Ménière’s Disease

Abdum Muneeb (202020)

Haroon Akbar Khan Niazi (202014)
 Ménière’s disease
• Ménière’s disease, also called endolymphatic hydrops, is a
disorder of the inner ear where the endolymphatic system is
distended with endolymph.
• It is characterized by
(i) vertigo
(ii) sensorineural hearing loss
(iii) tinnitus
(iv) aural fullness.
 PATHOLOGY
The main pathology is distension
There is a marked bulging of
of the endolymphatic system,
The dilatation of the cochlear Reissner’s membrane, which may
mainly affecting the cochlear duct
duct is such that it may even herniate through the
(scala media) and the saccule,
completely fill the scala vestibuli helicotrema into the apical part
and to a lesser extent the utricle
of scala tympani
and semicircular canals.

The utricle and saccule may show

The distended saccule may come
outpouchings into the
to lie against the stapes footplate.
semicircular canals.
 AETIOLOGY
• The main pathology in Ménière’s disease is distension of endolymphatic system due to
increased volume of endolymph.
• This can result either from increased production of endolymph or its faulty absorption or
both.
1. Defective absorption by endolymphatic sac
• Defective absorption by the sac may be responsible for raised endolymph pressure.
• Ischaemia of sac has been observed in cases of Ménière’s disease undergoing sac surgery,
indicating poor vascularity and thus poor absorption by the sac.
• Distension of the membranous labyrinth leads to rupture of Reissner’s membrane and thus
mixing of perilymph with endolymph, which is thought to bring about an attack of
vertigo.
2. Vasomotor disturbance
• There is sympathetic overactivity resulting in spasm of internal auditory artery and/or its
branches, thus interfering with the function of cochlear or vestibular sensory
neuroepithelium.
3. Allergy
• The offending allergen may be a foodstuff or an inhalant.
• inner ear acts as the “shock organ” producing an excess of endolymph
4. Sodium and water retention
• Excessive amounts of fluid are retained leading to endolymphatic hydrops.
5. Hypothyroidism
• About 3% of cases of Ménière’s disease are due to hypothyroidism
6. Autoimmune and viral aetiologies
• have also been suggested on the basis of experimental, laboratory and clinical
observations.
 CLINICAL FEATURES
1. Vertigo
• The onset is sudden.
• Patient gets a feeling of rotation of himself or his environment.
• Usually, an attack is accompanied by nausea and vomiting with ataxia and nystagmus.
• Tullio phenomenon - It is a condition where loud sounds or noise produce vertigo and is due to the distended
saccule lying against the stapes footplate.
2. Hearing loss
• This fluctuating nature of hearing loss is quite characteristic of the disease
• Distortion of sound. Some patients complain of distorted hearing.
• A tone of a particular frequency may appear normal in one ear and of higher pitch in the other leading to
diplacusis.
• Intolerance to loud sounds.
• Patients of Ménière’s disease cannot tolerate amplification of sound due to the recruitment phenomenon.
• They are poor candidates for hearing aids.
• 3. Tinnitus.
• It is the low-pitched roaring type and is aggravated during acute attacks.
• Sometimes, it has a hissing character. It may persist during periods of remission.
• Change in intensity and pitch of tinnitus may be the warning symptom of an attack
• 4. Sense of fullness or pressure.
• Like other symptoms, it also fluctuates.
• It may accompany or precede an attack of vertigo.
• 5. Other features
• Patients of Ménière’s disease often show signs of emotional upset due to apprehension of the repetition of
attacks.
• Earlier, emotional stress was considered to be the cause of Ménière’s disease.
 EXAMINATION
1. Otoscopy
• No abnormality is seen in the tympanic membrane.
2. Nystagmus. It is seen only during an acute attack.
• The quick component of nystagmus is towards the unaffected ear.
3. Tuning fork tests.
• They indicate sensorineural hearing loss.
• Rinne test is positive, absolute bone conduction is reduced in the affected ear and Weber is lateralized to the
better ear.
 INVESTIGATIONS
• 1. Pure tone audiometry
• There is sensorineural hearing loss.
• In the early stages, lower frequencies are affected and the curve is of a rising type.
• When higher frequencies are involved curve becomes flat or a falling type
2. Speech audiometry
• Discrimination score is usually 55–85% between the attacks but discrimination ability is
much impaired during and immediately following an attack.
3. Special audiometry tests
• They indicate the cochlear nature of the disease and thus help to differentiate from retro
cochlear lesions, e.g. acoustic neuroma
• 4. Electrocochleography.
• Ménière’s disease, SP/AP ratio is greater than 30%
• 5. Caloric test

• 6. Glycerol test
• Glycerol is a dehydrating agent.
• When given orally, it reduces endolymph pressure and thus causes an improvement in
hearing.
 VARIANTS OF MÉNIÈRE’S DISEASE
• 1. Cochlear hydrops.
• Only the cochlear symptoms and signs of Ménière’s disease are present
• Vertigo is absent
• 2. Vestibular hydrops
• Patient gets typical attacks of episodic vertigo while cochlear functions remain normal.
• 3. Drop attacks (Tumarkin’s otolithic crisis)
• there is a sudden drop attack without loss of consciousness.
• There is no vertigo or fluctuations in hearing loss.
• 4. Lermoyez syndrome.
• Here symptoms of Ménière’s disease are seen in reverse order.
• First there is the progressive deterioration of hearing, followed by an attack of vertigo, at which time the
hearing recovers.
 Differential Diagnosis
• Labyrinthitis
• Otitis Media
• Sinusitis
• Lyme Disease
 Treatment
GENERAL MEASURES
• Reassurance
• Cessation of smoking
• Low salt diet
• Avoid excessive intake of water
• Avoid over indulgence in coffee, tea and alcohol
• Avoid stress and bring a change in lifestyle
• Avoid activities requiring good body balance

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B. MANAGEMENT OF ACUTE ATTACK
• Reassurance
• Bed rest
• Vestibular sedatives
• Vasodilators
• Inhalation of carbogen
• Histamine drip.

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C. MANAGEMENT OF CHRONIC PHASE
• Vestibular sedatives
• Vasodilators
• Diuretics
• Propantheline bromide
• Elimination of allergen
• Hormones

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 SURGICAL TREATMENT
1.Conservative 2.. Destructive
procedures. procedures

a) Decompression of endolymphatic sac Labyrinthectomy.

b) Endolymphatic shunt operation.
c) Sacculotomy
d) Section of vestibular nerve.
e) Ultrasonic destruction of vestibular labyrinth

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A 45 years old patient is complaining of sudden attacks of imbalance
for past 3 years, which last for more than 20 minutes and associated
with tinnitus. These episodes of imbalance and tinnitus occur now
more frequently and with increasing ear fullness.

1. What specific questions will you ask to reach the diagnosis?

2. Give differential diagnosis?
3. Give management plan of your diagnosis? (diagnosis and
treatment)
4. What complications can develop?
5. Write the prescription for your patient?

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