Ménière’s Disease
Sam Maleki, Jordan Braun, Alex Wohl, Rob Whittaker
Etiology
 Female Caucasians most prone to disorder.
 157/100k in England
 46/100k in France
 Peak incidence 40-60 y.o. (1.3:1 female to male ratio)
 2-50% of symptoms arise in opposite ear
 Prevalence rates caused by differences in environment,
genetics, or diagnostic criteria is unclear
 Familial occurrence reported in 10-20% cases (autosomal
dominant mode of inheritance)
 Genetics – human leukocyte antigens B8/DR3 & Cw7 have been
associated
Anatomy/Physiology
 Vestibular System
 Detects forces from gravity & movement, maintains clear vision
during head motion (VOR) by head positioning
 Semicircular canals – ring-shaped, fluid filled (endolymph)
oriented in 3D provides sensory input to velocity & angular
acceleration (ampulla deflected away from direction of head
movement by endolymph)
 Speed & direction of deflection of hair cells of ampulla determines
the rate of firing of the vestibular nerve
 Ends of semicircular duct open into otolith (utricle & saccule) –
contained hair cells covered in otolithic membrane (otoconia produce
shear force)
 Signals carried by vestibular nerve - If lesion in vestibular nerve,
brain can possibly adapt from intact opposite nerve & recalibrate
 Motor output through vesibulospinal reflexes (VSRs) – automatic
control of postural muscles in trunk & limbs
Anatomy/Physiology Cont’d
 Audition
 Tympanic membrane  Ossicles  cochlea via oval
windows
 Scala vestibula & scala tympani (perilymph), Scala media
(endolymph)
 Pressure waves travels through scala vestibuli, helicotrema, &
scala tympani  pressure changes onto basilar membrane &
into Organ of Corti  exits round window at end of scala
tympani
 Inner ear (cochlea – fluid filled tube dived by organ of Corti)
 Fluid incompressible & bony wall rigid, important to maintain
fluid volume
 Sound through ossicles  oval window  scala vestibuli
(perilymph)  scala tympani  round window
 Endolymph in scala media
Pathology
 Endolymphatic hydrops – over accumulation of
endolymph compromising perilymphatic space
 Lack of absorption of endolymph in endolymphatic duct &
fluid backs up into system
 Characterized by episodic vertigo; fluctuating, sensorineural
hearing loss; sensation of fullness in the ears; & tinnitus
 Vertigo most debilitating symptom with intervals of hours to
days
 Simultaneous hearing deterioration of involved ear
 Reduction in responsiveness of involved peripheral vestibular
system can occur
Pathology cont’d
 Multifactorial causes
 Fibrosis, atrophy of the sac, obstruction of the
endolymphatic duct, infection, or the vascularity in the
region in the inner ear.
 Otosyphilis (involvement of the inner ear in collagen
vascular disease)
 Immune responses likely within the complex related to
allergic reactions & histamine
 Viral infection – more susceptible to changes in thyroid, Na+
or hormone dysfunction
 Overproduction of endolymph by stria vasularis
 Blow to head, a fall, or flexion/extension injury
Pathology cont’d
 Pathogenesis of symptoms uncertain
 Deficits related to volume/pressure changes within
closed fluid system
 Membranous labyrinth progressively dilate until the wall
makes contact with the stapes footplate & the cochlear duct
fills the entire scala vestibula  vestibular & cochlear
dysfunction
 Distension of otoliths can put pressure on the ampulla,
creating sensation of spinning that is characteristic of acute
unilateral dysfunction
 Membrane rupture  leak of K+ into endolymph  Nerve palsy
Pathology cont’d
 Typical attack of hydrops – initial sensation of fullness of
the ear, reduction in hearing, & tinnitus
 Followed by rotational vertigo (30 min – 24 hours), postural
imbalance, nystagmus, & nausea
 Permanent loss of hearing over time
 Tinnitus is commonly described as low-pitched roaring or
seashell like
History
 General Questions:
 Age, date of onset, previous history of falls
 Triad of Associated Symptoms:
 Vertigo
 Tinnitus
 Fluctuating Hearing Loss
 Family History
 7-10% affected
 Employment: Current work, community, & leisure
actions, tasks, or activities
History Cont’d
 Functional status & activity level: current/prior functional
status in self care/home & in work
 Medications
 Other clinical tests: lab & diagnostic tests, review of
available records, review of other clinical findings
 Employment: Current work, community, & leisure
actions, tasks, or activities
 General health status: general health perception,
physical function, psychological function, role function,
social function
 Other clinical tests: lab & diagnostic tests, review of
available records, review of other clinical findings
Systems Review
 CV: BP, edema, HR, RR
 Integumentary: pliability, scar formation, skin
color/integrity
 Musculoskeletal: ROM, strength, symmetry, height/weight
 Neuromuscular: coordination (balance, gait, locomotion,
transfers, transitions), motor function
 Cranial Nerve Testing
 Nystagmus testing
 Communication, affect, cognition, language, & learning style
 Ability to make needs known, consciousness, expected
emotional/behavior responses, learning preferences, orientation
(person, place, time)
Global outcomes
 Functional Limitations – Nottingham health profile, SF –
12/36, Quality of Well being (self administered), dizziness
handicap inventory1
 Visual analogue scale (VAS), dizziness handicap inventory
(DHI), functional disability scale, motion sensitivity quotient
(MSQ)2
 Gait, locomotion, & balance
 Elderly mobility scale, Fugl-Meyer assessment scale, functional
standing test, hop tests, obstacle course, seated postural
control measure, TUG, trunk control
 Berg balance scale, Romberg Tests, sit to stand tests, tilt board
balance tests, Tinetti performance-oriented mobility scale
 Functional ambulation profile, gait abnormality rating scale, gait
speed, Rivermead visual gait assessment
PT tests
 Smooth pursuits (nystagmus)
 Saccadic eye movements (look back/forth 2 objects)
 VOR (focus on an object while turning head)
 Head thrusts (quick passive movements by PT)
 Head shaking (pt. actively move head quickly)
 Dix-Hallpike maneuver (BPPV test)
 Lab (by physician)
 Caloric (air/water injected - alter temp)
 Rotational (Barany test, rotate in chair, watch eyes, balance
master)
Special Tests

Dix-hallpike Dx of BPPV
 http://youtu.be/kEM9p4EX1jk
 Sensitivity – 79% [95% CI: 65-94%]
 Specificity – 75% [95% CI: 33-100%]
 LR+ – 3.14 [95% CI: 0.58-17.58]
 LR- – 0.28 [95% CI: 0.11-.0.69]

Sidelying Test
 Sensitivity – 90% [95% CI: 79-100%]
 Specificity – 75% [95% CI: 33-100%]
 LR+ – 3.59 [95% CI 0.65-19.67]
 LR- – 0.14 [95% CI: 0.04-0.46]

Establishing a Diagnosis of Benign
Paroxysmal Positional Vertigo
Through the Dix-Hallpike & SideLying Maneuvers (2008)
Special Tests Cont’d

Vestibular evoked myogenic potentials (VEMPs)
 Sensitivity – 50.0%
 Specificity – 48.9%
 LR+ – 1.04
 LR- – 1.00

Caloric Test
 Sensitivity – 37.7%
 Specificity – 51.2%
 LR+ – 0.75
 LR- – 0.72

No significant difference in hearing level between patients appropriately
or inappropriately identified by VEMPs, whereas significant difference in
those of the caloric test.
 Combined VEMP & caloric test increased sensitivity to 65.8%

The diagnostic value of vestibular evoked myogenic potentials in patients
with Meniere’s disease (2013)
Evaluation
 Rule out differential diagnosis
 Potential referral for diagnosis
 Describe frequency & duration of symptoms
 Refer to previous slides for other testing
Differential Diagnosis
 Pathology Implications for the Physical Therapist
 Presence of neurologic signs or symptoms such as syncope, visual
aura, & motor weakness suggest another diagnosis
 Disorders that present with similar symptoms include: migraine,
acoustic neuroma, perilmyphatic fistula, dehiscence of the superior
semicircular canal, labyrinthitis, autoimmune inner ear disorder, &
MS
 Vertigo – a feeling of spinning & loss of balance, caused by disease
affecting the inner ear or the vestibular nerve
 Migraines – a regular aching/throbbing headache that usually
affects one side of the head usually goes along with nausea &
troubled vision.
 Vestibular Neuronitis – Can be a series/single attack of vertigo or a
persistent condition that decreases over 6 weeks
 Postural muscle weakness, reflex integrity/peripheral nerve
 BPPV
Differential Diagnosis Cont’d
 Factors that may differentiate Ménière’s disease from
benign recurrent vertigo
 Based on case-control study of 112 patients with Ménière’s
disease & 63 patients with benign recurrent vertigo
 Vertigo attacks with unilateral tinnitus & unilateral hearing
loss more likely to be Ménière’s disease in multivariate
analysis
 Earlier age at onset & shorter duration of vertigo attacks,
female preponderance, & presence of migraine more
common in benign recurrent vertigo
Differential Diagnosis (Reference)
 Other problems to be considered include the following:
 Trauma, Endocrine abnormalities, Thyroid dysfunction,
Hyperlipidemia, Diabetes, Congenital anomalies, Autoimmune
problems/inner ear inflammation,
 Differential Diagnosis
 Anterior Circulation Stroke, AVM, Basilar Artery Thrombosis, Benign
Positional Vertigo in Emergency Medicine, Benign Skull Tumors,
Brainstem Gliomas, Cerumen Impaction Removal, Ear Foreign Body
Removal in Emergency Medicine, HIV-1 Associated CNS Conditions –
Meningitis, Hypothyroidism & Myxedema Coma in Emergency
Medicine, Inner Ear Labyrinthitis, Intracranial Hemorrhage, Ischemic
Stroke in Emergency Medicine, Lyme Disease, Migraine Headache,
Multiple Sclerosis, Neurosyphilis, Otitis Media in Emergency
Medicine, Polyarteritis Nodosa, Posterior Cerebral Artery Stroke,
Primary Malignant Skull Tumors, Rheumatoid Arthritis, Salicylate
Toxicity in Emergency Medicine, Subarachnoid Hemorrhage in
Emergency Medicine, Syncope & Related Paroxysmal Spells,
Temporal Lobe Epilepsy, Transient Ischemic Attack, Vestibular
Neuronitis, Viral Encephalitis, Viral Meningitis, Wernicke
Encephalopathy
Diagnosis
 Practice Pattern 5A: Primary Prevention/Risk Reduction
for Loss of Balance & Falling
 ICD-9-CM Code – 386.0
 Pathology Implications for the Physical Therapist
 2 or more definitive episodes of spontaneous rotation
vertigo lasting at least 20 minutes (nausea & vomiting
abates by 24 hours)
 Low frequency sensorineural hearing loss documented by
audiometry
 Tinnitus or aural fullness in the affected ear
 Exclusion of other causes for the symptoms
Prognosis
 Expected Range of Number of Visits Per Episode of Care
 2-18 visits
 Range affected by: accessibility/availability of
resources, adherence, age, cognitive status, comorbidities, concurrent medical interventions, level
of impairment/physical function, living environment,
nutritional status/overall health status, psychological
& socioeconomic factors, social support, stability of
condition
Prognosis Cont’d
 Highly variable, Attacks increase in frequency in first years
then decrease
 Clusters of attacks may be separated by periods of long
remission – balance function between attacks can be
normal, although a sense of disequilibrium often persists
later in the disorder
 2-6% of patients experience “drop attacks” (otolithic crisis
of Tumarkin) – abruptly thrown to the ground without LOC
& with little/no vertigo
 Initially 1 ear – bilateral disease ranges from 2-78% with an
average incidence of 45%
 If bilateral involvement has not occurred within 5 years of
onset of first ear, then unlikely will occur
 Hearing loss fluctuating, low-frequency sensorineural loss
early becomes irreversible often progressing in severity with
higher frequencies & loss of speech discrimination
Problem list/Symptoms
 Inner ear condition of vestibular & cochlear systems
 Recurrent vertigo
 Hearing loss & tinnitus in one year
 Feeling of pressure differences in ears
 Nausea
 Balance deficits
 Risk of fall
Goals
 Patient will reduce the risk of falling through therapeutic
exercise, balance training, & lifestyle modification within
4-6 weeks to improve quality of life.
 Reduce nystagmus
 Improve dizziness
 Increase smooth pursuit
 Independent HEP
 Increase balance
 Improvement via functional test (Berg, MiniBEST, ect.)
Surgical Intervention
 Cochlear implant
 Improved hearing reported with cochlear implantation in case
series of 9 patients (mean age 61 years) with Ménière’s disease
for at least 10 years & severe sensorineural hearing loss
 Vertigo may decrease with/without surgical intervention
 Endolymphatic sac surgery does not appear effective for
Ménière’s disease
 Endolymphatic sac shunt & ventilating tube insertion appear
similarly effective both treatments associated with significant
reductions in dizzy spells at 6 & 12 months, but no significant
differences between groups
 Middle ear injections
 Gentamicin
 Steroids
Post-Surgical Timeline
 Goal: 30 days before return to work
 Physician will assess need for continued interventions, or
possible use of medications
 PT may be utilized to address any functional limitations
 Neuromuscular Reeducation
 Strengthening
 Aerobic Conditioning
 PT will continue to monitor for signs/symptoms that
indicate referral back to a physician is necessary
Intervention Cont’d
 Patient will need referral from Physician
 PT alone cannot diagnose Ménière’s Disease
 Precautions/Contraindications
 Sudden loss of hearing
 Increased feeling of pressure or fullness to discomfort in
ears
 Severe ringing in ears
 Severe increase in symptoms
 Severe nausea
Intervention
 Addressing required functions, collaboration & coordination with
agencies (equipment, payers, home care), communication
(education/documentation), data collection/analysis, documentation
 Therapeutic exercise – aerobic/endurance training,
balance/coordination/agility, body mechanics/postural stabilization,
flexibility, gait training Treatment
 Diuretics can control vertigo & stabilize hearing in more than ½ of
individuals
 Restricting salt, caffeine, alcohol, & nicotine (reduces endolymph
volume by fluid removal)
 Antivertiginous meds, antiemetic, sedatives, antidepressants, &
psychiatric treatment
 Corticosteroid infusion of the middle ear via a transtympanic route –
autoimmune & inflammatory injury
 Intratympanic gentamicin used for chronic unrelenting unilateral
hydrops
 Surgery for endolymphatic decompression
Inpatient/Outpatient Care
(nonsurgical)
 Balance/Vestibular training program progressions
 Gaze stabilization exercises
 Hip, knee, & ankle strategies
 Therapeutic Exercises
 Aerobic
 Strength
 Assess Vertigo if needed
 Gait analysis
 Patient Education
 Home analysis
 HEP
 The use of different devices (hearing, AD)
Surgical Intervention
 Vestibular nerve section:
 Selective vestibular nerve section (AKA vestibular
neurectomy)
 Goal is to disconnect diseased labyrinth from brainstem
while preserving hearing
 Complications may include hearing loss, facial nerve injury,
CSF leak, & headache
 Retrosigmoid approach of vestibular nerve section reported
to control vertigo in patients with Meniere's disease
 Translabyrinthine vestibular nerve section may be
superior to labyrinthectomy for improving balance, but
appears to have similar efficacy for vertigo
Home Program
 Hearing Aid
 Meniette device-positive pressure device administer 3x/day
for five min/session. Equalizes pressure in patient with
persistent problems
 Home assessment
 Continue inpatient/outpatient care
 Dietary Changes
 Limit Caffeine & sodium
 Lifestyle changes
 Stop smoking
 Manage stress/anxiety
 Eat regularly
Patient Education
 Who’s affected/prevalence
 What causes the disease
 How the disease impacts function (hearing/balance?)
 Identifying signs/symptoms
 Options for treatment (refer/balance training)
 HEP
 Potential of extra help
 Secondary complications in life
 Three systems that control balance
 Somatosensory, visual, vestibular
Discharge
 Occurs when anticipated goal & expected outcomes have
been achieved
 Patient has met goals
 Improved functional ability
 Improved quality of life
 Dependent on medical/psychosocial status
 Significant improvement on functional assessments
 PT determines pt. will no longer benefit
References
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