Meniere's Disease

An Evidence Based Approach to Assessment and Management

I. Syed, C. Aldren

Disclosures
Int J Clin Pract. 2012;66(2):166-170.

Abstract and Introduction

Abstract

Menire's disease (MD) is frequently over-diagnosed in both primary and

secondary care. This is unfortunate given the significant medical and social
implications of such a diagnosis. Difficulties may arise in differentiating the patient
with true MD from those individuals with less clearly defined disorders of cochleovestibular function. In this review, we suggest a practical evidence based
approach to assessment and management of the patient with MD.

What is Menire's Disease

Menire's disease (MD) is a condition characterised by recurrent attacks of
vertigo associated with tinnitus, a sense of fullness in the ear and a fluctuating
hearing loss. The condition was first described in the 19th Century by Prosper
Menire who suggested that the underlying abnormality lay in the inner ear. The
precise aetiology remains unclear.

What Causes Menire's Disease?

The pathology of MD is poorly understood. A popular suggestion is the theory of
endolymphatic space dilatation (endolymphatic hydrops). It is suggested that the
distension of the endolymphatic space may cause damage to hair cells and
vestibular neuro-epithelium. In advanced disease the primary histopathological
finding is endolymphatic hydrops with most of the distension seen in the cochlear
and saccular ducts. The cause of endolymphatic hydrops remains unclear and
whilst this theory is popular, histopathological evidence is sparse.
Some patients with the symptoms of MD have an underlying inner ear disorder
which may cause these symptoms. This is referred to as Meniere's syndrome and
causes include autoimmune vasculitis, syphilis, mumps and trauma. Therefore,
MD is sometimes defined as an idiopathic Menire's syndrome.

How Does Menire's Disease Present?

The symptoms of MD usually start in middle age but rarely in the elderly. It
probably affects men and women equally, although women present slightly more
often. There is a family history in about one patient in 20. The natural history is
that of a relapsing-remitting disease that eventually 'burns out'.
Patients with MD classically present with a history of recurrent episodic bouts of
severe rotational vertigo associated with hearing loss, a sensation of fullness in
the ear and tinnitus. A key feature to identify in the patient history is the
synchronicity of the vertigo with the other otological symptoms. The aural fullness
is reported in most patients and usually precedes the attack of vertigo. The
associated tinnitus is often rushing in nature and again may precede the vertigo.
The duration of the vertigo is highly significant and is between 20 min and 24 h
(most usually 12 h). The vertigo, often severely disabling, is frequently
associated with nausea and vomiting. Occasionally, in the later stages of the
disease, there may be episodes of acute postural imbalance where the patient
drops to the ground without warning. As these episodes are not associated with
vertigo they may be mistaken for cardiovascular instability and so-called 'drop
attacks' in the elderly. They are, however, never associated with loss of
consciousness.
Clinical signs are often only apparent during the attacks and cannot necessarily
be identified in the out-patient clinic. Horizontal rotatory nystagmus during attacks
is a consistent finding. Patients should be encouraged to video any abnormal eye
movements as they are rarely seen in the clinic during an attack.
There is typically a fluctuating low frequency sensorineural hearing loss which
recovers after the attacks. After recurrent attacks, there may be a persistent and
worsening hearing loss eventually leaving a severe sensorineural hearing loss
affecting all frequencies. The symptoms are initially unilateral but the second ear
may become involved, often many years later (figures in the literature vary from
9% up to 50%).[13]

How is Menire's Disease Diagnosed?

Menire's disease is a clinical diagnosis the certainty of which may be
augmented by a number of investigations.
The American Academy of Otolaryngology Head & Neck Surgery has proposed
some useful criteria for the diagnosis of MD which are summarised in Table 1.[4]

Definite MD is associated with two or more definitive episodes of vertigo with

hearing loss, plus tinnitus, aural fullness or both.
We would recommend that the diagnosis of MD should only be made when the
full AAO HNS criteria for 'definite' MD are met. Patients not meeting the full
criteria should be referred to a specialist clinic.
Table 1. Guidelines for classification of Menire's disease from the Committee on Hearing
and Equilibrium of the American Academy of Otolaryngology Head & Neck Surgery
Certain Menire's disease
Definite Menire's disease plus histopathological confirmation
Definite Menire's disease
Two or more definitive spontaneous episodes of vertigo 20 min or longer
Audiometrically documented hearing loss on at least one occasion
Tinnitus or aural fullness in the treated ear
Other causes excluded
Probable Menire's disease
One definitive episode of vertigo
Audiometrically documented hearing loss on at least one occasion
Tinnitus or aural fullness in the treated ear
Other causes excluded
Possible Menire's disease
Episodic vertigo of the Menire type without documented hearing loss, or
Sensorineural hearing loss, fluctuating or fixed, with disequilibrium but without definitive episodes
Other causes excluded

What Other Conditions Can Resemble Menire's Disease?

For the non-specialist, it is useful to be able to differentiate MD from other

common causes of vertigo. The key factors are the duration of vertigo and the
presence of co-existing symptoms.
Benign positional paroxysmal vertigo is associated with very short episodes of
vertigo which last around 1020 s unlike the longer episodes of MD. The
symptoms are positional, e.g. rolling out of bed, unlike MD which involves
spontaneous attacks. Diagnosis is easily confirmed by the DixHallpike positional
test.
Acute labyrinthine dysfunction ('labyrinthitis' or 'vestibular neuronitis') is
associated with acute spontaneous vertigo. Typically a longer initial episode lasts
hours to days. There may be shorter episodes of vertigo resulting from
decompensation at a later stage.
The first presentation of MD may be perceived to be a bout of acute labyrinthine
dysfunction. However, the patient with acute labyrinthine dysfunction is often
unsteady for a number of days unlike the patient with MD who usually has normal
balance in between attacks of vertigo. Significantly there is no hearing change or
the typical synchronous otological symptoms of MD. Migraine associated
dizziness/vertigo is a recognised phenomenon associated with a clear history of
headaches with aura and visual symptoms e.g. zig-zag lines across the visual
field. There is usually no history of aural fullness or hearing impairment unlike
MD.
Brainstem pathology may also masquerade as Meniere's disease and can
present with ataxia, disorders of ocular motility and sensory dysfunction.
A thorough history and examination (in particular neurological examination) is
important to identify conditions like multiple sclerosis which can present like MD
in the early stages.

Investigations
Pure Tone Audiogram
All patients should have this basic formal hearing assessment. An initial pure tone
audiogram may be normal. Serial audiograms may confirm a fluctuating
sensorineural hearing loss typically in the lower frequencies which may progress
to leave a persistent sensorineural hearing loss across all frequencies.

Electrocochleography
An elevated summating potential (SP):action potential (AP) ratio is believed to
reflect endolymphatic hydrops and, by association, MD. Whilst previously popular
this technique has low sensitivity and has limited diagnostic use. A recent well
constructed retrospective case review compared SP/AP ratios in patients with
definite MD with a combined group of probable and possible MD. Overall only
59.8% had abnormally elevated SP/AP ratios and around 30% of patients with
clinically definite MD would not have abnormal electrocochleography results. [5]

Caloric Testing
Bithermal caloric testing identifies unilateral vestibular loss that occurs in MD.

Magnetic Resonance Imaging

We would advocate an MRI scan of the brain and internal acoustic meatus in all
suspected cases of MD to rule out any retrocochlear lesion as the cause for the
symptoms.

Blood Tests
There are no diagnostic blood tests for MD. We would suggest that blood tests
are considered on an individual patient basis depending upon specific features
that are raised in the history. Conditions like anaemia, hypothyroidism, diabetes
mellitus and vasculitic auto-immune diseases may be associated with MD
symptoms but these are rarely presenting features of the systemic disease.
Congenital or acquired syphilis may masquerade as MD and some would
advocate a reactive fluorescent Treponema antibody test on all suspected cases
of MD. There is no role for routine batches of tests.

How Do I Manage a Patient With a Diagnosis of Menires

Disease?
The primary goals of management are to provide the patient with (i) treatment for
the acute attacks and (ii) therapy to reduce the severity & frequency of attacks
(initial measures).
When the attacks are debilitating and refractory to these measures the goal is to
provide definitive (sometimes destructive) treatment to stop the vertigo as it is this

symptom which troubles the patient the most. These treatments involve ablation
of vestibular function with/without cochlear preservation (vestibular ablation).

Initial Measures
Anti-emetics The severe nausea or vomiting of MD may be treated with
vestibular sedatives e.g. buccal or intramuscular prochlorperazine. This should
be confined to treating acute symptoms only, as prolonged use can delay the
compensation process required for recovery and may produce extra-pyramidal
side effects.
There are no trials that investigate the efficacy of drugs to relieve the acute
symptoms of MD attacks.
Diet There is low level evidence (level IV) which suggests that salt loading
induces attacks in MD patients and that salt reduction to urinary sodium levels of
less than 50 mmol per day reduces the frequency of vertigo attacks in these
patients.[6,7]These studies also involved the use of diuretic therapy and there are
no high quality randomised trials that investigate the role of dietary salt restriction
in isolation.
It is worth explaining to the patient that a low sodium diet may be beneficial.
However, they should be made aware of the limited evidence for dietary salt
restriction as such an endeavour can impinge upon a patient's quality of life.
Severe salt restriction should not be recommended in patients on diuretic therapy
as this can result in significant electrolyte imbalance.
Although caffeine and alcohol reduction have been advocated in patients with
MD, there is no evidence base to suggest a role of either in
worsening/precipitating Menire's symptoms.
Self Management A recent well constructed randomised control trial examined
the effectiveness of booklet based education to manage the symptoms of MD.
[8]
Patients were randomised to three groups: vestibular rehabilitation booklet,
symptom control booklet or waiting list control. The symptom control booklet aims
to reduce the stress which exacerbates the severity of nausea and vertigo during
attacks using breath control and relaxation techniques. The vestibular
rehabilitation booklet helps the patient to design a programme of basic exercises
and activities designed to improve recovery.

Benefits observed in the booklet groups included reduced symptoms, anxiety,

handicap and negative beliefs about dizziness.
Diuretics Although widely used in the treatment on MD a recent Cochrane
review found no good evidence for the use of diuretics. [9] Given the potential for
adverse effects with diuretic therapy, particularly in elderly patients, we would not
advocate the use of diuretics in the routine management of MD.
Betahistine Betahistine is commonly prescribed to patients with MD in primary
care. A recent Cochrane systematic review including six randomised controlled
trials concluded that there is not enough evidence to show whether it is helpful for
treating symptoms of MD.[10] Betahistine is a cheap well tolerated drug that has a
limited side effect profile. It is reasonable to try betahistine but if there appears to
be no improvement in the frequency of attacks it should be withdrawn.
Steroids The use of steroids in the management of MD has been extensively
investigated.
A recent Cochrane review identified a well constructed prospective double blind
randomised placebo controlled trial that showed that intratympanic injection of
dexamethasone under local anaesthetic resulted in significant improvement in
control of vertigo, tinnitus, aural fullness and hearing loss compared with controls.
[11,12]
Improvements in control of vertigo was also identified in two other smaller
prospective double blind randomised placebo controlled trials. [13,14] There are no
studies that investigate the efficacy of steroid therapy alone via any route versus
placebo for the treatment of MD symptoms.

Vestibular Ablation
When initial measures fail, it may be necessary to destroy the vestibular function
on the affected side in order to treat the debilitating vertigo attacks. It is important
to inform the patient that there is a risk of symptoms (vestibular loss and
sensorineural deafness) developing in the currently unaffected ear. If this does
occur, and the patient has no vestibular (and/or cochlear) function in the original
ear as a result of the disease or its treatment, they may suffer from a significant
disability.
Intratympanic Gentamicin The symptoms of intractable vertigo in MD patients
may be reduced by chemical ablation of the vestibular labyrinth. Damage to the
cochlea is dose related and gentamicin is more toxic for vestibular than cochlear

cells. Nevertheless, there is a substantial risk of sensorineural hearing loss with

this treatment.
Two recent randomised double blind placebo controlled trials suggest significant
improvement of vertigo symptoms following intratympanic gentamicin perfusion in
patients with unilateral active MD.[13,14]Strategies involving fewer and smaller
doses of gentamicin aim to limit dose-related damage to the cochlea as complete
ablation of function is not a pre-requisite to obtain good control of vertigo. [15]
Currently intratympanic gentamicin has a clear role in MD patients with
intractable vertigo who already have significant sensorineural hearing loss of the
affected side.
Vestibular Nerve Section Surgical section of the vestibular nerve whilst
preserving the cochlear nerve and hence the hearing, may be used to treat the
vertigo attacks of MD. The combined retrolabyrinthine-retrosigmoid vestibular
neurectomy (RRVN) is associated with a complete cure of vertigo in around 85%
patients with a loss of significant hearing in only 4% of patients. [16]
Labyrinthectomy Labyrinthectomy is a destructive treatment which may be used
to treat intractable symptoms of vertigo in patients who have failed conservative,
medical and other surgical options where there is non-serviceable hearing. Such
a decision needs careful discussion with the patients.

Other Treatments
Ventilation Tube Insertion Ventilation tube (grommet) insertion has historically
been a popular treatment option for MD patients especially prior to the use of
intratympanic steroids/gentamicin. Evidence to support any benefit above a
placebo effect is sparse but they may be used for intratympanic delivery of drugs
for which there is good evidence (see above).
Pressure Treatment It has been observed that MD patients' symptoms may
improve in response to a relative over-pressure created in the middle ear.[17] A
Meniett low pressure pulse generator device has been devised that provides
positive pressure into the ear canal. There are two small multi-centred
randomised double blind placebo controlled studies investigating the use of the
Meniett in MD patients which show improvement of vertigo symptoms. [18,19] Larger
trials are required before pressure treatment can be advocated in the routine
management of MD.

Endolymphatic Sac Surgery (ESS) Whilst some trials cite high rates of
alleviation of intractable vertigo long term with low risk of severe sensorineural
hearing loss.[20,21] the evidence for benefit is equivocal and a small placebo
controlled trial comparing endolymphatic sac surgery with a sham operation
showed no differences in the control of vertigo attacks. [22]
A recent Cochrane review found a distinct lack of randomised controlled trials for
the surgical management of MD and concluded that there was no sufficient
evidence for a beneficial effect.[23] Given that the number of patients refractory to
all medical treatment and consequently considered for surgery is very small it
may prove difficult to obtain high level evidence in this field.

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