MedicineToday 2014; 15(3): 18-26

PEER REVIEWED FEATURE

2 CPD POINTS

Ménière’s
disease
A stepwise approach
MELVILLE DA CRUZ FRACS, MSc, MD

Key points Ménière’s disease is an uncommon cause of recurrent vertigo that

• Ménière’s disease is should be distinguished from other causes of balance disturbance such
characterised by recurrent
as vestibular migraine. Stepwise introduction of dietary restriction of
attacks of vertigo, fluctuat­ing
hearing loss, tinnitus and a sodium, lifestyle changes and medication can reduce the frequency and
sensation of aural fullness. severity of attacks in most patients.
• Attacks are sudden in onset,

M
typically accompanied by an
intense sensation of move­
ment, nausea, vomiting,
diarrhoea and sweating, and
can last hours.
• Treatment options for acute
attacks include ondanse­tron,
prochlorperazine and
diazepam.
• In most patients, attack
frequency and severity can
be reduced by a stepwise
approach of dietary restriction
of sodium, lifestyle changes
and medi­ca­­tion; surgical
treatment is rarely required.
• Referral to a specialist is
best for initial diagnosis and
evaluation.
• GPs have an important role
in ongoing management of
patients with Ménière’s
disease.
énière’s disease is characterised by
recurrent attacks of vertigo accom-
panied by fluctuating sensorineural
hearing loss, tinnitus and a sense of
aural fullness. Prosper Ménière in 1861 cor-
rectly attributed the attacks to a disorder of the
inner ear, suggesting that the mechanism of
causation could be similar to migraine or inner
ear vasospasm, a differential diagnosis which
is still relevant for the disease today.1
MÉNIÈRE’S DISEASE TODAY
The differential diagnosis of vertigo is broad,
often leading to all cases of vertigo being labelled
as Ménière’s disease. To clarify the diagnosis,
treatment and prognostication of patients with
Ménière’s disease, the American Academy of
Otolaryngology – Head and Neck Surgery
(AAO-HNS) has published guidelines for the
classification of Ménière’s disease (Box 1).2
Although the category of ‘certain Ménière’s
disease’ is used only as a research definition, as
it requires postmortem histological examina-
tion of the temporal bone, the other categories
are clinically useful.
CLINICAL FEATURES AND NATURAL
HISTORY
The hallmark of an acute Ménière’s attack is
prolonged vertigo. The vertigo is characterised
by a sudden unheralded intense sensation of
movement, most commonly rotation or spinning,
lasting at least 20 minutes and accompanied by
nausea, vomiting, diarrhoea and sweating. The
duration and character of the vertigo is impor-
tant in the diagnosis of Ménière’s disease
because episodes lasting a few seconds or min-
utes are more likely to be due to benign parox-
ysmal positional vertigo (BPPV) or, if longer
than eight to 12 hours, to vestibular n
­ euritis or
cerebellar stroke. Feelings of light-headedness,
pressure or vague disturbances of balance are
more likely to be due to non-otological causes,
such as postural hypotension, hyperglycaemia,
hyperventilation, panic attacks or anxiety.
In the early stages of Ménière’s disease,
patients may report only the vertigo symptoms,
as the fluctuations in hearing thresholds are
minor and may go unnoticed. However, as the
disease progresses the fluctuating hearing loss
(generally affecting low tones) becomes more

Associate
Copyright _Layout Professor
1 17/01/12 1:43da
PMCruz is an4Ear Nose and Throat Surgeon at Westmead Hospital, University of Sydney, and a
Page
Cochlear Implant Surgeon at Sydney Cochlear Implant Centre, Sydney, NSW.

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 1. CLASSIFICATION OF MÉNIÈRE’S
DISEASE BASED ON AAO-HNS
CRITERIA 2*

Certain Ménière’s disease

• Definite Ménière’s disease plus
­histopathological confirmation
Definite Ménière’s disease
• Two or more definite spontaneous
episodes of rotational vertigo for
20 minutes or longer
• Audiometrically documented hearing loss
(unilateral or bilateral) on at least one
occasion
• Tinnitus or aural fullness in the affected
ear
• Other causes excluded, such as v­ estibular
schwannoma
Probable Ménière’s disease
• One definite episode of rotational vertigo
© CHRISTY KRAMES, 2014
• Audiometrically documented hearing loss
(unilateral or bilateral) on at least one
balance control causes them to drop to the
occasion
ground without losing consciousness.
• Tinnitus or aural fullness in the affected
The natural history of Ménière’s disease is
ear
highly variable. Attacks of vertigo can occur
• Other causes excluded
daily in clusters over periods of several weeks
Possible Ménière’s disease or as isolated episodes interspersed with variable
• Episodic vertigo of the Ménière’s type periods of remission, sometimes lasting many
without documented hearing loss, or months or several years. In the later stages of
• Sensorineural hearing loss (unilateral or the disease, the episodes of vertigo tend to ‘burn
bilateral), fluctuating or fixed, with out’, and severe hearing loss dominates the
disequilibrium but without definite clinical picture. In long-term follow-up studies
episodes of vertigo of patients with Ménière’s disease, 30% to 45%
• Other causes excluded developed the disease in the contralateral ear
* American Academy of Otolaryngology-Head and Neck within a period of 30 years.
Foundation. Otolaryngol Head Neck Surg 1995; 113: 181-185.
There are many variants of classical Ménière’s
disease, in which vertigo precedes the fluctua-
marked, and a permanent hearing loss may tions in hearing loss. The reverse occurs in
persist between attacks (middle stages of the Lermoyez syndrome: hearing loss precedes
disease). Later in the disease course, the hearing vertigo and improves following vertigo attacks.
loss becomes permanent and nonfluctuating. Some patients experience a fluctuation in their
At this stage, the attacks of vertigo usually hearing thresholds (usually low tone) accom-
lessen, and hearing loss becomes the dominant panied by tinnitus and fullness but no vertigo.
symptom (‘burnt out’ Ménière’s disease). The This has been labelled ‘cochlear hydrops’.
sensation of tinnitus similarly may be less
noticed in the early stages, only to become more EPIDEMIOLOGY
prominent in the later course of the disease. Several epidemiological studies of Ménière’s
Late in the disease, patients may experience disease have been performed over the past few
drop attacks (caused by dysfunction
Copyright _Layout of
1 the ves-
17/01/12 decades
1:43 with
PM Page 4 widely contrasting results.
tibulospinal reflex), where a sudden loss of ­Estimated prevalence rates range from as low

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Ménière’s disease CONTINUED

late in the disease, and the responses to

Autoimmune disease
various treatments, particularly surgery
Allergic responses
to the endolymphatic system.
Obstructed endolymph drainage Vertigo
Although no single theory of Ménière’s
Excess endolymph production Endolymphatic Hearing loss
causation is accepted by all workers in
Autonomic imbalances hydrops Tinnitus
this field, a more contemporary concept
Viral infections Aural fullness
of the disease is that it represents a failure
Dietary deficiencies
of the complex inner ear homeostasis
Vascular irregularities
caused by any of a range of pathologies.
The symptoms during an attack can be
Figure 1. Proposed causation of Ménière’s disease.
more ­usefully viewed as a syndrome than
as a single disease entity. The inner ear
as 3.5 per 100,000 population to as high as In 1964, further histological studies changes leading to symptoms may be
513 per 100,000 population. The wide range showed evidence of ruptures of Reissner’s caused by a range of pathologies (see Fig-
is likely to result from methodological dif- membrane, one of the membranous divi- ure 1). They include autoimmune diseases
ferences, changes over time in criteria for the sions between the potassium-rich endo- (Cogan’s syndrome and systemic lupus
diagnosis of Ménière’s disease, difficulty in lymph and the perilymph of the cochlea. erythematosus), congenital rubella and
distinguishing Ménière’s d ­ isease from related The ensuing ‘rupture theory’ proposed other viral infections (possibly herpes
conditions such as migraine-associated that the acute mixing of potassium-rich simplex) of the inner ear (viral labyrinthi-
­vertigo, and differences in the populations endolymph with perilymph leads to tran- tis), syphilis, chronic ear disease, acoustic
surveyed. However, it is clear that Ménière’s sient hair cell dysfunction within the coch- neuroma, trauma, allergy and hormonal
disease is more common in women, and lear and vestibular end organs, resulting changes with the menstrual cycle. In most
the prevalence increases dramatically with in the attacks seen in Ménière’s disease. patients the cause is unknown.
age, peaking in the 60 to 69 years age On the basis of this theory, many different
group.3,4 It is very rare in people younger forms of surgery to alter the function of DIAGNOSIS
than 20 years. the endolymphatic system were designed, In the vast majority of cases, Ménière’s
with the aim of preventing pressure build disease can be diagnosed on the basis of
PATHOPHYSIOLOGY up within the inner ear and minimising an accurate history and a few relevant tests
History the membrane ruptures implicated in to exclude differential causes of recurrent
Before Ménière’s description of the disease attacks of Ménière’s disease. vertigo. Clinical examination of patients
in 1861 correctly attributed it to a disorder with Ménière’s disease often finds no
of the inner ear, vertigo attacks were Current theories abnormalities or at most shows evidence
thought to be due to a form of epilepsy.1 As the understanding of the complex of a unilateral sensorineural hearing loss
Ménière suggested the mechanism could physiology of the inner ear evolved and (or bilateral asymmetrical hearing loss in
be similar to migraine or vasospasm the outcomes of various surgical and med- the case of bilateral disease). Clinical tests
within the inner ear. Early treatments, ical treatments for Ménière’s disease were of balance such as Romberg’s test (stand-
including cervical sympathectomy and reviewed, it seemed less likely that the ing feet together with eyes closed) and
vasodilators such as nicotinic acid, were rupture theory could explain the attacks Unterberger’s stepping test (walking on
based on this understanding. of vertigo and fluctuating hearing loss. the spot with eyes closed) may show only
In 1938, the first reports of temporal Contemporary understanding of the a mild disturbance of balance. This is
bone histology in patients with Ménière’s radial and longitudinal endolymph flow because most patients are examined only
disease were published, showing an patterns in the healthy and diseased coch- between attacks, long after their symp-
increased fluid volume in the endolymph lea led to a theory in 1991 that Ménière’s toms have resolved. If there are associated
compartment of the cochlea, termed endo- disease is caused by episodic disturbances persisting neurological signs (cranial
lymphatic hydrops. It was assumed that of endolymph formation and resorption.5 nerve palsies or nystagmus) then other
increased endolymphatic volume led to the The theory encompasses current anatom- intracranial pathologies need to be
attacks of Ménière’s disease. However, fur- ical, physiological and pathophysiological considered.
ther histological analysis of human tempo- knowledge about the functions of the In the few cases where patients have
ral bones showed many examples of endo- cochlear and balance system. It also been observed during an acute attack, clin-
lymphatic hydropsCopyright
in patients with1 no
_Layout explains
17/01/12 thePage
1:43 PM stages
4 of Ménière’s disease, ical examination has varying results. In
clinical symptoms of Ménière’s disease. including the occurrence of drop attacks general, patients appear quite unwell

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because of the unpleasant sensation of ver- First test Second test
Figure 2. Serial
Air conduction
tigo. They may be sweaty and pale, unable pure tone
Bone conduction [ [
to stand up safely, nauseated and violently audiograms of
vomiting. There may be horizontal nystag- 0 [ [ [ the right ear in a
mus that changes direction as the attack
progresses. Following an attack, patients [ [ [ [ patient with uni­

are left with a sense of ‘hangover’ for a 20 [ lateral Ménière’s

day or two before recovering to normal

[ disease showing
fluctuating
function.
Hearing loss (dB) 40 [ sensorineural ­
[ [ low-tone hearing
INVESTIGATIONS loss in that ear.
Pure tone audiogram 60
The most useful investigation for diagnos-
ing Ménière’s disease is a standard pure tone
audiogram. In classical Ménière’s disease, 80
this may show varying degrees of sensori-
neural hearing loss. Most commonly the
100
hearing loss affects low tones, although 250 500 1000 2000 4000 8000
other patterns are also seen (Figure 2). Frequency (Hz)
Serial audiograms recorded at intervals
during the progression of Ménière’s dis- Figure 3. A
ease may show fluctuations in the degree contrast-
of hearing loss. In the earliest stages of enhanced MRI
Ménière’s disease, an audiogram (typically scan showing a
recorded after the attack has abated) usu- large acoustic
ally appears normal. As the disease pro- neuroma (arrow)
gresses, the hearing loss becomes more in a patient who
marked with some degree of hearing loss presented with
persisting between attacks (middle stages low-tone hearing
of the disease). Later in the disease course, loss and a mild
the hearing loss becomes permanent and disturbance of
nonfluctuating. balance.

Vestibular function tests and

electrocochleography
Vestibular function tests are highly spe- be useful in confirming the diagnosis of demonstrated dilation of the endolymph
cialised tests of the balance system and are Ménière’s disease in atypical cases.6 compartments in some patients with
particularly useful in evaluating patients advanced Ménière’s disease.7 Further
whose cases are unusual, for example with Imaging studies refinements of these imaging techniques
an atypical clinical history or bilateral Imaging studies such as MRI and CT scans may allow a more definite diagnosis in
disease.6 Vestibular testing is mandatory are useful in excluding acoustic neuromas patients with Ménière-type symptoms,
before considering interventions that (Figure 3) and other intracranial pathol- and strengthen the evidence base for the
involve permanent ablation of vestibular ogies that disturb balance and hearing (e.g. many treatments available for patients with
function (e.g. surgery, labyrinthectomy or acoustic tumours, hydrocephalus and Ménière’s disease.
vestibular nerve division). Vestibular func- multiple sclerosis). At current diagnostic
tion tests are best ordered and interpreted resolutions, MRI imaging has no specific Other tests
by a specialist (a neurologist or ear nose findings to indicate the presence of endo- General haematological and biochemistry
and throat surgeon) with experience in lymphatic hydrops. However, there have tests show no specific abnormalities in
managing otological conditions. been recent advances in MRI imaging Ménière’s disease but are useful in evaluat-
An electrocochleogram,
Copyrightwhich records
_Layout using
1 17/01/12 intratympanic
1:43 PM Page 4 injections of gado- ing patients for other causes of vertigo. A
the hair cell responses to sound, may also linium contrast material, which have full blood count may show anaemia. Renal

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Ménière’s disease CONTINUED

distinguished by its response to antimi- that the two be distinguished. If doubt

2. SOME DIFFERENTIAL DIAGNOSES
graine medication (see below). exists, a trial of antimigraine management
FOR BALANCE DISTURBANCE
Simultaneous loss of vestibular and should be undertaken before considering
cochlear function is most commonly due irreversible interventions for Ménière’s
Nonvestibular (most likely
nonvertigo) to Ménière’s disease but occasionally can disease. Vestibular migraine was discussed
Anaemia have other causes. Herpes zoster causes in detail in a previous issue of Medicine
Anxiety pain, vertigo and hearing loss, with vesicles Today.8
Cardiac arrhythmias in the ear canal (and if accompanied by
Medication and other drug side effects facial palsy is termed Ramsay Hunt syn- MANAGEMENT
Panic attacks drome). Meningitis due to bacteria or fungi, Modern management of patients with
Postural hypotension carcinoma, lymphoma or sarcoid can cause Ménière’s disease aims to reduce the
vestibular and cochlear dysfunction asso- ­frequency and severity of symptoms and
Vestibular (with and without
hearing loss)
ciated with other cranial nerve lesions. to improve the quality of life of patients
Benign paroxysmal positional vertigo
Vasculitides, including some ear- and and their families. A flexible management
Cerebellar stroke
eye-specific syndromes such as Cogan’s strategy needs to be formulated for each
Ménière’s disease
syndrome and Susac’s syndrome (retino- patient and for different stages of the dis-
Migraine
cochleocerebral vasculopathy), and syphilis ease. Treatments are best considered from
Viral labyrinthitis
can mimic Ménière’s disease. Brainstem a symptom control viewpoint, leading to
lesions involving the vestibular nerve root a stepwise introduction of available ther-
or nucleus, such as multiple sclerosis, rarely apies depending on their toxicities and
function tests are important for patients cause a similar syndrome. ease of administration (Figure 4).
who may require treatment with diuretics In general, episodic vertigo can be con-
as part of their overall management. MIGRAINE AND MÉNIÈRE’S DISEASE trolled in most patients by current inter-
Over the past 25 years, awareness has ventions (70% controlled within two years
DIFFERENTIAL DIAGNOSIS increased that migraine can be associated of presentation), but it may take time to
Patients with disturbances of balance are with disturbances of balance, including establish a satisfactory treatment regimen.
extremely common in general practice, dizziness, imbalance and vertigo, with or In the advanced stages of Ménière’s disease,
with many of the underlying causes being without headache, mimicking early especially when it is bilateral, hearing loss
nonvestibular (see Box 2). The key to eval- Ménière’s disease. The overall prevalence has greater impact, often requiring pow-
uating the differential diagnosis of dis- of migraine in the general population is erful hearing aids or at times a cochlear
turbed balance lies in obtaining an accurate 13%, and a quarter of migraine patients implant. Tinnitus and the sensation of
history of the balance problem and asso- experience dizziness or vertigo along with aural fullness are more difficult to manage.
ciated symptoms (e.g. hearing loss, tinnitus other more typical migraine symptoms. Patients often habituate to these symp-
and a feeling of aural fullness). Descriptions Because the prevalence of migraine in the toms, but they can persist and remain
such as light-headedness, disorientation general population is far greater than that troublesome.
and floating are likely to have nonvestibular of Ménière’s disease, a patient presenting
causes, whereas a history of vertigo (a true with recurrent vertigo is much more likely Treatment of acute vertiginous
sense of movement, spinning, rocking or to have migraine-associated vertigo than attacks
tilting) is highly likely to be due to a dis- Ménière’s disease. The dominant symptom during acute
turbance of the vestibular system, including The distinction between vestibular attacks is vertigo. This is usually associated
Ménière’s disease. migraine and Ménière’s disease may not with intense nausea, vomiting, sweating
Further clues in the history, such as the be considered important in the early stages and sometimes diarrhoea. As the attacks
duration of the vertigo, its frequency (rate of Ménière’s disease as initial management are usually unheralded, it is wise to advise
of recurrence), positional elements, and strategies for the two disorders overlap (diet patients of strategies:
the association of hearing loss, tinnitus and lifestyle modification), and prescribed • to ensure their safety (especially
and aural fullness, allow Ménière’s disease medications generally have few side effects. while driving or working in situa-
to be distinguished from other vestibular However, in the later stages of Ménière’s tions of danger)
causes of vertigo such as BPPV, vestibular disease, when more invasive and irrevers- • to allow the attack to pass (which
neuritis and cerebellar haemorrhage.6 ible treatments are being considered (e.g. may take several hours) and recovery
Vestibular migraine can mimic
Copyright _Layoutearly gentamicin
1 17/01/12 1:43 PM or surgical
Page 4 labyrinthectomy or to ensue (often after a period of a day
Ménière’s disease but can usually be endolymphatic sac surgery), it is crucial or two of feeling ‘washed out’).

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Ménière’s disease CONTINUED

Complete destruction of the inner ear Figure 4.

3. SUPPORT SOCIETIES AND
Ladder of INFORMATION SOURCES FOR
Vestibular nerve section treatment MÉNIÈRE’S DISEASE
options for
Aminoglycoside injections
ive
ct preventing Support societies are an important
tr u
es attacks in source of information and reassurance
Surgery of endolymphatic sac rd
d /o patients with to patients and their families. They can
l an Ménière’s let patients and their families know that
Micropressure ca
r gi disease. they are not alone or helpless in the
Diuretics/Betahistine/ Su
challenge of managing their symptoms.
Corticosteroids
They commonly provide authoritative
ive guidelines on low-salt diets and
Low-salt diet t
r va exercise programs, and updates on
n se
Co new management strategies as they
become available. Much information of
a general nature is available from their
Once an attack is established, little can individual requirements can be helpful. A websites and regular newsletters.
be done to alter its natural course. Vestib- regular and vigorous exercise program is Support societies and sources of
ular suppressants with antinausea effects also useful in helping patients regain con- information on Ménière’s disease
(ondansetron, prochlorperazine) are fidence in their sense of balance. A regular include:
­useful. My preferred strategy is to use sub- brisk walk of 30 minutes, three to five days • Meniere’s Australia
lingual ondansetron wafers (4 to 8 mg each week, is achievable by most patients. (www.menieres.org.au)
three times daily), as these can be taken Formal vestibular exercises, conducted by • US National Institute on Deafness
despite intense nausea and have a rapid a physiotherapist, may be useful for those and Other Communication Disorders
onset of action. Diazepam 2 mg orally who need help designing a specific and (www.nidcd.nih.gov/health/balance)
twice daily for 24 hours is a reasonable sustainable training program. • UK Ménière’s Society
alternative. Rarely, hospital admission for Support societies such as Meniere’s (www.menieres.co.uk)
a severe or prolonged attack is necessary Australia (www.menieres.org.au) provide
for intravenous rehydration, especially for useful and accurate information for
elderly patients or during hot weather. patients about vertigo, hearing loss, tinni- processed and fast foods. Although food
tus and management of Ménière’s disease. may initially taste bland and unappetising,
Preventive strategies They can also provide reassurance for most patients who persist habituate to the
Education, lifestyle changes and patients and their families (see Box 3). new dietary conditions after a few weeks.
support societies Input from a dietitian or an NAS recipe
Patient education is an important part of Low-salt diet book can be useful. Reducing caffeine,
the treatment of Ménière’s disease. Knowl- A simplistic understanding of diet and caffeinated soft drinks, chocolate and alco-
edge about the disease, likely natural his- Ménière’s disease suggests that dietary salt hol intake seems to make a difference in
tory and treatment options and dispelling intake is related to the degree of endolym- some patients.
of myths surrounding treatment are phatic hydrops and influences the frequency
important to improve the quality of life of and severity of vertiginous episodes. Diuretics
patients and their families. Accurate infor- Although there is little formal evidence to Diuretics have been a mainstay for treatment
mation can help alleviate the feelings of support this view, there is anecdotal evidence of Ménière’s disease since the early 1900s.
frustration and helplessness experienced from clinical practice, with some patients Theoretically, the sodium loss produced by
by many patients at first diagnosis. reporting acute attacks of Ménière’s disease diuresis reduces the extracellular fluid in
Simple interventions such as regularity following a salt binge. the body and in turn the amount of endo-
with diet, sleep and exercise go a long way Sensible recommendations for dietary lymphatic hydrops. Hydrochlorothiazide is
to improving patients’ coping mechanisms. sodium restriction suggest a salt intake the most widely used diuretic, but frusemide,
Stress, both physiological and emotional, ranging from 1 to 2 g/day. In practice, this spironolactone and combination diuretics
plays a role in triggering attacks. Formal involves following a ‘no added salt’ (NAS) such as hydrochlorothiazide plus triamterene
stress managementCopyright
programs_Layout
delivered by diet,1:43
1 17/01/12 avoiding obviously
PM Page 4 salty foods, taking are also used. Careful monitoring of serum
a psychologist and tailored to patients’ note of food labelling and avoiding electrolyte levels is required. Despite the

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widespread use of diuretics, a recent Hearing aids and tinnitus inner ear from a portable pressure gener-
Cochrane review found that there is no good management ator. Its use is based on the observation that
evidence to support or reject their use in Later in the course of Ménière’s disease, pressure changes applied to the inner ear
patients with Ménière’s disease.9 hearing loss starts to become intrusive. result in improved vertigo control in
Unilateral hearing loss has limited impact patients with Ménière’s disease. A standard
Betahistine in quiet listening environments but ventilation tube (or pre-existing perforation
Vascular insufficiency to the inner ear has becomes significant with background of the ear drum) is required for a micro-
long been proposed as a mechanism for noise. Directional hearing and music pressure device to be used. A treatment
Ménière’s disease. Betahistine (a histamine appreciation are also impaired. Bilateral cycle takes a few minutes and is repeated
analogue) has been used in this context for hearing loss in bilateral Ménière’s disease three times a day. Several studies have sug-
decades with the aim of improving inner is more intrusive. Hearing aids can be use- gested beneficial vertigo control with min-
ear perfusion. Testing in animals suggests ful in this situation, but a particular chal- imal risk of complications.
that this improves blood flow in the stria lenge is the fluctuating hearing loss seen
vascularis of the cochlear duct. A recent in Ménière’s disease. This has been partially Aminoglycoside treatment
clinical trial in humans showed betahistine addressed with self-programming and For patients in whom disabling vertigo
to be useful in reducing the frequency and adjustable hearing aids, which allow continues to be the dominant symptom
severity of vertiginous episodes and to some patients to adjust the power and processing despite reasonable trials of medical man-
degree in helping tinnitus. There was no setting of the hearing aid to suit their cur- agement, chemical labyrinthectomy should
effect on hearing loss or aural fullness. rent hearing thresholds. If hearing loss is be considered. Gentamicin is the most
Betahistine has minimal side effects severe then cochlear implants are highly commonly used ototoxic agent. The aim
(gastric irritation) and the dose is easily effective in restoring hearing.10 of treatment is to greatly reduce hair cell
varied (8 to 32 mg/day), allowing it to be Tinnitus can be a particularly distress- function in the vestibular system of the
titrated against the frequency and severity ing symptom. Management strategies affected ear. Gentamicin is relatively ves-
of vertiginous attacks. An initial regimen include education, sound therapy, short- tibulotoxic but leaves the cochlear hair cells
of 16 mg twice daily is a useful starting term drug therapy with benzodiazepines intact, making it suitable for treatment of
point, with the dose reduced by half each or sedating antidepressants, tinnitus intractable vertigo in ears with functional
month. There is very little interaction with retraining therapy and cognitive behav- hearing. Gentamicin can be administered
other medications, making it safe for use ioural therapy.11 under local anaesthesia by a transtympanic
in older patients with Ménière’s disease. injection or via a grommet to the affected
Semi-invasive and surgical ear, thereby sparing toxicity to the unaf-
Corticosteroids treatments fected ear in unilateral disease or the inac-
Corticosteroid treatments for Ménière’s Grommets tive ear in bilateral disease.
­disease are a more recent intervention, based Several nonevidence-based surgical oper- A commonly used fixed-dose transtym-
on the possible autoimmune basis of the ations and ‘sham’ procedures have been panic protocol involves a single injection
disease and recent experience with use of used in the past with some benefit, most of gentamicin (40 mg in a 2 mL solution)
intratympanic corticosteroids to treat sudden likely because of a strong placebo effect. to the middle ear, with a second injection
sensorineural hearing loss. In addition to Most have now become historical, but four weeks later if the vertiginous episodes
their possible immune-modulating effects, insertion of grommets (tympanostomy continue. Most outcome studies of gen-
corticosteroids are likely to influence the tubes) is still used and may have a benefit tamicin report greatly improved vertigo
sodium and fluid dynamics of the inner ear on vertigo control and aural fullness. As it control. The effect on the vestibular hair
through their mineralocorticoid properties, is a simple procedure able to be performed cells is permanent and irreversible, with a
making them a theoretically attractive treat- under local anaesthesia, is temporary and small risk of associated hearing loss.
ment. However, few clinical trials have inves- has a low risk of complications (persistent
tigated the use of corticosteroids for vertigo perforation, otorrhoea) grommet insertion Endolymphatic sac surgery,
control to date. It may be reasonable to is worth considering. labyrinthectomy and vestibular nerve
administer a short course of oral cortico­ section
steroids (prednisone 1 mg/kg/day for 10 days) Micropressure therapy Surgery on the endolymphatic sac has been
or to consider intratympanic injection Micropressure therapy is a minimally inva- a major treatment for intractable vertigo.
­(dexamethasone 4 mg, single dose) for sive therapy for Ménière’s disease that uses However, more recent analysis of the out-
patients with Ménière’s disease_Layout
Copyright who experi- a soft
1 17/01/12 probe
1:43 PM inserted
Page 4 into the external ear comes of sac surgery have shown it to be
ence a sudden drop in hearing thresholds. canal. This delivers pressure pulses to the no better than placebo treatments (simple

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Ménière’s disease CONTINUED

changes can go a long way to improving

4. PRACTICE POINTS ON MÉNIÈRE’S DISEASE
patients’ quality of life.
Referral to a specialist is preferable to
• Ménière’s disease is characterised by recurrent attacks of vertigo accompanied by establish the correct diagnosis and to
fluctuating sensorineural hearing loss, tinnitus and a sense of aural fullness. It is ­formulate a treatment plan. Some practice
caused by a disturbance of inner ear physiology. points about Ménière’s disease are sum-
• The prevalence of Ménière’s disease increases with age, peaking at ages 60 to marised in Box 4.
69 years; it is rare under the age of 20 years.
• The hallmark of an acute Ménière’s attack is prolonged vertigo, which is characterised CONCLUSION
by a sudden unheralded intense sensation of movement, most commonly rotation or An attack of Ménière’s disease can be a
spinning, lasting at least 20 minutes. frightening experience for patients and
• The differential diagnosis of recurrent vertigo includes migraine-associated their families. It occurs suddenly and with-
vertigo, which is far more common than Ménière’s disease and should be strongly out warning. Later in the disease, hearing
considered in the initial assessment and treatment of patients with vertigo. loss and tinnitus can become intrusive,
• Management aims to reduce the frequency and severity of symptoms and to particularly in bilateral cases. In patients
improve the quality of life of patients and their families. A combination of lifestyle with classical Ménière’s disease, a careful
changes, medical and surgical interventions should be considered. history and a simple audiogram are usually
• Episodic vertigo can be controlled in most patients by current interventions
sufficient to make the diagnosis. A care-
(70% controlled within two years of presentation), but it may take time to establish
fully designed, stepwise treatment plan
a satisfactory treatment regimen.
involving dietary and lifestyle changes and
medication can stabilise the symptoms
and greatly improve patients’ quality of
mastoidectomy), leading to a decline in its This is usually because of insufficient resid- life.  MT
popularity as a treatment of Ménière’s ual vestibular function in longstanding
disease. end-stage disease. REFERENCES
Complete unilateral surgical deaffer- A current experimental intervention
entation of the vestibular system in the is the vestibular electronic implant. This A list of references is included in the website version
affected ears via labyrinthectomy or comprises an implanted device with three (www.medicinetoday.com.au) and the iPad app
­vestibular nerve section have also lost electrodes, one placed in each of the end version of this article.
popularity. Although highly effective in organs of the affected semicircular canals,
controlling vertigo, these procedures carry attached to a microprocessor (analogous COMPETING INTERESTS: None.
the risk of total hearing loss, facial nerve to a cochlear implant), which can sense
palsy and neurosurgical complications changes in head posture and position. The
related to entering the posterior fossa. aim of the device is to simulate vestibular Online CPD Journal Program
Surgical treatment of Ménière’s disease function during head movement with the
has been largely replaced by transtym- hope of improving balance control and
panic application of gentamicin, because quality of life in patients with end-stage
of the latter’s ease of administration and vestibular disease.
relative safety.
ROLE OF THE GP
Experimental treatments GPs have an important role in the man-
Management of active Ménière’s disease agement of patients with Ménière’s disease.
focuses on reducing the frequency and It is important to recognise the chronic
What are the characteristic features
severity of vertiginous attacks. This can be nature of Ménière’s disease and to differ-
of vertigo in Ménière’s disease?
satisfactorily achieved in most patients with entiate it from more common causes of
a combination of the interventions transient vertigo, such as BPPV, vestibular Review your knowledge of this topic and
described above. However, in some patients neuritis and vestibular migraine. Provid- earn CPD points by taking part in
MedicineToday’s Online CPD Journal Program.
with severe disease and particularly those ing education about the natural history of
with bilateral Ménière’s disease, disabling Ménière’s disease, ways of implementing Log in to
disequilibrium (poor balance)
Copyright persists
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4 (low sodium) and www.medicinetoday.com.au/cpd
between each cluster of vertiginous attacks. lifestyle (regular sleep and exercise)

26 MedicineToday x MARCH 2014, VOLUME 15, NUMBER 3

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 MedicineToday 2014; 15(3): 18-26

Ménière’s disease
A stepwise approach
MELVILLE DA CRUZ FRACS, MSc, MD

REFERENCES

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new theory of the attacks occurring in Ménière’s disease. In:
Kaufmann AI, ed. Inner ear surgery. The Hague (The Netherlands):
Kugler Publications; 1991. p. 17-23.
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with acute vertigo. Med Today 2012; 13(6): 25-32.
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Suppl 2009; 560: 5-8.
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Med Today 2012; 13(7): 16-22.

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