Meniere’s disease

MENIERE’S DISEASE

Saurabh GuptaProf. (Dr.) S. K. Jaiswal unit

IntroductionMeniere's disease (idiopathic endolymphatic

hydrops) is a disorder of the inner ear associated with a symptoms consisting of spontaneous, episodic attacks of vertigo; sensorineural hearing loss which usually fluctuates; tinnitus; and often a sensation of aural fullness.

dramatic variability is the hallmark of this disease.

Introduction : HistoryFirst 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 sacculeBulging of reissner’s membrane Saccule may come to lie against the stapes

footplate.

EtiologyA. Defective absorption by endolymphatic sac-• Poor vascularity of sac• Less absorptive tubular epithelium• increased perisaccular fibrosisB. 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 hair cells

EtiologySpasm of int. auditory artery – Sym.

OveractivityAllergy – inner ear is shock organSodium & water retentionHypothyroidismAutoimmuneViral

Clinical featuresAffects in 4th -5th decade of lifeMale:Female 1:1Prevalence more in whites.VERTIGO : episodic attacks , asso. with nystagmus,

nausea & vomiting , vagal disturbanceTullio phenomenon may be seen

Clinical featuresHEARING LOSS 1. Fluctuating2. SNHL3. Progressive 4. Unilateral5. Distortion of sound6. Intolerance to loud sound

Clinical featuresTINNITUS1. Low pitched roaring2. Subjective3. Unilateral AURAL FULLNESS1. Fluctuates , in prodromal phase

Diagnosis

InvestigationsTuning forks tests :

SNHLPTA Speech audiometryRecruitment test

+veSISI >70%Tone decay <20 dB

Investigations Caloric testing – canal paresisENGHead Thurst testECoG – SP is larger & more negativeSP/AP ratio increases > 30%Glycerol testVEMP – elevated threshold

VEMPs

StagingSTAGE PURE TONE AVERAGE IN dB IN PREVIOUS 6

MONTHS

1 = < 25

2 26-40

3 41-70

4 >70

Variants Cochlear hydrops – no vertigoVestibular hydrops – no heaing lossDrop attacksLermoyez syndrome- hearing loss followed by

vertigo

Treatment Medical management –ACUTE stage : labyrinth sedatives + anti-

emeticsCarbogen, Histamine dripFrustenberg Regimen -1. Low salt diet2. Diuretics + Pot. chlor3. High protein Beta histine – to relieve vascular ischemia Stop caffeine, nicotine, alcohol & tobacco

Non ablative proceduresPortman -1926Endolymphatic sac surgery1. Subarachnoid shunt2. Mastoid shunt

Non ablative proceduresIntratympanic steroids May benefit in autoimmune causes of

meniere’s syndrome.Sacculotomy Cochleosacculotomy

Ablative proceduresIntratympanic gentamicin – Schuknecht

(1957)

Ablative proceduresSelective Vestibular nerve sectioning

Ablative proceduresUltrasonic destruction of vest. Labyrinth CryodestructionLabyrinthectomy - when cochlear function

has been totally deteoriated ,higher rate of vertigo control seen than that typical for vestibular neurectomy

Recent advancesdecrease hydrops by pulsing pressure in the

middle earMeniett device - handheld air pressure

generator that the patient self-administersThe 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.

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