A wide variety of clinical tests are required when
assessing a patient presenting with vertigo and
dizziness (Table 2.1). Whilst a single test may produce clear signs that support a working diagnosis,
Table 2.1. Summary of the clinical tests performed for patients with dizziness and vertigo.
Examination
Normal findings
Abnormality (correlation)
Otoscopy/microscopy
Intact tympanic membrane
Cholesteatoma or deep retraction pocket
Pneumatic otoscopy
No nystagmus
Transient horizontal nystagmus – perilymph fistula, or
erosive inner ear breach due to cholesteatoma.
Vertical or torsional nystagmus – superior semicircular
canal dehiscence
Gait
Normal gait
Wide based gait – cerebellar pathology or bilateral
vestibular hypofunction, shuffling gait – Parkinsonism,
antalgic gait – osteoarthritis
Nystagmus
(spontaneous and
gaze-evoked)
No nystagmus
Horizontal nystagmus intensity increases in one
direction, increases with Frenzel’s glasses – peripheral
Direction changing, increasing with fixation – central
Pendular – central
Dix-Hallpike test
No nystagmus
Latency, geotropic torsional nystagmus, complete
cessation – posterior canal BPPV
Short latency, high frequency horizontal nystagmus
– lateral canal BPPV
Persistent lateral or vertical nystagmus – significant
central pathology
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Table 2.1. (Continued) Summary of the clinical tests performed for patients with dizziness and vertigo.
Examination
Normal findings
Abnormality (correlation)
Head thrust test
No saccades
Catch up saccades on rapid head movement –
peripheral vestibular deficit
Head shake test
No nystagmus
Brief horizontal nystagmus – peripheral vestibular
deficit. Vertical nystagmus – central pathology
Finger-nose
Accurate coordination of
movement
Dysemmetria – lateral cerebellar pathology
Rapid alternating
movement
Accurate coordination of
movement
Dysdiadochokinesia – lateral cerebellar pathology
Heel-shin
Accurate coordination of
movement
Poor coordination – lateral cerebellar pathology
Tandem gait
Normal gait
Instability/unable to perform – median cerebellar
pathology, bilateral vestibular hypofunction, poorly
compensated peripheral vestibular deficit
Romberg’s test and
sharpened Romberg
(on foam)
Normal stance
Widened stance – bilateral peripheral vestibular loss,
mixed, proprioceptive loss, posterior column loss
Unterberger test
Less than 30 degree
rotation
Greater than 30 degree rotation – peripheral
vestibular loss to the rotated side
Smooth pursuit
Accurate movement
Broken – central, drugs, inattention, but commonly age
related
Saccades
Accurate
Overshoot – central pathology (vermis)
Undershoot – central pathology. Disconjugate – central
for example geotropic torsional nystagmus on
Dix-Hallpike testing in posterior canal BPPV, it is
often prudent to complete the entire clinical test
battery in order to exclude associated pathology, for
example a peripheral vestibular deficit and pcBPPV.
References
1 Brandt T, Baloh RW. Rotational vertebral
artery occlusion: a clinical entity or various syndromes? Neurology. 2005;65:1156–1157.
2 Minor LB, Cremer PD, Carey JP, Della Santina
CC, Streubel SO, Weg N. Symptoms and signs
in superior canal dehiscence syndrome. Ann NY
Acad Sci. 2001;942:259–273.
3 Halmagyi GM, Curthoys IS. A ­clinical
sign of canal paresis. Arch Neurol.
1988;45:737–739.
4 Halmagyi GM, Cremer PD. Assessment and
5
6
7
8
treatment of dizziness. J Neurol Neurosurg
Psychiatry. 2000;68:129–134.
Bertholon P, Bronstein AM, Davies RA, Rudge
P, Thilo K. Positional down beating nystagmus
in 50 patients: Cerebellar disorders and possible
anterior semicircular canalithiasis. J Neurol
Neurosurg Psychiatry. 2002;72:366–372.
Fukuda T. The stepping test. Two phases of
the labyrinthine reflex. Acta Otolaryngologica.
1958;50:95–108.
Hickey SA, Ford GR, Buckley JG, Fitzgerald
O’Connor AF. Unterberger stepping test: A useful indicator of peripheral vestibular dysfunction? J Laryngol Otol. 1990;104:599–602.
Honaker JA, Boismier TE, Shepard NP, Shepard
NT. Fukuda stepping test: sensitivity and specificity. J Am Acad Audiol. 2009;20:311–314.
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3
Imaging in dizziness
and vertigo
Neshe Sriskandan and Steve Connor
Contents
Introduction
Imaging modalities
Imaging appearances
Peripheral vestibular disturbance
Ménière’s disease
Inflammation
Neoplasia
Middle ear inflammatory disease
Post trauma
Semicircular canal dehiscence
Post surgical
Developmental
Central vestibular disturbance
Vascular
Demyelination
Tumours
Other
Conclusion
References
19
20
21
21
21
21
21
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23
25
25
26
26
26
28
28
29
30
30
Introduction
Imaging is of limited value and questionable cost
effectiveness when applied to non-selected patient
populations with dizziness.1,2,3 However, imaging
may be diagnostically useful in certain clinical
situations.
Where isolated dizziness or vertigo is suspected
to originate from the inner ear or vestibulocochlear nerve (peripheral), imaging may not be
required. The most frequent causes of peripheral
vertigo are benign paroxysmal positional vertigo (BPPV) and acute peripheral vestibular loss
(e.g. vestibular neuronitis).4 These are clinical
diagnoses and seldom require support from conventional imaging.
Patients with a peripheral vestibular disturbance benefit from imaging if they are resistant
Imaging in dizziness and vertigo 19
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to ­treatment5 or if clinical features are atypical.
If there is an associated unilateral or asymmetric ­sensorineural hearing loss or tinnitus, then
magnetic resonance imaging (MRI including
thin section T2 weighted imaging) is indicated to
evaluate the cerebello-pontine angle (CPA) cistern,
internal auditory meatus (IAM) and labyrinthine
structures. When dizziness and non-positional vertigo present in patients with vascular risk factors,
brain imaging ensures that a peripheral vestibular
presentation is not confused with a posterior fossa
infarct.6 Imaging with computed tomography (CT)
is indicated in the setting of peripheral vertigo
when semicircular canal dehiscence is suspected,
following trauma or surgery, and when there is
otoscopic evidence of cholesteatoma.
Imaging is usually indicated if there are clinical features to suggest pathology of the cerebral vestibular
connections (central). MRI is focused on whole
brain imaging and considerations include neuroinflammatory, vascular, infectious and developmental
causes. Diagnostic yield is increased if there are
additional focal neurological findings. Imaging
is of value in patients suspected of experiencing
vestibular migraine (vertiginous migraine), as other
central pathology must be excluded to arrive at this
diagnosis.
Although MRI is more sensitive for posterior fossa
pathology, CT may be appropriate for an emergency
clinical presentation, for example when associated with severe headache. Computed tomography
angiography (CTA) and magnetic resonance angiography (MRA) are used to assess for significant
vertebrobasilar arterial disease. Cervical vertigo is
a poorly defined syndrome and the role of CT and
MRI of the cervical spine is controversial.7
Imaging modalities
Computed tomography is generally focused on the
bony appearances of the petrous temporal bones,
where the high spatial resolution is optimal for
the evaluation of the otic capsule and for demonstration of developmental, traumatic or erosive
pathologies. CT may also be performed to assess
for intracranial pathology such as haemorrhage or
infection in the emergency setting or when MRI is
contraindicated.
The use of MRI permits accurate assessment of
the labyrinthine structures, the cranial nerves
within the IAM, CPA cistern, and the cerebral parenchyma. Dedicated imaging of the
IAMs and the fluid containing structures of
the l­ abyrinth is performed with thin s­ ection
T2 weighted imaging sequences (such as CISS,
DRIVE and CFIESTA depending on the MRI
­system), which permits high resolution imaging of structures within the cerebrospinal
fluid (CSF) spaces, such as the cranial nerves.
Gadolinium is administered in selected cases
to assess for inflammation and to characterise
a tumour. Diffusion weighted imaging (DWI)
is a sequence which may be used to i­ dentify
acute ­cerebral ischaemia ­demonstrated by
increased ­signal. Contraindications to MRI
include some o
­ tological implants, intra-orbital
metallic ­foreign bodies, along with most neurostimulators, pacemakers and aneurysm clips.
Young children and ­claustrophobic patients may
require a ­general anaesthetic in order to obtain
­satisfactory MRI.
Vascular imaging may be performed with CTA or
MRA. CTA requires administration of contrast
medium with the bolus time optimised for arterial enhancement. MRA may be performed using
‘time of flight’ imaging (that is using the properties of flowing blood rather than contrast medium
to image the vascular system), although contrast
enhanced MRA (CEMRA) allows improved
spatial resolution when imaging the cervical
vessels. Both iodinated contrast and gadolinium
may be contraindicated in patients with renal
impairment.
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Imaging appearances
A number of the conditions and pathologies
­associated with dizziness and vertigo have well
recognised imaging appearances.
Neoplasia
Ménière’s disease is generally a clinical diagnosis
and the main role of imaging is to exclude central
causes. Earlier imaging observations demonstrated
a decreased conspicuity of the endolymphatic sac
and duct in severe cases of Ménière’s disease.8 More
recent experimental studies have shown that intratympanic and intravenous gadolinium may delineate endolymphatic hydrops at 3 Tesla (T) using
delayed and high resolution MRI (the standard MR
magnetic strength for clinical application is 1.5 T,
however, 3 T is becoming increasingly available).9,10
The most frequent peripheral vestibular pathway
neoplasm is the vestibular schwannoma. Whilst
usually arising in the internal auditory canal, they
may occasionally extend into the vestibule via a
transmacular route. Whilst more likely to present
with unsteadiness rather than true vertigo, secondary to cerebellar or brain stem impingement7
(Figure 3.2), peripheral features may result from
vascular compression and labyrinthine i­ schaemia.
Neoplastic involvement of the labyrinthine
structures may also occur secondary to intrinsic
labyrinthine tumours, such as intralabyrinthine
schwannoma (Figure 3.3) and endolymphatic sac
tumours (Figure 3.4), or due to secondary erosion
by primary petrous temporal bone or middle ear
neoplasms13,14 There may be encroachment on the
otic capsule by expansile non-neoplastic processes
such as Paget’s disease15 (Figure 3.5).
Inflammation
Middle ear inflammatory disease
An acute peripheral vestibular deficit is generally a
clinical diagnosis. Inflammation within the inner
ear structures may be demonstrated on MRI by
subtle signal dropout on T2 weighted thin section imaging, and enhancement post gadolinium,
whereas isolated vestibular neuronitis11 and other
cranial nerve inflammation may be demonstrated
as linear neural gadolinium enhancement12
(Figure 3.1).
Acute otitis media or chronic middle ear effusions in children may be associated with vertigo
and unsteadiness.6,16 Otoscopic evidence of cholesteatoma should raise the possibility of erosion
of the otic capsule usually in relation to the lateral
semicircular canal, CT may demonstrate nondependent soft tissue within the middle ear with
erosion of adjacent bony structures, whereas diffusion weighted MR demonstrates the lesion as high
Peripheral vestibular
disturbance
Ménière’s disease
Figure 3.1. Neurolabyrinthitis: T1 post gadolinium axial magnetic resonance imaging through the internal
auditory meatus and labyrinthine structures demonstrates linear neural enhancement within the left internal auditory meatus, and further enhancement within the left labyrinthine structures in a patient with syphilitic infection.
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Figure 3.2. Vestibular schwannoma: T1 Axial post gadolinium magnetic resonance imaging demonstrates
typical appearances of a left internal auditory meatus and cerebellopontine angle cistern vestibular schwannoma indenting the left cerebellar peduncle.
Figure 3.3. Intralabyrinthine schwannoma: T2 CISS Axial magnetic resonance imaging demonstrates a
nodule corresponding to an intralabyrinthine schwannoma within the anterior vestibule.
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Figure 3.4. Endolymphatic sac tumour: Axial computed tomography image demonstrates scalloped erosion
and spicules of residual bone related to the left posterior petrous pyramid at the site of endolymphatic sac
tumour.
Figure 3.5. Skull base Paget’s disease: Axial computed tomography image demonstrates well marginated
lucency corresponding to Paget’s disease encroaching on the labyrinth and the internal auditory meatus.
signal and is useful in primary diagnosis and post
operative follow-up (Figure 3.6).17
Post trauma
Whilst post traumatic vertigo is usually due to
BPPV and rarely hydrops,18 these entities do not
demonstrate conventional imaging correlates.
CT is used to demonstrate fractures involving the
otic capsule (Figure 3.7) or the IAM, which may
damage the vestibular pathway. Post traumatic
sequelae associated with vertigo, such as perilymphatic fistula (with pneumolabyrinth demonstrated
on CT) or intralabyrinthine haemorrhage (with
increased T1 signal demonstrated within the inner
ear) (Figure 3.8) may be apparent on imaging.19
Imaging in dizziness and vertigo 23
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(a)
FPO
(b)
Figure 3.6. Middle ear cholesteatoma with lateral semicircular canal erosion: (a) CT demonstrates opacification of the right middle ear with erosion of the scutum and lateral semicircular canal. (b) Coronal DWI
(non-epi) image confirms the presence of cholesteatoma.
Figure 3.7. Transverse petrous temporal bone fracture: Computed tomagrophy image of the petrous bone
(bone algorithm with wide window width) demonstrates a transverse fracture extending from the vestibular
aqueduct through the vestibule to the epitympanum.
24 Dizziness and Vertigo: An Introduction and Practical Guide
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Figure 3.8. Intralabyrinthine haemorrhage: T1 axial magnetic resonance imaging (without gadolinium)
demonstrates T1 hyperintense haemorrhagic degradation products within the cochlea.
Figure 3.9. Superior semicircular canal dehiscence: Coronal computed tomography study demonstrates
dehiscence of the superior semicircular canal.
Semicircular canal dehiscence
The diagnosis of semicircular canal dehiscence is
based on the demonstration of a small defect within
the bony wall of the (usually superior) semicircular
canal on CT (Figure 3.9). This is best demonstrated
in the coronal plane and there is little benefit from
oblique reformatted planes.20 MRI is less accurate
unless high resolution sequences are used.21 The
dehiscence leads to formation of a third mobile
window and symptoms of vertigo, particularly
manifesting as Tullio’s phenomenon (sound induced
vertigo and nystagmus). Large dehiscences are associated with increased vestibular symptoms.22
Post surgical
Post stapedectomy vertigo occurs in 5–6% of
procedures.23 This may result from compression of
the utriculo-saccular organ by bony fragments or a
medially located prosthesis (Figure 3.10). The presence of a perilymph fistula and intralabyrinthine
Imaging in dizziness and vertigo 25
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Figure 3.10. Stapes prosthesis displacement: Coronal computed tomography of the petrous bones demonstrates medial displacement of the stapes prosthesis into the vestibule.
haemorrhage may be demonstrated on CT or
MRI, as in the post traumatic situation. Following
cochlear implantation, vertigo may result from
a gusher, and this may be predicted on pre
implant CT or MRI imaging by detecting labyrinthine anomalies, including cochlear modiolar
deficiency.24
Developmental
A range of developmental normalities and variations may be seen in patients with dizziness,
however causation may be difficult to establish.
Some congenital vestibular disturbances are secondary to functional deficiency of the vestibular
epithelium, however, there are some macroscopic
labyrinthine malformations, such as large vestibular aqueduct syndrome (Figure 3.11), which are
associated with vertigo. 25 Anomalies associated
with deficiency of the modiolus of the cochlea or
lamina cribrosa may also result in vertigo, due to
perilymph hydrops and translabyrinthine fistulae
through the oval window. Non-labyrinthine
developmental variations, such as high riding jugular bulbs and jugular diverticulae,
may erode the inner ear structures such as the
vestibular aqueduct and posterior semicircular
canal, resulting in hearing loss, vertigo, and tinnitus. 26,27 Such labyrinthine and developmental
anomalies are usually well demonstrated on both
MRI and CT.
Central vestibular disturbance
Vascular
The brain stem, cerebellum and the labyrinthine
structures are all supplied by the vertebrobasilar
arterial system, so central and peripheral ischaemic
vestibular syndromes may occur concurrently.
Ischaemic infarction is secondary to embolus
(­cardio-embolic or artery to artery) or in situ
thrombosis of the large or small (perforating)
­vessels within the posterior fossa.
Cerebellar infarction presenting with vertigo is
usually related to involvement of the medial branch
of the posterior inferior cerebellar (PICA) territory28 or the anterior inferior cerebellar artery
(AICA)29,30 and such infarcts may present with
26 Dizziness and Vertigo: An Introduction and Practical Guide
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Figure 3.11. Large endolymphatic sac anomaly (LESA): T2 CISS Axial magnetic resonance imaging demonstrates an enlarged endolymphatic duct and intra osseous sac.
Figure 3.12. Lateral medullar infarct: T2 Axial magnetic resonance imaging demonstrates T2 hyperintensity
within the right posterolateral medulla secondary to a posterior inferior cerebellar infarction (in the setting of
vertebral dissection).
isolated vertigo or imbalance in 10% of cases
(Figure 3.12). This may be confused with peripheral vestibular syndromes, as symptoms of nausea
and vomiting are often prominent. Ischaemia of
the labyrinth may also occur concurrently, since
the internal auditory (labyrinthine) artery, usually
arises from the AICA.7,29
Posterior fossa haemorrhage and large infarcts can
readily be demonstrated on CT in the ­emergency
Imaging in dizziness and vertigo 27
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setting, however, MRI is more likely to demonstrate
smaller lesions and enables the ­diagnosis of acute
ischaemia through diffusion imaging. There is
evidence that detection of v­ ertebrobasilar stenosis
with vascular i­ maging following posterior circulation infarction may detect groups of patients who
have a high early recurrent stroke risk and may
benefit from stenting.31
Migrainous vertigo (vestibular migraine) represents the most common cause of central vertigo.32,33
There are usually no specific findings on imaging,
although areas of posterior circulation infarction
may be associated, particularly in those patients
with an aura.34 It has been speculated that neurovascular contact of the AICA with the vestibular
nerve may result in audiovestibular symptoms
including vertigo and dizziness, however evidence
is conflicting35,36,37 (Figure 3.13).
Demyelination
Isolated central vertigo may be an initial symptom
of multiple sclerosis in up to 5% of cases and 70% of
multiple sclerosis patients report some abnormality
in balance.7,38 MRI is used to demonstrate demyelinating lesions. Involvement of the medial vestibular nucleus and the superior cerebellar peduncle by
demyelinating plaques (Figure 3.14) has been noted
in the context of acute positional vertigo. Proton
density (PD) sequences are particularly useful in
standardised multiple sclerosis MRI protocols, in
order to demonstrate lesions within the posterior
fossa with greater sensitivity.39
Tumours
Intra axial lesions may occasionally present with
vertigo, however, are more typically associated
Figure 3.13. Intrameatal vascular loops: T2 CISS Axial magnetic resonance imaging demonstrates loops of
the anterior inferior cerebellar artery extending to both internal auditory meatii and contact with the cochlear
and vestibular nerves bilaterally.
Figure 3.14. Demyelination: T2w axial magnetic resonance imaging shows demyelinating plaques in the
superior cerebellar peduncles and upper pons.
28 Dizziness and Vertigo: An Introduction and Practical Guide
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with ataxia and disequilibrium. Tumours
in the posterior fossa in childhood are typically p
­ ilocytic astrocytoma, medulloblastoma
and ependymoma whilst intra axial posterior
fossa lesions in adults are most frequently
metastatic.
Other
A number of other neurological conditions with
typical imaging appearances may be associated
with vertigo or dizziness, although it is rarely the
dominant or isolated feature. A Chiari 1 malformation is characterised by caudal displacement of the
cerebellar tonsils through the foramen magnum
(Figure 3.15). Both intracranial hypotension and
intracranial hypertension may demonstrate characteristic MRI features. Partial seizures may result in
epileptic vertigo, as a result of vestibular representation within the cortex,40 and a focal epileptogenic
lesion may be evident on MRI. Superficial siderosis
(Figure 3.16), usually due to recurrent subarachnoid
bleeding and haemosiderin deposition secondary to
neoplasms, trauma and surgery, is associated with
Figure 3.15. Chiari 1 malformation: T1 Sagittal magnetic resonance imaging demonstrates descent of the
cerebellar tonsils which appear angulated and compacted at the foramen magnum.
Figure 3.16. Superficial siderosis: T2* Axial magnetic resonance imaging demonstrates low T2 signal
haemosiderin lining the pial surfaces.
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cerebellar ataxia and a specific vestibular defect
due to siderosis of the vestibular nerve.41 Dizziness
and disequilibrium may also be a manifestation of
hereditary or acquired cerebellar degeneration, such
as paraneoplastic syndrome or ethanol consumption,
which is characterised by cerebellar volume loss.
Conclusion
Imaging is of diagnostic benefit in those patients
who present with atypical features, resistant
symptoms or with central vertigo. However, it is of
limited value in those with peripheral symptoms,
where the diagnosis is usually clinical. CT and MRI
may be complimentary or used alone to aid diagnosis and the value of these modalities rely on their
appropriate use based upon the patient’s history,
symptoms and signs.
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32 Dizziness and Vertigo: An Introduction and Practical Guide
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4
Special investigations
used in the assessment
of the dizzy patient
Presanna Premachandra
Contents
Introduction
Audiological examination and testing
Oculomotor tests
Smooth pursuit
Saccades
Optokinetic nystagmus
Vestibulo-ocular reflex (VOR)
Spontaneous nystagmus
Caloric testing
Rotatory chair testing
Vestibular evoked myogenic potential
Posturography
Summary
33
33
35
36
36
36
38
40
41
43
44
45
46
Introduction
In the management of every patient p
­ resenting with
dizziness, a detailed history and t­ horough c­ linical
assessment are mandatory. Audiovestibular ­testing
establishes baseline vestibular function and determines whether a vestibular abnormality is peripheral, central or of mixed origin, and hence often
confirm a working diagnosis. This allows appropriate
treatment to be planned. In 5–10% of cases, the
results of this test battery will reveal unexpected
pathology, such as bilateral vestibular hypofunction,
or broken smooth pursuit. These investigations may
also be used as a screening tool in order to identify
those patients who may also require additional
investigations such as brain imaging.
Audiological examination and testing
Otoscopy is always performed prior to testing.
Wax occlusion, otitis externa or a middle ear
effusion will affect stimulus presentation to the
inner ear and in such cases, microsuction may be
required or testing deferred until an active infection or middle ear effusion has resolved.
Special investigations used in the assessment of the dizzy patient 33
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A pure tone audiogram (PTA) is required in every
patient. A sensorineural hearing loss may be present in patients suffering from Ménière’s disease, for
example. In such patients, the sensorineural hearing loss follows a recognised pattern (a fluctuating
low frequency loss in the initial stages, followed by
a high frequency loss which then plateaus across all
thresholds).
Labyrinthitis may also cause a sensorineural hearing loss, whilst superior semicircular
canal dehiscence is associated with an apparent
conductive hearing loss that may be mistaken for
early otosclerosis.
Tympanometry, an audiological test of admittance
and impedance, is always required in order to
exclude a middle ear effusion or tympanic membrane perforation.
Auditory brainstem response (ABR) testing records
the neurophysiological response to an auditory
stimulus (Figure 4.1). Acoustic energy is transferred
Auditory
cortex
Thalamus
V. Inferior
colliculus
IV. Lateral
lemniscus
II. Cochlear nuclei
SOUND
I. Auditory nerve
III. Superior olive
Figure 4.1. Auditory brain stem response pathway.
34 Dizziness and Vertigo: An Introduction and Practical Guide
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[µV]
V
4
IV
3
VI
III
I
2
VII
II
1
1
2
3
4
5
6
7
8
9
[ms]
Figure 4.2. A normal auditory brainstem response trace.
Table 4.1. Normative data for ABR.
Time post stimulus
presentation (ms)
Wave I
Wave III Wave V
Females
1.49
3.50
5.23
Males
1.55
3.63
5.65
to the inner ear resulting in stimulation of the
auditory nerve pathway and higher cortical centres.
Bursts of activity occur along this pathway which
can be recorded via electrodes placed on the head
resulting in an ABR trace (Figure 4.2).
Our normative data (for 18–24 years olds using
insert earphones with clicks at 80dBHL) is set out
in Table 4.1.
Abnormalities in wave latency may indicate a
conductive hearing loss (where each wave form is
delayed by the same amount), cochlear loss (with
absence of waveforms if severe/profound) or retrocochlear (with increased latencies of wave III and V).
ABRs have approximately 90% sensitivity in
detecting extracanalicular neuromas and 75%
sensitivity for intracanalicular neuromas.
Although ABR testing has been largely superseded by magnetic resonance imaging for detecting retrocochlear pathology, it remains a useful
assessment tool for those unable to undergo
imaging.
Oculomotor tests
Oculomotor tests are routinely performed as part
of a full audio-vestibular battery. These allow
a functional assessment to be made of a number of
anatomically distinct pathways.
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The vestibular and visual systems combine in
order to stabilise vision via four pathways: smooth
pursuit, saccadic movement, the optokinetic reflex
and vestibulo-ocular reflex. The smooth pursuit
pathway enables tracking of slow moving ­targets,
whereas the saccade system rapidly enables the
eyes to fixate on one point and then another.
The optokinetic pathway is believed to be a primitive vestigial reflex to alert both hunter and prey of
movement in their peripheral field. The vestibularocular reflex requires head movement and enables
gaze to be maintained on fast moving objects.
Eye movements are recorded using one of two different detection systems:
1 Electronystagmography (ENG) measures
the corneo-retinal potential, but ­recordings
are susceptible to electrical noise, are invasive
with electrode application, and re-­calibration is
required due to corneo-retinal potential
change with altered ­lighting ­conditions that
take place during testing.
2 Videonystagmography (VNG) detects
pupil movement using an infra-red camera set
in swim-type goggles. It is often favoured as
it is non-invasive and re-calibration is seldom
required.
Testing is performed in relative darkness to
optimise stimuli definition. Vestibular sedatives
are prohibited 48 hours prior to testing and eye
make-up removed to optimise monitoring. Patient
alertness is required as drowsiness will affect gain
measurement. Eye muscle weakness and reduced
visual acuity may also affect testing.
Smooth pursuit
A subject is asked to visually track a target as it
moves from side to side in the horizontal plane
(Figure 4.3). As with saccadic movement and
optokinetic nystagmus (OKN), the visual target
may be either projected onto a screen or on a liquid
crystal display (LCD). The smooth pursuit pathway
stabilises a moving target on the fovea for velocities less than 60 degrees per second or with 1 Hz
periodicity. At velocities greater than this intrusive
saccades may be introduced. Table 4.2 describes
normal test results and interpretation of abnormal
recordings, Figure 4.4 smooth pursuit tracings.
Saccades
Saccades move the eye rapidly between points and
are recorded by asking a subject to visually track a
target as it is randomly presented in the horizontal
or vertical plane (± 20 degrees from primary position, horizontally or vertically). Velocity, latency
and accuracy are measured.
Saccade velocity is not affected substantially by
age or gender. Saccades are either voluntary or
involuntary (Figure 4.5). Voluntary saccades
occur in response to flashing or moving stimuli,
or a remembered target on the peripheral retina.
Involuntary saccades form the fast phase of nystagmus. Table 4.3 sets out normal and abnormal
parameters.
Optokinetic nystagmus
Optokinetic nystagmus (OKN) is produced when a
subject tracks visual field movement. This results in
rapid eye movement (an involuntary saccade) that
relocates gaze onto new targets entering the field.
A common example of this is when an individual
looks out on the scenery when travelling on a moving train. Although nystagmus occurs the p
­ assenger
sees a seamlessly continuous moving scene.
Active OKN is smooth pursuit with involuntary
saccade. Passive OKN involves (extrafoveal) reflex
tracking of visual field movement followed by
a corrective saccade. OKN is more robust than
pursuit because it is less affected by inattention and
central vestibular sedatives. The pathway involved
in generating the passive response is illustrated in
Figure 4.6.
Assessment of OKN is either performed by using a
striped rotating drum, or by exposure to a ­projected
moving striped pattern. Table 4.4 below sets out
­normal values and how to interpret abnormal
tracings:
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Moving
target
Lateral
geniculate
body
Retina
Frontal
Pursuit
Area
Superior
colliculus
Primary
visual
cortex
Inferior
parietal
cortex
Middle
temporal
cortex
Pons
Cerebellum
Vestibular
nucleus
Optic
motoneurones
Figure 4.3. The smooth pursuit pathway. Pursuit (solid arrows) is followed by the corrective biofeedback
pathway (dashed). Patients keep their head steady whilst tracking a sinusoidal moving target (horizontally or
vertically).
Table 4.2. Smooth pursuit normative data and interpreting abnormal results (for target velocity <30% and
0.3 Hz (oscillation).
Normal
Gain
Eye velocity/target
velocity >0.8
Morphology Without saccadic
interruption
Abnormal
Reduced (0.2–0.8): Poor
attention, reduced visual
acuity, central system drugs,
alcohol
Absent (0–0.2): Central nervous
system disturbance, reduced
visual acuity (poor central
vision)
Poor: Reduced visual acuity,
drug and alcohol ingestion,
poor attention
Asymmetry: Eye muscle
weakness for example
congenital or latent nystagmus,
central system dysfunction
Special investigations used in the assessment of the dizzy patient 37
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Figure 4.4. Traces of smooth pursuit, with upward tracing denoting rightward eye movement and downward
tracing denoting leftward eye movement. The top trace is normal pursuit, poor morphology pursuit due to
­inattention is shown in middle trace and broken pursuit due to a central system abnormality is shown with the
bottom trace.
Vestibulo-ocular reflex (VOR)
Whilst smooth pursuit is used for tracking slow
moving targets, head movement is required for
faster targets and utilises the vestibulo-ocular reflex
(Figure 4.7).
The semi-circular canals are functionally paired
(anterior ipsilateral to superior contralateral, and
the lateral with each other). Head movement causes
at least one pair to be stimulated; that of the lateral
canals are now considered.
Rightward head rotation causes endolymph flow
within both lateral semicircular canals. This results
in movement of the cupulae within each ampulla
and relative shearing of the underlying stereocilia.
This causes an increase in right neural impulses
38 Dizziness and Vertigo: An Introduction and Practical Guide
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DETECTION
Visual
target
RECOGNITION
Lateral
geniculate
body
Retina
Primary
visual
cortex
I
V
I
Superior
colliculus
V
Posterior
parietal
cortex lobe
Frontal
(cortex) eye
fields lobe
RESPONSE INITIATION
Pons
(PPRF)
Memory
Key
Optic
motor
neurones
I
V
PPRF
Involuntary
Voluntary
Parapontine reticular
formation
Figure 4.5. The saccadic pathway. Saccade production is shown above. Biofeedback (involving the
­cerebellum) repositions the eye on target.
Table 4.3. Saccadic normal measurements and interpreting abnormal results.
Normal
Abnormal
Velocity
>350 and <750 degrees
per second
Slow: Drugs/fatigue/oculomotor
weakness
Fast: Optic flutter (normal
variant)/opsoclonus
(uncontrolled eye movement)
Accuracy
100% +/−5%
Undershoot: Peripheral visual
disorder/Superior colliculus
disorder (if occurs >50%)
Overshoot: Cerebellar
dysfunction
Latency
200–400 ms (milli
seconds)
Long: Visual difficulty for
example cataract
Short: Anticipated
Pattern
Macrosaccadic oscillation:
Microsaccadic oscillation:
Hypermetric saccades overshoot Tiny back-to-back saccadic
their target resulting in
oscillation; usually benign
oscillation indicating cerebellar
disorder
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Moving
target
Retina
Lateral
geniculate
body
Optic
motoneurone
Vestibular
nucleus
Cerebellum
Figure 4.6. Pathways involved in optokinetic nystagmus.
Table 4.4. Optokinetic nystagmus (OKN) normal measurements and interpreting abnormal results.
Normal
Abnormal
Gain
>0.75
Reduced: Inattention, visual system
disorders/congenital nystagmus
Absent: Bilateral vestibular failure/
central vestibular abnormality
Morphology
Symmetrical
Asymmetry: Complete unilateral
peripheral vestibular disorder
Failure to reverse: Congenital
nystagmus
Slow phase: Pursuit system disorder/
medication
Fast phase: Saccade system disorder
Pattern
and decrease in left resulting in contraction of the
left lateral rectus and right medial rectus to stabilise
gaze.
The eyes move until they reach a physiological end
point, when a corrective saccade returns them to
the primary position, creating a nystagmic beat;
VOR is the slow phase and saccade the fast phase.
The VOR causes eye movement in the opposite
direction to head movement. However, visual suppression overrides VOR (Figure 4.8).
The eye movement tests described above examine
normal pathways for gaze stabilisation. However,
recording the presence of spontaneous nystagmus
indicates the presence of pathology.
Spontaneous nystagmus
Patients keep their head steady, then maintain their
eye position in primary and lateral eye positions
(30 degrees from centre) with and without fixation
on a visual target. Normally, eyes are maintained in
these positions without deviation.
Nystagmus observed in the absence of head or
body motion is called spontaneous nystagmus
and over six degrees per second is significant.
Visual fixation usually suppresses the nystagmus produced by a peripheral vestibular deficit,
but may be impaired with medicinal, alcohol or
recreational drug use or if the patient has ocular
muscle fatigue, reduced visual acuity or a central
vestibular dysfunction.
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Medial rectus
Lateral rectus
Oculomotor nucleus
Abducens nucleus
Vestibular nucleus
Neural firing rate
Head turning
Figure 4.7. The vestibulo-ocular reflex.
Caloric testing
This assesses the VOR of each lateral canal by aural
irrigation using water or air. Water caloric testing has been long established with the American
National Standards Institute providing test standard
conditions. The British Society of Audiology has
approved standards for both water and air caloric
testing and these parameters are listed in Table 4.5.
Air caloric testing can be used in patients with
tympanic membrane perforations or with mastoid
cavities, but interpretation of results must be taken
with caution due to altered ear structure.
Patients lie on a couch inclined at 30 degrees to
horizontal. Irrigating the ears with stimuli different
to body temperature creates a thermal gradient in
the middle ear. This results in endolymphatic flow
by convection in the lateral canal due to its proximity to the middle ear. Cold air/water ear irrigation
causes endolymph flow in the opposite direction
to warm air/water, creating decreased or increased
stimulation respectively of the vestibular nerve of
the ear being irrigated.
Four irrigations are performed, with the test
order being warm air/water irrigation to the
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Retina
Head
movement
Fixation
light
Semicircular
canal
Lateral
geniculate
body
Vestibular ocular reflex
Optic
motoneurone
Vestibular
nucleus
Suppression
Cerebellum
Figure 4.8. Neurological pathways of the vestibulo-ocular reflex (suppression pathway in dashed arrows).
Table 4.5. Testing parameters for caloric testing.
Temperature (°C)
Warm
Cold
Irrigation time (s) Flow volume
Water
44
30
30
250 mL
Air
24
50
60
8L
right ear, then warm left, cold right and finally
cold left. Each irrigation causes stimulation of
the lateral canal, simulating head movement,
which results with VOR production and corrective ­saccade. The slow phase velocity (SPV)
of the VOR is monitored and the maximum
velocity is recorded. Suppression of the VOR is
then assessed with the patient fixating on a light
(Figure 4.9).
Canal paresis:
The irrigation test results are interpreted using
Jonkee’s formula that calculates canal paresis and
directional preponderance. Canal paresis is reduced
activity of one of the lateral canals. Directional
preponderance indicates imbalance within the vestibular system due to a poor peripheral or central
deficit.
(Right cold + Right warm)–(Left cold + Left warm)
× 100
(Right cold + Right warm) + (Left cold + Left warm)
Directional preponderance:
(Right warm + Left cold)–(Left warm + Right cold)
× 100
(Right cold + Right warm + Left cold + Left warm)
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Retina
Air/water
Irrigation
Vestibular ocular reflex
Fixation
light
Lateral
semicircular
canal
Optic
motoneurone
Lateral
geniculate
body
Vestibular
nucleus
Suppression
Cerebellum
Figure 4.9. Flow pathway for the caloric response (suppression pathway in dashed arrows).
Table 4.6. Caloric test normal measurements and interpreting abnormal results.
Normal
Abnormal
Responsivity from slow
phase velocity of each
irrigation
>5 and <60 degrees/second
Total reactivity (sum of all 4 responses) ≤20
degrees/second: Bilateral peripheral
vestibular hypofunction (failure is confirmed
using ice cold water irrigation)
Canal paresis
<20%
≥20%: unilateral peripheral vestibular deficit
Directional preponderance
<20%
≥20%: peripheral vestibular deficit/central
system dysfunction
Suppression
Present
Absent: central vestibular dysfunction/visual
impairment
Caloric testing helps to confirm a diagnosis,
establish baseline function and detect unsuspected
vestibular hypofunction or failure. Table 4.6 shows
how to interpret the results.
are non-physiological at < 0.01 Hz and therefore the
caloric test is often performed in conjunction with
rotatory tests that are more representative of real
body motion.
Caloric testing is unique in that it assesses the function of each lateral canal. However, caloric stimuli
Rotatory chair testing
Rotatory chair tests produce stimuli of 0.1–1.2 Hz,
which are more representative of body motion. They
are used when patients have chronic ear infection,
stenotic ear canals or if patients refuse caloric testing. As rotatory tests assess both inner ears simultaneously, unilateral dysfunction is difficult to discern
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Chair
rotation
Vestibular ocular reflex
Retina
Lateral
semicircular
canal
Optic
motoneurone
Fixation
light
Lateral
geniculate
body
Vestibular
nucleus
Suppression
Cerebellum
Figure 4.10. Flow pathway for rotatory chair testing. Phase, gain and symmetry of the responses are
­measured and abnormality usually indicates either unilateral or bilateral vestibular dysfunction.
but rotatory tests are good at establishing bilateral
vestibular hypofunction of the lateral canals.
In a darkened room, patients are secured in a
motorised chair that moves at controlled velocities
in a circular motion. Stimulation of both lateral
canals occurs resulting in VOR and corrective saccade production.
In sinusoidal motion, the chair oscillates at
various frequencies with phase, gain and symmetry measurements of the VOR recorded. Phase
measurements describe the timing relationship
between eye movement to head movement and
these movements are usually around 180 degrees
out of phase. Gain measurement is the ratio of
eye movement velocity to chair velocity (usually 1) and symmetry measurement is a comparison of VOR for rightward and leftward chair
motion (usually equal). Patients with bilateral
hypofunction may have no VOR but if VOR is
present the phase difference is greater than normal and the gain measurement is much reduced.
Suppression ability of VOR is also assessed in
sinusoidal testing using a fixation light whilst
the chair is in motion. Patients with central vestibular disorders may have impaired suppression
(Figure 4.10).
Vestibular evoked myogenic potential
Vestibular evoked myogenic potential (VEMP) testing assesses otolith function and is complementary
to caloric and rotary testing that assess lateral canal
function. This is often used in patients suspected of
having superior semi-circular canal dehiscence.
Loud sounds stimulate the otolith organs leading
to contraction of antigravity muscles of the neck
and spine via the vestibular spinal reflex (VSR),
causing the body to turn to the sound source
(Figure 4.11). Increased electrical activity in the
neck muscles can be recorded. The cervical VEMP
(C-VEMP) assesses the VSR that involves the saccule, inferior branch of the vestibular nerve and
sternocleidomastoid (SCM) muscle. Loud sounds
may also provoke another type of vestibular
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Loud sound
Vestibular
sensory
cells
Vestibular
nucleus
Spinal/ocular
muscle
innervation
Figure 4.11. Flow pathway for vestibular evoked myogenic potential (VEMP) testing.
Table 4.7. VEMP test normal values and interpreting abnormal results.
Normal
Abnormal
P1 latency (ms)
13–14
N1 latency (ms)
21–23
Delayed: Central vestibular system
dysfunction or endolymphatic hydrops.
Threshold (dBHL)
80–95
Lower: Superior canal dehiscence
ocular reflex by utricule stimulation. The ocular
VEMP (O-VEMP) may provide information of
the activity of this organ by assessing change in
eye muscle potentials but this technique has not
yet been fully established. C-VEMP recording is
however well used.
For C-VEMP measurements, patients lie
down with their neck elevated, or sit with
their head turned sideways to provide resting muscle ­tension in the ipsilateral SCM for
C-VEMP ­comparison. An electrode is placed
on the SCM belly, clavicle and forehead (earth).
Auditory stimuli (usually 500 Hz tones) are then
presented via an ipsilateral insert earphone or
headphone and the VEMP is recorded in the SCM.
The P1–N1 (peak–trough) complex is measured at
different stimulus intensities. Parameters recorded
are: amplitude, threshold and latency. Normally,
increased stimulus intensity increases amplitude of
response yet this does not increase P1–N1 latency.
Table 4.7 below shows normative data using 50 Hz
tones and pathologies associated with abnormal
traces.
Posturography
This assesses the overall balance maintenance of
a patient who stands on a platform that monitors sway. The patient stands on a platform and is
attached to a safety harness. The platform is able to
pivot antero-posteriorly in response to the patient’s
sway (sway referenced). The patient’s ­corrective
mechanisms to this altered proprioceptive input
are then measured. The patient is enclosed in front
and at the sides by a pictured frame that is also able
to move in a sway referenced way and the effect
of altered visual input on sway is thus assessed.
Six different test conditions are presented here and
the sway is measured to assess the subject’s effective
use of visual, v­ estibular and proprioceptive inputs
in ­maintaining balance.
Condition 1: Eyes open, platform steady, curtain
fixed.
Condition 2: Eyes closed, platform steady, curtain
fixed.
Condition 3: Eyes open, platform steady, curtain
sway referenced.
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Condition 4: Eyes closed, platform steady, curtain
sway referenced.
Condition 5: Eyes open, platform sway referenced,
curtain sway referenced.
Condition 6: Eyes closed, platform sway referenced,
curtain sway referenced.
Normal subjects have little sway with eyes
open and closed conditions in all six ­situations.
Patients with functional loss tend to have
­i nconsistent responses whereas those with
­vestibular failure are unable to maintain
their balance in the platform sway referenced
­conditions. The test is not sensitive to other
balance deficits but is useful for monitoring the
effectiveness of rehabilitation.
Summary
Audio-vestibular tests provide a sensitive and
reliable method for detecting subclinical eye signs
related to peripheral and central vestibular deficits.
They are an essential adjunct to a ­t horough
­clinical assessment in most balance disorder
patients.
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5
Differential diagnosis
Rahul Kanegaonkar
Most medical conditions are diagnosed on the
basis of an accurate history, thorough clinical
examination and necessary special investigations.
Vestibular conditions are no exception. Pattern
recognition based on the frequency, setting and
duration of spells, a neuro-otological examination,
audio-vestibular tests supported where necessary
by imaging studies will in the majority of cases
allow an accurate diagnosis to be made. As previously described, special investigations both support
a working diagnosis and also on occasion produce
an unexpected result (e.g. a unilateral or bilateral
vestibular hypofunction).
However, in some cases despite a full work-up
a diagnosis cannot be reached. An alternative
approach that can be adopted in these situations
is based on excluding possible diagnoses. This
method essentially considers the sensory pathways and central nervous system, and a list of
potential differential diagnoses (note Figure 5.1,
Table 5.1).
Once a differential diagnosis has been reached,
it may then be appropriate to manage patients
medically in the first instance, and combine treatment with appropriate vestibular physiotherapy
support. Early Ménière’s disease, for example,
may mimic vestibular migraine. In such instances
maximal medical therapy of both conditions may
be appropriate with regular outpatient follow up.
A new management plan can be introduced once
a clear diagnosis has been made as the condition
evolves.
As methods available to test the vestibular system continue to evolve, our ability to accurately
diagnose and hence appropriately treat patients
will improve significantly. Although many
medical practitioners reach a diagnosis and plan
a management pathway in isolation, many units
have adopted a multi-disciplinary approach,
whereby the history, examination, and special
investigations are discussed amongst the medical, audiovestibular and physiotherapy teams in a
multi-disciplinary team setting. This allows each
aspect of the assessment to be taken in context to
gain a much better understanding of the entire
patient. A customised treatment plan can then be
instituted.
The subsequent chapters aim to allow a clinician to
come to a diagnosis and also recognise associations
between common vestibular conditions.
Differential diagnosis 47
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Input
Output
Integration and
interpretation
Gaze stabilization
Vision
Peripheral vestibular system
Templates
Postural control
Proprioception
Hearing
Spatial
awareness
Figure 5.1. An overview of normal human balance.
Table 5.1. Relative prevalence of vestibular
pathology presenting to a specialist balance service.
Benign paroxysmal positional vertigo
Unilateral peripheral vestibular deficit
Vestibular migraine (vertiginous migraine)
Multilevel vestibulopathy
Bilateral peripheral vestibular deficit
Cholesteatoma
Bilateral vestibular hypofunction
Hyperventilation syndrome/psychological
Ménière’s disease
Superior semicircular canal dehiscence
Perilymph fistula
Central pathology e.g. cerebrovascular accident,
multiple sclerosis, space occupying lesions
Pharmacological/drug induced
Paraneoplastic syndrome
48 Dizziness and Vertigo: An Introduction and Practical Guide
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6
Benign paroxysmal
positional vertigo
Nitesh Patel
Contents
Introduction
49
Aetiology
49
Pathogenesis
50
Symptoms
50
Signs50
Dix-Hallpike positional test
50
Management
51
Epley manoeuvre
52
Semont manoeuvre
52
Lateral SCC variant
55
Superior SCC variant
55
Surgery for intractable BPPV
56
Case studies
58
Conclusion
59
References
59
Introduction
Benign paroxysmal positional vertigo (BPPV) is
a clinical disorder characterised by brief ­episodes
of vertigo precipitated by changes in head ­position
with respect to gravity. It is the most common cause
of vertigo presenting to primary care and specialist
balance clinics alike, and has a lifetime prevalence
of 2.4% in the general population.1 The posterior semicircular canal (SCC) is most c­ ommonly
involved (85–95%) followed by the lateral SCC
(5–15%) and rarely the superior SCC.2
Aetiology
In the majority of cases (48%) no cause is found.3
BPPV may occur as a complication of head trauma,
vestibular neuronitis, surgery (stapedectomy or
cochlear implant surgery) or develop during the
course of an inner ear disorder such as Ménière’s
disease or autoimmune inner ear disease. Patients
with BPPV have recently been found to have
more osteopenia and osteoporosis than matched
Benign paroxysmal positional vertigo 49
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controls, and those with recurrent BPPV the
lowest bone density scores.4 This suggests bone
demineralisation may be involved in otoconia
release (see below).
Pathogenesis
Schuknecht5 proposed the theory of cupulolithiasis in which otoconia (calcium carbonate crystals)
from a degenerating utricular macula form otoliths
that settle on the cupula of the posterior SCC. These
otoliths are more than twice as dense as endolymph
and so move in response to gravity and acceleration with displacement of the cupula and resulting
vertigo.
An alternative canalithiasis theory was proposed by
Hall et al.6 in which the otoconia enter the posterior
SCC and float freely in the endolymph under the
influence of gravity. Considering the upright position,
the posterior SCC is gravitationally vertical and any
otoconia will come to rest near the cupula. When
laid back and turning to the same side, the otoconia
fall from the cupula and rest in the middle of the
duct. As they fall away from the cupula they create
a negative fluid pressure which exerts a pull on the
cupula producing an ampullofugal deflection that is
excitatory for the posterior SCC afferent neurones.
In most typical cases it is likely that canalithiasis is responsible due to the nystagmus having a
latency (brief duration), the nystagmus reverses on
returning to a sitting position, and the condition
is p
­ aroxysmal. It is assumed that due to anatomical factors free otoconia are less likely to enter the
lateral and superior SCC. However these variants
do exist and are discussed later.
Symptoms
Patients classically describe rotatory vertigo on
turning in bed or rising in the morning. Although
the vertigo usually lasts seconds, patients may estimate their symptoms to last minutes.3 There is no
associated hearing loss nor tinnitus. Patients suffer
marked nausea and vomiting. On occasion patients
describe rotating vertigo or tumbling on looking
up (“top shelf syndrome”) or down quickly. Most
patients become wary of certain head movements
as they anticipate onset of their symptoms.
Signs
A neurotological examination is usually normal in
patients with idiopathic BPPV. Diagnosis rests on
history together with the finding of a characteristic
positional nystagmus induced by rapidly moving
the patient from the sitting to head-hanging position as originally described by Dix and Hallpike.7
Dix-Hallpike positional test
It is important to fully explain the test and ensure
the patient is aware that dizziness may be induced.
Explanation can be aided by a short demonstration
video.8 They should be asked to keep their eyes open
and look straight ahead to enable any nystagmus
to be clearly observed. Nystagmus detection can be
aided with either Frenzel glasses or videonystagmography if available. They should be reassured
that the examiner will be fully supporting their
head to encourage them to relax their neck muscles.
They should be seated on a couch such that when
laid supine their head can be tilted backwards into
a hanging position 30 degrees below horizontal.
The steps of the manoeuvre are demonstrated
in Figure 6.1. Typically a torsional nystagmus
50 Dizziness and Vertigo: An Introduction and Practical Guide
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Figure 6.1. (A) Dix-Hallpike positioning manoeuvre to the right. (B) Dix-Hallpike manoeuvre to the left. Observe
for a latency of 5 to 20 seconds after positioning before the onset of nystagmus. Observe for nystagmus that is
upbeating and torsional with duration of 10 to 30 seconds. The side with the downward ear is the affected side
in benign paroxysmal positional vertigo of the posterior canal. Reprinted with permission from Fife, Terry. D;
Benign Paroxysmal Positional Vertigo; Seminars in Neurology; 29/5. Barrow Neurological Institute © 2009.
is observed with the upper pole of the eye beating towards the ground (geotrophic). A reversed
nystagmus of lesser amplitude can be observed on
returning the patient to the sitting position. There
is latency with initiation of n
­ ystagmus between five
and 20 seconds after reaching the head-hanging
position. The provoked subjective vertigo and the
nystagmus increase, and then resolve within a time
period of 60 seconds from the onset of nystagmus.9
With repeated positioning the nystagmus tends to
fatigue in the majority of patients.
It is important to note that a negative DixHallpike test does not necessarily rule out BPPV,
as there is a sensitivity of 82% and specificity of
71%.10 In this group with a good clinical history
it may be necessary to repeat the test on another
occasion.
Management
A thorough clinical history and examination are
essential in order to differentiate BPPV from other
causes of dizziness. However, a similar presentation can occur with cervical vertigo. Cervical
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vertigo has been described as vertigo arising in
conjunction with degenerative cervical spine disease caused by abnormal proprioceptive input.11
In cervical vertigo, symptoms may be triggered
by rotation of the head relative to the body while
in an upright posture (as opposed to vertigo
triggered by changes in head position relative to
gravity), and the Dix-Hallpike manoeuvre will be
negative.
Further investigations, such as radiological ­imaging
or vestibular function tests, are not w
­ arranted
where there is a history, examination and DixHallpike test consistent with BPPV. These tests
should however be considered in persistent cases,
treatment failures or atypical presentations.
It is important to reassure the patient that this is a
benign inner ear disorder and is treatable. The aim
of treatment is to move the otoconia from the
posterior SCC through the crus communis back
into the utricle through a repositioning manoeuvre. Once the otoconia are back in the vestibule,
they are absorbed within a period of days in most
patients.
Brandt and Daroff 12 were the first to propose exercises, based on the theory of cupulolithiasis, which
can be effective (Figure 6.2). The exercises are
repeated many times a day for two to three weeks
aiming to habituate symptoms in which the patient
repeatedly moves from the sitting to the head
hanging position. However, this repeatedly induces
vertigo and is less well tolerated by patients.
The Epley ‘canalith repositioning procedure’13 and
Semont ‘liberatory’ manoeuvre14 are more widely
used to treat posterior SCC BPPV with good outcome. BPPV has been reported to be immediately
eliminated in more than 85% of patients.9
Patients given instructions to self-treat with canalith repositioning manoeuvres at home show better
improvement than self-administered Brandt Daroff
exercises.15
As an alternative, vestibular rehabituation exercises have also been suggested to be an effective
treatment for BPPV, although costs of repeat visits
to the balance therapist and a significant recurrence
rate have to be considered.16
Epley manoeuvre
The steps of this manoeuvre are demonstrated in Figure 6.3. The manoeuvre begins by
­placing the patient into the Dix-Hallpike position for the affected side and inducing vertigo.
The head is then turned in two 90 degree increments towards the opposite side, stopping until
any nystagmus resolves, until the nose is pointing 45 degrees from the ground. The patient is
then brought into a sitting position. The manoeuvre is repeated until no further nystagmus is
elicited.
It is worthy of mention that posterior SCC BPPV
can occasionally be converted to lateral SCC BPPV
during an Epley manoeuvre in 6–7% of cases.17
Some clinicians advocate the use of a vibrator on
the mastoid bone to aid canalith repositioning,
however this does not seem to offer any added
benefit.18 Patient co-operation can be aided by
showing a short video demonstrating the test.19
Caution should be exercised when treating patients
with cervical spine problems.13 In such patients
a new manoeuvre, suggested by Rashad20 can be
considered which is designed to avoid passive neck
manipulation. A rigid neck collar is fitted and the
head positions needed to direct particles along the
semicircular canal achieved with the help of an
adjustable operating table.
Semont manoeuvre
The ‘liberatory’ manoeuvre of Semont (Figure 6.4)
aims to dislodge the otoconia from the cupula
and move them out of the posterior semicircular
canal into the vestibule where they are harmless.
In the sitting position the patient’s head is turned
45 degrees to the unaffected ear. The patient is
rapidly swung sideways onto the involved side
provoking vertigo. After the vertigo subsides
the patient is rapidly swung sideways onto the
uninvolved side through a 180 degree cartwheel
motion.
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Figure 6.2. Brandt-Daroff exercises begin by sitting the patient upright on the edge of the bed, with the
head turned 45 degrees to one side. The patient is instructed to move rapidly down into the side-lying
­position, keeping the head turned in the same direction. This position is maintained until the precipitated vertigo subsides, or at least for 30 seconds. The patient then returns to the upright, and holds this position for an
additional 30 seconds. The head is then turned in the opposite direction, and the same procedure is repeated
on the other side. Patients repeat the whole sequence until vertigo is no longer experienced with changes in
position. These exercises may be performed several times each day, and should continue until at least one
full day after no symptoms of vertigo are experienced. Reprinted with permission from Fife, Terry. D; Benign
Paroxysmal Positional Vertigo; Seminars in Neurology; 29/5. Barrow Neurological Institute © 2009.
Benign paroxysmal positional vertigo 53
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Figure 6.3. Canalith repositioning manoeuvre. Step 1: Seat the patient on a table positioned so they may
be taken back to the head hanging position with the neck in slight extension. Stabilize the head with your
hands and move the head 45 degrees toward the side you will test. Move the head, neck and shoulders
together to avoid neck strain or forced hyperextension. Step 2: Observe for nystagmus and hold the position
for ~10 ­seconds after it stops. Step 3: Keeping the head tilted back in slight hyperextension, turn the head
~90 degrees toward the opposite side and wait 20 seconds. Step 4: Roll the patient all the way on to his or
her side and wait 10 to 15 seconds. Step 5: From this side-lying position, turn the head to face the ground
and hold it there 10 to15 seconds. Step 6: Keeping the head somewhat in the same position, have them sit up
then straighten the head. Hold on to the patient for a moment because some patients feel a sudden but very
brief tilt when sitting up. REPEAT: After waiting 30 seconds or so, repeat the whole manoeuvre. If there is not
paroxysmal nystagmus or symptoms during Dix-Hallpike positioning (Steps 1, 2) then there is a high likelihood
of success. Reprinted with permission from Fife, Terry. D; Benign Paroxysmal Positional Vertigo; Seminars in
Neurology; 29/5. Barrow Neurological Institute © 2009.
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Figure 6.4. Semont liberatory manoeuvre. Step 1: Start with the patient sitting on a table or flat surface
with head turned away from the affected side. Step 2: Quickly put the patient into the side-lying position,
toward the affected side with the head turned up. Nystagmus will occur shortly after arriving at the side-lying
position. Keep the patient here until at least 20 seconds after all nystagmus has ceased. Step 3: Quickly move
the patient back up and through the sitting position so that he or she is in the opposite side-lying position
with head facing down (head did not turn during the position change). Keep the patient in this position for
~30 seconds (some recommend up to 10 minutes). Step 4: At a normal or slow rate, bring the patient back
up to the sitting position. Reprinted with permission from Fife, Terry. D; Benign Paroxysmal Positional Vertigo;
Seminars in Neurology; 29/5. Barrow Neurological Institute © 2009.
Some clinicians advise patients avoid lying flat for
two days after canalith repositioning manoeuvres
or to wear a cervical collar to reduce the likelihood
of the otoliths returning back to the posterior SCC,
however this has not been shown to be of significant benefit.21
Lateral SCC variant
In lateral SCC BPPV symptoms can be provoked
on turning over in bed, turning the head to the
side when lying back in a chair, and occasionally turning the head to the side whilst sitting or
walking.
The diagnosis is confirmed by the supine roll test
(Figure 6.5). The patient is first placed supine on
the couch with the head in the neutral position.
The head is then rapidly turned 90 degrees to one
side with the ear resting on the couch and the eyes
observed for nystagmus. After return to the midline, the head is turned 90 degrees to the o
­ pposite
side. Horizontal geotropic direction changing (right
beating in head right position, left beating in head
left position) or horizontal ­apogeotropic direction
changing (left beating in head right position, right
beating in head left position) can occur. The nystagmus is stronger when turning to the affected side.
The latency is often brief, and the duration may be
15–60 seconds.
The positioning manoeuvre for lateral SCC ­variant
involves log rolling the patient (Figure 6.6).
The patient is placed supine and then rapidly rolled 90 degrees to the normal side. Then
rolled in 90 degree steps into the prone position,
affected side and then supine again. An alternative ­treatment can be to advise the patient to sleep
only on the unaffected side so that the otoconia can
find their way out of the lateral SCC back into the
vestibule.22
Superior SCC variant
In superior SCC BPPV torsional downbeat nystagmus is observed (apogeotrophic) during the
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Figure 6.5. Diagrammatic views of the supine roll test. (1) The patient is in the starting neutral position. The
patient’s head is turned rapidly to the right side (2) to examine tor characteristic nystagmus. Then the head is
returned to the face-up position (1). allowing all nystagmus to subside, and then turned rapidly to the left side
(3) to examine once again for nystagmus. Reprinted with permission from Fife, Terry. D; Benign Paroxysmal
Positional Vertigo; Seminars in Neurology; 29/5. Barrow Neurological Institute © 2009.
Dix-Hallpike test, as opposed to torsional upbeat
nystagmus (geotrophic) with posterior SCC
BPPV. The affected ear is uppermost as opposed
to lowermost with posterior SCC BPPV. Superior
SCC BPPV can be treated with the Epley manoeuvre starting with the affected ear uppermost
and progressing in similar fashion as shown in
Figure 6.3.
However this diagnosis should be considered
with caution as downbeating positional nystagmus can also be related to brainstem or cerebellar
lesions. In a review of 50 patients with downbeat
positional nystagmus, three quarters had central
nervous system (CNS) disease, with the remaining quarter of cases thought to have superior SCC
BPPV.23
Surgery for intractable BPPV
The vast majority of patients will be cured
by particle repositioning manoeuvres, however some patients may not respond especially
when the o
­ toconia are immobile or attached
to the cupula. In such cases surgery may be
indicated.
Gacek 24 has developed a procedure to section the
ampullary nerve of the posterior SCC (singular neurectomy). In 102 operated ears there was
elimination of positional vertigo in 97% of cases
although sensorineural hearing loss occurred
in 4%. 25 Parnes and McClure26 have described a
technically easier procedure that involves occluding the posterior SCC. A cortical mastoidectomy
is performed and the membranous labyrinth of
the posterior canal blue lined and filled with bone
dust and fibrinogen glue. They report resolution in 44 out of 44 occluded ears, with only 1
case developing late atypical recurrence. 2 A total
56 Dizziness and Vertigo: An Introduction and Practical Guide
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Figure 6.6. Lempert 360- (Barbeque) degree roll manoeuvre to treat horizontal canal BPPV. When the
patient’s head is positioned with the affected ear down, the head is then turned quickly 90 degrees toward
the unaffected side (face up). A series of 90-degree turns toward the unaffected side is then undertaken
sequentially until the patient has turned 360 degrees and is back to the affected ear-down position. From
there, the patient is turned to the face-up position and then brought up to the sitting position. The successive
head turns can be done in 15- to 20-second intervals even when the nystagmus continues. Waiting longer
does no harm, but may lead to the patient developing nausea, and the shorter interval does not appear
to detract from the effectiveness of the treatment. Reprinted with permission from Fife, Terry. D; Benign
Paroxysmal Positional Vertigo; Seminars in Neurology; 29/5. Barrow Neurological Institute © 2009.
osseous labyrinthectomy is rarely performed for
BPPV.
Patients should be informed that recurrence is
not uncommon with up to 50% patients having a
relapse by 5 years.27 Patients over 40 years of age
with a pre-treatment duration of BPPV attacks of
three years or more are associated with a higher
chance of recurrence.28
Magnetic resonance imaging (MRI) scanning
should be considered when there is atypical nystagmus, failure of repositioning manoeuvres or
asymmetrical sensorineural hearing loss to rule out
a retrocochlear or central lesion. Vestibular sedatives
are not indicated in BPPV as they do not suppress
the onset of acute episodes and symptoms are of
brief duration.
In some patients although positional vertigo is
abolished after particle repositioning manoeuvres, symptoms of general imbalance can persist.
An associated or underlying peripheral vestibular
deficit may exist in approximately 30% of such
cases. Formal audiovestibular testing is hence of
benefit in these cases and customised vestibular
­physiotherapy required.29
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Case study 6.1
A 56-year-old woman presented with a three-month history of intermittent spells of rotatory
vertigo when rising in the morning and rolling over in bed. As a result she avoided turning to
her left. Spells of vertigo lasted less than 30 seconds but she was left unsteady for an hour
thereafter. There was no hearing loss nor tinnitus, but she felt sick during each episode. Her
pure tone audiogram demonstrated early symmetrical presbyacusis.
On examination, her cranial nerves were intact, and head thrust testing and Unterberger
testing were both normal. Dix-Hallpike testing with her head turned to her left clearly demonstrated, following a latency of 5 seconds, geotropic torsional nystagmus that completely
settled after 20 seconds.
A diagnosis of left posterior canal benign paroxysmal positional vertigo (pc-BPPV) was
made and she underwent an Epley maneouvre.
A second left Dix-Hallpike maneouvre was performed. She demonstrated geotropic
torsional nystagmus that settled after 10 seconds. She therefore underwent a second Epley
maneouvre.
A third left Dix-Hallpike maneouvre did not provoke nystagmus.
She was reviewed six weeks later. Her vertigo had settled completely and she was
discharged.
Case study 6.2
A 45-year-old woman presented with a three-year history of intermittent vertigo. Spells of
vertigo were provoked by rolling over in bed to her right. Looking down when emptying the
tumble dryer, and looking up when taking tins out of a kitchen cupboard also provoked transient tumbling of her environment.
Neuro-otologocal testing was unremarkable other than head thrust testing that demonstrated a clear catch up saccade on rapid head movement to the left.
Right Dix-Hallpike testing provoked, following a latency of 10 seconds, geotropic torsional
nystagmus that persisted for 10 seconds. This settled completely. An Epley manouvre was
performed and the patient reviewed after eight weeks.
Although her symptoms had improved, she continued to complain of short spells of nystagmus on rolling over in bed to the right. Repeat Dix-Hallpike testing revealed a 5-second burst
of geotropic nystagmus following a latency of 10 seconds. A repeat Epley manouvre was
performed and she was reviewed eight weeks later.
On review, her vertigo had settled completely but she described intermittent disequilibrium.
Audiovestibular testing confirmed a right peripheral vestibular deficit.
She underwent a course of customized vestibular rehabilitation and her symptoms settled
completely.
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Case study 6.3
A 45-year-old male sustained a head injury following an alleged assault. He complained of a
three-year history of rotatory vertigo on turning to his right. There was neither hearing loss nor
tinnitus.
On Dix-Hallpike testing, following a short latency, geotropic torsional nystagmus was
clearly evident. A diagnosis of right pc-BPPV was made.
He underwent successive Epley manouvres, and attempted Semont manouvres. His symptoms, however, persisted.
Following a lengthy discussion a right posterior canal plugging procedure was recommended. He was warned of the risks of surgery including sensorineural hearing loss and
facial nerve palsy.
He underwent an uncomplicated right posterior canal plugging procedure and a course of
customized vestibular rehabilitation exercises.
His symptoms of vertigo settled completely and he was subsequently discharged.
Conclusion
BPPV is a common cause of vertigo. It can be
successfully treated by particle repositioning
manoeuvres. Although rarely required, surgical
intervention should be reserved for those patients
with intractable BPPV.
7 Dix MR, Hallpike CS. The pathology, symp-
References
1 Fife TD. Benign paroxysmal positional vertigo.
Seminars in Neurology. 2009;29(5):500–508.
2 Parnes LS, Agrawal SK, Atlas J. Diagnosis and
management of benign paroxysmal positional
vertigo (BPPV). CMAJ. 2003;169:681–693.
3 Baloh RW, Honrubia V, Jacobson K. Benign
positional vertigo: clinical and oculographic features in 240 cases. Neurology.
1987;37:371–378.
4 Jeong SH, Choi SH, Kim JY, Koo JW, Kim HJ,
Kim JS. Osteopenia and osteoporosis in idiopathic benign positional vertigo. Neurology.
2009;72(12):1069–1076.
5 Schuknecht HF. Cupulolithiasis. Arch
Otolaryngol. 1969;90:765–778.
6 Hall SF, Ruby RR, McClure JA. The mechanics
of benign paroxysmal vertigo. J Otolaryngol.
1979;8:151–158.
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12
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tomatology, and diagnosis of certain common
disorders of the vestibular system. Proc R Soc
Med. 1952;45:341–354.
benfranklinfu YouTube http://www.youtube.
com/watch?v=xLsv5mOUkXk&feature=related
(accessed 22 June 2013).
Bhattacharyya N, Baugh RF, Orvidas L, et al.
American Academy of Otolaryngology – Head
and Neck Surgery Foundation. Clinical practice
guideline: benign paroxysmal positional ­vertigo.
Otolaryngol Head Neck Surg. 2008;139(5, Suppl
4):S47–S81.
Lopez-Escamez JA, Lopez-Nevot A, Gamiz
MJ, et al. Diagnosis of common causes of
vertigo using a structured clinical history. Acta
Otorrinolaringol Esp. 2000;51:25–30.
Bracher ES, Almeida CI, Almeida RR, et al.
A combined approach for the treatment of
cervical vertigo. J Manipulative Physiol Ther.
2000;23:96–100.
Brandt T, Steddin S, Daroff RB. Therapy for
benign paroxysmal positioning vertigo, revisited. Neurology. 1994;44:796–800.
Epley JM. The canalith repositioning procedure: for treatment of benign paroxysmal
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positional vertigo. Otolaryngol Head Neck Surg.
1992;107:399–404.
Semont A, Freyss G, Vitte E. Benign paroxysmal
positional vertigo and provocative maneuvers
[in French]. Ann Otolaryngol Chir Cervicofac.
1989;106:473–476.
Radtke A, von Brevern M, Tiel-Wilck K, MainzPerchalla A, Neuhauser H, Lempert T. Selftreatment of benign paroxysmal positional
vertigo: Semont maneuver vs Epley ­procedure.
Neurology. 2004;63(1):150–152.
Banfield GK, Wood C, Knight J. Does vestibular habituation still have a place in the treatment of benign paroxysmal positional vertigo?
J Laryngol Otol 2000;114:501–505.
Yimtae K, Srirompotong S, Sae-Seaw P. A
randomized trial of the canalith repositioning
procedure. Laryngoscope 2003;113:828–32.
Hain TC, Helminski JO, Reis IL, Uddin MK.
Vibration does not improve results of the canalith repositioning procedure. Arch Otolaryngol
Head Neck Surg. 2000;126(5):617–622.
benfranklinfu YouTube http://www.youtube.
com/watch?v=NQr7MKJBAJY (accessed 22
June 2013).
Rashad UM. Patients with benign paroxysmal
positional vertigo and cervical spine problems:
is Epley’s manoeuvre contraindicated, and is a
proposed new manoeuvre effective and safer?
J Laryngol Otol. 2010;124:1167–1171.
Fife TD, Iverson DJ, Lempert T, et al. Quality
Standards Subcommittee, American Academy
of Neurology. Practice parameter: ­therapies
for benign paroxysmal positional vertigo
(an evidence-based review): report of the
Quality Standards Subcommittee of the
American Academy of Neurology. Neurology.
2008;70(22):2067–2074.
22 Nuti D, Agus G, Barbieri M-T, Passali D. The
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management of horizontal canal paroxysmal
positional vertigo. Acta Otolaryngol (Stockh).
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Bertholon P, Bronstein AM, Davies RA, Rudge P,
Thilo KV. Positional down ­beating nystagmus
in 50 patients: cerebellar disorders and possible
anterior semicircular canalithiasis. J Neurol
Neurosurg Psychiatry. 2002;72(3):366–372.
Gacek RR. Transection of the posterior ampullary nerve for the relief of benign paroxysmal
positional vertigo. Ann Otol Rhinol Laryngol.
1974; 83:596–605.
Gacek RR. Singular neurectomy update.
II. Review of 102 cases. Laryngoscope.
1991;101(8):855–862.
Parnes LS, McClure JA Posterior semicircular
canal occlusion for intractable benign paroxysmal positional vertigo. Ann Otol Rhinol
Laryngol. 1990;99:330–333.
Nunez RA, Cass SP, Furman JM. Short- and
long-term outcomes of canalith repositioning for benign paroxysmal positional
vertigo. Otolaryngol Head Neck Surg.
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Rashad UM. Long-term follow up after Epley’s
manoeuvre in patients with benign paroxysmal positional vertigo. J Laryngol Otol.
2009;123:69–74.
Hong SM, Park MS, Cha CI, Park CH, Lee JH.
Subjective visual vertical during eccentric
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positional vertigo. Otology and Neurology.
2008;29(8):1167–1170.
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7
Acute peripheral
vestibular loss
Ambrose Lee
Contents
Introduction
Aetiology
Pathogenesis
Symptoms and signs
Audiovestibular testing
Management (medical/surgical/physiotherapy)
Visual vertigo
Bilateral vestibular hypofunction
Case study
Conclusions and recommendations
References
61
61
62
64
66
68
69
69
70
70
70
Introduction
An acute peripheral vestibular loss (APVL) is a
common cause of vestibular dysfunction and
accounts for approximately 30% of patients seen in
a specialised dizzy clinic.1 There are various terms
used to describe this clinical condition that consists
of profound prolonged vertigo. These include
‘vestibular neuritis’ implying inflammation of the
vestibular nerve (Figure 7.1), ‘vestibular neuronitis’ involving inflammation of Scarpa’s ganglion
(sensory neurones of the vestibular ganglion) and
‘labyrinthitis’. Although these terms are often used
interchangeably, the term ‘labyrinthitis’ should be
reserved for cases where a simultaneous vestibular
and sensorineural hearing loss occur.
Aetiology
The causes of an APVL are listed in Table 7.1 with
vestibular neuritis considered the most common.2
Although the cause of vestibular neuritis remains
obscure, a viral aetiology has been suggested
including members of the herpes simplex virus
(HSV) and varicella zoster virus families. There is
also evidence to suggest that a vascular cause may
be implicated in APVL.3 Disruption to the blood
Acute peripheral vestibular loss 61
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Figure 7.1. Diagrammatic representation of vestibular neuronitis.
Table 7.1. Causes of an acute unilateral peripheral
vestibular deficit.
Vestibular neuritis
Labyrinthine concussion
Trauma
Post surgical
Iatrogenic vestibular ablative treatments
Infective (labyrinthitis, complications from acute
otitis media and chronic suppurative otitis media)
Cholesteatoma
Advanced Ménière’s disease
flow of the inner ear may result in abrupt vestibular hypofunction. Patients may have a personal or
familial history of cardiac risk factors including
hypercholesterolaemia, or autoimmune disorders
such as anti-phospholipid antibody syndrome
(­anticardiolipin antibody).4
Labyrinthitis may be the result of a bacterial or
viral infection. Pathogens, and their associated
toxins, can spread into the membranous labyrinth in four ways: haematogenously, via a direct
bony breach, from the subarachnoid space via the
modiolus, or by way of the cochlear aqueduct.5
Labyrinthitis ossificans is the pathological ossification of the inner ear with inflammation followed by fibrosis and formation of new bone.6 It is
typically bilateral and can be seen as early as 3–4
months following an episode of bacterial meningitis. Five per cent of children who survive bacterial
meningitis develop bilateral sensorineural hearing
loss.7 It is imperative that clinicians consider this
diagnosis early when evaluating patients with a
history of bacterial meningitis and hearing loss.
Once the labyrinth becomes ossified it can make
cochlear implantation very difficult to carry out.
Computed tomography is commonly used in the
preoperative assessment to detect the degree of
sclerosis.
Pathogenesis
Within the central vestibular system, neurons in
the vestibular nuclei maintain an intrinsic firing
rate at rest of approximately 80 spikes per second.
This intrinsic baseline activity is believed to be
characteristic of all vestibular neurons and allows
for a greater degree of accuracy in calculating spatial
positioning. Two types of lateral semicircular canal
driven neurons have been found in the medial
62 Dizziness and Vertigo: An Introduction and Practical Guide
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Right
Middle line
Left
Lateral
semicircular
canal
Type II
GAD+
Type II
GAD-
Scarpa’s
ganglion
Type I
GAD+
Vestibular
nuclei
Oculomotor
neurons
R†MR
L†LR
L†MR
R†LR
R†MR
L†LR
L†MR
R†LR
Figure 7.2. Different neurons in vestibular ocular reflex: the relationship between type I and type II secondary vestibular neurons. (adapted from Baloh and Honrubia 2001). Central neural connections and ocular
movement (Rt = right, Lt = left, MR = medial).
vestibular nucleus, type I and type II neurones
(Figure 7.2). At rest both types fire spontaneously, but
the discharge rate of type I neurons increases during
ipsilateral head rotation, and decreases during contralateral head rotation. In contrast, type II neurons
increase their firing rates on contralateral head
rotation and reduce their discharge rate on ipsilateral head rotation. The excitatory type I neurons are
connected to the inhibitory type II neurons on the
opposite side. Therefore, they receive a ­combination
of stimuli from both ears, corresponding to the sum
ampullopetal activity (excitatory signals) from the
ipsilateral side and the inverted ampullofugal activity (disinhibitory) mediated by the type II neurons.8
In addition, most cells within the vestibular nuclei
respond to visual stimuli via the cerebellum.9
Immediately after a unilateral loss of peripheral vestibular function, the ipsilateral type I neurons lose
their spontaneous activity and become unresponsive
to angular stimulation. In addition, the contralateral
healthy type I neurons lose their inhibitory input
from the injured type II neurons. This increases
their spontaneous discharge rate. An imbalance in
muscle tone occurs, resulting in the clinical signs of
nystagmus, pastpointing and dysequilibrium.
Following an abrupt peripheral vestibular loss,
vestibular compensation begins with the damaged type I neurons responding to stimulation
via the contralateral labyrinth as a result of their
­connections with ipsilateral type II neurons.
The mechanism of this renewed tonic input has
not been fully elucidated, but may be due to
changes in membrane properties of the vestibular neurons,10 from sprouting of axons from
other sources, or from an increased efficacy of
the remaining intact synapses.11 This is referred
to as the static phase of compensation, whereby
patients may be asymptomatic when lying still,
but become unsteady on movement. Due to the
rapid and robust mechanisms outlined above
this process takes approximately 3 days in most
patients, although younger patients may compensate within hours.
The dynamic phase of compensation may take
weeks or months and allows habituation and
Acute peripheral vestibular loss 63
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compensation for self movement and changes in
posture. At this stage new behavioural strategies
may be learned to allow gaze and posture control to
operate as if normal.12
Vestibular neuronitis has been shown to selectively
involve the superior part of the vestibular labyrinth
(lateral and superior semicircular canals, and
utricle) with sparing of the inferior part (posterior
semicircular canal and saccule). The former is supplied by the superior vestibular nerve whilst the
latter by the inferior vestibular nerve.13 Anatomical
differences in the bony canals of the two divisions
might explain this relative vulnerability.14
Symptoms and Signs
An APVL results in profound, prolonged and severe
rotatory vertigo accompanied by postural imbalance,
nausea and profuse persistent vomiting. The onset
occurs gradually over minutes to hours, although
many patients wake with vertigo. The patient may
give a history of a viral illness prior to the start of
vertigo. Head movements worsen symptoms because
they accentuate imbalance within the vestibular
pathways. Autonomic symptoms such as nausea and
sweating are common due to the many interconnections between vestibular and autonomic centres.
The initial signs following an APVL are spontaneous, horizontal ocular nystagmus with the slow
component towards the affected ear and the fast
phase (saccade) beating away from it. In addition,
an otolithic response results in: (a) head tilt towards
the lesioned side; (b) skew deviation to the lesioned
side; (c) conjugate ocular torsion towards the
lesioned side.15 In skew deviation the eye ipsilateral
to the lesioned labyrinth is positioned lower in the
orbit than the contralateral eye resulting in diplopia. In ocular torsion both eyes roll with the upper
pole towards the lesioned ear.
This acute stage of severe vertigo lasts between
hours and days but long lasting symptoms such as
chronic dizziness and dysequilibrium provoked by
head movements, stress and darkness remain in up
to 50% of patients.16 Vertigo is usually not a predominant feature in slow, unilateral loss of vestibular function or in symmetrical bilateral vestibular
loss as seen with ototoxic drugs. Both VN and
labyrinthitis are rarely painful. When there is pain
it is particularly important to intervene rapidly
as there may be a treatable bacterial infection or
herpes zoster oticus. Patients with the latter typically p
­ resent with deep burning pain of the auricle
followed by a vesicular eruption a few days later.
At some point after the onset of this eruption hearing loss, vertigo and facial weakness may develop.
The neurotological examination of a patient presenting with dizziness should follow a systematic
order as outlined in Table 7.2 and the results be
clearly documented. The findings suggestive of
APVL include a persistent, horizontal direction
fixed spontaneous nystagmus, a positive headthrust examination, a positive head-shake examination, rotation on Unterberger testing and an
absence of central neurological signs.
The amplitude of the spontaneous nystagmus
increases with gaze in the direction of the fast
phase and decreases with gaze away from it
(Alexander’s Law). The head-thrust test is a bedside
test of the horizontal vestibulo-ocular reflex first
reported by Halmagyi. It is performed by grasping
the patient’s head and applying a brief, small-amplitude, high-acceleration head turn, first to one side
and then to the other. The patient is asked to fixate
on the examiner’s nose as the examiner watches
for corrective rapid eye movements (saccades),
which is a sign of a decreased vestibular response
(i.e. the eyes move with the head rather than staying fixed on the nose).17 A patient with unilateral
APVL is expected to have a corrective eye movement (saccade) in the direction contralateral to the
lesion (Figure 7.3). The positive predictive value
(PPV) of the head-thrust test with respect to caloric
stimulation for patients with unilateral peripheral
vestibulopathy ranges from 64 to 100% and the
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Table 7.2. Essential components of a neurotological examination.
Test
Specific components
Tuning fork test
Otoscopy
Cranial nerve examination
Tests of oculomotor function
Spontaneous nystagmus
Gaze holding
Smooth pursuit
Saccades
Convergence
Vestibulocular reflex cancellation
Tests of the vestibulocular reflex
Head-thrust test
Head-shake test
Dynamic visual acuity
Gait and balance testing
Romberg
Unterberger/Fukuda stepping test
Tandem gait
Ceberellar testing
Positioning tests
Dix-Hallpike testing
Other tests as clinically indicated
Hyperventilation test
Fistula test
A
B
C
D
E
Figure 7.3. Head thrust test. The subject is asked to look at the examiner’s nose and their head rapidly
rotated from 30 degrees to the midline. A single corrective saccade will bring the eyes to focus on the target
in a normal subject (A and B). In those with a peripheral vestibular deficit, however, multiple corrective
­saccades will be seen (C to E).
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negative predictive value (NPV) from 61 to 100%
(Table 7.3). In Halmagyi’s study the test was found
to have a 100% sensitivity and specificity as their
patient population consisted of those who underwent a unilateral peripheral vestibular neurectomy
for reasons other than VN. A positive head-thrust
test alone cannot reliably differentiate APVL from
cerebellar strokes as up to 30% of patients with a
clinical history consistent with APVL and a positive head-thrust may have suffered an infarct.18
One must take into account additional features
such as audiological symptoms, ataxia, and the
nature of the nystagmus seen before a central cause
can be excluded.
The head-shake nystagmus test reflects the
dynamic asymmetry in the gain of the vestibulocular reflex first observed by Ewald.23 Known as
Ewald’s Second Law, it is interpreted as excitation
being a better vestibular stimulus than inhibition.
It is performed at the bedside by first grasping the
patient’s head and moving it briskly at approximately 2 Hz in the horizontal plane for 20 cycles
before abruptly stopping. Eye movements are then
observed via a Frenzel’s lens. In normal subjects
or patients with symmetrical vestibular loss no
nystagmus is seen. However, in patients with an
APVL a horizontal nystagmus is initially seen
with the fast phase towards the ‘better’ ear. This
decays over 30 seconds. This is the first phase
of nystagmus, with a second phase producing,
a weaker nystagmus that may be seen to beat
towards the lesioned ear and decay more slowly.
It is often attributed to short term vestibular adaptation, probably reflecting adaptive processes in
both the peripheral and central structures.24 The
PPV of the head-shake test with respect to caloric
stimulation for patients with unilateral peripheral
vestibulopathy ranges from 32 to 80% and the
NPV 15 to 94% (Table 7.4).
The Unterberger stepping test was first described by
Unterberger and modified by Fukuda.30,31 Subjects
are asked to march like a stationary soldier with
their eyes closed, until 50 steps are achieved at a
rate of 110 steps/minute. Patients with abnormal
peripheral vestibular function may rotate towards
the side of their lesion past 30 degrees although
there is no correlation between the degree of
canal paresis and the angle of rotation in patients
with poorly compensated peripheral labyrinthine
dysfunction.32 This test has to be taken into context
with the Romberg’s test as an indicator for further
investigations of the vestibular system. Conversely,
a negative stepping test does not reliably exclude
the presence of APVL and should not preclude
further testing if clinically suspected.
During the initial encounter with a patient with
vertigo it can be difficult to distinguish between a
peripheral and central vestibular cause. In contrast
to APVL, spontaneous nystagmus of central origin
typically changes in direction when the patient
looks away from the direction of the fast phase and
is not inhibited with visual fixation. It often persists
for weeks to months. Therefore, if the cause of the
vertigo cannot be localised after the initial visit, the
patient should be followed up to see if the course is
typical of APVL.
On the basis of the appearance of the nystagmus,
a positive head-thrust and head-shake test, rotation in the stepping test and a negative neurologic
examination, one can usually be confident in the
diagnosis of a unilateral peripheral loss. Although it
has been shown that the head-shake and the headthrust test have limited PPVs and NPVs at the very
least they complement other aspects of the neurotologic examination and the clinician should take the
overall findings in consideration before a diagnosis
is reached.
Audiovestibular testing
An APVL due to VN is unlikely to result
in a sensorineural hearing loss, although
this may be apparent in cases of true
labyrinthitis. Tympanometry will produce
a type A trace. Videonystagmography, or
­electronystamography, is routinely used in order
66 Dizziness and Vertigo: An Introduction and Practical Guide
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K17350.indb 67
Table 7.3. Head-thrust test compared with caloric test for unilateral vestibular loss.
Sensitivity (%)
Specificity
(%)
Positive
predictive
value (%)
Negative
predictive
value (%)
Number of
patients
Study
100
100
100
100
24
Halmagyi et al.17
Dizziness
39
97
68
90
112
Harvey et al.19
Dizziness
35
95
64
86
105
Harvey et al.20
Vertigo
34
100
100
61
150
Benyon et al.21
Vestibular failure and nonvestibular dizziness
75
82
87
65
111
Schubert et al.22
Condition
Unilateral vestibular neurectomy
Table 7.4. Head-shake test compared with caloric test for unilateral vestibular loss.
Sensitivity (%)
Specificity
(%)
Positive
predictive
value (%)
Negative
predictive
value (%)
Number of
patients
Dizziness
35
92
50
86
105
Harvey et al.22
Vertigo
40
68
32
75
108
Wei et al.25
Dizziness
94
62
63
94
85
Dizziness
44
65
N/A
N/A
115
Burgio et al.27
Canal Paresis
42
85
41
15
214
Goebel et al.28
Dizziness
40
82
80
43
290
Asawavichianginda et al.29
Condition
Study
Acute peripheral vestibular loss 67
Takahashi et al.26
2/21/14 8:31 PM
to demonstrate u
­ nilateral caloric hypoexcitability
­signifying ­u nilateral vestibular loss. Rotational
chair ­testing may also be used to investigate
patients with APVL. Unfortunately the results
of this test are often indefinite because at low
frequencies the oculomotor response to the
acceleration can be controlled by a variety of
different sensory and motor systems apart from
the vestibular system. 33 In order to measure
dynamic vestibular function at high accelerations the head impulse test is used. It uses short
duration angular accelerations in the natural
range (2000–3000 deg/sec) that in practice are
very similar to the head-thrust test. In normal
subjects, the vestibular-ocular reflex (VOR) gain
(the ratio of eye velocity to head velocity) is close
to 1.0. For a patient with APVL, the VOR gain is
drastically reduced in ipsilesional head rotation.
However, it is often not necessary and impractical
to have patients ­u ndergoing this test during the
acute phase of APVL.
Patients with a confirmed APVL should undergo
a fasting lipid screen to exclude hypercholesterolaemia, and young female patients an autoimmume screen to exclude antiphospholipid antibody
syndrome. Plasma D-Dimer has been shown to
be elevated in patients with APVL compared to
Ménière’s disease. However the significance of this
test remains unknown.34
The use of magnetic resonance imaging (MRI) to
screen patients for cerebellopontine angle lesions
presenting with acute vestibular loss remains
controversial as there have been no published
guidelines regarding this topic. An MRI may demonstrate an isolated enhancement of the vestibular
nerve in patients with VN.35 However, the prevalence of MRI abnormalities in patients with audiovestibular disorders presenting in a tertiary care
setting could be up to 34%.36 We therefore advocate clinical prudence when considering MRI to
evaluate patients with APVL. It should be used in
cases where the diagnosis remains d
­ oubtful or in
others where central findings are present.
Management (medical/surgical/physiotherapy)
The treatment of APVL can be divided into
­symptomatic drug therapy and vestibular rehabilitation (VR).
Symptomatic drug therapy is used to reduce vertigo,
nausea and vomiting immediately following an
APVL. Prochlorperazine is a vestibular suppressant that is commonly prescribed to patients with
an APVL. Although usually prescribed as an oral
preparation, an intramuscular injection may be
required in those with marked nausea and vomiting.
Vestibular suppressants should not be used on a
long term basis because they may interfere with
central compensation. Their use should be limited to seven days to cover the static phase of
compensation.
It has been proposed that a short course of corticosteroids may improve the recovery and long
term outcome of patients with APVL. However, a
Cochrane review concluded there is insufficient evidence for the routine administration of this drug as
the studies examining this issue lacked robustness
in their design.37 The combination of corticosteroids and an antiviral agent such as acyclovir has,
however, been reported to be effective in treating
herpes zoster oticus.38
Compensatory mechanisms involve adaptation
via changing the gain, phase or direction of the
vestibular response. This results from the substitution of other sensory input mechanisms,
alternate motor responses and strategies based
on prediction or anticipation, and form the
foundation of effective VR. A Cochrane Review
has shown VR to be a safe and effective treatment in patients with an APVL. 39 The Cooksey
Cawthorne exercises, for example, are readily
available on the internet.40 Individual or group
68 Dizziness and Vertigo: An Introduction and Practical Guide
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based VR should be instituted if possible depending on institutional availability. This is because
patients can have varying degrees of visual or
vestibular preference and their exercises can be
individually tailored. Patients with poor visual
and vestibular inputs do better with computer
dynamic posturography whilst those with poor
visual preference may benefit from optokinetic
stimulation.41
The restoration of static equilibrium is robust
whereas dynamic compensation is heavily dependent on intact visual, vestibular and
proprioceptive sensory inputs and is often incomplete.42 Recovery involves the interaction of the
contralateral labyrinth, somatosensory and visual
substitution and central vestibular compensation. Patients should be advised that recovery
from APVL typically takes several weeks although
longer periods are not uncommon. Many patients,
however, notice mild disequilibrium when tired,
stressed or generally unwell, spells described as
‘decompensation’.
Surgery has no role in the treatment of VN nor
labyrinthitis.
Visual vertigo
Visual vertigo represents a form of vertigo in which
symptoms are provoked by specific visual environments (e.g. driving, supermarkets, or crowds).
It may arise due to either central or peripheral
pathology.
This condition is thought to arise due to an over
reliance on vision to maintain balance. A thorough history remains the cornerstone to reaching
this diagnosis.43 Neuro-ophthalmological, neurological and neuro-otological examinations are
often normal.44 Posturography tests in patients
with this condition show abnormally large
body sways induced by full field visual motion
­stimulation.45 The cornerstone of treating visual
vertigo is VR combined with appropriate visual
stimulation. Virtual reality training involving
computer generated imagery has been demonstrated to b
­ enefit patients by synchronising the
visual sensory to vestibular and somatosensory
inputs.46
Bilateral vestibular hypofunction
Bilateral vestibular loss is rare, yet profoundly
disabling. It is idiopathic in 50% of cases.47
Known aetiological factors include ototoxicity,
autoimmune disease, meningitis and Ménière’s
disease.48 The most common presenting symptoms are those of disequilibrium (91%) and
oscillopsia, (77%) where objects in the visual
field appear to oscillate. A history of falls
may also be present in up to 34% of patients.
Other otologic symptoms include hearing loss
(37%), tinnitus (23%), and vertigo (17%).49 This
­condition is primarily treated by vestibular
rehabilitation with modest goals, as few patients
completely recover. Gene therapy to the inner
ear may provide a method of restoring peripheral function and has been explored in conjunction with cochlear implantation. 50 Work is
also underway to create an effective vestibular
implant to restore function to those with bilateral
impairment. 51
Acute peripheral vestibular loss 69
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Case study 7.1
A 44-year-old woman presented with a three-year history of unsteadiness.
She described an episode of true rotatory vertigo prior to the onset of her symptoms.
She had awoken with severe continuous vertigo that had persisted for two days. This was
associated with nausea and vomiting. There was no associated hearing loss nor tinnitus.
Her symptoms of unsteadiness persisted and she complained of marked unsteadiness on
rapid head movement. She also found crowds and supermarkets disorientating and hence
avoided these environments.
Following a thorough assessment a left canal paresis was noted and she subsequently underwent customised vestibular rehabilitation exercises. As her symptoms were provoked by visually
rich environments, her exercises were performed whilst exposed to a checkerboard pattern.
Her symptoms almost settled completely, her confidence improved and she was subsequently discharged.
Conclusions and Recommendations
APVL results in spontaneous profound prolonged
vertigo. Although some patients recover fully without intervention, the dynamic phase of compensation may be limited and these individuals benefit
from customised vestibular rehabilitation. Those
with visual vertigo may also require exposure to a
visually rich environment whilst undertaking their
physical exercises.
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8
Vestibular migraine
Nitesh Patel
Contents
Introduction
Aetiology
Symptoms
Signs
Investigations
Management
Treatment of the acute episode
Prophylactic treatments
Case studies
Conclusion
References
73
74
74
75
75
75
75
75
76
77
77
Introduction
Vestibular migraine (VM) is a syndrome of
episodic vertigo in patients with a current or past
history of migraine. Various other terms, including
migraine-associated vertigo, migraine-­associated
dizziness, migraine-related vestibulopathy,
migrainous vertigo and vertiginous migraine,
have all been applied to this patient population.
However, Brandt and Strupp1 have suggested the
term VM as most appropriate as it emphasises the
particular vestibular manifestation of migraine,
and helps differentiate this entity from nonvestibular dizziness or motion sickness associated with
migraine.
The third commonest cause of vertigo in the adult
population is VM2 and accounts for as many as
14% of adults with vertigo presenting to a specialist unit.3 In the general population, the lifetime
prevalence of definite VM (as per criteria discussed
later in this chapter) has been estimated to be 1%4
whereas dizziness and vertigo may be found in up
to 30% of people with migraine, definite VM is
found in 9%.5 It can occur at any age but is more
common in women with a female to male ratio
of 5:1.6 In children, it has been suggested that
benign paroxysmal vertigo of childhood is an early
­manifestation of VM.7
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Aetiology
The cause of VM is unknown. However, the traditional vascular theory of migraine suggesting the
cause to be cranial vasodilation has recently been
challenged.8 Current concepts consider migraine
primarily to be a brain disorder. There is good
evidence that the brain is hyper-excitable in people
who suffer with migraine.9 It is also evident that
migraine sufferers are more s­ ensitive to sensory
stimuli such as bright light, loud noises, strong
smells and motion. These ­sensory inputs can trigger
cortical events in which sensory overload triggers subsequent temporary neuronal shutdown.
It has been shown that there is an elevated level of
calcitonin gene related peptide (CGRP) in acute
migraine, which returns to normal after treatment
and is independent of vascular effects.10,11 Triptans
are thought to block release of CGRP via action at
presynaptic 5–hydroxy tryptamine receptors.12
The mechanism of VM is still not fully ­understood.
One hypothesis suggests VM to result from a
spreading depression in neuronal activity affecting
the central cortical areas which process vestibular signals including the posterior insula and the
­temporoparietal cortex.13,14 Other hypotheses
include dysfunctional neurotransmitter release
in the central or peripheral vestibular neurons,13
a genetic defect in ion channels,15 or cross-talk
between neighbouring neuronal systems such as
trigeminal and vestibular nuclei.16
Symptoms
The diagnosis of VM relies mainly on patient history. Frequently, patients presenting with vertigo do
not volunteer a history of migraine so it is important to specifically enquire about this. Neuhauser
et al.17 have proposed the following criteria essential
for the diagnosis of definite VM:
vestibular symptoms of at least mod• Episodic
erate severity (interfere with activities of daily
living).
or past history of migraine.
•• Current
One of the following during two or more
•
attacks of vertigo: migrainous headache,
­photophobia, phonophobia, visual or other
aura.
Other causes ruled out by appropriate
investigations
However, this diagnosis may also need to be considered (probable VM) where:
i Migrainous symptoms have occurred during
vestibular symptoms.
ii Classic migraine triggers such as food, irregu-
lar sleep and hormonal changes precipitate
vertigo.
iii When migraine medications abort episodes of
vertigo.
It is worthy of mention that positional vertigo can
occur with VM and has been reported in 24% of
patients, in contrast to 67% of patients describing
spontaneous episodic vertigo.18 Head motion intolerance, visual vertigo, nausea and imbalance can be
associated. Hearing loss and tinnitus are infrequent
symptoms and tend to be mild and non-progressive
when present.6 Fluctuating hearing loss similar to
endolymphatic hydrops has also been described.19
VM and Ménière’s disease have a complex a­ ssociation
as both can coexist. Migraine may even damage the
inner ear causing a delayed endolymphatic hydrops.20
The duration of vertigo varies widely from seconds
to days, with further episodes occurring after
days, months or years. In many women spells are
associated with their menstrual cycle, occurring
2–3 days before the start of a period, and lasting
2–3 days in total. Vertigo may occur as a prodromal aura before a migraine headache, occur with
the migraine headache or occur independently.
Most episodes have no temporal relationship with
the headaches.
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Signs
Neurotological clinical examination between
­episodes is usually normal. During an acute
episode there may be non-specific nystagmus and
imbalance with swaying on Romberg, Unterberger
and Tandem gait testing.
Investigations
There is no specific diagnostic test for VM. The
diagnosis relies on a clinical history and investigations to exclude other peripheral or central
vestibular disorders. Vestibular function tests
(videonystagmography with caloric function testing) are usually unremarkable but may show a
concomitant unilateral vestibular hypofunction in
20.9% of patients.13 A pure tone audiogram usually
demonstrates normal thresholds. A magnetic
resonance imaging (MRI) scan of the internal
auditory meati and brain is indicated to rule out
retrocochlear or brain pathology. Blood investigations should include urea and electrolytes, glucose,
lipids, thyroid function tests, autoantibodies and
syphilis serology to exclude specific vestibular
disorders.
Management
Management of this condition is medical and can
be considered for the acute episode or ­prophylaxis
against further episodes. Various m
­ odalities
can be helpful including patient education,
dietary ­modification, drug therapy, vestibular
­rehabilitation and psychotherapy. In considering
treatment, other co-morbidities should be taken
into account such as hypertension, anxiety or
depression, asthma, and epilepsy due to the side
effects and interactions of drug therapy. A dizziness diary can be useful in assessing an individual’s
response to treatment.
Treatment of the acute episode
Although acute episodes of vertigo can be treated
along similar lines to other vestibular disorders with
antivertiginous and antiemetic m
­ edications, studies
suggest that nonsteroidal anti-­inflammatory drugs
(NSAIDs) address the processes that produce symptoms, including pain, inflammation, vasodilation,
and central and peripheral sensitisation.21,22
Patients can also be treated with acute migraine
medications such as serotonin antagonists
(triptans), although there is a lack of conclusive evidence for their use. Zolmitriptan has been shown to
have some benefit in a single randomised controlled
trial, although this pilot study involved a small
number of subjects.23
Prophylactic treatments
Dietary modification with reduction in c­ lassic
migraine triggers such as cheese, ­chocolate, caffeine, red wine, and glutamate rich foods may be
beneficial in many patients. Lifestyle modification
should be considered where other triggers such as
stress and irregular sleep pattern may be contributory. Regular exercise may be b
­ eneficial in reducing
stress and improving sleep.24
Onset of symptoms may be associated with the
combined and contraceptive pill and hence some
patients may benefit from changing to the progesterone only pill.
In clinical practice, drugs used for VM are
similar to those used in the treatment of classical migraine (note Table 8.1). The classes of
Vestibular migraine 75
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Table 8.1. Prophylactic drug therapy options for Vestibular migraine.25
Drug
Group
Initial dose
Target dose
Cautionary note
Nortriptyline Tricyclic
20 mg at night
(unlicensed
antidepressant for 2 weeks
use)
Increased in 10 mg steps every
2 weeks until control; usual
maintenance dose 30–70 mg/
day
Can cause drowsiness,
dry mouth
Topiramate
Anticonvulsant
25 mg at night
for 1 week
Increased in 25 mg steps at
weekly intervals; usual
maintenance dose
50–100 mg/day in divided
doses; maximum 200 mg/day
Can cause secondary
closed angle
glaucoma typically
occurring within one
month of treatment
Propranolol
Beta-blocker
40 mg/day
80–240 mg/day in divided
doses
Avoid in asthma,
hypotension
Pizotifen
Serotonin
antagonist
and
antihistamine
Initially 500
micrograms at
night
Increase gradually to usual dose
1.5 mg at night or 3 divided
doses; maximum 4.5 mg/day
Can cause increased
appetite, weight gain,
closed angle
glaucoma, urinary
retention
Verapamil
(unlicensed
use)
Calciumchannel
blocker
120 mg/day
2 mg/kg per day26
Avoid in patients with
constipation, acute
phase myocardial
infarction, heart
failure, heart block,
hypotension, on
beta-blockers
drugs most commonly used are antidepressants,
antihistamines, anticonvulsants, beta-blockers,
and calcium channel blockers (see Table 8.1).
The choice will be guided by coexisting conditions, physician experience and preference, efficacy, and side effect profile.
There is some evidence to suggest that vestibular
rehabilitation (VR) can help improve physical
­ erformance and self-perceived abilities26 although
p
this may be a placebo effect.27
Up to 65% patients with VM may exhibit anxiety
disorder.28 In this group, a tricyclic antidepressant should be considered to help with prophylaxis
as well as alleviating anxiety. Cognitive behavioural therapy (CBT) in these patients may also be
beneficial.
Case study 8.1
A 39-year-old woman presented complaining of intermittent spells of marked
disequilibrium.
She described her first spell clearly. She experienced vertigo that lasted for several hours
that came on gradually. She also experienced nausea but did not vomit. She described
‘everything being too loud’. She spent that day in bed with the curtains drawn, as the bright
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light made her uncomfortable. She felt unsteady for the following two days but managed to
return to work the day after.
She suffered similar spells that were less intense every four weeks. She noticed that the
spells occurred just before her period started.
She described suffering from classical migraine during her late teens and early 20’s and
her mother had also suffered with migraine.
Full clinical and audiovestibular testing was normal as was the MRI of her internal auditory
meati.
A diagnosis of vestibular migraine was made and she was commenced on a restricted diet
(avoiding all chocolate, cheese, red wine, caffeine, citrus, bananas, monosodium glutamate,
processed meats and full fat milk and cream). Her symptoms gradually settled over the following 12 weeks and she was able to gradually introduce several possible dietary triggers.
Case study 8.2
A 44-year-old woman presented with a history of intermittent spells of marked disequilibrium associated with photophobia and phonophobia. Spells lasted 48 hours before settling
completely.
Full clinical and audiovestibular testing was unremarkable, as was her brain imaging.
A diagnosis of vestibular migraine was made and she was commenced on a strict diet
avoiding common triggers.
Unfortunately her symptoms persisted and she was therefore offered antimigraine prophylaxis. Pizotifen was introduced and her symptoms gradually settled.
Unfortunately, she developed significant weight gain and low mood and was hence
reviewed by a cognitive behavioral therapist.
She was successfully managed and discharged.
Conclusion
Vertigo is often seen in patients with migraine,
however this alone does not implicate migraine as
the cause. Several dizziness and vertigo syndromes,
including Ménière’s disease, benign paroxysmal
positional vertigo, motion sickness and orthostatic
hypotension, are more common in migraineurs than
in the general population6 and these need exclusion. The diagnosis of VM can be challenging since
vestibular symptoms often occur independently of
migraine headache and the clinical presentation may
mimic other syndromes of positional and spontaneous episodic vertigo. In some cases the diagnosis can
only be made by exclusion and on the basis of a positive treatment response to migraine medication.
References
1 Brandt T, Strupp M. Migraine and vertigo:
classification, clinical features, and special
treatment considerations. Headache Currents.
2006;3(1):12–19.
2 Kanegaonkar R, Corcoran J, Jones G. A
modern balance clinic. Otorhinolaryngologist.
2010;3(2):52–57.
3 Kentala E, Rauch SD. A practical ­assessment
algorithm for diagnosis of dizziness. Otolaryngol
Head Neck Surg. 2003;128:54–59.
4 Neuhauser H. Epidemiology of vertigo. Curr
Opin Neurol. 2007;20:40–46.
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5 Neuhauser H, Leopold M, von Brevern M,
6
7
8
9
10
11
12
13
14
15
16
et al. The interrelations of migraine, vertigo, and migrainous vertigo. Neurology.
2001;56:436–441.
Lempert T, Neuhauser H. Epidemiology of
­vertigo, migraine and vestibular migraine.
J Neurol. 2009;256(3):333–338.
Abu-Arafeh I, Russell G. Paroxysmal vertigo as a migraine equivalent in children:
A ­population-based study. Cephalalgia.
1995;15:22–25.
Goadsby PJ. The vascular theory of migraine –
a great story wrecked by the facts. Brain.
2009;132(1):6–7.
Aurora SK, Wilkinson F. The brain is
hyperexcitable in migraine. Cephalalgia.
2007;27(12):1442–1453.
Goadsby PJ, Edvinsson L, Ekman R. Vasoactive
peptide release in the extracerebral circulation
of humans during migraine headache. Ann
Neurol. 1990;28:183–187.
Petersen KA, Birk S, Lassen LH, Kruuse
C, Jonassen O, Lesko L, et al. The CGRP­antagonist, BIBN4096BS does not affect
cerebral or systemic haemodynamics in healthy
volunteers. Cephalalgia. 2005;25:139–147.
Levy D, Jakubowski M, Burstein R. Disruption
of communication between peripheral and
central trigeminovascular neurons mediates the antimigraine action of 5-HT1B/1D
receptor agonists. Proc Natl Acad Sci USA.
2004;101(12):4274–4279.
Cutrer FM, Baloh RW. Migraine-associated
­dizziness. Headache. 1992;32(6):300–304.
Fasold O, von Brevern M, Kuhberg M,
et al. Human vestibular cortex as identified with caloric stimulation in functional
magnetic resonance imaging. Neuroimage.
2002;17(3):1384–1393.
Ophoff RA, Terwindt GM, Vergouwe MN,
et al. Familial hemiplegic migraine and episodic ataxia type-2 are caused by mutations
in the Ca21 channel gene CACNL1A4. Cell.
1996;87:543–552.
Marano E, Marcelli V, Di Stasio E, et al.
Trigeminal stimulation elicits a peripheral
17
18
19
20
21
22
23
24
25
26
27
28
vestibular imbalance in migraine patients.
Headache. 2005;45(4):325–331.
Neuhauser H, Lempert T. Vestibular migraine.
Neurol Clin. 2009;27:379–391
Neuhauser H, Radtke A, von Brevern M, et al.
Migrainous vertigo. Prevalence and impact on
quality of life. Neurology. 2006;67:1028–1033.
Johnson GD. Medical management of migrainerelated dizziness and vertigo. Laryngoscope.
1998;108(Suppl 85):1–28.
Lee H, Lopez I, Ishiyama A, Baloh RW. Can
migraine damage the inner ear? Arch Neurol.
2000;57:1631–1634.
Jakubowski M, Levy D, Goor-Aryeh I,
Collins B, Bajwa Z, Burstein R. Terminating
migraine with allodynia and ongoing central
sensitization using parenteral administration of COX1/COX2 inhibitors. Headache.
2005;45:850–861.
Jakubowski M, Levy D, Kainz V, Zhang X-C,
Kosaras B, Burstein R. Sensitization of central trigeminovascular neurons: blockade by
intravenous naproxen infusion. Neuroscience.
2007;148:573–583.
Neuhauser H, Radtke A, von Brevern M,
Lempert T. Zolmitriptan for treatment
of migrainous vertigo: a pilot randomized placebo-controlled trial. Neurology.
2003;60(5):882–883.
Johnson GD. Medical management of migrainerelated dizziness and vertigo. Laryngoscope.
1998;108(1 Pt 2):1–28.
BNF No.63 British National Formulary (2012
March). London: BMJ Publishing Group;
2012.
Cherchi M, Hain TC. Migraine-associated vertigo. Otolaryngol Clin N Am. 2011;44:367–375.
Whitney SL, Wrisley DM, Brown KE, Furman
JM. Physical therapy for migraine-related
vestibulopathy and vestibular dysfunction with history of migraine. Laryngoscope.
2000;110(9):1528–1534.
Eckhardt-Henn A, Best C, Bense S,
et al. Psychiatric comorbidity in different organic vertigo syndromes. J Neurol.
2008;255:420–428.
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9
Multilevel vestibulopathy
Mudit Jindal
Contents
Introduction
Risk of falls
Aetiology of multi-level vestibulopathy
Cardio-vascular causes of dizziness
Effect of polypharmacy
Neurological causes
Autonomic failure
Visual problems
Case study
Summary
79
79
80
80
80
81
81
81
81
82
Introduction
Dizziness is common in the general population.
A variety of disorders may result in dizziness or
symptoms of ‘light-headedness’, ‘fainting’ and
‘unsteadiness’.
Elderly patients present a diagnostic and therapeutic
challenge as they frequently suffer from abnormalities of their visual, vestibular and proprioceptive
systems. Central nervous system changes that occur
with age may also limit their ability to compensate
for these peripheral abnormalities.
This cumulative pathology and the symptoms and
signs seen are described as multi-level vestibulopathy or ‘multi-modal vestibulopathy’.
Risk of falls
Vertigo and dizziness in elderly patients are major
risk factors for falls. In one study, over 70% of patients
referred for falls risk assessment were found to have
an impaired vestibular system. Elderly patients
presenting with vertigo or ­balance problems should
be assessed for the risk of falls. The aim is to establish
­strategies that will prevent falls, tailored to the individual patient’s risks. These strategies include providing supportive or reducing medication, ­addressing
visual impairment, managing postural ­hypotension
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and cardiac arrhythmias, providing supportive
­footwear and modifying the home environment.
The vestibular apparatus in the inner ear suffers
from age related loss of inner hair cells and vestibular neurons resulting in reduced vestibular
function. The vestibular-ocular reflex (VOR) and
vestibulospinal reflexes, which help maintain
­posture and balance, are hence affected by age.
This results in vestibular impairment and increases
the risk of falls.
Aetiology of multi-level vestibulopathy
Cardiovascular disease is common in the general
population and is also a major factor in the aetiology of dizziness. Patients presenting with dizziness
may actually be experiencing ‘light headedness’,
‘syncope’ or ‘pre-syncope’. Cardiac arrhythmias and
postural hypotension are also frequently encountered in the elderly patient. Postural hypotension is
defined as a drop of >20 mmHg in systolic pressure
or >10 mmHg in diastolic pressure within two minutes of standing from the supine position.
Many drugs widely used to treat cardiac disorders
such as atenolol, digoxin, nitrates, nifedipine, antiarrythmic and diuretic medications can cause bradycardia and hypotension resulting in ‘dizziness’.
Furthermore, cardiovascular disease can also lead
to ischaemic changes in the brain and this can also
cause global cerebral atrophy resulting in dizziness
and unsteadiness.
Cardio-vascular causes of dizziness
Light headedness with associated pallor or
sweating may suggest a cardiovascular cause
of dizziness, especially in a patient with preexisting cardiovascular disease or following a
recent change in cardiac medication. It is equally
­i mportant to identify vasovagal syncope and
orthostatic hypotension. Bradyarrhythmias
are also an increasingly common cause of
dizziness and syncope. These patients should
undergo a 12-lead echocardiogram (ECG),
supine and erect blood pressure and referral to
a cardiologist.
Effect of polypharmacy
Elderly patients are often on a variety of medication
for multiple illnesses and are also more vulnerable
to the adverse effects of these drugs. A combination
of age related physiological changes and polypharmacy affect balance and greatly increase the risk of
falling.
The following drugs can cause ­bradycardia or postural hypotension resulting in unsteadiness:
blockers
•• alpha
beta-blockers
• vasodilators
antidepressants
•• tricyclic
loop diuretics.
The following drugs have vestibulotoxic side effects:
•• aminoglycosides
chemotherapeutic agents
• diuretics.
Other drugs affecting balance include
statins, ­c arbamazepine, barbiturates and
benzodiazepines.
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Neurological causes
Neurological causes of dizziness and disequilibrium include cerebrovascular disease, transient
ischaemic attack, Parkinson’s disease, tumours,
multiple sclerosis, Guillain–Barré syndrome (GBS)
and myasthenia gravis (note Chapter 15 Central
pathology causing dizziness).
Autonomic Failure
Another recognised cause of dizziness and postural hypotension is autonomic neuropathy. This
may occur in Parkinson’s disease, amyloidosis, B12
deficiency, diabetes mellitus, hypothyroidism, and
hypothalamic or spinal cord lesions. Patients with
diabetes may develop somatosensory neuropathy
of lower extremities, which can give rise to loss
of proprioception and result in balance and gait
disturbance. Micro-angiopathic effects in diabetic
patients can also result in ischaemia of the vestibular apparatus.
Visual problems
Reduced visual acuity can result in balance disturbance. Elderly patients are at increased risk of falls
due to cataracts, age related macular degeneration,
the use of bifocals and diabetic retinopathy.
Case study 9.1
An 80-year-old male presented with marked dizziness on rolling over in bed to his right.
He also described a pervasive unsteadiness since his total knee replacements and advancing
diabetes. He described an episode of severe vertigo in his twenties that was diagnosed as
‘labyrinthitis’. His vision had changed recently and he had taken to wearing bifocal glasses,
although he had noticed he had become very wary about stepping off the kerb whilst out
shopping and walking down stairs. He had fallen three times in the previous six weeks.
On examination he demonstrated broken smooth pursuit and a consistent catch up saccade on rapid head movement to the right. He was unable to stand on foam and step testing
revealed a rotation of 90° to the left. Dix-Hallpike testing revealed a short burst of geotropic
torsional nystagmus that lasted for 10 seconds after a latency of 10 seconds with his head
turned to the right.
A diagnosis of right posterior canal BPPV was made and he underwent three successive
Epley manouvres.
At his subsequent review, his vertigo on head turning had settled but his general unsteadiness persisted. He was therefore referred to a falls clinic where he was entered into a strength
and balance class and his diabetic medication and analgesia optimized. An occupational
therapy review was undertaken and he was encouraged to use a walking stick, which he did
begrudgingly. He also replaced his bifocal lenses with separate lenses. He was subsequently
discharged having made good progress.
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Summary
There are often multiple causes for dizziness
and it can be difficult to ascertain exactly which
pathology is the principle cause of their symptoms
(see Table 9.1). Management is multidiscplinary as
part of a falls clinic, addressing each sensory system
in turn in order to optimise function and improve
quality of life.
Table 9.1. Common causes of multi-level vestibulopathy.
Poor co-ordination/restricted mobility
Neurological problems
Transient ischaemic attack/stroke
Diabetes
Polypharmacy
Poor vision
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10
Cholesteatoma
Attila Dezso
Contents
Introduction
Aetiology
Pathogenesis
Symptoms
Signs
Investigations
Management
Conclusion
References
83
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Introduction
Cholesteatoma is a three dimensional locally
destructive epidermal structure found within the
middle ear. It is an uncommon condition with an
incidence of approximately 10 per 100,000 per year.1
Although the commonest presenting complaint is
that of chronic foul smelling aural discharge and
hearing loss, symptoms of vertigo or disequilibrium
are estimated to be present in five per cent of patients
at diagnosis.2
Aetiology
Cholesteatoma may be either c­ ongenital or
acquired. Congenital, or primary ­cholesteatoma,
is rare and arises due to failure of ­re-absorption of
a rest of squamous ­epithelium within the middle
ear during embryological development. Hence,
congenital cholesteatoma forms behind an intact
tympanic membrane.
Secondary cholesteatoma forms as a result of:
(a) Tympanic membrane retraction (classically
affecting the pars flaccida).
(b) Implantation of squamous epithelium within the
middle ear secondary to surgery.
(c) Migration of squamous epithelium into the middle ear via a tympanic membrane perforation.
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Tertiary cholesteatoma is thought to arise as a
result of recurrent middle ear infection. This results
in squamous metaplasia of the respiratory mucosa
of the middle ear cleft.3
Pathogenesis
The complications of cholesteatoma include
chronic aural discharge, tympanic membrane perforation and erosion of the ossicular chain, each resulting in a conductive hearing
loss.
Intracranial complications include meningitis,
temporal lobe or cerebellar abscess, and sigmoid
sinus thrombosis. As a result, cholesteatoma may
lead to death.
Vertigo or dysequilibrium symptoms arise due to:
Erosion of the bony labyrinth: Erosion of the
bony labyrinth occurs due to gradual expansion
of the cholesteatoma sac and its associated osteolytic enzymes.
Toxin penetration of the membranous
­labyrinth: Recurrent infection may lead
to the accumulation of toxins within the
middle ear. These may pass directly into the
inner ear via the round or oval windows,
through a breach of the membranous labyrinth, or through intact bone via the Haversian
system.
Hearing loss: The association between
­normal balance function and ­hearing
loss has been discussed previously
(Chapter 1: Anatomy and physiology of the
­peripheral ­vestibular system). The relative weighting ­however remains to be determined, and
in cases of cholesteatoma may contribute
to the unsteadiness experienced by patients.
Symptoms
Chronic ear discharge and hearing loss are
­common. As the process of erosion is gradual, the
peripheral vestibular deficit is also gradual, and
many patients are able to compensate without
any overt symptoms. Rapid expansion or toxic
­metabolites passing into the inner ear may result in
disequilibrium or vertigo.
A peripheral vestibular deficit, however, if present
will result in intermittent disequilibrium on rapid
head movement and patients may also develop
unsteadiness in rich visual environments (visual
vertigo).
Signs
2–3% of patients with chronic ear discharge
demonstrate a positive fistula test (produced on
compression of the tragus, or pneumatic otoscopy),
either due to pressure transmission secondary to a
bony breach of the bony labyrinth or via a hypermobile stapes footplate.2
Head thrust testing may demonstrate clear
catch up saccades, and head shake testing transient ­nystagmus. Unterberger test may also result
in rotation towards the affected side.
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Malleus
Cholesteatoma
Chorda tympani
nerve
Promontory
Stapes
Round window
Figure 10.1. Otoscopical examination.
Investigations
A careful otoscopical examination will, in general, demonstrate an attic tympanic membrane
perforation and the presence of keratin (see
Figure 10.1).
Ear wax or discharge may obscure one’s view of the
tympanic membrane and the ear must be microsuctioned. A child may not tolerate this outpatient
assessment and an examination under anaesthetic
is occasionally required.
An assessment of vestibular function is often difficult in patients with cholesteatoma and seldom
required. Water calorics are contraindicated
as this may provoke an ear infection, and air
calorics may produce a response that can be difficult to interpret (due to evaporation of fluid and
subsequent cooling of the middle ear mucosa)
but may at least confirm underlying vestibular
function. Rotatory chair testing is preferable if
vestibular testing is required and allows a comparison of the function of both lateral semicircular canals (SCC) to be made. A fine section
temporal bone c­ omputed tomography (CT) scan
is often undertaken in order to assess the extent
of disease. Breaches of the lateral SCC and saccule
may be seen, and the ossicular chain assessed
(Figure 10.2 a and b).
Management
In the majority of cases surgery is indicated as the
lifetime risk of a life threatening complication is
estimated to be between 5–10%.
Surgical approaches for cholesteatoma generally
include an open cavity (canal wall down procedure)
or canal wall up procedure. The latter requires
subsequent ‘look’ procedures to exclude recurrent
or residual disease.
The aim of surgery is to remove all disease, graft
the tympanic membrane defect and reconstruct
the ossicular chain.
Erosion of the bone overlying the lateral semicircular canal (SCC) may occur in approximately
10% of cases. Although often evident on preoperative CT scans, one should always approach a
diseased ear with the expectation that the facial
nerve is dehiscent and the lateral SCC has been
breached.
Cholesteatoma is removed with great care from the
canal fistula. The risk of a dead ear is greater if the
fistula is large, but is still low overall. The fistula
is then repaired with bone dust or fascia. While
some surgeons may decide to leave a little of the
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Lateral semicircular
canal fistula
(a)
Lateral semicircular
canal fistula
(b)
Figure 10.2. a and b A fine section temporal bone computed tomography (CT) scan demonstrating a
fistula of the lateral semicircular canal.
cholesteatoma over the canal fistula and convert to
an open cavity operation, the cholesteatoma may still
progress to invade the canal, resulting loss of balance
and hearing.
The lateral SCC is exposed and prone to cooling.
On occasion, patients with a generous meatoplasty
experience similar symptoms on windy days.
Patients who have undergone an open ­cavity
­mastoidectomy are susceptible to spells of disequilibrium. This is often experienced during microsuction.
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Conclusion
Cholesteatoma is an uncommon cause of vertigo
and disequilibrium but requires exclusion in every
patient. The vast majority of patients require surgery to excise the disease.
References
1 Harker LA. Cholesteatoma: An incidence
study. In McCabe BF, Sadé J, Abramson M, eds.
Cholesteatoma: First International Conference,
Birmingham, AL: Aesculapius Publishing; 1977.
2 Gormley PK. Surgical management of
­labyrinthine fistula with cholesteatoma.
J Laryngol Otol. 1986;100(10):1115–1123.
3 Ferlito A. A review of the defini-
tion, ­terminology and pathology of
aural ­cholesteatoma. J Laryngol Otol.
1993;107(6):483–488.
4 Welkoborsky HJ. Current concepts of the
pathogenesis of acquired middle ear
cholesteatoma. Laryngorhinootologie.
2011;90(1):38–48.
5 Albino AP, Kimmelman CP, Parisier SC.
Cholesteatoma: A molecular and cellular
puzzle. Am J Otol. 1998;19(1):7–19.
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11
MÉNIÈRE’S disease
Neil Donnelly
Contents
Introduction
Aetiology
Pathogenesis
Symptoms
Natural history
Stage one
Stage two
Stage three
Investigations
Pure tone audiometry
Caloric testing
Electrocochleography
Vestibular evoked myogenic potentials
Magnetic resonance imaging
Treatment
Medical management
Surgical management
Case study
Conclusion
References
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96
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98
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Introduction
Ménière’s disease is a disorder of the inner ear resulting in symptoms of episodic vertigo, tinnitus, hearing
loss and aural pressure. It is a challenging condition
that requires access to a wide range of clinical expertise. Although the exact aetiology is uncertain, it is
associated with raised pressure in the endolymph of
the inner ear (endolymphatic hydrops).
The diagnosis of Ménière’s disease is p
­ rincipally
a clinical one. The 1995 guidelines from the
American Academy of Otolaryngology–Head
and Neck Surgery (AAO-HNS)1 form the
­cornerstone for diagnosis and classification of
this condition (Table 11.1).
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Table 11.1. Diagnosis and classification of Ménière’s disease.1
Diagnosis
Classification
Certain
Histopathological evidence of endolymphatic hydrops
Definite
≥ 2 spontaneous episodes of vertigo > 20 minutes
Audiometrically documented hearing loss ≥ 1 occasion
Tinnitus or aural pressure in same ear
Probable
One definitive episode of vertigo
Audiometrically documented hearing loss ≥ 1 occasion
Tinnitus or aural pressure in the same ear
Possible
Episodic vertigo of Ménière’s type without hearing loss
Sensorineural hearing loss with disequilibrium
Table 11.2. Gibson 10-point score: ‘Have you ever had…?’2
Gibson 10-point score
Score
Vertigo
Rotational vertigo
1
Attacks of rotational vertigo > 10 minutes
1
Rotational vertigo associated with one or more of hearing loss, tinnitus, aural pressure
1
Hearing
Sensorineural hearing loss
1
Fluctuating hearing loss
1
Hearing loss or fluctuation with one or more of vertigo, tinnitus, aural pressure
1
Tinnitus
Peripheral tinnitus lasting > 5 minutes
1
Tinnitus fluctuating or changing with one or more of vertigo, hearing loss, aural pressure
1
Pressure
Constant aural pressure lasting > 5 minutes
1
Pressure fluctuating or changing with one or more of vertigo, hearing loss, tinnitus
1
/10
The AAO-HNS classification is far from ideal and
ranges from diagnostic certainty, which is possible
only after post-mortem histopathological examination, to differential diagnostic speculation.
Another diagnostic tool, the Gibson 10-point
score,2 also relies on clinical symptoms and looks
to determine their presence and interaction with
each other (Table 11.2). The greater the number
of characteristic symptoms, the higher the score
and hence the greater the likelihood of Ménière’s
disease. A score of seven or more is highly
suggestive.
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Scala vestibuli
Expanded scala
media
Organ of Corti
Scala tympani
Figure 11.1. Cross section through the cochlea demonstrating endolymphatic hydrops in scala media.
Aetiology
Temporal bone studies have clearly established
that endolymphatic hydrops plays an important role in Ménière’s disease. Endolymph is
contained within the scala media of the inner
ear. Hydrops of various degrees is seen in the
scala media (see Figure 11.1) of individuals
diagnosed with Ménière’s disease during life. 3
Membranous structures within the vestibular
apparatus may also be displaced. The precise role
of hydrops in Ménière’s disease, however, is not
fully u
­ nderstood and the presence of hydrops in
an ear does not always result in symptoms of the
disease.
Less certain is the cause of endolymphatic hydrops
in Ménière’s disease. Normal endolymph homeostasis is regulated by a complex system of local ion
transport mechanisms.4 These systems operate
under close hormonal control. The endolymphatic
sac is thought to play a key role in endolymph pressure regulation. Disruption to these systems, at any
location in the ear, may contribute to changes in the
composition of endolymph and result in volume
disturbances. Ménière’s disease is likely to represent
a variety of different disease processes that result
in the final common pathway of endolymphatic
hydrops.
Pathogenesis
Two common theories have been proposed to
explain the episodic attacks of vertigo.
First the ‘rupture theory’ proposes that increased
pressure within the scala media results in a
rupture of the delicate Reissner’s membrane; the
subsequent mixing of endolymph with perilymph
resulting in an attack of vertigo. The theory is
based upon historical histological specimens of
individuals with Ménière’s disease, where disruptions were observed in Reissner’s membrane. It is
highly likely that these histological findings represent an artefact of specimen preparation, not least
because of the unlikelihood of so many individuals
dying during the throes of an attack. Even if the
structures of the inner ear were able to survive the
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endolymph within the scala media drains rapidly
from the cochlear duct, through the saccule and
into the endolymphatic sinus. It proposes that
the increased volume of endolymph exceeds the
capacity of the endolymphatic sinus. Once a critical
level is reached, the valve separating the utricle and
sinus is breached and endolymph overflows into
the utricle, stretching the cristae of the semicircular canals (SCC) thus causing vertigo. The inflow
and then outflow of endolymph from the utricle
may explain the initial irritative and subsequent
paralytic nystagmus that is observed with a
Ménière’s attack.
mixing of endolymph with perilymph, the theory
does not explain why a rupture in the apex of the
cochlea has an effect on the anatomically distant
vestibular apparatus.
Second, a more likely theory is the ‘drainage
theory’.5 It is recognised that the endolymphatic
sac plays a primary role in endolymph volume
regulation. The role of pressure detection is
thought to be played by the endolymphatic sinus,
a small structure between the saccule and the
utriculo-endolymphatic valve at the entrance to
the endolymphatic duct (Figure 11.2). The longitudinal drainage theory suggests that excess
Symptoms
The symptoms of Ménière’s disease are those
of episodic vertigo, tinnitus, hearing loss and
aural pressure. The vertigo is typically rotatory,
debilitating and associated with nausea, vomiting
and rarely diarrhoea. There may be a prodrome.
The vertigo usually lasts longer than 20 minutes
but rarely exceeds four hours. It is unusual to
experience more than one attack within a 24 hour
period. Between attacks, subjective balance
­f unction typically returns to normal. The hearing levels and symptoms of tinnitus and aural
­pressure are subject to variation.
Ampulla
n
mo
Com
ra
Du
er
at
m
cu Bon
t s e:
ur
fa
ce
a
Cranial cavity:
posterior fossa
cru
s
Utricle
“Valve”
Utricular
duct
Saccule
Fovea for
endolymphatic
sac
Duct
isthmus
sinus
Saccular duct
Sac
Reuniting duct
Temporal bone: petrous part – cut surface
Cochlear duct
Figure 11.2. Anatomical cross section of the membranous labyrinth demonstrating the close proximity of the
utricular valve to the endolymphatic sinus.
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Natural History
Ménière’s disease can be thought of as having three
stages.6
Stage one
The onset of the symptoms: Episodes of vertigo
are the dominant feature and are usually severe.
Hearing fluctuates, but recovers to normal levels
between attacks. The tinnitus and aural fullness
often improve or disappear between attacks, where
tests of hearing and balance function are often
normal.
Stage two
There continues to be repeated episodes of vertigo.
The hearing may still fluctuate, but does not fully
recover to normal thresholds. The tinnitus and aural
fullness usually do not fully disappear between
attacks.
Stage three
The vertigo is often the least problematic symptom and the Ménière’s disease is described as
‘burnt out’. Rarely, individuals might experience
a Tumarkin drop attack, where they suddenly fall
without warning, without loss of consciousness and
without any neurologic symptoms. Typically there
is no further fluctuation of hearing, which remains
poor (thresholds of approximately 60 dBHL).
Commonly, there is constant tinnitus and a continual sensation of aural pressure.
Investigations
Pure tone audiometry
This is an essential investigation to document
­hearing thresholds and monitor any fluctuation.
Early hearing losses frequently affect lower frequencies, l­eading to the assumption that endolymphatic hydrops initially affects the apex of the
cochlea.
Caloric testing
This provides some insight into the stage of
Ménière’s disease and helps guide management.
Lateral SCC function is usually within the normal
range during the first stage of disease, but by the
time of burnout it has declined by 50% or more.
Caloric testing provides essential information about
balance function of the unaffected ear prior to
treatment of the affected ear to prevent rendering an
individual alabyrinthine with symptoms of constant
imbalance and oscillopsia. Calorics can also be used
to monitor the effect of ablation treatment in the
affected ear.
While the diagnosis of Ménière’s disease is based
on clinical history, there are occasions when it
is desirable to support clinical suspicion with an
objective test. This may aid in the diagnosis of
‘probable’ or ‘possible’ cases. In less equivocal cases,
objective testing can support clinical diagnosis
prior to implementing ablative treatment. It may be
of particular benefit in establishing early evidence
of hydrops in the contra-lateral ear prior to ablative
management.
Electrocochleography
Electrocochleography measures electrical activity
within the cochlea in response to a sound stimulus.
The electrocochleography (ECOG) waveform (see
Figure 11.3) is characterised by three elements: the
cochlear microphonic (CM), the summating potential
(SP) and the action potential (AP). ECOG involves
the presentation of a sound stimulus to the ear. The
cochlear response to this stimulus is measured with a
recording electrode either positioned in the ear canal
near the tympanic membrane or through the ear
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SP
AP
1 ms
Figure 11.3. An electrocochleography trace.
drum onto the promontory near the round window
(‘transtympanic’ technique). Transtympanic electrocochleography provides a more robust waveform that
is less troubled by artefact.
An increase in the ratio between the amplitudes of the SP and AP waves in response to a
click stimulus is suggestive of Ménière’s disease.
Different d
­ iagnostic ratios used have ranged
between 0.3 and 0.5. When using SP:AP ratio alone,
the test tends to have a high specificity (low false
positive) but poor sensitivity (high false negative),
particularly marked in early Ménière’s disease.
Greater diagnostic accuracy is gained with tests
examining the SP alone. This reflects the fact that
the greater the hydrops, the greater the effect on the
amplitude of the SP. A tone burst stimulus at 1 kHz
has been found to be the most effective tool for
examining the SP resulting in increased sensitivity
and specificity for Ménière’s disease, even in the
early stages.7
Vestibular evoked myogenic
potentials
The role of vestibular evoked myogenic potentials (VEMPs) in the diagnosis of Ménière’s
disease is currently uncertain. However, there is
an e­ merging body of evidence that may support
their use as an objective test. The cervical VEMP
(cVEMP) represents activity of the vestibulo-collic
reflex, which stabilises the head position in space.
Stimulation of the saccule leads to a m
­ yogenic
potential recorded at the sternocleidomastoid
muscle. A typical normal cVEMP response is
shown in Figure 11.4 and is c­ haracterised by
two deflections: ‘p13’ and ‘n24’ referring to
the d
­ irection and latency of deflection.
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Amplitude µV
n24
p13
Latency ms
Figure 11.4. A normal cVemp response.
Studies of cVEMP in individuals with Ménière’s
disease have demonstrated that the response may
be absent, the latency of the response increased,
the amplitude of the recorded waves reduced and/or
the frequency tuning altered. To date, the inter-aural
amplitude difference (IAD) has been found to be the
most consistently abnormal finding and the most useful for detecting the relatively early stages of disease.8
Magnetic resonance imaging
Magnetic resonance imaging (MRI) has an
important role to play in ruling out a vestibular
schwannoma and other lesions that may mimic
the symptoms of Ménière’s disease. Although not
in widespread clinical use, recent developments
have demonstrated that it is possible to get a better
appreciation of inner ear anatomy by the transtympanic introduction of gadolinium into the middle ear
space.9 Gadolinium is absorbed into the inner ear via
the round window, where it enters the perilymphatic
space and diffuses throughout the entire inner ear.
An MRI scan is performed 24 hours after instillation
of the gadolinium. In a hydropic ear, imaging is able
to identify the expanded endolymphatic space as a
filling defect within the inner ear. These radiological
findings correlate well with temporal bone specimens demonstrating hydrops and confirm hydrops
in patients with ‘definite’ Ménière’s when compared
to normal controls. This technique means that it is
likely that imaging will soon be used to achieve a
‘certain’ diagnosis of Ménière’s disease in life.
Treatment
The stepwise management of this condition,
dependent upon disease severity, means that treatment of Ménière’s disease spans all levels of medical care from primary care practitioner to tertiary
centre super-specialist (see Table 11.3). Medical
treatment of Ménière’s disease in the form of lifestyle changes and medication is effective in controlling vertigo in approximately 85% of patients.
Surgical management becomes i­ ndicated when
disabling vertigo continues.
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Table 11.3. Stepwise management strategy for management of Ménière’s disease.
Management
Medical
Surgical
Intact hearing
Non-useful hearing
1st line: Lifestyle modification
1st line: Lifestyle modification
2nd line: Betahistine 16–24 mg tds
2nd line: Betahistine 16–24 mg tds
and/or
and/or
Bendroflumethiazide 2.5–5 mg od
Bendroflumethiazide 2.5–5 mg od
1st line: Intra-tympanic injection of steroid 1st line: Intra-tympanic injection of steroid
2nd line: Grommet +/− Meniett device
2nd line: Total osseous labyrinthectomy
Endolymphatic sac surgery
3rd line: Intra-tympanic injection of
gentamicin
Vestibular nerve section
and/or
Intra-tympanic injection of gentamicin
Management should aim to preserve hearing and balance function while maximising quality of life with
symptom control. The potential evolution of contralateral disease must always be kept in mind. The rate
of bilateral disease is thought to range between 20 to
50% with the majority becoming apparent within five
years. Every step should be taken to look for bilateral
disease prior to ablative management.
Medical management
A number of lifestyle modifications have been
proposed to reduce the frequency and severity of
Ménière’s attacks. It has been suggested that a diet
high in salt can contribute to an increase in endolymph within the inner ear; it is therefore recommended that salt intake is less than 1.5–2 grams per
day. Patients may also be advised to avoid alcohol,
caffeine and tobacco. Stress is a recognised factor
contributing to Ménière’s attacks and steps may be
taken to reduce this.
The role of pharmaceutical agents is twofold.
Firstly, to reduce the frequency of vertigo attacks
and mitigate the associated aural symptoms;
secondly, to limit the vegetative effects of an acute
attack:
is the principal pharmaceutical
• Betahistine
agent and is thought to prevent symptoms due
to its vasodilation effect on the inner ear.
diuretics (e.g. bendroflumethiazide)
• Thiazide
are believed to have an effect by reducing the
volume of the endolymph.
agents such as prochloperazine
• Antiemetic
or ondansetron have a role to play during the
acute attack.
Surgical management
When considering escalations in management,
the hearing of the affected ear in addition to
the hearing and balance function of the contralateral ear are important considerations.
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If available, ECOG is a useful adjunct. Hearing
­a ssessment with pure tone audiometry and
tests of speech discrimination can establish
whether there is socially useful hearing in the
affected ear (­t ypically speech discrimination score
of > 50%).
Many cases of Ménière’s disease are responsive
to steroids and for this reason, an intra-tympanic
steroid injection (methylprednisolone 40 mg/mL or
dexamethasone 4 mg/mL) can be an effective first
line intervention, regardless of underlying hearing
thresholds. More invasive or destructive interventions, such as intra-tympanic gentamicin, endolymphatic sac surgery, vestibular nerve section,
and total osseous labyrinthectomy are reserved for
patients with disabling vertigo whom have failed
other therapy.
If good serviceable hearing exists in the affected
ear, considerations should be given to interventions
with the least potential impact on hearing. These
include grommet insertion alone or combined with
the use of the Meniett® device (Medtronic Limited,
UK) and endolymphatic sac surgery. There is an
emerging evidence base to support the role of the
Meniett, especially as the risks of this therapy are
limited to those of the grommet.10 Endolymphatic
sac surgery has been used in the management of
Ménière’s disease for many years. Its role remains
controversial, with a conflicting evidence base.11,12
Endolymphatic sac surgery involves decompression
of the sac. This is achieved by a cortical mastoidectomy and exposure of the posterior fossa dura in a
region bounded by the sigmoid sinus posteriorly,
posterior SCC antero/superiorly and the jugular
bulb inferiorly (see Figure 11.5). The sac is incised
and a silastic tube (such as Austin endolymph
dispersement shunt) is inserted to maintain the
decompression.
If conservative techniques are unsuccessful at
symptom control or if hearing thresholds are
poor, then consideration is given to ablative techniques. Intra-tympanic gentamicin is first line
therapy for ablation. Gentamicin is an ototoxic
aminoglycoside with greater effects on vestibular
hair cells than cochlear hair cells. Vertigo control
is approximately 80–90% despite a variety of
dosing regimens and numbers of treatments in
the literature.13 An effective regimen might be
Gentamicin 30 mg/mL administered weekly with
an end point of symptom resolution, new vestibular symptoms secondary to treatment or new
hearing loss.
An extremely small number of patients will fail
to respond to the above management strategies
or may continue to suffer with Tumarkin drop
attacks. For these individuals, surgical labyrinthectomy or vestibular nerve section offer excellent
control of intractable vertigo. The risks of these
Lateral
semicircular canal
Posterior
semicircular canal
Endolymphatic
duct
Figure 11.5. Exposure of the endolymphatic duct.
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treatments are greater. Nerve section should be
considered if reasonable hearing remains. A total
osseous labyrinthectomy removes all vestibular
neuro-epithelium but results in profound ipsilateral hearing loss. To achieve this the bone of
the three SCCs along with their ampullae must
be removed. The horizontal section and s­ econd
genu of the facial nerve are skeletonised to enable
the removal of the posterior canal ampulla
and the opening of the vestibule in order to
remove the otolithic organs.
Case study 11.1
A 42-year-old man presented with a six month history of intermittent episodes of right aural
fullness, severe rotatory vertigo lasting several hours, tinnitus and hearing loss. A series of
pure tone audiograms demonstrated a fluctuating sensorineural hearing loss. An MRI scan
was reported as normal and formal vestibular testing confirmed a right canal paresis.
The patient’s symptoms were initially controlled with a salt free diet and betahistine
(16mg PO TDS).
However, his vertiginous episodes recurred. Surgical options were discussed and he subsequently underwent a right Shah grommet insertion under a general anaesthetic.
Postoperatively, his vertiginous spells settled and his sensorineural hearing thresholds
improved significantly. He continued to complain of intermittent unsteadiness on rapid head
movement and was referred for vestibular rehabilitation therapy. His symptoms continued to
improve and he remained under regular follow-up.
Conclusion
There remains much to discover about Ménière’s
disease. An improved understanding of the aetiology and disease process will facilitate the development of novel therapeutic agents and treatments.
Technologies to improve symptom relief and to
rehabilitate the hearing and balance loss continue to develop. Cochlear implantation offers
an effective means of hearing rehabilitation for
the patient with bilateral disease and a profound
hearing loss. Ongoing research into implantable
vestibular devices offers the prospect of control of
episodic vertigo and rehabilitation of vestibular
hypofunction.
References
1 Committee on Hearing and Equilibrium.
Guidelines for the diagnosis and evaluation of
therapy in Ménière’s disease. Otolaryngol Head
Neck Surg. 1995;113:181–185.
2 Conlon BJ, Gibson WP. Electrocochleography
3
4
5
6
7
in the diagnosis of Ménière’s disease. Acta
Otolaryngol. 2000;120(4):180–183.
Hallpike CS, Cairns HWB. Observations of the
pathology of Ménière’s syndrome. Proc R Soc
Med. 1938;31:1317–1336.
Salt AN, Plontke SK. Endolymphatic hydrops:
Pathophysiology and experimental models.
Otolaryngol Clin North Am. 2010;43:971–983.
Gibson W. Hypothetical mechanism for vertigo
in Ménière’s disease. Otolaryngol Clin North
Am. 2010;43:1019–1026.
Kumagami H, Nishida H, Baba M.
Electrocochleographic study of
Ménière’s ­disease. Arch Otolaryngol.
1982;108(5):284–288.
Iseli C, Gibson W. A comparison of
three ­methods of using transtympanic
­electrocochleography for the diagnosis of
Ménière’s disease: Click summating potential
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measurements, tone burst summating potential
amplitude ­measurements, and biasing of the
summating potential using a low frequency
tone. Acta Otolaryngol. 2010;130(1):95–101.
8 Young YH, Huang TW, Cheng PW. Assessing
the stage of Ménière’s disease using vestibularevoked myogenic potentials. Acta Otolaryngol.
2003;129:815–818.
9 Naganawa S, Sugiura M, Kawamura M, et al.
Imaging of endolymphatic and perilymphatic
fluid at 3T after intratympanic administration of gadolinium-diethylene-triamine
pentaacetic acid. AJNR Am J Neuroradiol.
2008;29(4):724–726.
10 Dornhoffer JL, King D. The effect of the
Meniett device in patients with Ménière’s
disease: long-term results. Otol Neurotol.
2008;29(6):868–874.
11 Huang TS. Endolymphatic sac surgery for
Ménière’s disease: experience with over
3000 cases. Otolaryngol Clin North Am.
2002;35(3):591–606.
12 Thomsen J, Bretlau P, Tos M, Johnsen NJ.
Ménière’s disease: endolymphatic sac decompression compared with sham (placebo) decompression. Ann NY Acad Sci. 1981;374:820–830.
13 Pullens B, van Benthem PP. Intratympanic
gentamicin for Ménière’s disease or syndrome.
Cochrane Database Syst Rev. 2011;3:CD008234.
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12
Superior semicircular
canal dehiscence
James Rainsbury and Richard Irving
Contents
Introduction
101
Aetiology
101
Pathogenesis
102
Symptoms
102
Signs102
Investigations
102
Management
104
Case study
105
Conclusion
105
References
106
Introduction
Superior canal dehiscence syndrome (SCDS) is a
relatively new condition, first described in a series of
eight patients by Lloyd Minor in 1998.1 It is uncommon2 and patients may have often been treated for
a number of other otological conditions before the
diagnosis is finally reached. The diagnosis is primarily based around the clinical history and physical
examination. Characteristic audio-vestibular and
imaging findings, in a patient with typical clinical
features, can lend further support to the diagnosis.
Aetiology
The underlying anatomical abnormality is a bony
dehiscence of the middle cranial fossa (MCF) floor
overlying the superior semicircular canal (SSC),
and more rarely in the posterior fossa involving
the posterior semicircular canal (SCC). It remains
unclear whether this condition is congenital or
acquired in origin. In favour of a developmental/
congenital aetiology, dehiscence of the superior canal
has been reported in young children;3,4 dehiscent
or paper-thin bone is often present bilaterally, with
lamellar bone surrounding the dehiscence rather
than an abrupt change;2 cadaveric and radiologic
studies5 demonstrate that in foetal life, the superior
canal protrudes significantly into the MCF since the
membranous labyrinth is adult-sized by 21 weeks
gestation. The SSC lies very close to the developing
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dura and brain, and one theory is that the cartilage
covering the SSC becomes too thin for endochondral ossification to occur, leading to a bony defect.5
Dehiscence may also occur next to the superior
petrosal sinus, possibly by a similar mechanism.6
In favour of an acquired mechanism is the fact that
most patients present in middle age, and may report
onset of their symptoms following minor head
trauma, lifting, coughing or straining, presumably as
a result of disruption of an already thin MCF floor.7,8
Pathogenesis
The auditory symptoms may be explained by the
presence of the third mobile window on the inner
ear seen in SCDS. Air conduction (AC) thresholds
are increased because sound pressure energy from
vibration of the stapes footplate in the vestibule is
diverted through a path of least resistance away
from the cochlea, up through the superior canal,
and out through the dehiscence. The presence of
the third mobile window increases the difference
in impedance between the scala tympani and scala
vestibuli, allowing the basilar membrane to vibrate
more easily, thus improving thresholds for bone
conducted (BC) sound,9 and also explaining the
presence of sound-induced vertigo (Tullio phenomenon). Pressure-induced vestibular symptoms
occur because the membranous labyrinth, no longer protected by the bony MCF tegmen, is directly
affected by changes in intracranial pressure.
Symptoms
The typical early auditory feature of SCDS is
autophony and consequently the condition is frequently misdiagnosed as Eustachian tube dysfunction or patulous Eustachian tube, the presence of an
associated unilateral conductive loss may suggest a
diagnosis of otoscleosis.9,10 Other auditory symptoms
frequently reported are aural fullness and pulsatile
tinnitus. A characteristic and perhaps unique feature
of this condition is hyperacusis to bone conducted
sound and any patient complaining of hearing their
own footsteps, joint movements or eye movements
in the affected ear demands to have SCDS excluded.
Other symptoms of the condition occur as a result of
abnormal stimulation of the vestibular system and
comprise of sound- or pressure-induced vertigo, and
sometimes oscillopsia or positional vertigo.11
Signs
The ears are usually otoscopically normal, but it
may be possible to elicit nystagmus with tragal
pressure (Henebert’s sign) or by exposing the ear to
loud sound (Tullio phenomenon). The nystagmus,
which is vertical downbeat with a torsional component, may be evident during a Valsalva manoeuvre.
Patients with a bilateral symptomatic dehiscence
may demonstrate a vertical downbeat nystagmus
with no torsional component on Valsalva, this
being ‘cancelled out’ due to the simultaneous stimulation of both sides. Tuning fork tests may suggest
a conductive hearing loss in the affected ear, the
Weber typically lateralising to the affected side.
Investigations
Pure tone audiometry (PTA) typically gives a
picture of a pseudoconductive hearing loss, with
negative BC thresholds in the lower frequencies, occasionally down to −15 dB (Figure 12.1).
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Frequency (Hz)
250
500
1000
2000
4000
8000
–10
0
0
10
10
20
20
30
30
40
40
50
50
60
60
70
70
80
80
90
90
100
100
110
110
120
120
130
130
140
140
Decibels sound pressure level (dB SPL)
–10
125
Figure 12.1. Pure tone audiogram in patient with right SCDS.
Stapedial reflexes are normal, differentiating it from
otosclerosis or ossicular discontinuity. Air conduction thresholds may be symmetrical but more typically a slight asymmetry is evident with thresholds
down for the lower frequencies on the affected side.
Clinical suspicion of SCDS may be supported with
radiological investigation and vestibular testing,
although both have a margin of error and the
results should be interpreted in conjunction with
the clinical picture.
the inferior vestibular nerve. The ocular VEMP
measures saccular function and detects excitatory
electrical activity (n10 potential) in the extraocular
muscles (inferior oblique and inferior rectus), and is
a crossed pathway involving the inferior vestibular
nerve.12,13 They are thought to have approximately
80% sensitivity and specificity for SCDS. There is
a wide variation in VEMP thresholds and amplitudes between normal individuals so the finding
of significant asymmetry between the normal and
abnormal sides may be useful for diagnosis.14
Vestibular evoked myogenic potentials (VEMPs)
are a test of otolith function (utricle and saccule)
and are elicited at abnormally reduced thresholds
and high amplitudes in patients with SCDS, they
complement clinical suspicion and imaging in the
diagnosis of this condition. The cervical VEMP
measures saccular function by detecting relaxation
in a tensed sternocleidomastoid muscle (p13 potential), and is an uncrossed neural pathway involving
High resolution computed tomography (CT)
using 0.5 mm slices or smaller, is currently the
gold standard for radiological diagnosis of superior canal dehiscence (Figure 12.2). However,
CT overestimates the size and incidence of the
dehiscence because of partial volume averaging,
with various studies reporting incidence on CT of
3–8%, compared to 0.5–1.4% in cadaveric studies.15,16,17 The positive predictive value (PPV) of
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Figure 12.2. (a) Coronal and (b) oblique reformatted CT demonstrating superior semicircular canal dehiscence (arrows).
CT might be improved from 50 to 93% by using
fine slice protocols and reformatting images in the
plane of, and at 90 degrees to, the SSC.18 However,
in larger series it seems that even when using
sub-millimetre slices with oblique reformats,
the actual PPV figure is much lower, between
57–67%.19 T2-weighted fast spin echo magnetic
resonance imaging (MRI) sequences may contribute to the radiological investigation of SCDS, with
one study finding a sensitivity of 96% and specificity of 98%, although this was using CT as the
gold-standard for comparison, so the actual figure
may be somewhat lower, for the reasons outlined
previously.20
Management
Many patients are effectively managed by avoidance
of provocative stimuli, but symptomatic patients
with a high suspicion of SCDS on clinical grounds,
imaging features and VEMP results may benefit
from surgical management. Minor1 originally
described a classical middle cranial fossa craniotomy approach to the SSC, while other authors have
described a transmastoid middle fossa approach21
and a simple transmastoid approach.22 On reaching the superior canal by either of the middle
fossa routes, the dehiscence may be resurfaced
using fascia, bone chip or cartilage, or sealed with
hydroxyapatite cement. In a standard transmastoid
approach, a cortical mastoidectomy is performed
until the SSC is seen. The anterior and posterior
limbs are blue-lined, the semicircular canal endosteum is depressed using a blunt needle, and the
dehiscence is isolated from the rest of the labyrinth
by plugging each limb with fascia and small bone
fragments. Due to the anatomical nature of the condition, the MCF floor may be too low to allow access
to the SSC via a transmastoid route, in which case,
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either a standard or a transmastoid middle fossa
approach may be employed and the dehiscence dealt
with from above. The authors favour a transmastoid
approach where this is deemed possible, since this
is quicker, avoids a craniotomy and avoids the risks
associated with temporal lobe retraction. The transmastoid approach however provides much poorer
visualisation of the defect, and t­ ypically the defect
is viewed only after occlusion, if at all. The surgeon
must therefore be extremely confident of the diagnosis prior to undertaking this procedure.
Post-operatively almost all patients will experience
a degree of imbalance that is best addressed with
vestibular therapy.
In addition a temporary hearing loss will be
evident which is typically mixed but with
a predominant conductive component. The
patient may not become aware of the full
­auditory b
­ enefits of surgery for up to six
weeks. Improvement in hyperacusis is however
­usually immediate, and patients will describe
­reduction or absence of these features in the early
­post-operative period.
The majority of patents reported following surgery
have derived benefits. One series 15 of 16 deriving
either complete resolution or significant improvement in symptoms.23 A number of cases have also
been operated bilaterally with encouraging results.
The debate continues over whether results are better with canal plugging or with resurfacing, with a
recent meta-analysis demonstrating the superiority of the canal plugging technique.
Case study 12.1
A 55-year-old man presented with a 12 month history of aural fullness and pulsatile tinnitus.
He also reported episodes of marked disequilibrium on straining and when exposed to loud
sounds. Tragal pressure produced transient nystagmus.
A pure tone audiogram demonstrated a mild mid frequency conductive hearing loss, and
a computed tomogram of his temporal bones an absent bony covering over the superior
semicircular canal.
His symptoms warranted surgical intervention and he underwent a transmastoid occlusion
procedure. He made an uneventful recovery and was subsequently referred for customised
vestibular physiotherapy. His symptoms resolved completely and he was discharged.
Conclusion
All involved in the management of patients with
disorders of balance are aware of the supreme value
of the clinical history in making a diagnosis, and
SCDS is no exception. The patient who can hear
their eyeballs moving, the patient with symmetrical air conduction thresholds within the normal
range, whose weber lateralises to the symptomatic
ear, these and the other features described in this
chapter should alert the clinician to the diagnosis.
The imaging can be very difficult to interpret as
we are working at the limits of resolution of HRCT
and we feel strongly that this should not be taken as
a diagnostic test but used in conjunction with the
other findings. VEMP testing can also be misleading and of little value especially in the 10% of cases
that are bilateral. Investigations can be supportive
and should be treated with caution if the picture
does not quite fit.
Superior semicircular canal dehiscence 105
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If the surgeon is confident of the diagnosis and the
patient describes the symptoms having a significant impact on their quality of life then surgery is a
very reasonable option. Complete resolution of all
References
1 Minor LB, Solomon D, Zinreich JS, et al.
Sound- and/or pressure-induced vertigo due
to bone dehiscence of the superior semicircular canal. Arch Otolaryngol Head Neck Surg.
1998;124:249–258.
2 Carey JP, Minor LB, Nager GT. Dehiscence
or thinning of bone overlying the superior semicircular canal in a temporal bone
survey. Arch Otolaryngol Head Neck Surg.
2000;126(2):137–147.
3 Zhou G, Ohlms L, Liberman J, et al.
Superior semicircular canal dehiscence in
a young child: implication of developmental defect. Int J Pediatr Otorhinolaryngol.
2007;71:1925–1928.
4 Lee GS, Zhou G, Poe D, et al. Clinical
­experience in diagnosis and management of superior semicircular canal
dehiscence in ­children. Laryngoscope.
2011;121(10):225–226.
5 Takahashi N, Tsunoda A, Shirakura S, et al.
Anatomical feature of the middle ­cranial
fossa in fetal periods: Possible etiology of
­superior canal dehiscence syndrome. Acta OtoLaryngologica. 2012;132:385–390.
6 McCall AA, McKenna MJ, Merchant SN,
et al. Superior canal dehiscence syndrome
associated with the superior petrosal sinus in
pediatric and adult patients. Otol Neurotol.
2011;32:1312–1319.
7 Minor LB. Classic manifestations of superior
semicircular canal dehiscence. Laryngoscope.
2005;115:1717–1727.
8 Watters KF, Rosowski JJ, Sauter T, et al.
Superior semicircular canal dehiscence presenting as postpartum vertigo. Otol Neurotol.
2006;27:756–768.
9 Merchant SN, Rosowski JJ, McKenna MJ.
Superior semicircular canal dehiscence
mimicking otosclerotic hearing loss. Adv
Otorhinolaryngol. 2007;65:137–145.
symptoms might be unrealistic for many but significant or full resolution of symptoms in over 90%
of cases is achieved with surgery in appropriately
selected cases.
10 Halmagyi GM, Aw ST, McGarvie LA, et al.
11
12
13
14
15
16
17
18
Superior semi-circular canal dehiscence
simulating otosclerosis. J Laryngol Otol.
2003;117:553–557.
Brantberg K, Bergenius J, Mendel L, et al.
Symptoms, findings and treatment in patients
with dehiscence of the ­superior semicircular
canal. Acta Otolaryngol. 2001;121(1):68–75.
Manzari L, Burgess AM, Curthoys IS. Ocular
and cervical vestibular evoked myogenic potentials in response to bone-conducted vibration
in patients with probable inferior vestibular
neuritis. J Laryngol Otol. 2012;126:683–691.
Colebatch JG, Halmagyi GM, Skuse NF.
Myogenic potentials generated by a clickevoked vestibulocolic reflex. J Neurol Neurosurg
Psychiatry. 1994;57:190–197.
Janky KL, Shepard N. Vestibular evoked
­myogenic potential (VEMP) testing:
­normative threshold response curves
and effects of age. J Am Acad Audiol.
2009;20(8):514–522.
Sequeira SM, Whiting BR, Shimony JS, et al.
Accuracy of computed tomography detection
of superior canal dehiscence. Otol Neurotol.
2011;32(9):1500–1505.
Stimmer H, Hamann KF, Zeiter S, et al.
Semicircular canal dehiscence in HR
­multislice computed ­tomography:
­distribution, ­frequency, and clinical
­relevance. Eur Arch Otorhinolaryngol.
2012;269(2):475–480.
Tavassolie TS, Penninger RT, Zuñiga MG,
et al. Multislice computed tomography in
the ­diagnosis of superior canal dehiscence:
how much error, and how to minimize it?
Otol Neurotol. 2012;33(2):215–222.
Crane BT, Minor LB, Carey JP. Threedimensional computed tomography of superior
canal dehiscence syndrome. Otol Neurotol.
2008;29(5):699–705.
106 Dizziness and Vertigo: An Introduction and Practical Guide
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19 Cloutier JF, Bélair M, Saliba I. Superior semi-
circular canal dehiscence: positive predictive
value of high-resolution CT scanning. Eur Arch
Otorhinolaryngol. 2008;265(12):1455–1460.
20 Krombach GA, Schmitz-Rode T, Haage P, et al.
Semicircular canal dehiscence: comparison
of T2-weighted turbo spin-echo MRI and CT.
Neuroradiology. 2004;46(4):326–331.
21 Teixido M, Seymour PE, Kung B, et al.
Transmastoid middle fossa craniotomy repair
of superior semicircular canal dehiscence
using a soft tissue graft. Otol Neurotol.
2011;32(5):877–881.
22 Agrawal SK, Parnes LS. Transmastoid superior
semicircular canal occlusion. Otol Neurotol.
2008;29(3):363–367.
23 Beyea JA, Agrawal SK, Parnes LS.
Transmastoid semicircular canal occlusion:
A safe and highly effective treatment for
benign paroxysmal ­positional vertigo and
superior canal dehiscence. Laryngoscope.
2012;122(8):1862–1866.
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13
Vestibular
schwannoma
James Tysome
Contents
Introduction
109
Aetiology
109
Symptoms
110
Signs110
Investigations
111
Management
111
References
112
Introduction
Vestibular schwannomas are benign tumours that
arise from the vestibular nerve. Although a rare
cause of dizziness, nearly three-quarters of patients
with vestibular schwannomas have problems with
balance at presentation.1 These symptoms result in
a significant negative effect on their quality of life.2,3
Up to two-thirds of these tumours do not grow and
are actively monitored with serial magnetic resonance imaging (MRI) scanning. Those that do grow
tend to do so slowly at around 2 mm each year.
Although these are benign tumours, their location
in the cerebellopontine angle means that growth
results in compression of the lower cranial nerves
and brainstem.
Aetiology
Over 90% of vestibular schwannomas are unilateral and sporadic with an estimated incidence of
1:100,000. Vestibular schwannomas most commonly arise from the superior division of the
vestibular nerve. Bilateral vestibular schwannomas
are diagnostic for neurofibromatosis type 2 (NF2)
which is characterised by multiple benign tumours
of the central and peripheral nervous systems, as
well as ocular and skin lesions. It is inherited in
an autosomal dominant manner and results from
mutation of the NF2 tumour suppressor gene on
chromosome 22q12.
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Figure 13.1. MRI scan of a left vestibular schwannoma.
Symptoms
The most common symptoms of vestibular
schwannomas are hearing loss, tinnitus and
balance disturbance; this may take the form of
vertigo or a general sense of imbalance. Symptoms
may progress as the tumour grows, causing
increased compression of the cochlear nerve.
Initially, hearing loss is in the high frequencies
and tends to progress slowly over time. However, a
small proportion of patients present with sudden
hearing loss.4 As the tumour expands into the
cerebellopontine angle and starts to compress the
cerebellum, balance may worsen (Figure 13.1).
Further growth results in brainstem compression
accompanied by ataxia as hydrocephalus develops.
Signs
Physical examination may be completely normal.
The most common examination finding is decreased
hearing in the ear on the side of the tumour; tuning
fork testing may find Weber’s lateralising away from
the side of the tumour and, Rinne’s test positive
in both ears due to a unilateral or asymmetrical
110 Dizziness and Vertigo: An Introduction and Practical Guide
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sensorineural hearing loss. When examining the
cranial nerves, it is rare that a vestibular schwannoma results in a motor weakness of the facial nerve.
In these cases, the possibility of a facial neuroma
should be considered. Decreased sensation of the
posterior external auditory canal skin (Hitselberger’s
sign) is due to compression of the sensory fibres
of the facial nerve and present in around 10% of
patients.5 Paraesthesia in the distribution of the
trigeminal nerve occurs with large tumours.
Patients with imbalance often have evidence of
uncompensated vestibular dysfunction. Romberg’s
is often normal. Unterberger’s stepping test may
show rotation towards the side of the tumour.
Spontaneous nystagmus is rare and head thrust
testing often normal, although post-head-shake
nystagmus towards the opposite side to the lesion
may be observed.
Investigations
Pure tone audiometry and speech discrimination
testing are useful in documenting the level of hearing
disability in order to aid the decision taken on treatment. Speech discrimination is worse than might be
expected from the pure tone audiogram.
An MRI of the internal auditory meati is essential in order to make the diagnosis of a vestibular
schwannoma. These tumours strongly enhance
after administration of gadolinium and tend to fill
the internal auditory canal before extending into
the cerebellopontine angle.
Prior to MRI, auditory brainstem reflexes (ABR)
were used as an aid to making the diagnosis of a vestibular schwannoma. They are abnormal in 95% of
patients, with wave V latency increased on the side
of the tumour and multiple waves absent in 50%.6
ABRs are no longer necessary. Vestibular testing is
not routinely performed unless patients’ symptoms
of imbalance are severe enough to require rehabilitation. A reduction in calorics is often seen on the
side of the tumour. Rotational testing may reveal
preponderance to the side opposite to the tumour.
Posturography testing is likely to be abnormal with
eyes closed, although this does not predict subjective balance function after surgery.7
Vestibular testing can help predict the degree of
balance dysfunction following surgery. Patients
with little loss of vestibular function prior to surgery may experience greater vestibular dysfunction
after surgery than those who have already compensated for a significant unilateral vestibular loss.
Management
Patients presenting with vestibular schwannomas
where the maximum intracranial diameter is less
than 20 mm are often initially managed conservatively with a watch, wait and rescan policy. However,
where growth is seen on serial scanning or tumours
are larger, treatment is recommended with either
surgery or stereotactic radiotherapy (single fraction
gamma knife or fractionated radiotherapy).
Patients with imbalance prior to radiotherapy
usually find that their symptoms do not improve.
As a result, severe balance problems not responsive
to vestibular rehabilitation are an indication for
surgery over radiotherapy in patients with growing
tumours. Thirty per cent of patients experience new
vestibular symptoms following radiotherapy for
vestibular schwannomas, most commonly in the
first six months after treatment.8 These may be due
to a direct effect of radiotherapy on the vestibulocochlear nerve or radiation induced neuritis affecting the labyrinth. Patients who are not responsive
to vestibular rehabilitation may benefit from a
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chemical or surgical labyrinthectomy to ablate
peripheral vestibular function on the side of the
tumour.
4 Moffat DA, Baguley DM, von Blumenthal H,
The most common surgical approaches used for
tumour removal are translabyrinthine, retrosigmoid and middle fossa. In all three, the vestibular
nerve is divided. In a translabyrinthine approach, a
complete labyrinthectomy is also performed where
all three semicircular canals and the vestibule are
drilled out. It is, therefore, not surprising that the
majority of patients experience balance problems
after vestibular schwannoma surgery. Symptomatic
patients usually improve within weeks as central
compensation takes place. Although satisfactory
recovery of balance control occurs by three months
after surgery in the majority of patients,9 improvements may take up to one year.10 This process can
be accelerated by vestibular rehabilitation therapy.11
Rarely, patients may experience persistent severe
vestibular problems several years after surgery.12
These are seen in patients with cerebellar ataxia,
although this is a rare complication of vestibular
schwannoma surgery as excessive retraction on the
cerebellum is avoided through decompressing CSF
cisterns. The loss of vestibular function experienced after treatment of vestibular schwannomas
is most effectively improved through vestibular
rehabilitation.
5
References
1 Moffat DA, Ballagh RH. Rare tumours of the
cerebellopontine angle. Clin Oncol (R Coll
Radiol). 1995;7:28–41.
2 Breivik CN, Varughese JK, Wentzel-Larsen T,
Vassbotn F, Lund-Johansen M. Conservative
management of vestibular schwannoma—a prospective cohort study: Treatment, symptoms,
and quality of life. Neurosurgery. 2012;70:1072–
1080; discussion 1080.
3 Myrseth E, Moller P, Wentzel-Larsen T, Goplen F,
Lund-Johansen M. Untreated vestibular schwannomas: Vertigo is a powerful predictor for
health-related quality of life. Neurosurgery,
2006;59:67–76; discussion 67–76.
6
7
8
9
10
11
12
Irving RM, Hardy DG. Sudden deafness in
vestibular schwannoma. J Laryngol Otol.
1994;108:116–119.
Thomsen J, Tos M. Diagnostic strategies in
search for acoustic neuromas. Findings in 300
acoustic neuroma patients. Acta Otolaryngol
Suppl. 1988;452:16–25.
Quaranta A, Scaringi A, Quaranta N. Auditory
brainstem responses, otoacoustic emissions and
efferent acoustic reflexes in ears with vestibular schwannomas. In: Baguley D, Moffat R D.,
eds. Fourth International Conference on
Vestibular Schwannoma and Other CPA Lesions.
Cambridge, UK: Immediate Proceedings Ltd;
2003:91–92.
Bergson E, Sataloff RT. Preoperative computerized dynamic posturography as a prognostic indicator of balance function in patients
with acoustic neuroma. Ear Nose Throat J.
2005;84:154–156.
Wackym PA, Hannley MT, RungeSamuelson CL, Jensen J, Zhu YR. Gamma
knife surgery of vestibular schwannomas:
­longitudinal changes in vestibular function and ­measurement of the dizziness
handicap ­inventory. J Neurosurg. 2008;109
Suppl:137–143.
Uehara N, Tanimoto H, Nishikawa T, et al.
Vestibular dysfunction and compensation after
removal of acoustic neuroma. J Vestib Res.
2011;21:289–295.
Parietti-Winkler C, Gauchard GC, Simon C,
Perrin PP. Long-term effects of vestibular compensation on balance control and
­sensory organisation after unilateral deafferentation due to vestibular schwannoma
surgery. J Neurol Neurosurg Psychiatry.
2010;81:934–936.
Enticott JC, O’Leary SJ, Briggs RJ. Effects of
vestibulo-ocular reflex exercises on vestibular compensation after vestibular schwannoma
surgery. Otol Neurotol. 2005;26:265–269.
Tufarelli D, Meli A, Labini FS, et al. Balance
impairment after acoustic neuroma surgery.
Otol Neurotol. 2007;28:814–821.
112 Dizziness and Vertigo: An Introduction and Practical Guide
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14
Perilymph fistula
Richard Gurgel
Contents
Introduction
Aetiology and pathogenesis
Symptoms and signs
Diagnostic investigations
Management
Case study
References
113
113
114
114
115
115
116
Introduction
A perilymph fistula (PLF) is an abnormal communication between the perilymph of the inner ear
and the spaces surrounding the otic capsule bone.
Fistulae can occur into the middle ear, mastoid and,
less commonly, the intracranial space. Modern definitions of PLF generally focus on a leak of perilymph
from the round window, oval window, or both.1
Perilymph fistulae are rare and the true incidence
is unknown. They can arise in both the paediatric and adult populations with no clear gender or
ethnic predilection. Fistulae are often mistakenly
diagnosed as Ménière’s disease, idiopathic sudden
sensorineural hearing loss, or benign paroxysmal
positional vertigo.2
Aetiology and pathogenesis
The underlying aetiologies for perilymph fistulae
are diverse and commonly include congenital,
acquired, iatrogenic, and idiopathic. A congenital
PLF can be due to malformation of the ossicles,
round window, or cochlea.3 Acquired, post traumatic fistulae can develop after barotrauma
(implosive or explosive), blunt or penetrating head
trauma, as well as abdominal or chest trauma with
sudden increases in intracranial pressure. This sudden increase in pressure is transmitted to the inner
ear via the spinal fluid or cochlear aqueduct.4,5,6
An iatrogenic fistula can arise after chronic ear
surgery or stapedectomy through a weakness in
the oval window.7,8
Perilymph fistula 113
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Figure 14.1. This figure illustrates the ‘fistula test.’ When pressure is applied by pneumatic otoscopy to the
left ear, nystagmus is induced.
Symptoms and signs
Patient presentation is variable, but symptoms of a
PLF are typically referable to the audio-vestibular
functions of the inner ear. In one large series, 83%
of patients with demonstrable perilymph fistulae
had auditory symptoms, whilst 82% had vestibular
symptoms.9 Fluctuating, sudden, or progressive
hearing loss, tinnitus, and aural fullness are other
possible auditory symptoms.
Acute, episodic vertigo or chronic, persistent
disequilibrium are common vestibular symptoms.
The vertigo can be positional, noise- or pressureinduced. The presence of a PLF can be demonstrated by provocative vestibular testing. One of the
more specific signs is the ‘fistula test’. This test is
performed by applying positive and negative pressure to the ear with pneumatic otoscopy. A positive response will illicit dizziness and nystagmus,
though the latter finding is only seen in about 25%
of patients (Figure 14.1).10
The ‘eyes-closed-turning’ test is reported to have
high sensitivity for detecting a PLF. This test
is performed by asking the patient to walk in a
straight line followed by an abrupt 180 degree
turn and stop. A positive test is seen when the
patient loses their balance after turning to the
side of the lesion.10
Diagnostic investigations
Routine audiometry may identify a sensorineural
hearing loss. Since PLFs are usually very small
(sub-millimetre), imaging has had a limited role in
confirming the diagnosis of PLF, unless a pneumolabyrinth is detected. Some have advocated
sampling fluid collected from the middle ear and
testing it for proteins specific to the cerebrospinal
fluid (CSF) or perilymph. These proteins include
β-2 transferrin, β-trace protein, and more recently,
cochlin-tomoprotein.11,12,13,14 The diagnostic accuracy and feasibility of these tests is still evolving.
The conventional gold standard for PLF detection is intraoperative microscopic visualisation
of perilymph leakage through an open fistula.13
However, in patients with suspected fistula, the
rates of positive intraoperative identification have
been reported at 40–50%.9,15,16
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Management
In the acute post traumatic PLF setting, conservative measures such as bed rest, head elevation,
use of stool softeners, and avoidance of Valsalva
manoeuvre are reasonable options. In the paediatric population, Prisman et al17 advocated surgical exploration for cases of CSF leak, persistent
vestibular symptoms, or progressive sensorineural
hearing loss (SNHL).17 When pneumolabyrinth
is present in a post traumatic PLF, surgical exploration has inconsistent results for improving
SNHL.18,19,20
Some studies have advocated injecting either an
autologous blood or fibrin glue into the middle ear
cavity as a method to hermetically seal the PLF.21,22
Despite the reported success of injections, surgical exploration of the middle ear remains one of
the most frequently cited ways to manage PLFs.
The goal of surgical intervention is to positively
identify the site of PLF and seal the leak. Typically
autologous tissue, such as fascia or perichondrium,
are used to seal the PLF. Fat has shown higher
recurrence rates than the other soft tissues mentioned.23 Most series cite some post surgical benefit
to patients.2,9,16,24
While surgery often provides resolution or stabilisation of vestibular symptoms, improvement in
hearing is less common. Interestingly, even in cases
where no fistulas are identified, patching tissue
around the oval or round windows is reported to
relieve symptoms in 30–40% of negative explorations, suggesting that some PLFs may be submicroscopic, intermittently active, or that there is a
placebo effect to exploration.15,16
Case Study 14.1
A 45-year-old man presented to the neurotology clinic with a one year history of intermittent
vertigo. The patient’s symptoms began while he was exercising and lifting heavy weights.
During his workout, he experienced a rapid-onset, room-spinning dizziness that lasted for
approximately 15 minutes. He had some residual disequilibrium for the following three days
which eventually resolved. He had no changes in hearing, tinnitus, or aural fullness during the
episode. The patient experienced similar episodes during subsequent, vigorous workouts and
once while fishing and straining to reel in his catch. He had no autophony or noise-induced
dizziness and was otherwise healthy.
On examination, the patient had intact tympanic membranes bilaterally with no evidence
of perforation, cholesteatoma, or chronic ear disease. He had pressure-induced vertigo on
pneumatic otoscopy, though no objective nystagmus. The rest of his neurotologic exam was
normal.
Routine audiometry revealed a mild, symmetric, high-frequency SNHL. A computed tomography (CT) scan was ordered that showed normal bony covering over the SSCs and no
abnormalities of the inner ear, middle ear, or mastoid. Given the history of consistent, pressureinduced, episodic vertigo and positive fistula test on examination, a middle ear exploration was
performed.
Intra-operatively, a small fistula was seen through inferior border of the annular ligament of
the oval window with leak of clear fluid during ventilator-induced valsalva. This was covered
with fascia and an autologous blood patch was injected to cover the fascia.
The patient’s symptoms of vertigo subsequently resolved.
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References
1 Hornibrook J. A balance test for chronic
perilymph fistula. Int J Otolaryngol.
2012;2012:163691.
2 Goto F, Ogawa K, Kunihiro T, Kurashima K,
Kobayashi H, Kanzaki J. Perilymph fistula–45
case analysis. Auris Nasus Larynx. 2001;28:29–33.
3 Weissman JL, Weber PC, Bluestone CD.
Congenital perilymphatic fistula: Computed
tomography appearance of middle ear and
inner ear anomalies. Otolaryngol Head Neck
Surg. 1994;111:243–249.
4 Fee GA. Traumatic perilymphatic fistulas. Arch
Otolaryngol. 1968;88:477–480.
5 Emmett JR, Shea JJ. Traumatic perilymph fistula. Laryngoscope. 1980; 90:1513–1520.
6 Goodhill V. Sudden deafness and round window rupture. Laryngoscope. 1971;81:1462–1474.
7 Ederies A, Yuen HW, Chen JM, Aviv RI,
Symons SP. Traumatic stapes fracture with
rotation and subluxation into the vestibule and pneumolabyrinth. Laryngoscope.
2009;119:1195–1197.
8 Hatano A, Rikitake M, Komori M, Irie T,
Moriyama H. Traumatic perilymphatic fistula
with the luxation of the stapes into the vestibule. Auris Nasus Larynx. 2009;36:474–478.
9 McCabe BF. Perilymph fistula: The Iowa experience to date. Am J Otol. 1989;10:262.
10 Singleton GT, Post KN, Karlan MS, Bock DG.
Perilymph fistulas. Diagnostic criteria
and therapy. Ann Otol Rhinol Laryngol.
1978;87:797–803.
11 Buchman CA, Luxford WM, Hirsch BE,
Fucci MJ, Kelly RH. Beta-2 transferrin assay
in the identification of perilymph. Am J Otol.
1999;20:174–178.
12 Bachmann-Harildstad G, Stenklev NC, Myrvoll
E, Jablonski G, Klingenberg O. Beta-trace protein as a diagnostic marker for perilymphatic
fluid fistula: a prospective controlled pilot study
to test a sample collection technique. Otol
Neurotol. 2010;32:7–10.
13 Ikezono T, Shindo S, Sekiguchi S, et al.
14
15
16
17
18
19
20
21
22
23
24
The ­performance of cochlin-tomoprotein
detection test in the diagnosis of perilymphatic
fistula. Audiol Neurootol. 2010;15:168–174.
Ikezono T, Shindo S, Sekine K, et al. Cochlintomoprotein (CTP) detection test identifies
traumatic perilymphatic fistula due to penetrating middle ear injury. Acta Otolaryngol. 2011;
31:937–944.
Shelton C, Simmons FB. Perilymph fistula: The
Stanford experience. Ann Otol Rhinol Laryngol.
1988;97:105–108.
Rizer FM, House JW. Perilymph fistulas: The
House Ear Clinic experience. Otolaryngol Head
Neck Surg. 1991;104:239–243.
Prisman E, Ramsden JD, Blaser S, Papsin B.
Traumatic perilymphatic fistula with pneumolabyrinth: Diagnosis and management.
Laryngoscope. 2011; 121:856–859.
Yanagihara N, Nishioka I. Pneumolabyrinth in
perilymphatic fistula: Report of three cases. Am
J Otol. 1987;8:313–318.
Lyos AT, Marsh MA, Jenkins HA, Coker NJ.
Progressive hearing loss after transverse temporal bone fracture. Arch Otolaryngol Head Neck
Surg. 1995;121:795–799.
Nishiike S, Hyo Y, Fukushima H.
Stapediovestibular dislocation with pneumolabyrinth. J Laryngol Otol. 2008;122:419–421.
Shinohara T, Gyo K, Murakami S, Yanagihara
N. [Blood patch therapy of the perilymphatic fistulas–an experimental study]. Nihon
Jibiinkoka Gakkai Kaiho. 1996;99:1104–1109.
Garg R, Djalilian HR. Intratympanic injection
of autologous blood for traumatic perilymphatic fistulas. Otolaryngol Head Neck Surg.
2009;141:294–295.
Seltzer S, McCabe BF. Perilymph fistula: the
Iowa experience. Laryngoscope. 1986;96:37–49.
Black FO, Pesznecker S, Norton T, et al.
Surgical management of perilymphatic fistulas: a Portland experience. Am J Otol.
1992;13:254–262.
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15
Central pathology
causing dizziness
C. Eduardo Corrales
Contents
Introduction
Aetiology
1. Migraine
2. Neoplasms
3. Cerebrovascular disorders
4. Craniovertebral junction disorders
5. Multiple sclerosis
6. Cerebellar ataxia syndromes
7. Focal seizure disorder
8. Normal-pressure hydrocephalus
9. Psychiatric dizziness
10. Toxins and medications
References
117
117
118
118
118
119
120
120
121
121
121
121
121
Introduction
One of the major undertakings for the physician
is differentiating between a peripheral or central
cause of dizziness.1 The main reason for the differentiation is that some central causes of acute
dizziness, such as central haemorrhage or infarction, can be life-threatening and may require
immediate intervention.2
Aetiology
Dizziness can be caused by multiple pathologies of
the central nervous system (CNS). A comprehensive
list follows outlining central causes of dizziness:
1 Migraine.
2 Neoplasms:
a Vestibular schwannomas.
b Brainstem neoplasms.
c Cerebellar neoplasms.
3 Cerebrovascular disorders:
a Vertebrobasilar insufficiency.
b Lateral medullary syndrome.
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c Lateral pontomedullary syndrome.
d Cerebellar infarction.
e Cerebellar haemorrhage.
4 Craniovertebral junction disorders:
a Basilar indentation.
b Assimilation of the atlas.
c Atlantoaxial dislocation.
d Chiari malformation.
5 Multiple sclerosis.
6 Cerebellar ataxia syndromes.
7 Focal seizure disorders.
8 Normal pressure hydrocephalus.
9 Psychiatric dizziness.
10 Toxins and medications.
The most common central disorders causing vertigo
are discussed below.
1. Migraine
Migrainous vertigo (vestibular migraine) is discussed in detail in Chapter 8.3
2. Neoplasms
Vestibular schwannomas. Please see Chapter 13 for
more details.
3. Cerebrovascular disorders
The vertebrobasilar system supplies blood to the
inner ear, brainstem and cerebellum. The most
common causes of ischaemia of the vertebrobasilar
system are embolism, large-artery atherosclerosis, penetrating small-artery disease and arterial
dissection.4
All patients suspected of posterior circulation ischaemia should have a thorough clinical evaluation
including a detailed patient history for vascular
risk factors, such as hypertension, diabetes, heart
disease and clinical examination, including an
accurate neurologic examination.
Specific cerebrovascular disorders associated with
vertigo include:
a Vertebrobasilar insufficiency
Cause: Atherosclerosis of the subclavian, vertebral
or basilar arteries is the underlying cause of
vertebrobasilar insufficiency.
Clinical examination: Vertebrobasilar insufficiency is the most common cause of ­vertigo in
the elderly population.5 Vertigo, oro-pharyngeal
dysfunction, headache, ­vomiting, diplopia,
visual loss, ataxia, facial numbness and weakness are common symptoms.
b Lateral medullary syndrome
(Wallenberg’s syndrome)
Cause: This occurs due to occlusion of the ipsilateral intracranial vertebral artery,6 and rarely
by occlusion of the posterior inferior cerebellar
artery (PICA).7
Clinical examination: The classic presentation
consists of:
symptoms including vertigo, facial
• General
pain, hoarseness, dysphagia and difficulty sitting without support.
signs including decreased
• Ipsilateral
facial pain and temperature sensation,
•
Horner’s syndrome, ataxia, laryngeal
paralysis.
Contralateral signs including decreased facial
pain and temperature in trunk, limbs or
both.
c Lateral pontomedullary syndrome
Cause: Occurs from ischaemia of the anterior cerebellar artery, which results in infarction of the
dorsolateral pontomedullary region and inferolateral cerebellum.8,9
Clinical examination: Acute severe vertigo, nausea,
vomiting are usually the presenting symptoms.
Other signs and symptoms may include: facial
palsy, hearing loss and tinnitus.
d Cerebellar infarction
Cause: A stroke confined to the cerebellum may
occur from occlusion of the vertebral, anterior
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inferior cerebellar, posterior inferior cerebellar
or superior cerebellar arteries.10
Clinical examination: Severe cerebellar signs of
gait ataxia and paretic gaze nystagmus with
associated severe vertigo, nausea and vomiting
suggest a cerebellar infarction.
e Cerebellar haemorrhage
Cause: Haemorrhage in the cerebellum may
­produce acute and severe vertigo.
Clinical examination: Severe vertigo with associated signs that might include severe headache
and neck stiffness.11,12
Investigations required
Non-contrast computed tomography (CT) scan is
usually performed because it is available in most
acute facilities and shows intracranial haemorrhage. Gadolinium magnetic resonance imaging
(MRI) and contrast enhanced magnetic resonance
angiography (MRA) are superior to CT for detecting cerebrovascular disorders, and when diffusionweighted sequences are performed, may detect early
infarcts within the first hour. Given the option,
MRI is preferred.
Treatment overview
Medical, interventional and surgical therapies have
and are being developed to treat occlusive disease
of the posterior circulation of the brain. Short-term
medical treatment may include the intravenous
administration of tissue plasminogen activator
(t-PA).
4. Craniovertebral junction
disorders
Compression of the central nervous system at the
level of the upper spinal cord and medulla, also
known as cervicomedullary compression.8,13 All
disorders listed below may present with tinnitus,
vertigo, hearing loss, pharyngeal dysfunction, and
hoarseness or airway obstruction and thus may
bring some patients to an otolaryngologist for
­initial management.
The disorders at the craniovertebral junction causing vertigo and dizziness are:
a Occipitalisation of the atlas
Characterised by fusion of the occiput to the
atlas.14 It is also know as assimilation of the
atlas. It is congenital in nature and it is the
most c­ ommon anomaly of the craniovertebral ­junction.15 It can be found isolated but
can also be associated with other anomalies
such as Klippel-Feil syndrome or Chiari I
malformation.14
Additional signs and symptoms
Facial paresis, tongue atrophy or hearing loss.
b Atlantoaxial dislocation
Characterised by instability of C1 (atlas) in relation
to C2 (axis). It occurs in 10–30% of patients with
Down syndrome16,17 and is associated in patients
with rheumatoid arthritis.18 Atlantoaxial dislocation
associated with inflammatory conditions affecting
the retropharyngeal region such as abscesses, osteitis and lymphadenitis is called Grisel syndrome.19
Additional signs and symptoms
Neck pain, sensory deficits and urinary incontinence, changes in gait and hyperreflexia may
occur.
c Basilar impression and basilar
invagination
Both disorders refer to the displacement of the
odontoid process into the foramen m
­ agnum;
the difference is their aetiology: basilar impression is caused by trauma, osteogenesis imperfecta, ­osteomalacia, rickets, rheumatoid
arthritis or Paget’s disease. Basilar i­ nvagination
is c­ ongenital in nature.20,21 Symptoms arise
as the odontoid process projects intracranially ­compressing the ventral aspect of the medulla.
Additional signs and symptoms
A short neck is common.
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d Chiari I malformation
Characterised by herniation of the cerebellar tonsils
caudally through the foramen magnum of more
than 5 mm.
Additional signs and symptoms
Classic presentation symptoms include upper
extremity weakness, sensory loss and pain.
Occipital headaches that are exacerbated by coughing, sneezing, bending over or heavy lifting are
common.22
5. Multiple sclerosis
Multiple sclerosis (MS) is a demyelinating central nervous system disorder of idiopathic origin
(likely autoimmune) that most commonly presents
between the ages of 20 and 40. Women are affected
twice as often as men.
Multiple sclerosis presents in 85% of patients with
an exacerbation or flare of disseminated neurological symptoms and signs that manifest in an
alternating manner over days to weeks. Vertigo is
the initial symptom of MS in approximately five per
cent of patients but 30–50% of patients eventually
develop vertigo throughout life23. However, benign
paroxysmal positional vertigo (BPPV) remains the
most common cause of vertigo in these patients, as
in the general population.24
Diagnosis
The cornerstone for diagnosis of MS remains a full
neurological history and physical examination. The
diagnosis can be aided with laboratory diagnostic
studies including cerebrospinal fluid (CSF) analysis
and neuroimaging.25 The preferred imaging modality for diagnosis and longitudinal follow-up is MRI.26
MRI findings in MS
MRI shows areas of
• Gadolinium-enhanced
brain inflammation, demyelination and loss of
axons.
lesions or plaques are focal and
• Characteristic
discrete, ovoid in appearance and are oriented
•
perpendicularly to the plane of the lateral
ventricles.
Plaques are typically located in the periventricular white matter, in or near the corpus
callosum, the deep white matter as well as deep
and cortical grey matter.
Treatment
These include disease-modifying and symptomatic
directed therapies to slow the progression of the
disease. The goal of therapy is to control symptoms and help maintain a normal quality of life.
Treatment of MS should be oriented toward these
basic goals:
or decrease in severity of symptoms;
•• relief
decrease the duration and frequency of an
acute exacerbation or relapse; and
• preventing disability progression.
The Food and Drug Administration (FDA) has
approved modifying-disease agents for MS and
patients should be referred to a neurologist
­familiar with the management and treatment
of MS.
6. Cerebellar ataxia
syndromes
Friedreich’s ataxia
This is an inherited autosomal recessive neurodegenerative disease caused by insufficient expression
of frataxin (FXN). The nervous system and heart are
the most severely affected organs.27 The underlying
pathology is axonal sensory neuropathy caused by an
unstable trinucleotide (GAA) repeat expansion in the
frataxin gene. Features include symptoms before 20
years of age, areflexia, dysarthria, positive Babinski
sign, propioceptive and vibratory sensory loss, scoliosis and diabetes.
Paraneoplastic cerebellar degeneration
(PCD)
Patients with undiagnosed or asymptomatic malignancies may develop paraneoplastic ­cerebellar
degeneration (PCD), where the c­ erebellum is
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not directly affected by tumour, but an immune-­
mediated response secondary to the tumour affects
the cerebellum. Small cell lung tumour, breast,
ovary and Hodgkin’s lymphoma are the most common tumours c­ ausing PCD. Neurologic symptoms
usually appear before the tumour is identified.
Symptoms appear rapidly and progress over
months and they may include: nausea, vomiting,
gait disturbances (ataxia), cerebellar-type oculor
motor symptoms (nystagmus, diplopia), dysarthria
and dysphagia.
Familial episodic ataxia
This is a rare, dominantly inherited disease characterised by episodes of ataxia and vertigo of early
onset. Episodes of ataxia are usually triggered by
stress, exercise and alcohol. Most patients have
completely normal cerebellar function between
episodes. Acetazolamide, a carbonic anhydrase
inhibitor, is the treatment of choice.3,28
7. Focal seizure disorder
Epileptic seizures that present as vertigo have been
attributed to epileptic foci surrounding the superior
temporal gyrus and the temporoparietal cortex.29,30
Vertigo can be part of an aura, but isolated vertigo
is rare in a seizure disorder.
8. Normal-pressure
hydrocephalus
Classical triad of idiopathic normal-pressure
hydrocephalus:
•• Dementia.
Urinary incontinence.
• Gait or balance disturbance.
Some patients may present with mild balance
difficulties associated with cognitive impairment.
New evidence-based guidelines have been developed for diagnosing normal-pressure hydrocephalus into probable, possible and unlikely
categories. 31 The diagnosis requires evidence from
the clinical history, physical exam and imaging (MRI) showing non-obstructive ventricular
enlargement disproportionate to the degree of
cerebellar atrophy.10
9. Psychiatric dizziness
This is dizziness that occurs exclusively as part of a
recognised psychiatric symptom cluster is not itself
related to vestibular dysfunction.32 Among psychiatric disorders, panic disorder is the only one in
which dizziness constitutes an important defining
characteristic (note Chapter 18). Other psychiatric
disorders include anxiety disorders such as generalised anxiety disorders, personality disorders such
as conversion disorder and depression.
Phobic postural vertigo
Is a syndrome characterised by a persistent sense of
unsteadiness or postural imbalance, and r­ ecurrent
dizziness.33,34 Phobic ­postural vertigo (PPV) disorder often begins after a major life event or stressor
but can develop f­ ollowing a vestibular disorder.
10. Toxins and medications
An excess of toxins may also induce imbalance
including alcohol, copper, mercury, talium, lead,
organic solvents.
Dizziness is a side-effect of many drugs including phenytoin, barbiturates, primidone,
­carbamazepine, 5-FU, methotrexate, piperazine,
lithium.17
References
1 Baloh RW. Clinical practice. Vestibular neuritis.
N Engl J Med. 2003;348:1027–1032.
2 Hotson JR, Baloh RW. Acute vestibular
­syndrome. N Engl J Med. 1998;339:680–685.
3 Baloh RW, Jen JC. Genetics of familial episodic vertigo and ataxia. Ann N Y Acad Sci.
2002;956:338–345.
4 Savitz SI, Caplan LR. Vertebrobasilar disease.
N Engl J Med. 2005;352:2618–2626.
5 Williams D, Wilson TG. The diagnosis of the
major and minor syndromes of basilar insufficiency. Brain. 1962;85:741–774.
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6 Fisher CM, Karnes WE, Kubik CS. Lateral
7
8
9
10
11
12
13
14
15
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18
medullary infarction – the pattern of
vascular occlusion. J Neuropathol Exp
Neurol. 1961;20:323–379.
Benglis D, Levi AD. Neurologic findings of
craniovertebral junction disease. Neurosurgery.
2010;66:13–21.
Cummings CW. Cummings Otolaryngology
Head And Neck Surgery. Philadelphia, PA:
Elsevier Mosby; 2005.
Oas JG, Baloh RW. Vertigo and the anterior
inferior cerebellar artery syndrome. Neurology.
1992;42:2274–2279.
Duncan GW, Parker SW, Fisher CM. Acute
cerebellar infarction in the PICA territory. Arch
Neurol. 1975;32:364–368.
Grad A, Baloh RW. Vertigo of vascular origin.
Clinical and electronystagmographic features in
84 cases. Arch Neurol. 1989;46:281–284.
Johkura K. Central paroxysmal positional ­vertigo: Isolated dizziness caused
by small cerebellar hemorrhage. Stroke.
2007;38:e26–27;author reply e28.
Galm R, Rittmeister M, Schmitt E. Vertigo in
patients with cervical spine dysfunction. Eur
Spine J. 1998;7:55–58.
Klimo P Jr., Rao G, Brockmeyer D. Congenital
anomalies of the cervical spine. Neurosurg Clin
N Am. 2007;18:463–478.
Karatas M. Central vertigo and dizziness:
Epidemiology, differential ­diagnosis, and common causes. Neurologist. 2008;14:355–364.
Taggard DA, Menezes AH, Ryken TC.
Instability of the craniovertebral junction and
treatment outcomes in patients with Down’s
syndrome. Neurosurg Focus. 1999;6:e3.
Ferguson RL, Putney ME, Allen BL, Jr.
Comparison of neurologic deficits with atlantodens intervals in patients with Down syndrome.
J Spinal Disord. 1997;10:246–252.
Clarke MJ, Cohen-Gadol AA, Ebersold
MJ, Cabanela ME. Long-term incidence of
subaxial cervical spine instability following cervical arthrodesis surgery in patients
with rheumatoid arthritis. Surg Neurol.
2006;66:136–140;­discussion 140.
19 Deichmueller CM, Welkoborsky HJ. Grisel’s
20
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syndrome–a rare complication following “small” operations and infections in the
ENT region. Eur Arch Otorhinolaryngol.
2010;267:1467–1473.
Rawal RB, Shah RN, Zanation AM. Endonasal
odontoidectomy for basilar impression and
brainstem compression due to radiation fibrosis. Laryngoscope. 2013;123:584–587.
Smith JS, Shaffrey CI, Abel MF, Menezes
AH. Basilar invagination. Neurosurgery.
2010;66:39–47.
Dyste GN, Menezes AH, VanGilder JC.
Symptomatic Chiari malformations. An analysis
of presentation, management, and long-term
outcome. J Neurosurg. 1989;71:159–168.
Noffsinger D, Olsen WO, Carhart R, Hart CW,
Sahgal V. Auditory and vestibular aberrations
in multiple sclerosis. Acta Otolaryngol Suppl.
1972;303:1–63.
Frohman EM, Kramer PD, Dewey RB, Kramer
L, Frohman TC. Benign paroxysmal ­positioning
vertigo in multiple sclerosis: diagnosis,
­pathophysiology and therapeutic techniques.
Mult Scler. 2003;9:250–255.
Polman CH, Reingold SC, Edan G, et al.
Diagnostic criteria for multiple sclerosis: 2005
revisions to the “McDonald Criteria”. Ann
Neurol. 2005;58:840–846.
Traboulsee AL, Li DK. The role of MRI in the
diagnosis of multiple sclerosis. Adv Neurol.
2006;98:125–146.
Xia H, Cao Y, Dai X, et al. Novel frataxin
isoforms may contribute to the pathological
mechanism of Friedreich ataxia. PloS One.
2012;7:e47847.
Jen J. Familial episodic ataxias and related ion
channel disorders. Curr Treat Options Neurol.
2000;2:429–431.
Hewett R, Guye M, Gavaret M, Bartolomei F.
Benign temporo-parieto-occipital ­junction
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31 Relkin N, Marmarou A, Klinge P, Bergsneider
M, Black PM. Diagnosing ­idiopathic
­normal-pressure hydrocephalus. Neurosurgery.
2005;57:S4–16;discussion ii–v.
32 Furman JM, Jacob RG. Psychiatric dizziness.
Neurology. 1997;48:1161–1166.
33 Brandt T. Phobic postural vertigo. Neurology.
1996;46:1515–1519.
34 Huppert D, Strupp M, Rettinger N, Hecht J,
Brandt T. Phobic postural vertigo–a ­long-term
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J Neurol. 2005;252:564–569.
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16
Systemic conditions
affecting balance
Stephen Broomfield
Contents
Introduction
Congenital disorders affecting balance
Immune disorders
Primary autoimmune inner ear disease
Generalised autoimmune conditions
Cogan’s syndrome
Central disorders
Ménière’s disease
Medications as a cause of vertigo
Aminoglycosides
Cancer chemotheraphy
Antimalarial agents
Otoprotection
Bilateral vestibular hypofunction
References
125
126
126
126
127
128
128
128
128
128
131
131
131
131
132
Introduction
The physiological control of balance is undoubtedly
complex, relying on accurate central ­coordination
of inputs from a number of ­peripheral systems.
It is therefore u
­ nsurprising that almost any
disease process can include ­imbalance (‘dizziness’, ‘presyncope’ or ‘­lightheadedness’) amongst
its presenting ­symptoms. Similarly, ‘­dizziness’
is one of the most commonly occurring medication side effects, reported in over 350 medications
in the British National Formulary.1 True vertigo,
though less common, may also be present in
a number of s­ ystemic i­ llnesses, summarised in
Table 16.1.
This chapter will highlight some of the systemic
conditions and medications that are associated with
vertigo. Approximately 90 medications list vertigo
or ototoxicity as side effects; these are summarised
in a table later in this chapter.
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Table 16.1. Systemic causes of vertigo.
Congenital/Perinatal
Labyrinthine malformation
Down, Usher, Waardenburg, Alstrom, Refsum, Alport, CHARGE, Arnold Chiari,
Goldenhar, Klippel-Feil, Treacher-Collins
Large vestibular aqueduct
Pendred, branchio-oto-renal
Infective
Rubella, cytomegalovirus, syphilis
Other
Hereditary ataxias, non-syndromic genetic disorders (e.g. DFNA9)
Acquired
Neurological
Vestibular migraine, multiple sclerosis, vestibular epilepsy, tumours,
psychological
Infective
Bacterial/viral labyrinthitis, meningitis, syphilis, cytomegalovirus, mumps,
herpes zoster oticus, Lyme disease
Haematological
Anaemia, malignancies (leukaemia, lymphoma)
Cardiovascular
Ischaemia of labyrinth/central pathways, hypotension, arrythmias
Metabolic
Diabetes mellitus
Autoimmune
Autoimmune inner ear disease
Immune-mediated systemic conditions
Drugs
Ototoxic agents
Alcohol
CHARGE – Coloboma, Heart defects, choanal Atresia, Growth retardation, Ear abnormalities.
Congenital disorders affecting balance
Congenital syndromes associated with labyrinthine
malformations resulting in balance disorders are
often associated with hearing loss. Infants with
hearing loss presenting with imbalance (which may
manifest as delayed motor milestones) should be
referred for o
­ phthalmological assessment to exclude
Usher syndrome. Enlarged vestibular aqueduct
can occur spontaneously or as part of a syndrome
(e.g. Pendred syndrome), and is associated with
­vestibular symptoms in one third of cases. These
range from generalised imbalance to episodic
vertigo. Non-syndromic hearing loss may also be
associated with vestibular impairment.
Immune disorders
There is good evidence that immune disorders
can affect the function of the vestibular system,
either as a disease process primarily affecting the
labyrinth, or as part of a generalised autoimmune
condition.
Primary autoimmune inner ear
disease
Autoimmune Inner Ear Disease (AIED), also known
as immune-mediated inner ear disease (IMIED)
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was first described as a case series by McCabe in
1979.2 Patients present with a rapid-onset bilateral,
often asynchronous and asymmetric, sensorineural hearing loss (SNHL) that fluctuates or becomes
progressive. Vestibular symptoms, including generalised imbalance, episodic or positional vertigo,
and ataxia, are also present in up to 50% of cases.3,4,5
AIED is more common in middle-aged adults,
with a 2:1 female predilection.6 A diverse range of
antigenic targets exist within the inner ear, meaning that a single diagnostic test for AIED has proved
elusive.7
Diagnosis depends upon the clinical findings
and a response to steroid therapy, the mainstay
of treatment. The mode of action of corticosteroids in the ear is thought to be through the
mineralocorticoid effect on regulation of inner
ear electrolyte balance, as well as the better recognised anti-inflammatory and immunosuppressive
glucocorticoid effects.8,9,10 Most authors recommend a dose of 1 mg/kg of prednisolone, starting
as soon as possible after onset of symptoms, with
up to four weeks of treatment required to see a
response prior to tapering down to a maintenance
dose.11,12 Such regimes report a response rate of
up to 70%, though long term hearing outcomes
remain poor.13 Recently, there has been interest in
the use of intratympanic administration of steroids
to prevent systemic complications, though reports
are limited to small series.7 Those who respond
to systemic steroid treatment but develop side
effects, or who require a high maintenance dose
to preserve hearing, are considered for immune
modulating treatment. A ­double-blind randomised
controlled trial has shown methotrexate to be no
superior to placebo for treating hearing loss in
AIED, though it may offer some improvement in
vestibular symptoms.14,15 Other medications used in
AIED include etanercept, azathioprine, intravenous
immunoglobulins, and cyclophosphamide, though
mixed results and high toxicity have prevented
routine use of these agents.7,16,17 More recently, the
use of m
­ onoclonal antibodies, such as rituximab,
­infliximab, given systemically or by intratympanic
injection, has shown some promise, though further
research is required.18,19
Generalised autoimmune
conditions
A variety of autoimmune conditions are associated
with immune-mediated inner ear disease, and are
found in approximately one third of patients with
a progressive sensorineural hearing loss (SNHL).20
These conditions (listed in Table 16.2) consist of primary forms of vasculitis such as Cogan’s syndrome,
Table 16.2. Systemic conditions associated with
immune-mediated inner ear disease.
Key features
Antiphospholipid
syndrome
Increased risk of
thromboembolism,
spontaneous miscarriage
Behcet’s disease
Oro-genital ulceration, uveitis
Cogan’s syndrome
Ocular inflammation
Crohn’s disease
Gastrointestinal tract
Goodpasture
syndrome
Haemoptysis, haematuria
Ménière’s disease/
syndrome
Fluctuating sensorineural
hearing loss
Multiple sclerosis
Visual change, fatigue
Myaesthenia gravis Muscle weakness
Polyarteritis nodosa Renal, cardiac, skin
Relapsing
polychondritis
Cartilage – ear, nose,
trachea
Rheumatoid arthritis Joint swelling and stiffness
Sarcoidosis
Erythema nodosum, lupus
pernio
Sjogren’s syndrome Dry eyes, mouth
Systemic lupus
erythematosus
Butterfly rash, malaise,
arthralgia
Systemic sclerosis
Calcinosis, Reynaud’s
phenomenum
Thyroiditis
Fatigue, weight gain, anxiety
Vogt-KoyanagiHarada disease
Meningeal irritation,
alopecia, vitiligo, uveitis
Wegener’s
granulomatosis
Renal failure, arthritis,
neuropathy
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polyarteritis nodosa, and systemic autoimmune
conditions including systemic lupus erythematosus.
Cogan’s syndrome
Cogan’s syndrome, named after the ophthalmologist David Cogan in 1945, is a rare condition
causing inner ear impairment with ocular inflammation. This is usually interstitial keratitis, but in
atypical forms may include conjunctivitis, scleritis,
uveitis, or retinal vasculitis.21 Audiovestibular
symptoms usually occur within two months of the
ocular symptoms, the commonest pattern being a
Ménière-like presentation with tinnitus and vertigo
followed by a progressive SNHL. Systemic symptoms are also common, the most significant due to
cardiovascular effects. Although Cogan’s syndrome
is categorised as autoimmune, no target antigen has
been identified, and the exact pathogenesis remains
unknown.22 Treatment is similar to that of AIED.
Response to systemic steroids is commoner for the
ocular than the audiovestibular symptoms, with
50% of cases developing profound deafness.23
Central disorders
It has been postulated that several n
­ on-infective central nervous system disorders may have an immunemediated aetiology. These include d
­ emyelinating
conditions, autoimmune ­encephalitis,
paraneoplastic encephalomyelitis, Susac’s syndrome,
and chronic hypertrophic ­pachymeningitis.24
The commonest is multiple sclerosis (MS), thought
to be an autoimmune condition occurring in those
with a genetic ­predisposition, triggered by unknown
environmental factors. The disease is characterised
by plaques of demyelination throughout the central
nervous system, including the vestibular nuclei.
Vertigo in MS may be spontaneous, positional, or
postural. Classically MS starts with a relapsingremitting phase, often followed by a secondary
progressive neurodegenerative phase. A primary
progressive type, without remissions, may also
occur. In most cases there are other signs and symptoms of central neurological deficits.
Ménière’s disease
Whilst the aetiology of Ménière’s disease remains
unknown, there is considerable experimental
evidence that autoimmunity may be responsible in
up to 6% of unilateral and 16% of bilateral cases.24,25
This has led to an increasing interest in the use of
systemic and intratympanic steroid treatment for
Ménière’s disease. In addition, a­ utoimmunity may be
responsible for contralateral delayed endolymphatic
hydrops, in which hydropic ­symptoms develop in
the ear opposite to one with longstanding sensorineural deafness or where p
­ revious surgery has been
performed.26 (See Chapter 11 for further details.)
Medications as a cause of vertigo
A wide variety of medications can affect the
­physiological control of balance resulting in dizziness or vertigo as a side effect (Table 16.3). These
symptoms are often reversible on cessation of the
medication. Some medications are well recognised
as being potentially ototoxic, with tinnitus, hearing
loss, and vestibular symptoms occurring frequently.
Risk factors for ototoxic effects of medications
include the dosage and d
­ uration of treatment, as
well as nutritional status, age (very young or old),
renal function, and genetic predisposition. In some
cases, such as loop ­diuretics and salicylates, the
effects are usually reversible. The most important
ototoxic agents that have potential for permanent
cochleovestibular ­toxicity are the aminoglycosides,
cancer chemotherapy and antimalarials.
Aminoglycosides
Aminoglycosides are an effective class of antibiotic
used in gram-negative sepsis and drug-resistant
tuberculosis. Agents include amikacin, gentamicin,
kanamycin, neomycin, netilmicin, streptomycin
and tobramycin. Ototoxic effects are often bilateral,
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Table 16.3. Medications in the British National Formulary listing vertigo or ototoxicity as a side effect
(March 2012).
Indication/Class
Cardiovascular
Central Nervous System
Ocular
Antibiotics
Other anti-infective
agents
Antineoplastic agents
Medication
Hypertension
Olmesartan, Candesartan, Telmisartan,
Losartan, Doxazosin, Enalapril, Lisinopril,
Propanolol, Co-tenidone
Antiplatelet
Clopidogrel, Ticagrelor
Anticoagulants
Fondaparinux
Hyperlipidaemia
Gemfibrozil
Angina
Ivabradine, Nifedipine
Arrythmias
Amiodarone, Flecainide
Analgesic
Opiods, Co-codomal, NSAIDs
Parkinson’s/Related
Rasigiline, Riluzole, Piracetam, Botox
Migraine
Frovatriptan, Rizatriptan
Multiple sclerosis
Cannabis extract
Antiemetic
Nabilone
Narcolepsy
Sodium oxybate
Epilepsy
Gabapentin, Retigabine
Antidepressants
Venlafaxine
Anxiolytics
Diazepam, Midazolam
Macular degeneration
Pegaptanib
Glaucoma
Brinzolamide
Aminoglycoside
Gentamicin
Tetracyclines
Minocycline
Polymyxin
Colistin
Combination
Co-trimoxazole
Other
Teicoplanin
Tuberculosis
Capreomycin, Cycloserine, Isoniazid
Threadworm
Piperazine
Protozoal infection
Sodium stibogluconate
Human immune virus
Enfuvirtide
Malaria
Quinine, Mefloquine
Fungal infection
Flucytosine
Malignant disease
Catumaxomab, Canakinumab
Leukaemia
Nilotinib, Imatinib
(Continued)
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Table 16.3. (Continued) Medications in the British National Formulary listing vertigo or ototoxicity as
a side effect (March 2012).
Indication/Class
Medication
Osteosarcoma
Mifamurtide
Prostate cancer
Cabazitaxel
Rheumatoid arthritis
Abatacept
Gout
Inosine, Allopurinol
Respiratory
Severe COPD
Roflumilast
Endocrine
Various indications
Glucocorticoids
Vitamin D deficiency
Ergocalciferol
Osteoporosis
Teriparatide
Diabetes insipidus
Vasopressin
Diabetes mellitus
Pioglitazone
Endometriosis
Danazol
Diuretics
Furosemide
Urinary frequency
Flavoxate, Tolterodine, Fesoterodine
Urinary incontinence
Duloxetine
Hyperphosphataemia
Lanthanum
Erectile dysfunction
Sildenafil, Alprostadil
Urticaria
Bilastine
Angioedema
Conestat alfa
Vaccines
Japanese encephalitis vaccine
Idiopathic
thrombocytopaenic
purpura
Eltrombopag
General anaesthesia
Propofol
Antirheumatic agents
Renal/Genitourinary
system
Antiallergens
Other
NSAIDs – non-steroidal anti-inflammatory drugs; COPD – chronic obstructive pulmonary disease.
and usually occur within days or weeks of systemic
administration. Effects from topical administration
and absorption into the inner ear are more immediate, a fact which is utilised in the ablation of vestibular function in the treatment of Ménière’s disease.
The relative toxicity of different aminoglycosides
remains controversial, and all are considered
­ototoxic. Gentamicin and tobramycin may be
­principally vestibulotoxic, and neomycin, kanamycin and amakacin more cochleotoxic.27 Unlike renal
toxicity, ototoxicity is irreversible. Overall, s­ ystemic
administration of aminoglycosides leads to vestibulotoxicity and cochleotoxicity in up to 15% and 25%
of patients, respectively.28 The incidence is known to
be higher in developing countries, where aminoglycosides are used more commonly, nutritional
status is generally poorer, and monitoring of serum
drug levels is less available.28 Co-administration
of loop diuretics (furosemide or ethacrynic acid)
potentiates the ototoxicity of aminoglycosides by
increasing uptake into the inner ear. Patients with
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the 1555 mutation of mitochondrial ribosomal
ribonucleic acid (RNA) have increased susceptibility to cochleotoxicity, but not vestibulotoxicity; this
mutation is estimated to have an incidence of 0.5%
in a European population.29 Genetic screening is
not used routinely in those receiving aminoglycosides, but relevant family h
­ istory should be sought.
The inner ear damage from aminoglycosides is
mediated initially by uptake into the outer and
inner hair cells of the cochlea (ototoxic effects),
and the type 1 and type 2 cells of the vestibular
ampullae (vestibulotoxic effects).28 Inside the cells,
the agents disrupt stereocilia and lead to apoptosis
through increased formation of reactive oxygen
species and free radicals.30
Cancer chemotheraphy
The platinum-containing chemotherapeutic agents
cisplatin and carboplatin are commonly used in
the treatment of solid tumours in both adults and
children, and are well recognised as being ­ototoxic.
The mechanism of action is similar to that of
­a minoglycosides, as production of free radicals,
and promotes cellular apoptosis. The cochlea is primarily affected, outer hair cells being affected first
followed by inner hair cell loss, starting at the basal
end of the cochlea and causing a high frequency
hearing loss (a so-called ‘cochleotopic gradient’
pattern of injury). Hearing loss in patients receiving cisplatin is common, occurring in more than
60% of children, vestibular effects are uncommon.31,32 There is a wide variation in the susceptibility of patients to cisplatin ototoxicity, and work
is ongoing to identify a genetic basis for this.33
Antimalarial agents
The association between the antimalarial agent
quinine and ototoxicity is well established. More
modern antimalarial agents such as chloroquine
and hydroxychloroquine, may also cause ototoxicity,
with audiovestibular symptoms in 2.6% of cases in
one large study.34 This is relevant given the increasing use of antimalarials in travellers as well as the
expansion of their use in systemic connective tissue
diseases, including rheumatoid arthritis, Sjogren’s
syndrome, systemic lupus erythematosus.34
Otoprotection
The fact that outer hair cell function is affected early
in the course of the ototoxic effects has led to the
development of monitoring protocols using high
frequency audiometry or otoacoustic emissions in
patients receiving cisplatin. Patients at increased risk
of ototoxicity, young children, and those reporting
any cochleovestibular symptoms, are monitored particularly carefully. There is some evidence that cessation of cisplatin therapy or adjustment of the dose
can prevent progression of the toxic effect. The toxic
effects of aminoglycosides are irreversible, and monitoring of serum concentrations is recommended.
Routine use of hearing monitoring in patients
undergoing long-term treatment is also advisable.
Vestibular monitoring is not routinely used.
An improved understanding of the molecular actions
of ototoxic agents has led to a series of potential routes
for protecting against their harmful effects. These
include the inhibition of a­ poptosis with neurotrophins and growth factors, and neutralisation of reactive
oxygen species using iron chelators and free radical
scavengers.30 Although showing potential in in-vitro
and animal studies, the long-term clinical safety and
efficacy of these agents is not proven, though use of
acetylsalicylates is particularly promising.35 Other
potential targets for otoprotection include prevention
of uptake of ototoxic agents into hair cells and the
use of gene therapy to counteract the interaction with
­ribosomal ribonucleic acid (RNA).
Bilateral vestibular hypofunction
Bilateral loss of vestibular function is rare, and
the aetiology remains unknown in half of all
cases. Patients present with oscillopsia, blurred
vision during head movements, gait disturbances
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and problems with navigation. In those cases
with known aetiology, it is unsurprising that
systemic diseases are common, most frequently
­ototoxicity, Ménière’s disease, autoimmune inner
ear disease or Cogan’s syndrome, and meningitis.
A subgroup have congenital malformations or
familial vestibulopathy. Bilateral loss of vestibular
function is an interesting area of research, as it
provides a platform for investigation of local gene
delivery to the inner ear and targeted molecular
therapy as an adjunct to cochlear implantation. 36
Work is also underway to create an effective
­vestibular implant to restore function to those
with bilateral impairment.
References
1 BNF No.63 British National Formulary (2012
March). London: BMJ Publishing Group; 2012.
2 McCabe BF. Autoimmune sensorineural
hearing loss. Ann Otol Rhinol Laryngol.
1979;88(5):585–589.
3 Hughes GB, Kinney SE, Barna BP, Calabrese LH.
Practical versus theoretical management of
autoimmune inner ear disease. Laryngoscope.
1984;94(6):758–767.
4 Rauch SD. Clinical management of immunemediated inner-ear disease. Ann N Y Acad Sci.
1997;830:203–210.
5 Bovo R, Aimoni C, Martini A. Immunemediated inner ear disease. Acta Otolaryngologica. 2006;126:1012–1021.
6 Stone JH, Francis HW. Immune-mediated
inner ear disease. Curr Opin Rheumatol.
2000;12:32–40.
7 Buniel MC, Geelan-Hansen K, Weber PC,
Tuohy VK. Immunosuppressive therapy for
autoimmune inner ear disease. Immunotherapy.
2009;1:425–434.
8 Rupprecht R, Reul JM, van Steensel B, et al.
Pharmacological and functional characterization of human mineralocorticoid and glucocorticoid receptor ligands. Eur J Pharmacol.
1993;247(2):145–154.
9 Trune DR, Kempton JB, Kessi M. Aldosterone
(mineralocorticoid) equivalent to prednisolone (glucocorticoid) in reversing hearing
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loss in MRL/MpJ-Fas1pr autoimmune mice.
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Trune DR, Kempton JB, Gross ND.
Mineralocorticoid receptor mediates glucocorticoid treatment effects in the autoimmune
mouse ear. Hear Res. 2006;212:22–32.
Ruckenstein MJ. Autoimmune inner ear
­disease. Curr Opin Otolaryngol Head Neck Surg.
2004;12(5):426–430.
Alexander TH, Weisman MH, Derebery
JM, et al. Safety of high-dose corticosteroids for
the treatment of autoimmune inner ear disease.
Otol Neurotol. 2009;30(4):443–448.
Broughton SS, Meyerhoff WE, Cohen SB.
Immune-mediated inner ear disease:
10-year experience. Semin Arthritis Rheum.
2004;34(2):544–548.
Harris JP, Weisman MH, Derebery JM,
et al. Treatment of corticosteroid responsive
autoimmune inner ear disease with methotrexate: A randomized controlled trial. JAMA.
2003;290(14):1875–1883.
Garcia-Berrocal JR, Ibanez A, Rodriguez A,
et al. Alternatives to systemic steroid therapy
for refractory immune-mediated inner ear
disease: A physiopathologic approach. Eur Arch
Otorhinolaryngol. 2006;263:977–982.
Matteson EL, Choi HK, Poe DS, et al.
Etanercept therapy for immune-mediated
cochleovestibular disorders: A multi-center,
open-label, pilot study. Arthritis Rheum.
2005;53:337–342.
Cohen S, Shoup A, Weisman MH, Harris
J. Etanercept treatment for ­autoimmune
inner ear disease: Results of a pilot
­placebo-controlled study. Otol Neurotol.
2005;26:903–907.
Cohen S, Roland P, Shoup A, et al. A pilot
study of rituximab in immune-­mediated
inner ear disease. Audiol Neurootol.
2011;16:214–221.
Van Wijk F, Staecker H, Keithley E,
Lefebvre PP. Local perfusion of the tumor
necrosis factor α-blocker infliximab to
the inner ear improves autoimmune
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2006;11(6):357–365.
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Haynes BF, Kaiser-Kupfer MI, Mason P,
et al. Cogan syndrome: studies in thirteen
patients, long-term follow-up and a review of
the literature. Medicine. 1980;59:426–441.
Murphy G, O’Sullivan M, Shanahan F,
Harney S, Molloy M. Cogan’s syndrome:
Present and future directions. Rheumatol Int.
2009;29:1117–1121.
Grasland A, Pouchot J, Hachulla E, et al.
Typical and atypical Cogan’s syndrome: 32 cases
and review of the literature. Rheumatology. 2004
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Bovo R, Ciorba A, Martini A. Vertigo and
autoimmunity. Eur Arch Otorhinolaryngol.
2010;267:13–19.
Yoo TJ, Sener O, Kwon SS, et al. Presence of
autoantibodies in the sera of Ménière’s disease.
Ann Otol Rhinol Laryngol. 2001;110:425–429.
Suzuki M, Hanamitsu M, Kitanishi T,
Kohzaki H, Kitano H. Autoantibodies in
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Matz GJ. Aminoglycoside cochlear ototoxicity. Otolaryngol Clin North Am.
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Forge A, Schacht J. Aminoglycoside
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Scrimshaw BJ, Faed JM, Tate WP, Yun K.
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Huth ME, Ricci AJ, Cheng AG.
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Brock PR, Knight KR, Freyer DR, et al.
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­predisposition, and protection, ­including
a new international society of pediatric
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Nakayama M, Riggs LC, Matz GJ.
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by gentamicin or cisplatin in guinea pig.
Laryngoscope. 1996;106:162–167.
Muckerjea D, Ryback LP.
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2011;12(7):1039–1050.
Jourde-Chiche N, Mancini J, Dagher N,
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17
Vestibular rehabilitation
– principles and practice
Rachel Ritchie
Contents
Introduction
Indications
Exercises and regimens
Outcomes
Case study
Conclusion
The future of vestibular rehabilitation
References
135
136
138
140
140
143
143
144
Introduction
Vestibular rehabilitation is an exercise based treatment program commonly used in the treatment
of patients presenting with dizziness and vertigo.
Over 70% of patients referred to our tertiary referral centre were considered to require vestibular
rehabilitation therapy.1,2
Vestibular rehabilitation exploits our understanding of the balance system, and allows a therapist
to formulate a unique patient-specific package of
exercises. The fundamental principles of vestibular rehabilitation requires an understanding of
postural control; the capacity to maintain one’s
relatively high centre of mass over a small base of
support in response to changes in self and environmental movement.
In order to plan rehabilitation, vestibular pathology
should be considered in terms of the wider balance
system. This systems approach considers sensory
inputs, central processing pathways and desired
motor output.
This chapter aims to give an overview of the principles and practice of vestibular rehabilitation, and
considers why some patients fail to fully compensate and how they can be treated with targeted
customised vestibular rehabilitation.
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INDICATIONS
Vestibular rehabilitation by means of exercised
based activities is well established for patients with
an acute peripheral vestibular loss.4 These promote
compensation via central nervous system plasticity. Early protocols for vestibular rehabilitation
used exercises of increasing hierarchical difficulty.5
These were later condensed into a generic list of
exercises that could be handed to the dizzy patient
in clinic. In young, otherwise healthy patients,
such exercises may be sufficient to initiate compensation and lead to the resolution of symptoms.
However, without therapist support, customisation
is not possible and compliance may be very poor.
Customised rehabilitation takes into account concomitant central and peripheral pathology, as well
as motor output and musculoskeletal limitations
(Figure 17.1).
Other factors that limit compensation are
long-term vestibular suppressant use, and the
psychological burden associated with dizziness
and balance (Chapter 18). Current principles of a
systems approach to balance rehabilitation6 and the
use of customised vestibular rehabilitation has been
shown to be more effective than generic exercise
advice alone.7,8
Symptoms vary widely for any given medical
diagnosis. Understanding the underlying cause
of their symptoms remains important in order
that patients can be reassured that vestibular
rehabilitation is an appropriate course of action.
Common sequelae of a vestibular dysfunction that
may indicate the need for vestibular rehabilitation
might include:
motion sensitivity;
•• self
space and motion sensitivity (visual vertigo);
instability;
•• gaze
postural instability;
SENSORY INPUTS AND PROCESSING
CENTRAL
Determination of Body
Position
Choice of
Movement
Information Processing:
Compare, select and
combine senses
Timing, sequencing,
error correction
Cerebellar Lesion
Somatosensation
Vision
PERIPHERAL
MOTOR PLANNING AND EXECUTION
Small vessel disease
TIA/Stroke
Parkinson’s Disease
MS
Psychological Trait
Myopia/
Cataracts/
diabetic
retinopathy
Vestibular
Peripheral
Neuropathy/
OA
Cerebellar Lesion
Cerebellar/
Brainstem/Cranial
Nerve/Spial Lesion
Unilateral/
Bilateral PVD
Environmental Interaction
Discordant Environmental
Stimluli
Spinal Lesion
Cervical
Muscles
Ocular
Muscles
Trunk and limb
muscles
Latent
squint
Pain/Weakness/Stiffness from
eg Peripheral Neuropathy/
Sarcopaenia/Disuse Atrophy/OA
Generation of
Movement
Figure 17.1. System model of balance control with examples of common challenges to balance (adapted
from Allison, 20013).
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and injuries resulting from these, dual
associated symptoms of panic
• falls
• psychological
task interference;
and behavioural adaptation;
confidence in balance;
(irrational thoughts that a
•• reduced
• catastrophisation
musculoskeletal strain/stiffness/disuse atrophy,
situation is worse than it actually is);
particularly cervical spine movement;
• daily activity limitation.
sarcopenia (loss of muscle mass
• accelerated
with ageing) in sedentary older adults;
Additional subjective tools that may be used to
assess patients are listed in Table 17.1.
• loss of cardio-respiratory fitness;
Table 17.1. Clinically available assessment tools to generate understanding of postural stability problems
for rehabilitation.
Problems assessed
Space and motion sensitivity
Daily function/activity
limitation
Assessment tool
Limitations
Situational vertigo questionnaire
Self reported score only
Visual analogue score for vertigo
in response to visual stimuli
Hard to replicate visual stimuli of reality
in clinical environment
Dizziness handicap inventory
Reliability dependent on accurate
description of use
Self motion sensitivity
Impact of timing use of questionnaire
with variable symptoms
Reduced confidence in
balance
Activities-specific balance
confidence scale/confidence in
maintaining balance
questionnaire
Self reported score only
Psychological associated
symptoms
Hospital anxiety and depression
score
Self reported score only
Catastrophisation
The Beck depression inventory
Gaze instability
Static versus dynamic visual acuity
Multiple systems interacting in control of
gaze stability, difficult to isolate and
standardise in clinical tests
Tests acuity at single speed of head
movement in isolated plane only, hard
to replicate challenge of reality
Muscle weakness
Manual muscle testing
Relies on subject motivation/adherence
Medical Research Council
sum-score
Inter-rater variability
Neglects strength endurance
MSK strain/stiffness
Visual analogue score for pain
Hard to replicate movement required in
reality in clinical environment
Range of movement measurement
Joint position sense accuracy
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Table 17.1. (Continued) Clinically available assessment tools to generate understanding of postural
stability problems for rehabilitation.
Problems assessed
Falls risk/Postural instability
and weighting of sensory
dependence
Assessment tool
Limitations
Modified CaTSIB test
Limited assessment of reactive balance
strategies and non-steady state postural
stability
Berg balance score
Hard to replicate postural control
demands required in reality in clinical
environment
Timed up and go
Timed walk
Tinetti gait and balance score
Functional gait analysis score
Lean and release/nudge test
Task interference
Single versus dual task gait speed
Reduced cardio-respiratory
fitness
HR recovery time
Hard to replicate complex task demands
required in reality in clinical
environment
Borg score of perceived exertion
6 minute walk test
CaTSIB – Clinical test of sensory interaction on balance
Exercises and Regimens
A customised programme of exercises is developed from a sound understanding of an individual’s problems and functional limitations.
The patient must also be reassured that moderate
provocation of symptoms during rehabilitation
is expected and counselled that the aim of treatment is to manage symptoms rather than cure the
underlying diagnosis. This enables the patient to
maintain a realistic expectation of outcome, maintain motivation and compliance with vestibular
rehabilitation.
In keeping with the diversity of problems associated with vestibular pathology, the nature, intensity
and duration of vestibular rehabilitation should be
individualised. With sufficient exercise tolerance, a
programme typically lasts between 30 and 60 minute. Initially, weekly therapist supervision allows
for re-evaluation and adjustment to the programme
as well as advice on technique. Expectation is for
the majority of exercises to be carried out independently at home. Ideally the weighting of therapist
involvement lessens over the course of 6–8 weeks
with a view to maximise patient led progression
and long term management should there be any
residual symptoms.
The programme looks to use provocation of symptoms through self or environmental movement to
drive the inherent plasticity within the balance
system, to become accustomed to or desensitised
to movement stimuli (habituation). Rehabilitation
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may also drive changes in the gain of the vestibular-ocular reflex (VOR) by altering the demands
placed upon it. This involves changing the timing,
amplitude and plane of head movements whilst
visually fixating on a target. As an example, an
early exercise may be fixating gaze to a simple
static visual target from a distance of 3m with
small amplitude horizontal head turns at 2.5–3Hz
set by auditory cue, whilst standing still. A later
progression may require fixating gaze with repetitive patterns in the peripheral field of vision with
larger amplitude horizontal head turns increased
to a rate of 3–3.5Hz with concurrent walking forwards and backwards.
Exercises to challenge postural stability can be used
for different purposes in the rehabilitation process. Balance exercises that deprive the individual
of reliable visual or proprioceptive cues aim to
strengthen central processing of asymmetrical vestibular input (for example walking with surrounding motion, such as generated by moving lights to
alter visual input or to maintain balance with feet
together on foam in a visually rich environment to
alter proprioceptive reliability). Conversely ‘substitution’ exercises encourage using intact sensory
pathways in place of those that are abnormal (for
example to stand feet together on a firm floor with
no upper limb support with eyes closed to encourage somatosensory focus or to maintain balance
on uneven flooring with eyes open to encourage
visual bias).
Any physical rehabilitation needs orientation around functional goals to ensure the
often abstract tasks of customised vestibular
rehabilitation look to achieve the ultimate aim of
improving daily symptoms and function. Early
education and counselling support are, therefore,
important adjuncts to the physical aspects of
vestibular rehabilitation. Once established, vestibular rehabilitation programmes must evolve so
that there is a stepwise progression of difficulty to
continuously drive compensation (for example, by
gradually incorporating clinic based exercises into
real life scenarios or to combine exercises such
as those describes for gaze stability whilst challenging postural stability). In practice, exercise
difficulty should be tailored to provoke symptoms of postural instability or dizziness without
becoming intolerable or impossible to complete.
A simple tool that may be used during vestibular rehabilitation is a symptom severity scale
(Table 17.2). A moderate provocation of symptoms during exercises should be encouraged and
reassurance given. Exercises that fail to provoke
symptoms beyond that at rest will fail to drive
compensation. Conversely, exercises that provoke
severe symptoms will fail to support desensitisation and offer a negative experience that may
reinforce negative beliefs and behaviours. By
using our understanding of the systems approach
to postural control, we can guide progression of
exercises and educate the patient to adopt these
principles for long term management. Subtle
changes to a programme in which one or more of
the systems of balance are challenged in a different way can be enough to see considerable change
in success or failure to complete a task and it is
here that the skill of the therapist is difficult to
replicate in the historical use of exercise templates
handed out to patients.
Table 17.2. Symptom provocation during VR exercises,
Bold indicates target level of difficulty.
Symptom Rating Score During VR Exercise
0
Normal level of symptoms at rest
1
Mild provocation of symptoms
2
Moderate provocation of symptoms
3
Severe provocation of symptoms
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OUTCOMES
Time should be invested at the initial physiotherapy
assessment to establish the presence and weighting of each problem. There are numerous widely
available and validated assessment tools that can be
used (see Table 17.1). It is well recognised that balance performance is dependent of other cognitive
or attentional demands.9,10 Our perceived limits of
stability may differ from our actual limit of stability
when there is a threat to our balance. Such threats
may be brought about by sensory conflict or apprehension. Clinically, this means that a person who
has a satisfactory tandem gait in clinic may well
display postural instability should they be asked to
repeat the task in a busy train station. Only through
thorough assessment can it be determined if the
problem is one of visual dependence, space and
motion sensitivity, dual task interference, anxiety,
or a combination of any of these problems. Hence,
determining problems for rehabilitation requires a
variety of assessment techniques, which are interpreted together rather than in isolation.
Overall outcome from vestibular rehabilitation is
dependent on the nature and location of vestibular pathology as well as the status of other aspects
of the balance system, specifically the visual
and proprioceptive pathways, the cerebellum,
spine and motor pathways. In otherwise healthy
adults, prognosis for recovery to previous level
of f­ unction is good. However, with concurrent
pathology affecting any other aspect of the wider
balance system, outcome is less easily predicted
and as such modest goals should be set (note
Table 17.3).
Case study 17.1. Acute peripheral vestibular deficit
33-year-old gentleman, previously well. Three months ago, he suffered severe sudden onset rotatory vertigo, associated with nausea and disequilibrium. Symptoms of vertigo lasted two days
with gradual improvement for ten days thereafter. He now presents with ongoing d
­ isorientation
in busy visual environments, unsteady balance and disorientation preventing return to sports and
loss of clarity of thought by the end of the working day. Clinical examination revealed a positive
left head thrust test with otherwise normal neurological exam. Romberg on foam was positive
at ten seconds with consistent leftward drift. Audio-vestibular testing identified normal hearing
thresholds, normal eye movement exam and a 30% left canal paresis on caloric testing. He was
diagnosed as having a left peripheral vestibular deficit and referred for vestibular rehabilitation.
Physiotherapy assessment revealed problems for rehabilitation as:
1 Gaze instability with significant loss on dynamic versus static visual acuity testing.
2 Space and motion disorientation with a significant Situational Vertigo Score.
3 Complex task interference with significant deterioration in balance performance with
added simple cognitive tasks.
A customised vestibular rehabilitation programme was developed.
Week 1 Exercises
1 Gaze fixation to target with horizontal head turns at 160–180 bpm set by auditory cue,
standing two metres from target standing on firm floor feet apart.
2 Maintain balance with feet together on firm floor and foam in visually rich environment.
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3 Walking with surrounding motion, such as generated by moving lights creating sensory
mismatch.
4 Standing and walking with head turns (60 bpm) on firm floor whilst answering cognitively
challenging questions.
5 Walking heel to toe on foam floor with eyes closed.
6 Football skill practice with added cognitive task.
Week 4 Exercises
1 Gaze fixation to target with horizontal head turns at 180–200 bpm set by auditory cue,
standing two metres from target firm floor with feet together. Repeat with repetitive patterns in
peripheral field of vision. Repeat with walking forwards and backwards to and from target.
2 Maintain balance with feet together on foam in visually rich environment. Repeat with
sharpened Romberg stance position.
3 Walking heel to toe with surrounding motion, such as generated by moving lights creating
sensory mismatch.
4 Walking heel to toe with head turns (60 bpm) on firm floor whilst answering cognitively
challenging questions. Repeat with uneven/foam mat flooring.
5 Walking heel to toe forwards and backwards on thick foam floor with eyes closed.
6 Football skill practice with added cognitive task in visually rich environment.
Progress at half way
Romberg on floor 20–30 seconds. Improvements noted in busy visual environments when
standing still but ongoing symptoms of imbalance when walking and talking.
Week 8 Adaptation Exercises
1 Gaze fixation to target with horizontal head turns at 180–200 bpm set by auditory cue,
walking forwards and backwards to and from target two metres from target firm floor.
Repeat with repetitive patterns in peripheral field of vision. Repeat walking to and from
target on uneven/foam mat flooring.
2 Maintain balance with feet in sharpened Romberg stance on foam position in visually rich
environment. Repeat with secondary upper limb balancing task.
3 Walking heel to toe with surrounding motion, such as generated by moving lights creating
sensory mismatch with cognitively challenging questions.
4 Walking heel to toe with head turns at variable speed and amplitude on firm floor whilst
answering cognitively challenging questions. Repeat with uneven foam mat flooring.
5 Walking heel to toe forwards and backwards on thick foam floor with eyes closed and
cognitive task.
6 Football skill practice with added cognitive task in visually rich environment.
Outcome
Negative Romberg on foam. Significant reduction in situational vertigo score and successful
return to football training. Ongoing exercise programme devised for independent management of symptoms with goal to return to full involvement in sports
Vestibular rehabilitation – principles and practice 141
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Table 17.3. Case studies of vestibular rehabilitation programmes tailored to diagnosis and individual
problems.
Case study
Individual
background
Problems for
vestibular
rehabilitation (VR)
Example
customised
programme
Adjunctive
management
1. Chronic
peripheral
vestibular
disorder
secondary to
Ménière’s
disease
15 year history of
unilateral hearing
loss and tinnitus,
now stable
dizziness. Anxiety
and depression,
no other past
medical history
Gaze instability
Self motion
hypersensitivity
Space and motion
disorientation
Unsteady balance with
proprioceptive or visual
dependence
Self motion
hyper-sensitivity
Neck pain and stiffness
Reduced cardiorespiratory fitness and
muscle de-conditioning
Catastrophisation
Horizontal head turns,
pace set by external
cue with fixation of
gaze two metres from
target in standing on
firm floor
Postural stability
retraining with
sensory mismatch
Postural retraining
with reduced visual
cue availability/
proprioceptive cue
availability
Repetitive motion of
self gradually
progressed in nature,
duration, frequency
and amplitude of
self-motion
Neck range of
movement exercises/
endurance
retraining/
proprioceptive
retraining
Cardio-respiratory
exercises, treadmill
walking, cycling,
aerobic step
exercises
Pharmacological
agents for acute
vertigo and
prophylaxis as
required
Dietary
management
Audiology for
hearing loss.
Cognitive
behavioural
therapy
concurrent with
physical VR
2. Multi-modal
Vestibulopathy
Diabetes with
peripheral
neuropathy and
mild retinopathy,
osteoarthritis with
bilateral total knee
replacements,
history of
presumed transient
ischaemic attacks
1 non-injurious fall
in last 12 months
Joint stiffness and
reduced power around
knees and ankles
Unsteady balance with
proprioceptive and
visual dependence
Poor reactive balance
mechanisms with slow
central processing
Dual task interference
Fear of falls
Falls prevention and
management
programme
Flexibility
Targeted strength
training
Postural stability–
proactive and
reactive re-training
Dual and complex
task practice
Wean from
vestibular
suppressants
(Continued )
142 Dizziness and Vertigo: An Introduction and Practical Guide
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Table 17.3. (Continued) Case studies of vestibular rehabilitation programmes tailored to diagnosis and
individual problems.
Problems for
vestibular
rehabilitation (VR)
Example
customised
programme
Case study
Individual
background
Adjunctive
management
3. Vertiginous
migraine
History of classical
migraine
Kinesiophobia
Space and motion
disorientation
Unsteady balance with
proprioceptive
dependence
Neck pain and stiffness
Global deconditioning
Slow progression of
exposure to visual
stimulus
Postural stability
Self motion activities
Neck ROM and
proprioceptive/
orientation exercises
CR exercises/strength
endurance training
4. Benign
paroxysmal
positional
vertigo
Nil of note
Transient positional
vertigo
Ongoing after particle
repositioning
manoeuvres: Self
motion hyper-sensitivity
Transient unsteady gait
on looking up
Particle repositioning Normally nil
manoeuvres initally
needed
On resolution of
Wean from
BPPV: Repetitive
vestibular
motion of self
suppressants
gradually progressed
in nature, duration,
frequency and
amplitude of self
motion
Dynamic postural
re-training with pitch
plane head
movements
Diet/migraine
prophylaxis
Note: not included are a wide range of possible functional restrictions such as activity and participation
problems. Duration and repetitions tailored to individual symptoms/capability.
Conclusion
Vestibular rehabilitation seeks to address the symptoms of vestibular pathology through a unique
patient-specific package of exercises that drive the
balance system to compensation. Indications for
vestibular rehabilitation vary widely for any given
diagnosis as they may originate directly from the
pathology, such as postural instability, or from
secondary complications such as joint stiffness.
Based on individualised problems, a therapist will
formulate a programme of exercises that typically
require moderate provocation of symptoms. Over
the course of several weeks, there is a stepwise
progression in exercise difficulty to support the
transition from clinic-based exercises to improving
daily function and achieving patient specific goals.
The future of vestibular
rehabilitation
With advances in our understanding of the complexities of human movement, the field of vestibular and balance rehabilitation is rapidly evolving.
Audio-vestibular displays and virtual reality
promise immersive ­technologies are currently
being trialled and are likely to improve vestibular
rehabilitation still further.11,12,13
Vestibular rehabilitation – principles and practice 143
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References
1 Lee A, Jones G, Corcoran J, Premachandra P,
Morrison GAJ. A UK hospital based multidisciplinary balance clinic run by allied health
professionals: First year results. J Laryngol
Otol. 2011;125,661–667. doi:10.1017/
S0022215111000624
2 Corcoran J, Jones G, Ritchie R. The
Guy’s Hospital Balance Clinic: An MDT
Approach. Otolaryngology Head Neck
Surg. 2010;143(2): s107. doi:10.1016/j.
otohns.2010.06.192
3 Allison, L.K. and Fuller, K. Balance disorders.
In: Umphred, D, ed. Neurological Rehabilitation,
Fourth Edition. St. Louis: Mosby. 2001:802–837.
4 Denham T, McKinnon Wolf A. Vestibular
­rehabilitation. Rehab Manag. 1997;10:93–4:144.
5 Cooksey FS. Rehabilitation in vestibular injuries. Proc R Soc Med. 1946;39:273.
6 Horak FB, Henry SM, Shumway-Cook A.
Postural perturbations: new insights for
treatment of balance disorders. Phys Ther.
1997;77(5):517–533.
7 Sherpard NT and Telian SA. Programmatic
vestibular rehabilitation. Otolayngol Head Neck
Surg. 1995;112:173–182.
8 Krebs DE, Gill-Body KM, Riley PO, Parker SW.
9
10
11
12
13
Double blind, placebo controlled trial of rehabilitation for bilateral vestibular hypofunction:
Preliminary Report. Otolayngol Head Neck Surg.
1993;109:735–741.
Woollacott M and Shumway-Cook A. Attention
and the control of posture and gait: A review
of an emerging area of research. Gait Posture.
2002:16:1–14.
Murdin L, Davies RA, Bronstein AM. Vertigo as
a migraine trigger. Neurology. 2009;73:638–642.
Pavlou M, Kanegaonkar RG, Swapp D,
Bamiou DE. The effect of virtual reality on visual
vertigo symptoms in patients with peripheral
vestibular dysfunction: A pilot study. J Vestib Res.
2012;22(5–6):273–281. doi: 10.3233/VES-120462.
Whitney SL, Sparto PJ, Hodges LF,
Babu SV. Responses to a virtual reality
­grocery store in persons with and without
­vestibular ­dysfunction. Cyberpsychol Behav.
2006;9(2):152–156.
Sparto PJ, Furman JM, Whitney SL, Hodges LF,
Redfern MS. Vestibular rehabilitation
using a wide field of view virtual environment. Conf Proc IEEE Eng Med Biol Soc.
2004;7:4836–4839.
144 Dizziness and Vertigo: An Introduction and Practical Guide
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18
Psychological
aspects of dizziness
Raj Attavar and Amalsha Vithanaarachichi
Contents
Introduction
Anxiety disorders
Signs and symptoms of anxiety
Specific anxiety disorders of importance
Panic disorder
Generalised anxiety disorder
Agoraphobia
Social phobias
Somatoform disorders
Somatisation disorder
Somatoform autonomic dysfunction
Depersonalisation–derealisation syndrome
Mood [affective] disorders
Depressive episode
Assessment of anxiety
Hospital anxiety and depression scale
Assessment of depression
Management of anxiety in patients with dizziness
Medical management of anxiety
Psychological management
Cognitive behavioural therapy
Case study
Conclusion
References
145
146
146
146
146
146
147
147
147
147
147
147
148
148
148
148
148
149
149
149
149
150
151
151
Introduction
Vertigo and dizziness are common presenting
complaints. Often associated, but frequently overlooked, are the psychological consequences that
may result.
Published data by Yardley et al.1 found that over
20% of patients of working age had sought medical attention for symptoms of dizziness. Of these,
almost half had reported anxiety or avoidance
Psychological aspects of dizziness 145
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behaviour. Other studies have recorded even
greater rates of psychiatric pathology when symptoms of depression are also included.2,3,4,5
Whilst a balance disorder may result in the onset of
psychological symptoms, a psychological disorder
may also be unmasked or accentuated by a dizzy
condition. Certain personality types and a previous
psychiatric or psychological history may make a
patient more susceptible to developing psychological symptoms, for example, anxiety and avoidant
personality. This personality disorder is characterised by feelings of tension and apprehension,
insecurity and inferiority.
Conversely, psychological conditions may in
isolation cause dizziness, notably anxiety and
depression. Of the anxiety disorders many of the
conditions listed under neurotic, stress-related
and somatoform disorders in the International
Classification of Diseases-10 code (ICD-10) include
dizziness as a symptom. Examples include panic
disorder, generalized anxiety disorder, phobic
anxiety disorder, agoraphobia, and somatisation
syndrome. Depressive disorder can also present
with complaints of dizziness. A less common cause
is eating disorders.
It is therefore essential that clinicians are aware of
the association between symptoms of dizziness and
psychological disorders, and explore the possibility of the presence of the latter in every patient
­presenting with dizziness or vertigo. Unless both
dizzy and psychological disorders are addressed
patients are unlikely to fully recover.
Listed below are commonly associated psychological disorders with their symptoms and signs.
Anxiety disorders
Signs and symptoms of
anxiety
The signs and symptoms that may be observed
in anxiety states are related to the body’s stress
response such as increased adrenaline levels and
its consequences. Hence, physical symptoms may
include: dizziness, drowsiness and tiredness, pins
and needles, palpitations, muscle aches and tension, a dry mouth, excessive sweating, shortness of
breath, stomachache, nausea, diarrhoea, headache,
excessive thirst, frequent urinating and insomnia.
Signs of anxiety include tachycardia, hyperventilation and tremor.
Anxiety can also give rise to psychological
symptoms such as: restlessness, a sense of dread,
feeling constantly being ‘on edge’, irritability,
impatience, being easily distracted and difficulty
concentrating.
Specific anxiety disorders of importance
Panic disorder
The essential feature of this condition is that of
recurrent attacks of severe anxiety (panic), which
are not restricted to any particular situation or set
of circumstances, and are therefore unpredictable.
As with other anxiety disorders, the dominant
symptoms include sudden onset of palpitations,
chest pain, choking sensations, dizziness, and
feelings of unreality (depersonalisation or derealisation). There is often also a secondary fear of
dying, losing control, or ‘going mad’.
Generalised anxiety disorder
This disorder is characterised by anxiety that is
generalised and persistent but not restricted to, or
even strongly predominating in, any particular
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environmental circumstance (i.e. it is ‘free-floating’).
In other words the anxiety is not specific to any
situation. The dominant symptoms are variable but
include complaints of persistent nervousness, trembling, muscular tensions, sweating, light headedness,
palpitations, dizziness, and epigastric discomfort.
Fears that the patient, or a relative, will shortly
become ill or have an accident are often expressed.
Agoraphobia
Agoraphobia is a well-defined cluster of phobias
embracing fears of leaving home, entering shops,
crowds and public places, or travelling alone in trains,
buses or planes. As a result patients may be misdiagnosed as suffering from visual vertigo or even mal de
debarquement syndrome. Panic disorder is a frequent
feature of both present and past episodes. Depressive
and obsessional symptoms and social phobias are
also commonly present as subsidiary features.
Avoidance of the phobic situation is often prominent,
and some agoraphobics experience little anxiety
because they are able to avoid their phobic situations.
Social phobias
A fear of scrutiny by other people leading to
avoidance of social situations characterises this
disorder. More pervasive social phobias are usually
associated with low self-esteem and fear of criticism. They may present as a complaint of blushing,
hand tremor, nausea, or urgency of micturition,
the patient sometimes being convinced that one
of these secondary manifestations of their anxiety
is the primary problem. Symptoms may progress to
panic attacks.
Somatoform disorders
The main feature of a somatoform disorder is
repeated presentation of physical symptoms
together with persistent requests for medical
investigations, despite negative clinical and special
investigation findings and reassurances by doctors
that the symptoms have no physical basis. If any
physical disorders are present, they do not explain
the nature and extent of the symptoms or the distress and preoccupation of the patient.
Somatisation disorder
This condition features multiple, recurrent and
frequently changing physical symptoms of at least
two years’ duration. Most patients have a long and
complicated history of contact with both primary and specialist medical care services, during
which many negative investigations or fruitless
exploratory operations, may have been carried out.
Symptoms may be referred to any part or system of
the body. The course of this disorder is chronic and
fluctuating, and is often associated with disruption of social, interpersonal, and family behaviour.
Short-lived (less than two years) and less striking
symptom patterns should be classified under undifferentiated somatoform disorder (F45.1).
Somatoform autonomic
dysfunction
A patient may present with symptoms that are in
keeping with a physical disorder of a system or
organ that is under autonomic innervation, such
as the cardiovascular, gastrointestinal, respiratory
and urogenital systems. The symptoms are usually
of two types, neither of which indicates a physical
disorder of the organ or system concerned. First,
there are complaints based upon objective signs of
autonomic arousal, such as palpitations, sweating,
flushing, tremor, and expression of fear and distress
about the possibility of a physical disorder. Second,
there are subjective complaints of a non-specific or
changing nature such as fleeting aches and pains,
sensations of burning, heaviness, tightness, and
feelings of being bloated or distended, which are
referred by the patient to a specific organ or system.
Depersonalisation–
derealisation syndrome
A rare disorder in which the patient complains
spontaneously that his or her mental activity, body,
and surroundings, are changed in their quality so
as to be unreal, remote or automatised. Among
the varied phenomena of the syndrome, patients
complain most frequently of loss of emotions and
feelings of estrangement or detachment from their
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thinking, their body, or the real world. In spite of
the dramatic nature of the experience, the patient is
aware of the unreality of the change. The sensorium
is normal and the capacity for emotional expression
intact. Depersonalisation–derealisation symptoms
may occur as part of a diagnosable schizophrenic,
depressive, phobic, or obsessive-compulsive disorder. In such cases the diagnosis should be that of
the main disorder.
Mood [affective] disorders
Depressive episode
In typical mild, moderate, or severe depressive
episodes, the patient suffers from lowering of
mood, reduction of energy, and decrease in activity. Capacity for enjoyment, interest, and concentration is reduced, and marked tiredness after
even minimum effort is common. Sleep is usually
disturbed and appetite diminished. Self-esteem
and self-confidence are almost always reduced
and, even in the mild form, some ideas of guilt or
worthlessness are often present. The lowered mood
varies little from day to day, is unresponsive to circumstances and may be accompanied by so-called
‘somatic’ symptoms, such as loss of interest and
pleasurable feelings, waking in the morning several hours before the usual time, depression worst
in the morning, marked psychomotor retardation,
agitation, loss of appetite, weight loss, and loss of
libido. Depending upon the number and severity of the symptoms, a depressive episode may be
specified as mild, moderate or severe. Complaint
of dizziness could be a feature of depression.
Assessment of anxiety
A detailed history is mandatory and the use of
clinical scales often beneficial. Widely used scales
are the Hospital Anxiety and Depression Scale,6 the
Hamilton Anxiety Scale, and the Clinical Anxiety
Scale. These scales are widely used and easy to
administer in patients with anxiety.
Hospital anxiety and
depression scale
The items on the questionnaire that relate to
­anxiety are:
tense or wound up.
•• II feel
get a sort of frightened feeling as if something
bad is about to happen.
thoughts go through my mind.
•• Worrying
I cannot sit at ease and feel relaxed.
sort of frightened feeling like butterflies
• Iingetthea stomach.
restless and have to be on the move.
•• II feel
get sudden feelings of panic.
Assessment of depression
A thorough history is key. The main features to
determine are the core features of depression
namely low mood, lack of energy and anhedonia.
Additional features to assess are:
mood most of the day, nearly every
• Depressed
day, as indicated by either a subjective report,
•
••
••
•
for example feels sad or empty, or observation
made by others such as appears tearful. In children and adolescents this may be characterised
as an irritable mood. Markedly diminished
interest or pleasure in all, or almost all, activities most of the day, nearly every day.
Significant weight loss when not dieting or
weight gain (for example a change of more
than five per cent of body weight in a month),
or decrease or increase in appetite nearly
every day.
Insomnia or hypersomnia nearly every day.
Psychomotor agitation or retardation nearly
every day.
Fatigue or loss of energy nearly every day.
Feelings of worthlessness or excessive or inappropriate guilt nearly every day.
Diminished ability to think or concentrate, or
indecisiveness, nearly every day.
148 Dizziness and Vertigo: An Introduction and Practical Guide
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thoughts of death (not just fear of
• Recurrent
dying), recurrent suicidal ideation without a
specific plan, or a suicide attempt or a specific
plan for committing suicide.
Management of anxiety in
patients with dizziness
Anxiety is the most common psychiatric disorder associated with dizziness. Treatment of anxiety disorders is generally addressed in primary
care, such as family doctors/General Practitioner
(GP). Treatment includes assessment of the
condition as well as medical and psychological
therapies.
It is important to rule out common organic causes
of anxiety that may also be present. Examples
include endocrine or metabolic disorders such as
hyperthyroidism or anaemia.
In addition to endocrine conditions, it is important to determine if a patient is consuming large
quantities of alcohol, or illicit substances, as these
addictions can mimic or mask both anxiety and
dizziness in subjects.
Some medications given to reduce the anxiety, panic or agitation can indeed worsen the
­d izziness (e.g. benzodiazepines). Antidepressants,
hypnotics such as Alprasolan and Clonazepam,
antipsychotics such as Clozapine and antiepileptic medication such as Gabapentin, and
Carbamazepine can give rise to dizziness. Hence,
it is important to take a detailed medical and
drug history.
As part of general management, the GP may offer
advice on reduction of caffeine and alcohol, smoking cessation, diet and exercise. This may require
general advice, the use of leaflets or referral to
other services such as addictions services. Some
GPs also offer smoking ceasing workshops. Other
areas of importance would be getting a regular
sleep pattern, development of a hobby, increase
of exercise and activities, leading to changes in
lifestyle.
Medical management of
anxiety
Antidepressants have been found to be beneficial
in the treatment of anxiety in randomised control
trials. Antidepressants have also been found to be
more effective than benzodiazepines in the treatment of anxiety and panic. As previously stated
both antidepressants and benzodiazepines do have
a potential to make the dizziness worse, hence
there is need for careful questioning and weighing up benefits versus risks. One should be careful
in titrating the dose of antidepressants, beginning
with the lowest possible dose and slowly increasing this. As the dose is increased, the beneficial
effects on anxiety must be weighed up against the
potential of these drugs to make dizziness worse.
Commonly used antidepressants are Escitalopram,
Citalopram, Paroxetine and Flouxetine.
Psychological management
These are also referred to as ‘talking therapies’ and
are carried out by trained therapists. A commonly
used method is cognitive behavioural therapy
(CBT). CBT is useful for many psychiatric conditions. It can be used in anxiety, depression, panic,
phobias (including agoraphobia and social phobia),
stress, bulimia, obsessive compulsive disorder,
post-traumatic stress disorder, bipolar disorder
and psychosis.
Cognitive behavioural therapy
The cognitive models of anxiety were first proposed
by Beck and colleagues in 1985. Sessions in CBT are
with a psychologist or a counsellor who is trained.
Treatment involves looking at beliefs and behaviour. A patient with anxiety may portray beliefs that
may be dysfunctional, leading to wrong assumptions of their anxiety symptoms, which then in
turn leads to maladaptive behaviour patterns such
as avoidance and worsening of symptoms.
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Case study 18.1: Cognitive behavioural therapy (CBT)
A 32-year-old lady was admitted to the psychiatric ward with palpitations, difficulty in
breathing, dizziness and feeling of losing control. She would experience these symptoms
without an identifiable trigger as well as when she was out in the community. Due to these
distressing episodes, she gradually stopped going out and became housebound. She did not
have a medical condition giving rise to these symptoms. Her diagnosis was agoraphobia
with panic.
An example of her difficulties would be:
Situation – need to go to the supermarket;
Thoughts – I am going to fall. I am going crazy. I am going to die;
Emotions – anxious, apprehensive;
Physical sensations – palpitations, dizziness, dyspnoea, chest pain;
Behaviour – stop going out, avoidance, anticipatory anxiety.
A diagrammatic representation would look something like this, Figure 18.1:
The thoughts, emotions, physical sensations and behaviours are all interconnected. The
thoughts of ‘I am going to die’ will cause her to feel anxious and cause her to get palpitations
etc. On the other hand getting the physical sensations of palpitations, dyspnoea with hyperventilation and dizziness, will cause her to feel as though she is going crazy and she is going
to die. This inter-linkage will lead to a vicious cycle leading to avoidance of the situation and
therefore inevitably worsening the symptoms.
The basis of CBT is challenging the automatic negative thoughts and thereby breaking the
vicious cycle.
Going back to the lady that was admitted, CBT therapy was used. She was encouraged
to tackle her anxieties gradually. She was also given skills to have a good structure to her
day, increase her life skills, increase her activities and interests including exercise, good sleep
hygiene and good dietary habits as standard input as an inpatient.
After several months of stay as an inpatient, she was able to venture to the local supermarket on her own and later on she was able to be discharged back into the community with
support.
The role of the clinician is to work alongside the patient in identifying patterns of maladaptive behaviour and dysfunctional assumptions leading to change in thoughts and
behaviour.
150 Dizziness and Vertigo: An Introduction and Practical Guide
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Situation
Needing to go to the shops
Thoughts
I am going crazy. I am going to die.
Behaviour
Emotion
Stop going out, avoidance
Anxious, apprehensive
Physical sensations
Palpitations, dyspnoea, dizziness
Figure 18.1. The interplay of thoughts, emotion, behaviour and physical sensations in CBT.
Conclusion
The psychological impact of dizziness should never
be underestimated. In some, symptoms of anxiety,
depression and agoraphobia persist long after its
organic cause is found and successfully treated.
Although medication may be ­appropriate in some
patients, side effects include dizziness, and hence
CBT is often most appropriate.
References
1 Yardley L, Owen N, Nazareth I, Luxon L.
Prevalence and presentation of dizziness
in a general practice community sample
of working age people. Br J Gen Pract.
1998;48(429):1131–1135.
2 Eckhardt-Henn A, Breuer P, Thomalske C,
et al. Anxiety disorders and other ­psychiatric
subgroups in patients complaining of ­dizziness.
J Anxiety Disord. 2003;17:369–388.
3 Grunfeld EA, Gresty MA, Bronstein AM,
Jahanshahi M. Screening for depression
among neurootology patients with and without identifiable vestibular lesions. Int J Audiol.
2003;42:161–165.
4 Persoons P, Luyckx K, Desloovere C, et al.
Anxiety and mood disorders in otorhinolaryngology outpatients presenting with
­dizziness: Validation of the self-administered
PRIME-MD Patient Health Questionnaire
and epidemiology. Gen Hosp Psychiatry.
2003;25:316–323.
5 Staab JP, Ruckenstein MJ. Which comes first?
Psychogenic dizziness versus otogenic anxiety.
Laryngoscope. 2003;113:1714–1718.
6 Zigmond, AS, Snaith, RP. The hospital ­anxiety and
depression scale. Acta Psychiatrica Scandinavica.
1983;67(6):361–370. http://­sideeffects.embl.de/se/
C0012833/ (accessed 21 June 2013).
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An Introduction and Practical Guide
Dizziness and vertigo are common symptoms. Patients may present to general practitioners,
ENT surgeons, neurologists or general medicine specialists but are often poorly managed.
Dizziness and Vertigo: An Introduction and Practical Guide is an essential text which
contains all the basic knowledge and practical skills necessary for managing patients
with these symptoms. It provides a comprehensive overview of dizziness and vertigo,
how to accurately diagnose patients and how to treat them.
Dizziness and Vertigo
Dizziness
and
Vertigo
Dizziness
and
Vertigo
An Introduction
and Practical Guide
An Introduction and Practical Guide
Key features
• Concise, practical and easy to read
• Highly illustrated throughout to aid understanding
• Written by experts in the field
• Companion volume to the successful ENT: An Introduction
and Practical Guide, from the same editors
Rahul G Kanegaonkar FRCS(ORL-HNS) is a Consultant in Otolaryngology at
Medway Maritime Hospital, Kent, UK, and an Honorary Senior Lecturer in Otorhinolaryngology at the Anglia Ruskin University.
James R Tysome MA, PhD, FRCS(ORL-HNS) is a Consultant in Otolaryngology
and Skull Base Surgery at Addenbrooke’s Hospital, Cambridge, UK.
Kanegaonkar
Tysome
K17350
Edited by
Rahul G. Kanegaonkar
James R. Tysome
ISBN: 978-1-4441-8268-2
90000
9 781444 182682
K17350_Cover.indd All Pages
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