menieres disesse

NOT NEUROLOGY FOR NEUROLOGISTSPract Neurol 2009; 9: 157–162

Meniere’sdiseaseMohamed A Hamid

Meniere’s disease affects about 1% of the population. Typically it presents asepisodic vertigo, fluctuating hearing loss, tinnitus and aural fullness. Thehistory and physical examination are critical in making the diagnosis.Laboratory tests are useful in tailoring and following up patients. Treatmentoptions are limited and usually targeted towards reducing endolymphatichydrops to stabilise the hearing loss and reduce the vertiginous episodes.

Although there are several different

disorders of the inner ear, and many

are very common, they all present

with hearing loss, vertigo or both.

The prevalence of hearing loss in industrial

populations is about 10% and of vestibular

and balance disorders about 20–30%. The

common peripheral vestibular disorders

amenable to medical and rehabilitative treat-

ments are Meniere’s disease, vestibular neu-

ronitis, vestibular migraine, benign

paroxysmal positional vertigo (BPPV), motion

intolerance and visual induced vertigo

(motion sickness), and age-related balance

deterioration (presbystasis). Here I will focus

on the aetiology, histopathophysiology, clin-

ical presentation and management of

Meniere’s disease which is a common otolo-

gical problem characterised by episodic

vertiginous spells, hearing loss, tinnitus and

aural fullness (there is a rather arcane debate

whether these symptoms should be grouped

under Meniere’s disease or Meniere’s syn-

drome).

EPIDEMIOLOGYThe prevalence of Meniere’s disease is about

1% and it is more common in industrialised

countries and in adult white populations.1

There is a slight female preponderance and

the typical age of onset is 30–50 years.

Bilateral disease is not uncommon (15–20%),

but both sides are seldom affected simulta-

neously except in patients with autoimmune

disease.1–3 Genetic predisposition has been

reported in families with Meniere’s disease.4, 5

Factors such as diet,6 weather changes (low

pressure fronts),7 emotional and physical

stress,8 and urban living can precipitate

attacks and make any symptoms worse.

AETIOLOGY ANDHISTOPATHOLOGYProsper Meniere was the first to realise that

vertigo and hearing loss could be attributable

to a disorder of the inner ear as opposed to

‘‘brain congestion’’ which was the accepted

cause prior to Meniere’s published case

report.9 Seventy seven years later, the first

M A HamidClinical Professor of

Otolaryngology and Audiology,

Founder and Director, The

Cleveland Hearing & Balance

Center, 29001 Cedar Road,

Cleveland, OH 44122, USA;

hamidma@clevelandhearingbalance.

com

157Hamid

www.practical-neurology.com

histopathological description of ‘‘endolym-

phatic hydrops’’ (fig 1) was simultaneously

and independently provided by Yamakawa,10

and Hallpike and Cairns.11 Since then the

histological entity of ‘‘endolymphatic

hydrops’’ has been associated with Meniere’s

disease, although not all patients with the

histopathology have the typical symp-

toms.12, 13 The conventional view is that

hydrops is caused by either increased produc-

tion of endolymph by the stria vascularis, or

decreased absorption by the endolymphatic

sac. Recently, however, it has been suggested

that hydrops is the result of dysfunction of

the spiral ligament fibrocytes, which inter-

feres with the recycling of K+ ions and results

in osmotic imbalance and expansion of the

endolymphatic compartment.14 Many factors

such as genetic abnormalities, infections,

allergies, autoimmune disorders, dietary,

endocrine and vascular problems could be

responsible for the development of hydrops

but the exact underlying cause is unclear.

NATURAL HISTORYThe natural history of Meniere’s disease is

poorly understood. Few studies1, 2, 15–19 have

had adequate length of follow-up (14 years19

and 21 years1) and sufficient numbers of

patients (53,16 11919 and 1612) to allow

reasonable conclusions about the natural

history of the syndrome. These studies also

underscore the difficulty in making the initial

diagnosis if the presenting symptom is just

hearing loss, tinnitus or vertigo, but not all of

the symptoms which make up the typical

presentation of ‘‘Meniere’s disease’’. What we

do know is:

N Hearing deteriorates over time to about50 db loss in 60–80% of patients.

N Speech scores (understanding/clarity)drop with time to below 50% which isthe minimal level at which a hearingdevice can be used.

N Audiometry (pure tone audiograms) showvariable configurations such as lowfrequency rising or high frequency fallingpatterns.

N Vertiginous episodes vary between 6 and11 per year (average 4),1 and decline withtime.

Vestibular testing (mostly electronystag-

mography) shows progressive decline of the

caloric response, relative to the normal ear,

over five years, and various types of sponta-

neous and positional nystagmus during or

between episodes.

The treatments used in the studies of

prognosis did not appear to alter the natural

history of the disease. An often quoted paper

in otology circles which reviewed treatment

results over 25 years15 reported that most

studies described ‘‘improvement’’ in 20–30%,

‘‘failure’’ in 10–25% and ‘‘recovery ’’ in 60%.

Recovery meant reduction of vertiginous

episodes, and this was independent of any

treatment in most studies. However, this

report, and other studies, did not address

hearing recovery (it is important to note that

most studies of Meniere’s disease have

focused on vertigo despite the fact that the

final disability is hearing loss1, 20).

CLINICAL PRESENTATION ANDDIAGNOSISThe typical Meniere’s patient is a middle aged

adult presenting with rotational vertigo (with

nausea and vomiting) lasting 30 minutes to

hours, unilateral fluctuating hearing loss and

tinnitus. The hearing loss frequently affects

the low frequencies, especially early in the

disease. Tinnitus is often described as a harsh

Figure 1Cochlear hydrops: the endolymphatic

space (EL) volume increases and

compresses Reissner’s membrane (RM)

so reducing the vestibular

perilymphatic space (PL) which

ultimately leads to the aural pressure

and vertiginous episodes of Meniere’s

disease.

Not all patientswith thehistopathologyhave the typicalsymptoms

158 Practical Neurology

10.1136/jnnp.2009.176602

roaring machine-like sound, especially during

vertigo attacks. Patients also may have

hyperacusis (hypersensitivity to sounds) and

duplacusis (distortion of sound) in the

affected ear. In atypical forms, the initial

symptom can be tinnitus or hearing loss. The

time between the initial symptom and the

subsequent typical constellation of symptoms

varies from months to years. Therefore, in

taking the history, the physician should focus

carefully on the patient’s symptoms, any

otological history (for example, ear disease or

surgery), the general medical history, allergies

and medications in order to tailor appropriate

laboratory tests and narrow the differential

diagnosis (see below).

The diagnosis of Meniere’s disease is

mainly clinical, based on the history and

audiometry. Therefore, it is important that

clinicians use standardised diagnostic criteria.

Laboratory tests are obtained to support the

diagnosis and to tailor and monitor treat-

ments. Guidelines have been proposed by the

American Academy of Otolaryngology - Head

and Neck Surgery (AAO-HNS) for the diag-

nosis as possible, probable, definite and

certain according to the criteria shown in

the box.21 However, physicians continue to

use the terms cochlear hydrops, when vertigo

is absent, and vestibular hydrops for cases

without hearing loss, which are considered

variants of the disease. This is important,

especially in the differential diagnosis. For

example, patients with only cochlear symptoms

may have genetic or immune mediated hearing

loss while patients with only vestibular symp-

toms may have vestibular neuronitis, BPPV or

vestibular migraine. Other atypical presenta-

tions include the otolith crisis of Tumarkin

(drop attacks usually of late Meniere’s disease

with sudden falls to one side with no warning

at all, and not necessarily in the middle of a

typical Meniere’s episode) and the Lermoyez

phenomenon (improvement of hearing after

vertigo episodes).

EXAMINATIONPatients presenting with dizziness or vertigo

should have a general medical, neurotological

and neurological examination, including using

either Frenzel’s glasses or video goggles to

pick up eye movement abnormalities that

would otherwise be missed on routine

examination.22 The important components of

the neurotological examination are the ear

itself, neck movements, eye movements,

vestibular responses (vestibulo-ocular (VOR)

and vestibulo-spinal (VSR)), cranial nerves,

motor and sensory signs, and cerebellum.

N Ear examination is generally normal withno evidence of middle ear effusion orvascular lesions.

N Hearing loss and hyperacusis are oftenpresent in Meniere’s ears, especiallyduring, or around, the time of an attack.

N Neck movements should be normalexcept in the acute or subacute stagesof a Meniere’s episode when patientsself-restrict movements to reduce dizzi-ness or vertigo.

N Saccadic, pursuit and optokinetic eyemovements are usually normal with nodysconjugate movements or gaze-evokednystagmus.

N Spontaneous nystagmus observed afterremoving visual fixation by Frenzel glassor video goggles, post head shakenystagmus and positional nystagmusare all frequently present in the acuteor subacute stages of an episode. Most, ifnot all, types of central nystagmus are

Classification of Meniere’s disease based on the AAO-HNScriteria

Certain Meniere’s diseasel Definite Meniere’s disease, plus histopathological confirmation

Definite Meniere’s diseasel Two or more definite spontaneous episodes of rotational vertigo for

20 minutes or longerl Audiometrically documented hearing loss (uni- or bilateral) on at least one

occasionl Tinnitus or aural fullness in the affected earl Other causes excluded such as vestibular schwannoma

Probable Meniere’s diseasel One definite episode of rotational vertigol Audiometrically documented hearing loss (uni- or bilateral) on at least one

occasionl Tinnitus or aural fullness in the affected earl Other causes excluded

Possible Meniere’s diseasel Episodic vertigo of the Meniere’s type without documented hearing loss, orl Sensorineural hearing loss (uni- or bilateral), fluctuating or fixed, with

disequilibrium but without definite episodes of vertigol Other causes excluded

159Hamid


enhanced with visual fixation and there-fore can be readily seen with the nakedeye. Frenzel’s glasses or video-electro-nystagmography remove visual fixationand enhance the detection of peripheralnystagmus which is commonly sup-pressed by visual fixation.

N The vestibulo-spinal response is examinedby asking patients to stand up unassistedand walk for several steps watching forany gross gait abnormalities or sidestepping (abnormalities are more evidentduring an acute episode and less soduring subacute and inactive stages ofthe disease).

N Romberg’s sign is conducted with eyesopen and eyes closed with the armsoutstretched and observing postural swayand post stepping (Unterberger-Fukuda)rotation with eyes closed.

N The vestibulo-cerebellar examination isconducted by observing the vestibulo-ocular visual suppression response.

N The remainder of the neurological exam-ination is usually normal in Meniere’spatients. Positive neurological findings onexamination should direct the physician’sattention to the presence of centralnervous system disease.

DIFFERENTIAL DIAGNOSIS

N Migraine vertigo is more common thanMeniere’s and often poses a diagnosticchallenge.23 Of course, significant hearingloss or fluctuation are common inMeniere’s but nonetheless migraine maybe associated with mild low frequencyunilateral or bilateral hearing loss.However, migraine vertigo is oftendescribed as a chronic non-paroxysmalmotion sensitivity, lightheadedness ordisequilibrium rather than the rotatoryvertigo experienced in Meniere’s. Previoushistory and family history of migraine,associated nausea, photosensitivity, sen-sitivity to sounds and visual changessuggest migraine.

N Retrocochlear lesion and disorders of theotic capsule should be ruled out withimaging when clinically indicated. MRI isthe test of choice for vestibular schwan-nomas and demyelination plaques, andCT for cochlear otosclerosis and superiorcanal dehiscence.

N Bilateral Meniere’s disease can be idio-pathic or associated with syphilis orautoimmune diseases such as systemiclupus erythematosus and rheumatoidarthritis. Appropriate laboratory work upfor such disorders should be consideredalthough their yield is usually low.

DIAGNOSTIC TESTSIn the course of evaluating patients with

hearing and balance disorders, the tests that

are commonly considered include audiometry,

vestibular tests, blood tests and imaging (CT

of the temporal bone and MRI of the brain).

These need to be tailored according to the

history and physical findings.

Figure 2Early and late audiometric profile of

Meniere’s disease. In the early stage, a

low frequency rising pattern is evident

in the right ear (red circles). The peak

pattern (red dots) is evident later in the

disease. Also note that the left ear

exhibits a peak pattern (black X) in the

late stage suggesting bilateral rather

than just unilateral disease.

Figure 3(A) Audiogram of a patient presenting

with right hearing loss and episodic

vertigo. (B) Although the clinical history

was in favour of Meniere’s disease, the

MRI (T2-weighted) showed a well

defined demeyelinating plaque (arrow)

in the right cochlear/vestibular nuclei

of the brainstem.


10.1136/jnnp.2009.176602

In Meniere’s patients the most valuable test

is audiometry to document the type and

degree of hearing loss. Pure tone and speech

audiometry should be obtained in all patients.

Typically it shows a low frequency rising

pattern in the early stage of the disease but

patients can also present with peak or high

frequency profiles (fig 2). If asymmetrical

hearing loss is present, a contrast MR scan of

the brain and internal auditory canals is

needed to rule out a vestibular schwannoma

or brain stem lesion such as a demyelinating

plaque (fig 3).

Other tests such as electrocochleography

and electro- or video-nystagmography are

not usually needed in the early phase of

management. Electronystagmography should

however be considered in patients undergoing

intratympanic gentamicin treatment, labyr-

inthectomy or vestibular nerve section to rule

out subclinical disease (vestibular weakness)

in the contralateral inner ear.

Laboratory tests are seldom needed.

However, thyroid function, syphilis serology,

autoimmune and routine chemistry should be

done in patients presenting with bilateral

Meniere’s.

MANAGEMENTMost Meniere’s patients can be managed as

an outpatient; however, elderly dehydrated

patients may need to be admitted.

The most distressing symptom of an acute

attack is the vertigo which should be treated

symptomatically; 5 mg diazepam (Valium)

with 2 mg glycopyrolate (Robinul) is very

effective. Sublingual administration of 2 mg

lorazepam (Ativan) is also very effective in

controlling and shortening the duration of an

attack.

The long-term treatments for Meniere’s

disease vary considerably worldwide, the main

aim being to reduce the frequency of the

vertiginous episodes and the development of

increasing hearing loss:24, 25

N Diuretics and a low-salt diet (if patientsare salt sensitive based on their diaries)may decrease the natural progression ofsensorineural hearing loss.2, 25

N Systemic and intratympanic corticoster-oids have the potential for controllingvertigo and improve hearing, and offer anew treatment leading to significant

speech discrimination recovery, allowingfor hearing aid use in ears that wouldotherwise have become deaf.26

N The prevalence of allergy appears to behigher in Meniere’s patients than in thegeneral population, or the population ofpatients visiting an otological clinic forother symptoms; allergy therapy mayimprove vertigo and tinnitus.27

N Betahistine (B-Serc) is commonly used inEurope, Canada and many other coun-tries.24 It is believed that it exerts its effectby increasing the blood circulation to theinner ear and it also acts as a pre-synaptic agonist to histamine receptors. Arecent meta-analysis28 showed its efficacyin treating non- Meniere’s vertigo.

N Other treatments include pressure equal-ising tubes, pressure machines, laser,acupressure, acupuncture, hydrotherapy,oxygen therapy and herbal medicationsbut there are no randomised trials.

N Inner ear gentamicin perfusion is effec-tive in controlling vertigo and Tumarkindrop attacks.29–31

N A recent study comparing vestibularnerve section to intratympanic gentami-cin treatment concluded that both inter-ventions are acceptable for Meniere’sdisease patients; however, gentamicincaused more hearing loss, and vestibularnerve section controlled vertigo better.32

Meniere’s disease can be very disabling,

physically and emotionally, to patients and

their families. It is critical to address the

emotional impact of the disease and aggres-

sively treat secondary anxiety and depression.

It is also important to assess the impact of

the disease on the functional capacity of the

patient at home and work. Certain jobs can be

PRACTICE POINTS

l Meniere’s disease is an infrequent cause of hearing loss, tinnitus andepisodic vertigo.

l The cause is unknown, it is probably multifactorial, and the final commonhistopathology is endolymphatic hydrops of the stria vascularis.

l Meniere’s disease and vestibular migraine often overlap and must bedifferentiated for proper management.

l Meniere’s is a lifelong disease with good prognosis with early detectionand targeted treatments. It has significant morbidity if not adequatelytreated.

l In resistant cases, emotional and physical disabilities are prominent andmust be aggressively managed.

161Hamid


hazardous to Meniere’s patients such as flying,

policing and firefighting, construction jobs,

working with heavy machinery, and most jobs

requiring critical control of balance. Physicians

should have low thresholds for approving

permanent or temporary disability to a

Meniere’s patient, particularly bilateral cases.

ACKNOWLEDGEMENTSI am grateful to Dr Marc Friedman,

Neurotology Consultant, for his invaluable

review of this manuscript.

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