Introduction
Meniere's disease (idiopathic
endolymphatichydrops)
 is a disorder of the inner ear
associated with a symptoms
consisting of spontaneous,
episodicattacks of vertigo;
sensorineural hearing loss
whichusually
fluctuates; tinnitus; and
often a sensation of aural
fullness.
dramatic variability is the
hallmark of this disease.
ENDOLYMPHATICSYSTEM
Functionsof ENDOLYMPHATIC
sac
1. Resorption of the water content of endolymph
2. Ability to participate in some ionicexchanges with endolymph
3. Removal of metabolic and cellular debris, includingotoconia
4. Immunodefense function ( perisaccular)
5. Inactivation and removal of viruses
6. Secretion of Glycoproteins to attract extra fluid (Glycoproteins act as a driving
force for longitudinalflow)
1. Secretion of Saccinto increase Endolymph production
Introduction : History
First described by Prosper
Meniere in 1861.
In 1902, Parry performed a
CN VIII division for vertigo
in a patient with suspected
Meniere’s disease.
 Portman did
endolymphatic sac
decompression via a
transmastoid approach in
1926.
In 1931,McKenzie
performed a selective
vestibular neurectomy.
Pathology
Distortion of the membranous labyrinth.
This condition reflects the changes in the anatomy of
the membranous labyrinth as a consequence of the
over-accumulation of endolymph.
Mainly affects scala media and saccule
Bulging of reissner’s membrane
Saccule may come to lie against the stapes footplate.
Etiology
A. Defective absorption by endolymphaticsac• Poor vascularity of sac
• Less absorptive tubularepithelium
• increased perisaccular fibrosis
B. Rupture of reissner’s membreane leading to mixing of
perilymph & endolymph- Schuknecht
• allow leakage of the potassium-rich endolymph into
the perilymph, bathing the eighth cranial nerve and
lateral sides of the haircells
Etiology
Spasm of int. auditory artery – Sym.Overactivity
Allergy – inner ear is shock organ
Sodium & waterretention
Hypothyroidism
Autoimmune
Viral
Clinical features
Affects in 4th -5th decade of life
Male:Female 1:1
Prevalence more in whites.
VERTIGO : episodic attacks ,
asso. with nystagmus, nausea &
vomiting , vagal disturbance
Tullio phenomenon may be seen
• Otolithic crisis of Tumarkin
o are thought to occur as a result of acute otolithic dysfunction.
o The patient simply drops to the ground
without warning and can sustain a fracture or
other serious Injury
• Lermoyezsyndrome
•
described an unusual clinical presentation in which tinnitus and hearing loss
precede the onset of vertigo
Clinical features
HEARING LOSS
1.
2.
3.
4.
5.
6.
Fluctuating
SNHL
Progressive
Unilateral
Distortion of sound
Intolerance to loud sound
Clinical features
TINNITUS
Low pitched roaring
2. Subjective
3. Unilateral
 AURAL FULLNESS
1. Fluctuates , in prodromal phase
1.
Diagnosis
Investigations
Tuning forks tests :
SNHL
PTA
Speech audiometry
Recruitment test +ve
SISI >70%
Tone decay <20 dB
Investigations
Caloric testing – canal paresis
ENG
Head Thurst test
ECoG – SP is larger & more negative
SP/AP ratio increases > 30%
Glycerol test
VEMP – elevated threshold
VEMPs
Staging
STAGE
PURE TONE AVERAGE IN dB IN PREVIOUS 6 MONTHS
1
= < 25
2
26-40
3
4
41-70
>70
Variants
Cochlear hydrops – novertigo
Vestibular hydrops – no heaing loss
Drop attacks
Lermoyez syndrome- hearing loss followed byvertigo
Treatment
Medical management –
ACUTE stage : labyrinth sedatives +anti-emetics
Carbogen, Histamine drip
Frustenberg Regimen 1.
2.
3.


Low saltdiet
Diuretics + Pot. chlor
High protein
Beta histine – to relieve vascular ischemia
Stop caffeine, nicotine, alcohol & tobacco
Non ablative procedures
Portman -1926
Endolymphatic sacsurgery
Subarachnoid shunt
2. Mastoid shunt
1.
Non ablative procedures
Intratympanic steroids
May benefit in autoimmune causes of meniere’s
syndrome.
Sacculotomy
Cochleosacculotomy
Ablative procedures
Intratympanic gentamicin – Schuknecht(1957)
Ablative procedures
Selective Vestibular nervesectioning
Ablative procedures
Ultrasonic destruction of vest.Labyrinth
Cryodestruction
Labyrinthectomy - when cochlear function has been
totally deteoriated ,higherrate of vertigo control seen
than that typical for vestibularneurectomy
Recent advances
decrease hydrops by pulsing pressure in themiddle
ear
Meniett device - handheld air pressure generator that
the patientself-administers
The pressure is delivered in complex pulses of up to
20 cm of water, over a 5 minute period.
The device requires a ventilation tube to be placed in
the tympanic membrane before initiation of therapy
Pressure at the RW passes to perilypmh and decreases
pressure in endolymph by redistributing it.