Ménière’s disease, also called endolymphatic hydrops, is a disorder of the inner ear where the

endolymphatic sys- tem is distended with endolymph. It is characterized by (i) vertigo, (ii)
sensorineural hearing loss, (iii) tinnitus and (iv) aural fullness.

PATHOLOGY

The main pathology is distension of endolymphatic sys- tem, mainly affecting the cochlear duct
(scala media) and the saccule, and to a lesser extent the utricle and semicircular canals. The
dilatation of cochlear duct is such that it may completely fill the scala vestibuli; there is marked
bulging of Reissner’s membrane, which may even herniate through the helicotrema into the
apical part of scala tympani (Figure 15.1). The distended sac- cule may come to lie against the
stapes footplate. The utricle and saccule may show outpouchings into the semicircular canals.

AETIOLOGY

The main pathology in Ménière’s disease is distension of endolymphatic system due to increased
volume of endo- lymph. This can result either from increased production of endolymph or its
faulty absorption or both. Normally, endolymph is secreted by stria vascularis, fills the mem-
branous labyrinth and is absorbed through the endolym- phatic sac (see p. 11 for inner ear
fluids).

The exact cause of Ménière’s disease is not yet known. Various theories have been postulated
(Figure 15.2).

1. deFectiVe Absorption by Endolymphatic Sac. Normally, endolymph is carried by the endolymphatic

duct to the sac where it is absorbed. Defective absorp- tion by the sac may be responsible for
raised endolymph pressure. Experimental obstruction of endolymphatic sac and its duct also
produces hydrops. Ischaemia of sac has been observed in cases of Ménière’s disease undergoing
sac surgery, indicating poor vascularity and thus poor ab- sorption by the sac. Distension of
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 over- activity resulting in spasm of internal auditory
artery and/ or its branches, thus interfering with the function of coch- lear or vestibular sensory
neuroepithelium. This is respon- sible for deafness and vertigo. Anoxia of capillaries of stria
vascularis also causes increased permeability, with transu- dation of fluid and increased
production of endolymph.

3. allergy. The offending allergen may be a foodstuff or an inhalant. In these cases, inner ear acts
as the “shock organ” producing excess of endolymph. Nearly 50% of patients with Ménière’s
disease have concomitant inhal- ant and/or food allergy.

It is possible that Ménière’s disease is multifactorial, resulting in the common end point of
endolymphatic hy- drops with classical presentation.
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. Such cases
benefit from thyroid replacement therapy.

6. autoimmune and Viral Aetiologies have also been suggested on the basis of experimental, laboratory
and clinical observations.

CLINICAL FEATURES
Age and sex. Disease is commonly seen in the age group of 35–60 years. Males are affected
more than females. Usu- ally, disease is unilateral but the other ear may be affected after a few
years.

Cardinal symptoms of Ménière’s disease are (i) episodic vertigo, (ii) fluctuating hearing loss,
(iii) tinnitus and (iv) sense of fullness or pressure in the involved ear.

1. Vertigo. It comes in attacks. The onset is sudden. Pa- tient gets a feeling of rotation of himself
or his environ- ment. Sometimes, there is feeling of “to and fro” or “up and down” movement.
Attacks come in clusters, with pe- riods of spontaneous remission lasting for weeks, months or
years. Usually, an attack is accompanied by nausea and vomiting with ataxia and nystagmus.
Severe attacks may be accompanied by other symptoms of vagal disturbances such as abdominal
cramps, diarrhoea, cold sweats, pallor and bradycardia. Usually, there is no warning symptom of
an oncoming attack of vertigo but sometimes the patient may feel a sense of fullness in the ear,
change in character of tinnitus or discomfort in the ear which herald an attack.

Some cases of Ménière’s disease show Tullio phenomenon. It is a condition where loud sounds
or noise produce ver- tigo and is due to the distended saccule lying against the stapes footplate.
This phenomenon is also seen when there are three functioning windows in the ear, e.g. a
fenestra- tion of horizontal canal in the presence of a mobile stapes.

2. hearing Loss. It usually accompanies vertigo or may precede it. Hearing improves after the
attack and may be

normal during the periods of remission. This fluctuating nature of hearing loss is quite
characteristic of the disease. With recurrent attacks, improvement in hearing during remission
may not be complete; some hearing loss being added in every attack leading to slow and
progressive de- terioration of hearing which is permanent.

• Distortion of sound. Some patients complain of distort- ed hearing. A tone of a particular

frequency may ap- pear normal in one ear and of higher pitch in the other leading to diplacusis.
Music appears discordant.

• Intolerance to loud sounds. Patients of Ménière’s disease cannot tolerate amplification of sound
due to recruitment phenomenon. They are poor candidates for hearing aids.
3. tinnitus. It is low-pitched roaring type and is ag- gravated during acute attacks. Sometimes, it
has a hiss- ing character. It may persist during periods of remission. Change in intensity and
pitch of tinnitus may be the warning symptom of attack.

4. sense oF Fullness or Pressure. Like other symp- toms, 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, the 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 acute attack. The quick component of nystagmus is towards the
unaffected ear.

3. tuning Fork Tests. They indicate sensorineural hear- ing 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 hear- ing loss. In early stages, lower frequencies are
affected and the curve is of rising type. When higher frequencies are involved curve becomes flat
or a falling type (Figure 15.3).

(A) Audiogram in early Ménière’s disease. Note: Hear- ing loss is sensorineural and more in lower
frequencies—the rising curve. As the disease progresses, middle and higher frequencies get involved and
audiogram becomes flat or falling type (B & C).

2. speech Audiometry. Discrimination score is usually 55–85% between the attacks but
discrimination ability is much impaired during and immediately following an at- tack.

3. special Audiometry Tests. They indicate the cochlear nature of disease and thus help to differenti-
ate from retrocochlear lesions, e.g. acoustic neuroma (Table 15.1).

(a) Recruitment test is positive.
 (b) SISI (short increment sensitivity index) test. SISI score is

better than 70% in two-thirds of the patients (normal

15%).
 (c) Tone decay test. Normally, there is decay of less than

20 dB.

4. electrocochleography. It shows changes diagnos- tic of Ménière’s disease. Normally, ratio of

summating po- tential (SP) to action potential (AP) is 30%. In Ménière’s disease, SP/AP ratio is
greater than 30% (Figure 15.4).

Electrocochleography. (A) Normal ear. (B) Ear with Mé- nière’s disease. Voltage of summating potential (SP)
is compared with that of action potential (AP). Normally SP is 30% of AP. This ratio is enhanced in Ménière’s
disease.

5. caloric Test. It shows reduced response on the af- fected side in 75% of cases. Often, it reveals a
canal pa- resis on the affected side (most common) but sometimes there is directional
preponderance to healthy side or a combination of both canal paresis on the affected side and
directional preponderance on the opposite side.

6. glycerol Test. Glycerol is a dehydrating agent. When given orally, it reduces endolymph pressure
and thus causes an improvement in hearing.

Patient is given glycerol (1.5 mL/kg) with an equal amount of water and a little flavouring agent
or lemon juice. Audiogram and speech discrimination scores are recorded before and 1–2 h after
ingestion of glycerol. An improvement of 10 dB in two or more adjacent octaves or gain of 10%
in discrimination score makes the test positive. There is also improvement in tinnitus and in the
sense of fullness in the ear. The test has a diagnostic and prognostic value. These days, glycerol
test is combined with electrocochleography.

VARIANTS OF MÉNIÈRE’S DISEASE

1. cochlear Hydrops. Here, only the cochlear symp- toms and signs of Ménière’s disease are present.
Vertigo is absent. It is only after several years that vertigo will make its appearance. It is
believed that in these cases, there is block at the level of ductus reuniens, thereby confining the
increased endolymph pressure to the cochlea only (Figure 15.5).

2. Vestibular Hydrops. Patient gets typical attacks of episodic vertigo while cochlear functions
remain normal. It is only with time that a typical picture of Ménière’s

disease will develop. Many of the cases of vestibular Mé- nière’s disease are labelled “recurrent
vestibulopathy” as endolymphatic hydrops could not be demonstrated in the study of temporal
bones in such cases.

3. drop Attacks (tumarkin’s Otolithic Crisis). In this, there is a sudden drop attack without loss of con-
sciousness. There is no vertigo or fluctuations in hearing loss. Patient gets a feeling of having
been pushed to the ground or poleaxed. It is an uncommon manifestation of Ménière’s disease
and occurs either in the early or late course of disease. Possible mechanism is deformation of the
otolithic membrane of the utricle or saccule due to changes in the endolymphatic pressure.

4. lermoyeZ Syndrome. Here symptoms of Ménière’s disease are seen in reverse order. First there is
progressive deterioration of hearing, followed by an attack of vertigo, at which time the hearing
recovers.
MÉNIÈRE’S DISEASE VS MÉNIÈRE’S SYNDROME

Ménière’s disease is an idiopathic condition while Mé- nière’s syndrome, though resembling
Ménière’s disease clinically (episodic vertigo, fluctuating hearing loss, tin- nitus and ear
fullness), results from a variety of condi- tions such as trauma (head injury or ear surgery), viral
infections (following measles or mumps), syphilis (con- genital or late acquired), Cogan’s
syndrome, otosclerosis or autoimmune disorders. It is also called secondary Mé- nière’s disease.

DIAGNOSIS OF MÉNIÈRE’S DISEASE

Committee on Hearing and Equilibrium of the American Academy of Otolaryngology—Head

and Neck Surgery (AAOHNS) classified the diagnosis of Ménière’s disease as follows:

1. Certain. Definite Ménière’s disease confirmed by histopathology. 


2. Definite. Two or more definitive spontaneous episodes of vertigo lasting 20 min or

longer.
 (a) Audiometrically documented hearing loss on at 
 least one occasion.

. (b) Tinnitus or aural fullness in the affected ear. 


. (c) All other causes excluded. 


3. Probable
 (a) One definitive episode of vertigo.
 (b) Audiometrically documented hearing
loss on at 
 least one occasion.
 (c) Tinnitus or aural fullness in the treated ear. (d) Other
causes excluded. 


4. Possible
 (a) Episodic vertigo of Ménière’s type without docu- 
 mented hearing loss
(vestibular variant) or

. (b) Sensorineural hearing loss, fluctuating or fixed, with disequilibrium but without
definitive epi- 
 sodes (cochlear variant). 


. (c) Other causes excluded. 


STAGING OF MÉNIÈRE’S DISEASE

This can be done in certain and definite cases of Ménière’s disease. It is based on the
average of pure tone thresholds at 0.5, 1, 2 and 3 kHz (rounded to the nearest whole) of
the worst audiogram during a period of 6 months before treatment (see Table 15.2).
TREATMENT
 A. GENERAL MEASURES
1. Reassurance. Patient anxiety can be relieved by reas- surance and by explaining the
true nature of disease. This is particularly important in acute attack.
2. Cessation of smoking. Nicotine causes vasospasm. Smoking should be completely
stopped. For some pa- tients, this may be the only treatment necessary.
3. Low salt diet. Patient should take salt-free diet as far as possible. No extra salt should
be permitted. Salt intake should not exceed 1.5–2.0 g/day.
4. Avoid excessive intake of water.
5. Avoid over-indulgence in coffee, tea and alcohol.
6. Avoid stress and bring a change in lifestyle. Mental relaxation exercises and yoga
are helpful to decrease stress.
7. Avoid activities requiring good body balance. As the attack of Ménière’s disease is
abrupt, sometimes with no warning symptom, professions such as flying, underwater
diving or working at great heights should be avoided.

B. MANAGEMENT OF ACUTE ATTACK

During the acute attack, there is severe vertigo with nausea and vomiting. Patient is
apprehensive. Head movements provoke giddiness. Therefore, treatment would consist
of:
1. Reassurance and psychological support to allay worry and anxiety.
2. Bed rest with head supported on pillows to prevent excessive movements.
3. Intravenous fluids and electrolyte administration to combat their loss due to
vomiting.
4. Vestibular sedatives to relieve vertigo. They should be administered intramuscularly
or intravenously, if vomit- ing precludes oral administration. Drugs useful in acute attack
are dimenhydrinate (Dramamine), promethazine theoclate (Avomine) or prochlorperazine
(Stemetil).

Diazepam (Valium or Calmpose) 5–10 mg may be giv- en intravenously. It has a tranquillizing

effect and also suppresses the activity of medial vestibular nucleus.

In some patients, acute attack can be stopped by atro- pine, 0.4 mg, given subcutaneously.

5. Vasodilators: Carbogen (5% CO2 with 95% O2) is a good cerebral vasodilator and its
inhalation improves labyrinthine circulation.

C. MANAGEMENT OF CHRONIC PHASE

When patient presents after the acute attack, the treat- ment consists of:

1.Vestibular sedatives. Prochlorperazine (Stemetil) 10 mg, thrice a day, orally for two months
and then reduced to 5 mg thrice a day for another month.

5. Vasodilators. Betahistine (Vertin) 8–16 mg, thrice a day, given orally, also increases
 labyrinthine blood flow by releasing histamine in the body. 


6. Diuretics. Sometimes, diuretic furosemide, 40 mg tablet, taken on alternate days with

potassium supple- ment helps to control recurrent attacks, if not con- trolled by
vasodilators or vestibular sedatives. Thi- azide diuretics (hydrochlorothiazide), 12.5 mg
daily can also be used. 


7. Propantheline bromide (Probanthine), 15 mg, thrice a day, can be given alone or in

combination with vas- odilator and is quite effective. However, they are not preferred by
many due to side effects. 


8. Elimination of allergen. Sometimes, a food or inhal- ant allergen is responsible for such
attacks. It should be found and eliminated or desensitization done. 


9. Hormones. Investigations should be directed to find any endocrinal disorder such as

hypothyroidism, and appropriate replacement therapy given. Control of stress by change
in lifestyle is important to prevent re- current attacks. 
 About 80% of the patients can be
effectively managed 


by medical therapy alone.

Intratympanic gentamicin therapy

(chemical labyrinthectomy)

Gentamicin is mainly vestibulotoxic. It has been used in daily or biweekly injections into the
middle ear. Drug is absorbed through the round window and causes destruc- tion of the
vestibular labyrinth. Total control of vertigo spells has been reported in 60–80% of patients with
some relief from symptoms in others. Hearing loss, sometimes severe and profound, has been
reported in 4–30% of pa- tients treated with this mode of therapy.

Microwick

It is a small wick made of polyvinyl acetate and meas- ures 1 mm × 9 mm. It is meant to deliver
drugs from external canal to the inner ear and thus avoid repeated in- tratympanic injections. It
requires a tympanostomy tube (grommet) to be inserted into the tympanic membrane

and the wick is passed through it. When soaked with a drug, the wick delivers the drug to the
round window to be absorbed into the inner ear. It has been used to deliver steroids in sudden
deafness and gentamicin to destroy vestibular labyrinth in Ménière’s disease.

D. SURGICAL TREATMENT
It is used only when medical treatment fails.

1. conserVatiVe Procedures. They are used in cases where vertigo is disabling but hearing is still
useful and needs to be preserved. They are:

(a) Decompression of endolymphatic sac.
 (b) Endolymphatic shunt operation. A tube is put,
connect-

ing endolymphatic sac with subarachnoid space, to

drain excess endolymph.
 (c) Sacculotomy (Fick’s operation). It is puncturing the

saccule with a needle through stapes footplate. A dis- tended saccule lies close to stapes footplate
and can be easily penetrated. Cody’s tack procedure consists of placing a stainless steel tack
through the stapes footplate. The tack would cause periodic decompres- sion of the saccule when
it gets distended. Both these operations were claimed to have shown good results but they could
not be reproduced by others and thus abandoned. Cochleosacculotomy is another similar
procedure in which, instead of saccule, cochlear duct is punctured and drained into the perilymph
(otic- periotic shunt). The procedure is performed with a curved needle passed through the round
window to puncture cochlear duct.

(d) Section of vestibular nerve. The nerve is exposed by ret- rosigmoid or middle cranial fossa
approach and se- lectively sectioned. It controls vertigo but preserves hearing.

(e) Ultrasonic destruction of vestibular labyrinth. Cochlear function is preserved.

2. destructiVe Procedures. They totally destroy cochlear and vestibular function and are thus used
only when cochlear function is not serviceable.

• Labyrinthectomy. Membranous labyrinth is completely destroyed either by opening through the

lateral semi- circular canal by transmastoid route or through the oval window by a transcanal
approach. This gives relief from the attacks of vertigo.

3. intermittent Low-Pressure Pulse Therapy [meniett DeVice Therapy (Figure 15.6)]. It is ob- served that
intermittent positive pressure delivered to inner ear fluids brings relief from the symptoms of
Mé- nière’s disease. Not only there is improvement in vertigo, tinnitus and ear fullness, but
hearing may also improve. Intermittent positive pressure waves can be delivered through an
instrument called Meniett device which has been approved by FDA. A prerequisite for such a
therapy is to perform a myringotomy and insert a ventilation tube so that the device when
coupled to the external ear canal can deliver pressure waves to the round window membrane via
the ventilation tube. Pressure waves pass

Mechanism of intermittent low pressure pulse therapy. Pressure waves pass through ventilation tube (1) to
round window mem- brane (2) and transmitted to perilymph (yellow) and compress endolymphatic labyrinth
(blue) to redistribute endolymph pressure to sac (3) and blood vessels (4).

through the perilymph and cause reduction in endo- lymph pressure by redistributing it through
various com- munication channels such as the endolymphatic sac or the blood vessels (Figure
15.6). Some believe they regulate secretion of endolymph by the stria vascularis.

Patient can self-administer the treatment at home. It may require a few months before complete
remission of disease is obtained. Meniett device therapy has been recommended for patients who
have failed medical treat- ment and the surgical options are being considered.