1

Journal of Vestibular Research 25 (2015) 1–7
DOI 10.3233/VES-150549
IOS Press

Diagnostic criteria for Menière’s disease
Jose A. Lopez-Escameza,k,∗, John Careyb , Won-Ho Chungc, Joel A. Goebeld , Måns Magnussone,
Marco Mandalàf , David E. Newman-Tokerg, Michael Strupph , Mamoru Suzukii, Franco Trabalzinif andAlexandre BisdorffjaOtology and Neurotology Group CTS495, Department of Genomic Medicine, Centre for Genomics and Oncology
Research – Pfizer/Universidad de Granada/Junta de Andalucía (GENyO), PTS, Granada, Spainb
Department of Otolaryngology, Head and Neck Surgery, John Hopkins University School of Medicine, Baltimore,
MD, USAc
Department of Otolaryngology, Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan Univerisity
School of Medicine, Seoul, KoreadDepartment of Otolaryngology, Head and Neck Surgery, Washington University School of Medicine, Saint Louis,
MO, USAe
Department of Otolaryngology, University of Lund, Lund, Swedenf
Otology and Skull Base Department, Azienda Ospedaliera Universitaria Senese, Siena, Italyg
Department of Neurology, John Hopkins University School of Medicine, Baltimore, MD, USAhDepartment of Neurology and German Center for Vertigo and Balance Disorders, Ludwig-Maximilians
University, Munich, GermanyiDepartment of Otolaryngology, Tokyo Medical University, Tokyo, Japanj
Department of Neurology, Centre Hospitalier Emile Mayrisch, Esch-sur-Alzette, LuxembourgkDepartment of Otolaryngology, Hospital de Poniente, El Ejido, Almeria, Spain

Received 2 March 2014
Accepted 10 Feburary 2015

Abstract. This paper presents diagnostic criteria for Menière’s disease jointly formulated by the Classification Committee of the
Bárány Society, The Japan Society for Equilibrium Research, the European Academy of Otology and Neurotology (EAONO),
the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) and the Korean
Balance Society. The classification includes two categories: definite Menière’s disease and probable Menière’s disease. The
diagnosis of definite Menière’s disease is based on clinical criteria and requires the observation of an episodic vertigo syndrome
associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus and/or
fullness) in the affected ear. Duration of vertigo episodes is limited to a period between 20 minutes and 12 hours. ProbableMenière’s disease is a broader concept defined by episodic vestibular symptoms (vertigo or dizziness) associated with fluctuatingaural symptoms occurring in a period from 20 minutes to 24 hours.
Keywords: Vertigo, sensorineural hearing loss, tinnitus, vestibular disorders, Meniere disease

List of acronyms∗ Corresponding author: Jose A. Lopez-Escamez, Otology and
Neurotology Group CTS495, GENyO, -Centre for Genomics andOncological Research – Pfizer/Universidad de Granada/Junta de Andalucía, PTS Avda de la Ilustracion, 114 18016 Granada, Spain.
E-mail: antonio.lopezescamez@genyo.es.

AAO-HNS – American Academy Otolaryngology
Head and Neck Surgery
CCBS – Classification Committee of the Bárány Society
EAONO – European Academy of Otology and Neurotology
ICVD – International Classification of Vestibular Dis-

c 2015 – IOS Press and the authors. All rights reserved
ISSN 0957-4271/15/$35.00 

2

J.A. Lopez-Escamez et al. / Diagnostic criteria for Menière’s diseaseordersMD – Meniere’s disease
SNHL – Sensorineural hearing loss
MRI – Magnetic resonance imaging
TIA – Transient ischemic attacks

1. Introduction
Menière’s disease (MD) is a multifactorial disorder where the combined effect of genetics and environmental factors probably determine the onset of thedisease. It is associated with the accumulation of endolymph in the cochlear duct and the vestibular organs
in histopathological studies [1], although endolymphatic hydrops (EH) per se does not explain all clinicalfeatures, including the progression of hearing loss or
the frequency of attacks of vertigo [2]. Menière’s disease is a clinical syndrome that consists of episodes of
spontaneous vertigo usually associated with unilateralfluctuating sensorineural hearing loss (SNHL), tinnitus
and aural fullness. Cochlear symptoms may also occur between vertigo episodes. The episodes of vertigo
are usually much more common in the first years ofthe disease, but hearing loss and vestibular hypofunction show a great variability among patients, making
the phenotyping of MD difficult. A taxonomy of MD
subtypes is needed to help categorize individuals who
manifest what may be incomplete phenotypes. Moreover, subsets of patients have been described who develop bilateral SNHL and other co-morbidities suchas migraine, benign paroxysmal positional vertigo andsystemic autoimmune diseases, further complicating
the diagnosis and management in these cases.
The Bárány Society, which represents the international community of basic scientists, otolaryngologists, oto-neurologists, physical therapists and otherexperts committed to vestibular research, has set up aClassification Committee for an International Classification of Vestibular Disorders (ICVD). Individual disorders are defined by classification groups, which include otolaryngologists and neurologists from at least
three continents. The ICVD has been organized in 3
levels: signs and symptoms, vestibular syndromes anddiseases. As a first step and prerequisite for defining
major syndromes and diseases of the ICVD, the Classification Committee of the Bárány Society (CCBS) has
published a consensus on the definitions of vestibular
symptoms [3]. Further, a consensus document of the
Bárány Society and the International Headache Society was published to define the diagnostic criteria ofvestibular migraine [4]. Current ongoing works include
definition for vestibular signs such as nystagmus, benign paroxysmal positional vertigo, unilateral and bilateral vestibulopathy and vestibular paroxysmia.
Different efforts have been sought to define in thepast a consensus diagnosis for MD. The JapaneseSociety for Equilibrium Research proposed clinicalcriteria for diagnosis of MD in 1974 (Appendix 1).
The American Academy of Otolaryngology-Head andNeck Surgery (AAO-HNS) developed the guidelines
for diagnosis and therapy evaluation of MD in 1972
and revised them in 1985 and 1995 [5]. These guidelines have been widely used in clinical research, andnearly all studies published since 1995 are based onthese criteria (Appendix 2). Despite the great contribution of these guidelines to evaluate the effectiveness of
medical and surgical treatments for MD, no biologicalmarker for diagnosis or prognosis has been identified
for MD so far, but an evolving understanding of MD
and vestibular migraine – the most relevant differential
diagnosis – points out the need to update these criteria.
The clinical variability observed in MD makes it necessary to improve the precision to characterize clinical findings during and between the episodes of vertigo
and the recognition of a familial history of hearing loss
and episodic vertigo. These will lead to more homogeneous cohorts of patients to conduct clinical multicenter studies, including large-scale genomic studies. The
EAONO had also established a working group to prepare European guidelines for diagnosis and treatment
of MD.
The principal aim of this effort has been to developinternational consensus diagnostic criteria for MD aspart of the ongoing work of the CCBS to improve thequality of clinical data obtained from patients to facilitate collaborative studies.

2. Methods
This work forms part of an ongoing multi-year
project to develop an ICVD, which uses a structured
process for developing international consensus definitions for vestibular symptoms, syndromes, disorders,and diseases. This process, overseen by the CCBS, isbased on expert, multi-disciplinary committees withinternational representation developing diagnostic criteria for subsequent comment and refinement prior to
publication. These criteria are built on a critical appraisal of current best scientific evidence. A collaborative work among the Equilibrium Committee of

J.A. Lopez-Escamez et al. / Diagnostic criteria for Menière’s disease

the AAO-HNS, the Japan Society for Equilibrium Research, the EAONO, the Korean Balance Society andthe Bárány Society has been established for joint development of diagnostic criteria for MD. All definitions
are supported by notes, comments, and written discussion according to a template established by the CCBS
for ICVD. MD criteria were developed iteratively overa four-year period (2010–14) through discussion, presentation, and refinement. Two diagnostic categories
were considered: definite and probable MD. The primary adjustments to prior criteria were to clarify thenature of auditory symptoms and signs present for definite MD.
3. Criteria for diagnosis of Menière’s disease
Previously used terms: Menière’s syndrome, endolymphatic hydrops (EH)
Definite MD
A. Two or more spontaneous episodes of vertigo(1,2),
each lasting 20 minutes to 12 hours(3) .
B. Audiometrically documented low- to mediumfrequency sensorineural hearing loss(4,5) in one
ear, defining the affected ear on at least one occasion before, during or after one of the episodesof vertigo(6,7).
C. Fluctuating aural symptoms (hearing, tinnitus or
fullness) in the affected ear(8) .
D. Not better accounted for by another vestibular
diagnosis(9) .
Notes:(1) Vertigo is the sensation of self-motion when no
self-motion is occurring or the sensation of distorted self-motion during an otherwise normal
head movement [3]. Episodic dizziness and unsteadiness are not considered as criteria to define MD, although patients with MD can reportdizziness and unsteadiness at long term.
(2) Although most patients report entirely spontaneous spells of vertigo, some patients identify
dietary triggers, such as excessive consumptionof sodium or caffeine. Some patients can experience episodes of vertigo lasting seconds tominutes triggered by high intensity and lowfrequency sound (Tullio phenomenon) and bychanges in pressure. These episodes tend tooccur later in the disease, perhaps as a resultof advanced hydrops bringing the membranous
labyrinth in close proximity to the stapes footplate.

3(3) The duration of the episodes is defined by thetime that the patient has to be on rest and cannot
move. It may last less than 20 minutes or morethan 12 hours, but neither is a common finding, and other disorders should be consideredwhen such durations are noted [6]. When shortepisodes occur they are usually spontaneous.
Brief episodes triggered by positional changesof the head should suggest other causes suchas BPPV. The duration of an episode may bedifficult to define as patients may have residual
symptoms after an episode.
(4) The affected ear is determined by hearing loss
defined in criteria B. Low-frequency SNHL isdefined as increases in pure tone thresholds
for bone-conducted sound that are higher (i.e.,
worse) in the affected ear than the contralateral ear by at least 30 dB HL at each of two
contiguous frequencies below 2000 Hz. In casesof bilateral low-frequency SNHL, the absolute
thresholds for bone-conducted sound must be
35 dB HL or higher at each of two contiguous
frequencies below 2000 Hz. If multiple audiograms are available, demonstration of recovery
of low-frequency SNHL at some point in timefurther supports the diagnosis of MD. Bilateralsynchronous SNHL (symmetric or asymmetric)
can occur in some patients [7–9], although sucha pattern should raise concern for the possibilityof autoimmune inner ear disease [10] and when
progressing slowly over years, may favor migraine as an alternate explanation for the vertigo
episodes or a comorbid condition [11]. Bilateral
low-frequency SNHL can also been observed inearly stages of non-syndromic progressive deafness (DFNA6/14) due to mutations in the WFS1
gene, but vertigo attacks have not been associated with this group of mutations [12]. SNHL in
MD can also involve middle and high frequencies after several episodes of vertigo, leading to
pan-tonal hearing loss.
(5) Non-simultaneous onset of vertigo and hearing loss. Sensorineural hearing loss may antedate the onset of vertigo episodes by several
months or years. This clinical variant has beencalled “delayed hydrops” [13,14], but the preferred term should be delayed MD, since EH is apathological finding. Episodic vertigo may precede the onset of hearing loss by several weeks
or months, but tinnitus or aural fullness is usually associated with the first episode of vertigo.

4

J.A. Lopez-Escamez et al. / Diagnostic criteria for Menière’s disease(6) A temporal association between hearing loss
and the vertigo episode is sometimes observedby the patient, generally as a change in hearing
within 24 hours of the vertigo episode. Hearing loss typically fluctuates spontaneously in thefirst few years of the disease. After repeated attacks, the hearing loss may progress and become permanent, and the episodes of vertigo
may likewise no longer be related with the aural
symptoms.
(7) Attacks of sudden loss of vestibulo-spinal reflexes resulting in sudden falls or less often
latero-pulsion and lasting seconds or, rarely, a
few minutes can occur (so called drop vestibular
attacks, otolithic crises or Tumarkin’s otolithic
crises) [15,16].
(8) An increase in tinnitus intensity or aural fullness in the affected ear is often associated withthe episode of vertigo in the first years. Tinnitus may be persistent once the hearing loss has
become permanent.
(9) The differential diagnosis should include transient ischemic attack, vestibular migraine, vestibular paroxysmia, recurrent unilateral vestibulopathy and other vestibular disorders. MRI may
be required to exclude vestibular schwannoma
or endolymphatic sac tumor. Migraine, benign
paroxysmal positional vertigo and some formsof systemic autoimmune conditions are considered co-morbidities and do not explain the diagnosis of MD. See Comments for discussion of
specific differential diagnoses.
Probable MD
A. Two or more episodes of vertigo or dizziness,each lasting 20 minutes to 24 hours.
B. Fluctuating aural symptoms (hearing, tinnitus or
fullness) in the affected ear(1) .
D. Not better accounted for by another vestibular
diagnosis(2) .
Notes:(1) Fluctuating symptoms must be reported during
the vertigo episode. Sensorineural hearing loss
is usually found, but conductive or mixed hearing loss may be also observed in the first yearsof the disease. An increase in tinnitus intensity
or aural fullness in the affected ear is usually associated with the episode of vertigo in the firstyears.

Table 1
Differential diagnosis of Menière’s disease
Autosomal dominant sensorineural hearing loss type 9 (DFNA9)caused by COCH gene
Autosomal dominant sensorineural hearing loss type 6/14
(DFNA6/14) caused by WSF1 gene
Autoimmune inner ear disease
Cerebrovascular disease (stroke/TIA in the vertebrobasiliar system/bleeding)
Cogan‘s syndrome. Some cases may have recurrences.
Endolymphatic sac tumor
Meningiomas and other masses of the cerebellopontine angle
Neuroborreliosis
Otosyphilis
Susac syndrome
Third window syndromes (Perilymph fistula, canal dehiscence, enlarged vestibular aqueduct)
Vestibular migraine
Vestibular paroxysmia (neurovascular compression syndrome)
Vestibular schwannoma
Vogt-Koyanagi-Harada syndrome(2) The differential diagnosis should include transient ischemic attack, vestibular migraine andother vestibular disorders. MRI may be required
to exclude vestibular schwannoma or endolymphatic sac tumor. Migraine, benign paroxysmal positional vertigo and some forms of systemic autoimmune conditions are considered
co-morbidities and do not explain the clinicalsyndrome of MD. See Comments for discussionof specific differential diagnoses.

4. Comments
4.1. Epidemiology
The prevalence of MD is approximately 34–190 per
100.000 [17–19]. The age of onset ranges from thethird to the seventh decades of life with a small female
predominance. The odds of MD are greater for older
subjects (adjusted OR per 10-year increase: 1.5), whiteethnicity (OR 1.7) and severe obesity (OR 1.7) [19].
Furthermore, MD is associated with several comorbid
conditions such as arthritis (OR 1.8), psoriasis (OR1.8), gastroesophageal reflux disease (OR 1.5), irritable bowel syndrome (OR 2.1) and migraine (OR2.0) [19]. MD is characterized by a variable course,
and the development of cochlear and vestibular symptoms may take years in individual patients [20]. A listof disorders mimicking MD is presented in Table 1.

J.A. Lopez-Escamez et al. / Diagnostic criteria for Menière’s disease5

4.2. Endolymphatic hydrops (EH)

4.5. Autoimmune inner ear disease

Histopathological studies in human temporal bones
have found EH in most patients with MD [1,2]. The recent development of 3 T MRI with gadolinium chelate
has allowed the visualization of EH in 93% of ears
with symptoms attributable to MD, but also in 65% ofasymptomatic contralateral ears [20]. In addition, electrocochleography is a neurophysiological test that correlates with EH in the cochlea; however, the fluctuating
symptoms limit their applicability as diagnostic tool in
the early course of MD [21].

Autoimmune inner ear disease (AIED) is a syndrome of rapidly progressive, often fluctuating, bilateral SNHL over a period of days to months. Vestibular symptoms may be present in almost 50% of patients, and a systemic autoimmune disease coexists in
15%–30% of patients [37]. In some cases, AIED begins as sudden SNHL in one ear progressing rapidly
to the second ear with tinnitus and balance symptoms,
which may resemble MD [38]. Rare cases of otologic
syphilis and neuroborreliosis can present similarly.

4.3. Familial MD
4.6. Transient ischemic attack and stroke
Familial MD should be considered if at least one
other relative (first or second degree) fulfills all the criteria of definite or probable MD [22]. Familial MD isobserved in 8%–9% of sporadic cases in populationsof European descent [22,23]. It has been described in
Caucasians from United Kingdom [24], Brazil [25],Sweden [26], Finland [23,27], Germany [28] andSpain [23]. Although most families described havean autosomal dominant pattern of inheritance, familial MD shows genetic heterogeneity, and mitochondrial and recessive inheritance patterns are also observed in some families [22]. Mutations in DTNA
and FAM136A genes have been described in familial
MD [29]. A family history of SNHL, migraine or recurrent vertigo in several members of the family would
justify a familial investigation of these patients to confirm if any of them fulfill diagnostic criteria for MD.
4.4. Overlap with vestibular migraine
Some studies suggest that migraine is more common
in patients with MD than in healthy controls [30,31].
Patients with features of both MD and vestibular migraine have been repeatedly reported [32]. In fact, migraine and MD can be inherited as a symptom cluster [33]. Fluctuating hearing loss, tinnitus and auralpressure may occur in vestibular migraine, but SNHL
does not typically progress to severe hearing loss over
the years in migraine. When hearing loss develops investibular migraine, it is often bilateral [32] whereas
involvement of both ears from the onset is rare in
MD [34]. Although migraine headache and phonophobia may be present during MD attacks, the pathophysiological relationship between vestibular migraine and
MD is unclear. Some authors have suggested a common pathophysiology, perhaps linked to an ion channel dysfunction [35] and endolymphatic hydrops has
been reported in patients with vestibular migraine andauditory symptoms [36].

Combined audio-vestibular symptoms are also a
hallmark of transient ischemic attack (TIA) and stroke,
since the blood supply to the inner ear derives fromthe vertebro-basilar circulation [39]. Such symptoms
typically result from ischemia in the anterior inferior
cerebellar artery (AICA) territory [40,41], since theinternal auditory artery (IAA) most often arises as abranch of the AICA. However, the IAA can arise directly from the basilar artery in 15%–20% of individuals [42], so mixed vestibular and auditory symptoms
may be a harbinger of basilar artery occlusion.
Patients may present initially with transient or episodic symptoms (TIA) that culminate in stroke withacute permanent hearing loss, permanent vestibularloss, or both [43]. Transient dizziness, hearing loss,
and/or tinnitus may occur as a premonitory TIA preceding acute audio-vestibular loss due to AICA infarction in up to 42% of cases. [40,43] Although neurologic symptoms such as facial palsy or hemiparesis
can occur in patients with basilar strokes, some patients with partial syndromes can manifest with isolated audio-vestibular symptoms almost indistinguishable from MD [44]. Furthermore, TIA symptoms canculminate in isolated labyrinthine infarction (without
cerebellar or brainstem involvement) [45].
Most strokes after TIA occur within 90 days [45],but isolated vestibular symptoms have been reported
for up to two years or more preceding stroke. Therefore, great care should be taken in patients with symptoms that are very recent in total illness duration (<3 months–6 months, especially < 30 days), particularly if the total number of episodes is relatively small(e.g., < 5) or has followed an escalating temporal profile of episode frequency (“crescendo TIA”) [44]. Careshould also be taken in patients with known vascularrisk factors (including older age at first onset) and those6

J.A. Lopez-Escamez et al. / Diagnostic criteria for Menière’s disease

presenting to acute care settings such as the emergency
department. When clinically-important doubt exists,
appropriate diagnostic testing for stroke and TIA (e.g.,
MRI with magnetic resonance angiography) should beconsidered. The first episode of MD may not differ
from a TIA, and sudden SNHL associated with vertigo
may be caused by a labyrinthine infarction.

Acknowledgments
The Barany Society, the EAONO and Neuro+,
Berlin have supported the working meetings to develop
a consensus definition for MD. The authors are thankful for all the comments and suggestions received fromthe vestibular community worldwide.

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Appendix 1. Criteria for diagnosis of Menière’s
disease. Menière’s disease Research Committee ofJapan. 1974.
Conditions
1. Repeated attack of whirling vertigo.
Dizzy spells without specific cause which are accompanied by nausea or vomiting lasting several minutes to several hours. There may be some
episodes of nonwhirling dizziness included in theseries of whirling vertigo; mixed types of sponta-

7

neous nystagmus (horizontal and rotatory is observed in most cases during the attacks; in caseswith a single first attack, differential diagnosis
with sudden SNHL is specially important.
2. Fluctuating cochlear symptoms.
Tinnitus and hearing loss often show fluctuationsynchronous with the vertiginous attacks; many
patients complain of fullness in the ear and hypersensitivity to intense sound in the affected ear.
The hearing tests reveal a marked fluctuation of
the threshold of hearing in the low and middle
tone range, recruitment of loudness will be observed; usually only one ear is affected, however,bilateral involvement is not rare.
3. Exclusion of central nervous system involvement, VIIIth nerve tumor and other cochleovestibular diseases.
To exclude these other disorders, a through history, neurological examination and specific clinical examination including equilibrium function
tests, audiological tests must be performed sometimes it is necessary to follow the patient’s course
in order to obtain the required chronological information necessary for establishing the correct
diagnosis.
Diagnostic criteria
I. Definite Menière disease: Condition 1–3.
II. Suspicious or uncertain Menière disease 1 and 3
or 2 and 3.

Appendix 2. Menière’s disease diagnostic scale.
committee on hearing and equilibrium american
academy otolaryngology-head neck surgery. 1995.
Certain Menière’s disease. Definitive Menière’s disease plus histopathological confirmation
Definite Menière’s disease. Two or more definitve
spontaneous episodes of vertigo lasting 20 minutes or
longer, audiometrically documented hearing loss on
at least one occasion, tinnitus or aural fullness in thetreated ear. Other causes excluded.
Probable Menière’s disease. One definitive episodeof vertigo, audiometrically documented hearing loss on
at lest one occasion, tinnitus or aural fullness in thetreated ear. Other causes excluded.
Possible Menière’s disease. Episodic vertigo without documented hearing loss or sensorineural hearing
loss fluctuating or fixed with disequilibrium but without definitive episodes. Other causes excluded.