REVIEW ARTICLE

Millennie et al., JPHV 2021;2

DOI: 10.21776/ub.jphv.2021.002.01.5

MENIERE’S DISEASE
Helena Era Millennie1, Badrul Munir2, Zamroni Afif2, Ria Damayanti2, Shahdevi Nandar Kurniawan2
1Doctor Profession Study Program, Medical Faculty, Brawijaya University, Saiful Anwar General Hospital, Malang, Indonesia.
2Neurology Department, Medical Faculty, Brawijaya University, Saiful Anwar General Hospital, Malang, Indonesia.
Correspondence : [email protected]

Abstract
Meniere’s disease is a disorder of the inner ear resulting in symptoms of episodic vertigo, tinnitus, hearing loss and aural pressure.
Although the exact etiology is uncertain, it is associated with raised pressure in the endolymph of the inner ear (endolymphatic
hydrops). The diagnosis of Meniere's disease is based on the clinical setting of the patient. This disease usually presents with
unilateral ear symptoms but can be also bilateral. Meniere's disease attacks are usually random and episodic (approximately 6-11 per
year), with periods of remission that can last from months to years. Investigations are audiometry, electronystagmogram, vestibular
evoked myogenic potentials (VEMPs) and imaging.The management consist pharmacological and non pharmacological. Meniere's
disease is initially progressive but fluctuates unexpectedly. It is difficult to distinguish natural resolutions from treatment effects.

Keyword : Meniere’s disease, episodic vertigo, tinnitus, hearing loss and aural pressure.

INTRODUCTION first decade of diagnosis, however the patient continues to

experience a deterioration that makes this a chronic disease
Meniere's disease is characterized by spontaneous attacks of (4,5).
vertigo, fluctuating sensorineural hearing loss, aural pressure,
and tinnitus. Meniere's disease is one of the clinically ETIOLOGY
significant diseases because it has serious implications for the
affected person. Unanticipated attacks that keep repeating Genetic
will disrupt the patient's daily life, it can even bother the Several lines of epidemiological evidence support a genetic
patient's professional career (1). contribution in Meniere's disease, including (a) the higher
prevalence observed in the European population over other
DEFINITION ethnicities and (b) a strong familial aggregation found in
Europeans and South Koreans.
Meniere's disease is a disorder of the inner ear that results in
symptoms of episodic vertigo, tinnitus, hearing loss, and Two genes for the immune response have been associated
aural pressure (2). Clinically, fluctuating sensorineural with the development of hearing loss in Meniere's disease. In
hearing loss tends to be resistant to treatment (progressive), a separate study, the common variant rs11096955 in the
but the symptoms of episodic vertigo are usually quite TLR10 gene also influenced the development of
responsive to treatment (3). sensorineural hearing loss in patients with bilateral Meniere's
disease. The MIF gene mediates the production of pro-
EPIDEMIOLOGY inflammatory cytokines and increases autoimmune-mediated
nerve inflammation.
Meniere's disease occurs approximately 50 to 200 per
100,000 adults and is most common in between 40 and 60 Additional evidence for genetic contribution is also supported
years old. Several studies have suggested that the prevalence by a study conducted in four families with Meniere's disease
of Meniere's disease is slightly higher in women than in men, with an autoimmune background.
with a reported female: male ratio in the United States of Autoimmune
1.89: 1.30. Becker-Bense et al. hypothesized that the main The autoimmune mechanism involved in this disease is still
cause of this female predominance was because there was a uncertain. There are several hypotheses have been proposed
mixture of patients who had vestibular migraine which the to explain the development of autoimmune responses in the
symptoms is similar with Meniere's disease. In some inner ear: (a) a cross-reaction due to shared epitopes between
Meniere's disease patients, the symptoms of decreased inner ear proteins and microbial proteins, such as cochlin,
hearing function are the most significant symptoms in the

Article History:
Received: January 25, 2021; Accepted: February 17, 2021; Published: March 1, 2021

Cite As:
Millennie HE, Munir B, Afif Z, Damayanti R, Kurniawan SN. Meniere’s disease. Journal of Pain, Vertigo and Headache; 2021.1:18-21

Page 18 of 4
Millennie HE et al., JPHV 2021;1 Page 19 of 4

(b) collateral damage due to persistent levels of cytokines and dBHL). Usually, there is tinnitus and a persistent sensation of
chemokines that can trigger the reaction delayed immunity, aural pressure (2,8).
possibly explaining the relapsing / remitting course of
Meniere's disease, (c) intolerance to inner ear antigens, and DIAGNOSIS
(d) immunogenetic factors leading to a persistent
inflammatory. A strict clinical classification for the diagnosis of Meniere's
disease has been established by the American Academy of
PATHOPHYSIOLOGY Otolaryngology– Head and Neck Surgery (AAO-HNS). The
diagnostic criteria for Meniere's disease has been revised by
Attacks of episodic vertigo can be explained by two theories. the Classification Committee of the Barany Society and
The first is the rupture theory which states that an increase in resulted in 2 categories: Definitive Meniere's disease; two or
pressure within the scala media results in the rupture of more spontaneous vertigo attacks, each lasting 20 minutes to
Reissner's membrane. Endolymph and perilymph will come 12 hours, audiometric examination reveals low-to-moderate
together which in turn causes vertigo attacks. This theory is frequency fluctuation of sensorineural hearing loss in the
based on historical histological specimens of individuals with affected ear at least 1 time before, during, or after one vertigo
Meniere's disease (2). episode, fluctuating aural symptoms (hearing loss, tinnitus, or
The second theory is the drainage theory. Drainage theory aural pressure) in the affected ear, other causes excluded by
suggests that excess endolymph at the scala media will other tests. Possible Meniere's Disease; at least 2 episodes of
eventually flow rapidly from the cochlear duct, saccule and vertigo or dizziness lasting 20 minutes to 24 hours,
into the endolymphatic sinus. Once the capacity of the fluctuating aural symptoms (hearing loss, tinnitus, or aural
endolymphatic sinus is reached, the valve that separates the pressure) in the affected ear, other causes excluded by other
utricle and sinus is breached and the endolymph overflows tests (4,6,9–11).
into the utricle, stretching the crest of the semicircular canal The diagnosis of Meniere's disease was made clinically. This
causing vertigo (2). Furthermore, if there is blockage of the disease usually presents with unilateral ear symptoms but can
endolymphatic duct or Bast valve which is responsible for the be also bilateral. Meniere's disease attacks are usually
drainage function, it can cause endolymphatic hydrops. random and episodic (approximately 6-11 per year), with
Endolymphatic hydrops can also result from overproduction periods of remission that can last from months to years. Thus,
of endolymph (7) which can make a damage to the organs of the diagnosis of Meniere's disease is usually not made at 1
the corti (6). point in time, it may take months or even years to fully lead
to a definitive diagnosis. To maximize treatment, it is
CLINICAL MANIFESTATIONS important to differentiate Meniere's disease from other
independent causes of vertigo and present with hearing loss,
Symptoms of Meniere's disease are episodic vertigo, tinnitus, tinnitus, for example, BPPV, acute labyrinthitis, migraine
hearing loss, and aural pressure. The vertigo is typically which are responding to different treatments (4,12).
rotatory, makes the patient feeling weak and may be Furthermore, there is a tool to make a diagnosis, the Gibson
accompanied by nausea, vomiting and the less common 10 point score, where a score of 7 or more leads to a
symptom is diarrhea. Vertigo usually lasts more than 20 diagnosis of Meniere's disease (2).
minutes but rarely exceeds four hours and rarely has more Table 1. Gibson 10 point score (2)
than one attack within 24 hours. Between attacks, the balance
function subjectively returns to normal. Hearing loss, tinnitus Gibson 10 point score Score
symptoms and aural pressure are fluctuating. There are three Vertigo
Rotational vertigo 1
stages in Meniere's disease:
Duration of rotational vertigo > 10 minutes 1
Stage one Rotational vertigo associated with at least one symptom
1
Dominant symptom in the stage one is the attack of vertigo : hearing loss, tinnitus, or aural pressure
and usually occurs with severe intensity. Hearing levels Hearing loss
Sensorineural hearing loss 1
fluctuate, but recover to normal levels between attacks.
Fuctuating hearing loss 1
Tinnitus and aural pressure often improve or disappear Hearing loss or fluctuation associated with at least one
between attacks, with hearing function and balance tests often 1
symptom : vertigo, tinnitus, or aural pressure
normal. Tinnitus
Stage two Duration of tinnitus > 5 minutes 1
Fluctuative tinnitus associated with at least one
There continues to be repeated episodes of vertigo. Hearing 1
symptom : vertigo, hearing loss, or aural pressure
may still fluctuate but not fully recover to normal levels.
Aural Pressure
Tinnitus and aural pressure usually don't completely Aural pressure lasting >5 minutes 1
dissapear between attacks. Fluctuative aural pressure associated with at least one
1
symptom : vertigo, hearing loss, or tinnitus
Stage three INVESTIGATIONS
The patient may also experience a Tumarkin drop attack, in Audiometry
where the patient suddenly falls without warning, without
Audiometry is important to determine the hearing threshold
loss of consciousness and without neurological symptoms but
and monitor any fluctuation. Early hearing losses often
this is rare. Hearing level remains poor (threshold around 60
affects the lower frequencies, which suggests that
Millennie HE et al., JPHV 2021;1 Page 20 of 4

endolymphatic hydrops initially affects the apex of the Table 2. Differential Diagnosis of Meniere’s Disease (4)
cochlea2. This examination can occur for 30 minutes (4). Differentiation from
Condition Clinical Manifestation
Video- or electronystagmogram Meniesre’s Disease
It is not accompanied with
This examination is used to evaluate the vestibulo-ocular Benign
Vertigo due to a change hearing loss, tinnitus, or
reflex. This examination is performed in a dark room while paroxysmal
in head position that aural
positional
eye’s movement is recorded as warm or cold water or air is vertigo
lasts less than 1 minute fullness. Vertigo occurs in
added to each canalis acusticus externus. This examination short duration
The symptoms are often
can trigger vertigo and nausea and it can occur for one hour Vertigo lasting in a few permanent and do not
(4). minutes with other fluctuate, comorbid with
Vestibular evoked myogenic potentials (VEMPs) Stroke/ symptoms: nausea, dysphagia, dysphonia, or
ischemia vomiting, imbalance, other neurologic symptoms
The role of vestibular evoked myogenic potentials (VEMPs) and also visual blurring and signs, usually not
in the diagnosis of Meniere's disease is currently uncertain. and drop attacks accompanied with hearing
loss and tinnitus
However, there is some evidence that might support its use as
Duration of attacks may be
an objective test. Reflexes that occur in response are obtained The attack lasting for
shorter or longer than
through the sternocleidomastoid and orbital muscles due to Vestibular hours but can also
Meniere’s disease. Hearing
migraine lasting for minutes or
strong acoustic stimulation. This can be used as bone loss less likely. Patients
>24 hours
conduction or air conduction to stimulate the otolith. often have a migraine history
The symptoms of
Currently VEMP is more widely used to monitor muscle vestibular schannoma
function and the effect of intratimpanic gentamicin (13). such as chronic Chronic imbalance more
Vestibular imbalance, asymmetric likely than profound episodic
Imaging
schannoma hearing loss and Vertigo. Hearing loss does
Magnetic resonance imaging (MRI) of the inner ear after tinnitus. Vertigo can not typically fluctuate.
intratimpanic gadolinium injection may be performed for also occurs in this
disease
evaluation of visualization of endolymphatic hydrops.
Sudden severe vertigo Vertigo, nausea with hearing
Gadolinium will be perfused through the membrana tympani Labyrinthitis (>24 hours) with loss but it doesn’t occur
secundaria or membrana fenestrae cochleae or round window hearing loss episodic and fluctuating
membrane that allowing the examiner to distinguish the Acute vertigo that have
boundary between the endolymphatic space and the a long duration of
attack, can be
perilymphatic space. A delay of 4 hours is required after dual Vestibular
associated with nausea,
Vertigo, nausea without
dose gadolinium injection. Both ears can be assessed but neuritis hearing loss
vomiting, without
there is a risk of systemic toxicity due to the use of high hearing loss, tinnitus,
doses of gadolinium. On the MRI results, if the and aural fullness
endolymphatic duct is expanded by more than 33%, this can
be said to be endolymphatic hydrops. However, visualization Pharmacological
of endolymphatic hydrops is not required to determine The role of pharmacological agents is to reduce the frequency
Meniere's disease and MRI imaging should not be used to of vertigo attacks and reduce the associated ear symptoms:
replace the diagnostic criteria for Meniere's disease if all Betahistine
defined criteria are fulfilled (13).
Betahistine dihydrochloride is an oral drug that has been used
for the treatment of peripheral vertigo. This drug is a
DIFFERENTIAL DIAGNOSIS histamine analogue that is highly histamine H3 receptor
Before making a diagnosis of Meniere's disease, other antagonist and acts as a weak agonist at histamine H1
conditions must be excluded which can be seen in the Table receptors4. Betahistine is considered to prevent symptoms
2. due to its vasodilating effect on the inner ear. Based on
clinical experience, the use of Betahistine 48 mg for 3-6
months to prevent Meniere's attack can be recommended
MANAGEMENT (13).
Non Pharmacological Diuretic
Diet and lifestyle modification Diuretics are generally given as first-line therapy for
A low-sodium diet (1500-2300 mg / day) and high water Meniere's disease (13). Diuretics are believed to alter the
intake can prevent vasopressin release and help maintain electrolyte balance in the endolymph, thereby decreasing the
inner ear homeostasis. The AAO-HNS suggests caffeine endolymph volume. The most commonly prescribed diuretics
restriction because caffeine can induce endolymph volume are thiazides with or without potassium-sparing diuretics such
modification. Some assume that a low amount of caffeine, as triamterene. The second line is the carbonic anhydrase
such as 100 mg/ day, will not trigger Meniere's symptoms inhibitor such as acetazolamide. Contraindications to the use
(4,13). Patients are also advised to avoid alcohol and tobacco. of thiazides are patients with gout and potassium-sparing
In addition, stress is a known factor contributing to Meniere's diuretics are contraindicated in patients with acute or severe
attacks, so things need to be done to reduce stress (2). renal failure. Patients using diuretics should be monitored for
electrolytes and blood pressure (4).
Vestibular rehabilitation
Vestibular rehabilitation is exercise and physical maneuvers
to treat chronic balance disorders (4).
Millennie HE et al., JPHV 2021;1 Page 21 of 4

Corticosteroids operative if it fails on non-pharmacological therapy and

The mechanism of action of intratympanic steroid therapy is pharmacological therapy.
to stabilize the vascular endothelium and increase cochlear ACKNOWLEDGMENT
blood flow through anti-inflammatory effects, as well as
effects on cochlear ion and fluid homeostasis. None.
Methylprednisolone and dexamethasone are commonly
corticosteroids that are usually prescribed. CONFLICT OF INTEREST
Methylprednisolone penetrates the round window more easily
The authors declare that there is no conflict of interest.
and achieves a higher concentration in the endolymph after
intratympanic injection than dexamethasone. However,
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