Meniere syndrome …

Douglas J Lanska MD MS MSPH

Neuro-Ophthalmology & Neuro-Otology

Updated 05.24.2024
Released 12.07.2004
Expires For CME 05.24.2027

Meniere syndrome

Author: Douglas J Lanska MD MS MSPH

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Meniere syndrome

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Introduction

Overview

The author discusses the clinical presentation, pathophysiology, diagnostic workup, and management of Meniere syndrome. Meniere disease can be confused with migraine-associated vertigo, and current diagnostic criteria may not adequately differentiate the two in all patients. Although some analytic epidemiologic studies have suggested an association between migraine and Meniere disease, more recent studies have not found an elevated incidence of migraine headache in patients with Meniere syndrome compared to the general population. A low-sodium diet and diuretics are considered the mainstays of treatment for Meniere disease. Ablative therapy is a second-line treatment approach for failures of medical therapy. Among the various forms of ablative therapy, the most common are intratympanic aminoglycoside administration and vestibular neurectomy.

Key points

	• The key clinical features of Meniere syndrome are recurrent episodes of spontaneous vertigo, fluctuating hearing loss, aural fullness, and subjective tinnitus, with a progressive loss of audiovestibular function. The vertiginous episodes typically last hours (with a range generally of 30 minutes to 12 hours), with residual nonvertiginous disequilibrium lasting possibly several days.
	• With otolithic crises (also called “otolithic catastrophe”), patients may report the sensation of being suddenly thrown to the ground without warning.
	• Audiometry is essential for diagnosis, staging, and monitoring.
	• Caloric tests and either electronystagmography or video nystagmography are recommended, particularly before and after surgical treatment or aminoglycoside therapy.
	• In at least two thirds of patients, vertigo is successfully controlled with medical management and the natural history of the disease, and many individuals reach a steady-state phase of the disease.
	• Most studies report a frequency of bilateral disease of between 10% and 33%.
	• A family history of Meniere disease is convincing (confirmed or probable) in nearly a quarter of patients, and genetic studies suggest that Meniere disease may be transmitted as an autosomal dominant trait with reduced penetrance and anticipation.
	• Staging is based on the average of pure-tone thresholds at 0.5 kHz, 1 kHz, 2 kHz, and 3 kHz of the worst audiogram in the 6-month period prior to treatment.
	• The aims of management of Meniere disease are to (1) reduce the number and severity of acute vertiginous attacks and their associated symptoms (eg, hearing loss and tinnitus), (2) alleviate chronic symptoms (eg, hearing loss and tinnitus), and (3) prevent progression of the disease and its associated symptoms (eg, hearing loss and tinnitus).
	• A low-sodium diet and diuretics are considered the mainstays of treatment for Meniere disease.
	• Ablative therapy is a second-line treatment approach for failures of medical therapy. Among the various forms of ablative therapy, the most common are intratympanic aminoglycoside administration and vestibular neurectomy.

Historical note and terminology

In 1861 French otologist Prosper Meniere (1799-1862) described a group of 11 patients, mostly young men in their 30s and 40s, with recurrent episodes of vertigo, deafness, and tinnitus with associated nausea, vomiting, gait ataxia, and falls (34; 158; 261; 09; 80; 222; 179; 06; 21; 163; 150).

Prosper Meniere (1799-1862)

French otologist Prosper Meniere (1799-1862) identified lesions of the vestibular labyrinth as a cause of vertigo and deafness. (Courtesy of the U.S. National Library of Medicine. Contributed by Dr. Douglas J Lanska.)

The intermittent vertigo attacks were accompanied by diminution of hearing and increased tinnitus. Only one case had mention of aural pressure and fullness, but Meniere did not include this as part of the clinical description. Over time, the hearing loss progressively worsened. He localized the responsible lesion to the peripheral vestibular apparatus and, specifically, the semicircular canals.

Meniere’s localization of responsible pathology to the semicircular canals was apparently based in part on a controversial autopsy (158; 08; 261; 222; 179). Meniere seemed to suggest hemorrhage into the semicircular canals as the responsible pathology, but subsequent historians have carefully reviewed his 1861 report (and an earlier published comment) and have concluded that Meniere was using a separate case of hemorrhage into the semicircular canals to support his contention that the disease was localized to the peripheral cochleo-vestibular system rather than the central nervous system (179).

In 1874 French neurologist Jean Martin Charcot (1825-1893) labeled the disease “Maladie de Meniere” and noted that episodic symptoms ceased when the deafness became complete (34).

French neurologist Jean-Martin Charcot (1825-1893)

(Courtesy of the U.S. National Library of Medicine. Contributed by Dr. Douglas J Lanska.)

Surgical treatments for Meniere disease began in the 1900s. In 1904, Richard Lake at the Royal Ear Hospital in London performed a labyrinthectomy and radical mastoidectomy on a 21-year-old woman with a 5-year history of episodic vertigo, nausea, vomiting, deafness, and tinnitus. In 1928, American neurosurgeon Walter Dandy (1886-1946) described nine cases of intracranial auditory nerve section for Meniere disease: all of the cases survived, and all had symptomatic relief (49).

In 1938, endolymphatic hydrops was found to be the pathological correlate of Meniere syndrome (90; 178), a finding subsequently confirmed by other investigators (91; 92; 178). The initial discovery was made independently by English otologist Charles Skinner Hallpike (1900-1979) and Australian-born neurosurgeon Hugh Cairns (1896-1952) in England, and Japanese otolaryngologist Kyoshiro Yamakawa (c1891-1980) in Japan (178).

Meniere disease is generally defined as the idiopathic syndrome of endolymphatic hydrops, whereas the term Meniere syndrome is generally used for patients with the same clinical features but an identified cause. Other authors, however, use the term Meniere disease for both idiopathic and secondary cases (175; 176).

So-called delayed endolymphatic hydrops develops in an ear that was previously damaged, usually from a bacterial or viral infection. A number of variants of Meniere disease have also been proposed, including vestibular and cochlear Meniere disease (characterized, respectively, by isolated episodes of vertigo or hearing loss, without the complete Meniere syndrome); however, there are no clinical-pathologic studies correlating isolated vestibular and auditory disorders with selective endolymphatic hydrops of the vestibular and auditory labyrinth.

Clinical manifestations

Presentation and course

	• The key clinical features of Meniere syndrome are recurrent episodes of spontaneous vertigo, fluctuating hearing loss, aural fullness, and subjective tinnitus, with a progressive loss of audiovestibular function.
	• In Meniere syndrome, the vertiginous episodes typically last hours (with a range generally of 20 minutes to 12 hours), with residual nonvertiginous disequilibrium lasting possibly several days.
	• In Meniere syndrome, vertigo may be severe and prostrating, with associated autonomic manifestations (eg, nausea, vomiting, diaphoresis, pallor, hypertension, tachycardia).
	• In Meniere syndrome, hearing loss is classically described as fluctuating, particularly early in the disease course, but not all patients have fluctuating hearing loss.
	• In Meniere syndrome, the tinnitus is subjective, classically described as a low-frequency “buzz” or “roar,” but patients may develop a high-pitched component late in the disease.
	• In Meniere syndrome, aural fullness incorporates sensations of pressure, discomfort, or fullness in the ears.
	• With otolithic crises (also called “otolithic catastrophe” or "Tumarkin attacks"), patients may report the sensation of being suddenly thrown to the ground without warning.

The key clinical features of Meniere syndrome are recurrent episodes of spontaneous vertigo, fluctuating hearing loss, aural fullness, and subjective tinnitus, with a progressive loss of audiovestibular function. The vertiginous episodes typically last hours (with a range generally of 20 minutes to 12 hours), with residual nonvertiginous disequilibrium lasting possibly several days (171; 176; 157; 18; 19). About a quarter of patients have vertiginous attacks lasting less than 1 hour, 50% have attacks lasting 1 to 2 hours, and a quarter have attacks lasting more than 2 hours (176). Vertigo may be severe and prostrating, with associated autonomic manifestations (eg, nausea, vomiting, diaphoresis, pallor, hypertension, tachycardia). Hearing loss is classically described as fluctuating, particularly early in the course of the disease, but not all patients have fluctuating hearing loss. Furthermore, the hearing loss is characteristically a low- to midfrequency sensorineural hearing loss (18; 19). The tinnitus is subjective and classically described as a low-frequency “buzz” or “roar” (168), but late in the disease, patients may develop a high-pitched component. Aural fullness incorporates sensations of pressure, discomfort, or fullness in the ears.

Diagnostic criteria for the diagnosis of Meniere disease have been established by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology--Head and Neck Surgery (05) and subsequently by a consortium of 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 (see Tables 1 and 2) (77; 139; 140; 190). These criteria have evoked some criticism (85). Similar criteria have been established by other professional societies (157; 18; 19).

Table 1. Diagnostic Criteria for Definite Meniere Disease

	A. At least two spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours.
		1. Vertigo is considered to be a “sensation of self-motion when no self-motion is occurring, or the sensation of distorted self-motion during an otherwise normal head movement”.
		2. This does not include episodes of nonvertiginous dizziness and unsteadiness, brief or very prolonged episodes of vertigo (which should raise suspicion for other diagnostic possibilities), or provoked episodes of vertigo, such as with sound (Tullio phenomenon) or pressure (Hennebert sign). Provoked episodes of vertigo usually occur late in the course of the disease and are not part of the diagnostic criteria.
	B. Low-frequency (125 to 250 Hz) to medium-frequency (500 to 2000 Hz) sensorineural hearing loss, documented audiometrically.
		1. Low- to medium-frequency sensorineural hearing loss is defined as increases in pure-tone thresholds for bone-conducted sound that are worse in the affected ear than the contralateral ear by at least 30 dB HL at two contiguous frequencies below 2 KHz.
		2. In cases with bilateral low-frequency sensorineural hearing loss, the absolute thresholds for bone-conducted sound must be 35 dB or higher at two contiguous frequencies below 2 KHz.
	C. Fluctuating aural symptoms (ie, hearing, subjective tinnitus, or fullness) in the affected ear(s).
		1. Subjective tinnitus may become persistent once hearing loss has become permanent.
	D. Not better accounted for by another vestibular diagnosis.

Table 2. Diagnostic Criteria for Probable Meniere Disease

	A. At least two spontaneous episodes of vertigo, each lasting 20 min to 24 hours.
	B. Fluctuating aural symptoms (ie, hearing, subjective tinnitus, or aural fullness) in the affected ear(s).
		1. Fluctuating symptoms must be reported during the vertigo episodes.
		2. Hearing loss is typically sensorineural, but conductive or mixed hearing loss may be observed early in the disease.
	C. Not better accounted for by another vestibular diagnosis.

The diagnosis of Meniere disease must be made separately for each ear for a case to be considered bilateral Meniere disease.

Current diagnostic criteria probably do not represent patients with monosymptomatic presentation or an early stage very well (106), nor do they consider diagnostic modalities other than audiometry. Atypical presentations with normal vestibular function and hearing are diagnostic challenges that delay treatment initiation (95).

In patients with unilateral or asymmetric disease, the degree of low-frequency hearing loss (250 Hz to 1 kHz) in combination with auditory symptoms during attacks reliably identifies the ear with the greater extent of endolymphatic hydrops (87).

Other clinical features of Meniere syndrome and Meniere disease may include diplacusis (ie, perception of the same sound with different pitches in the normal and affected ear), recruitment of loudness (suspected when a patient complains that loud sounds are annoying or painful in the ear with poor hearing), a positive Romberg test (although the direction of fall may vary, either toward or away from the affected ear), a positive Fukuda marching test (with significant turning if the patient can stand with eyes closed), Tullio phenomenon (ie, sound-induced vertigo, sound-induced nystagmus, or both), Hennebert sign (ie, pressure-induced vertigo, pressure-induced nystagmus, or both elicited by insufflation of the external auditory canal) (165), a positive Dix-Hallpike test for positioning vertigo (in patients with secondary positioning vertigo), and Tumarkin otolithic crises (ie, drop attacks without warning, associated autonomic or neurologic symptoms, loss of consciousness, or postictal signs) (28; 13; 125; 242; 200; 120). With unilateral disease, the Weber tuning fork test usually lateralizes away from the affected ear, whereas the Rinne test usually indicates better air conduction than bone conduction. Downbeat nystagmus may occur during attacks of Meniere disease due to an asymmetry in the vertical vestibulo-ocular reflex or saccular dysfunction (126).

With otolithic crises (also called “otolithic catastrophe” or "Tumarkin attacks"), patients may report the sensation of being suddenly thrown to the ground without warning. These crises are thought to result from inappropriate stimulation of one of the otolith receptors, probably the saccule (28; 242). Such stimulation causes the brain to interpret the gravity vector as suddenly changing before visual and proprioceptive input can be assessed for accuracy, resulting in a sudden fall (28). Patients with Meniere disease who develop Tumarkin attacks are more disabled, have more severely impaired hearing in the asymptomatic ear (especially at low frequencies), and experience autonomic symptoms that are more severe and more frequent than those without Tumarkin attacks (189; 197). They are also more fearful and anxious, self-report greater impairment in workability, and are more likely to have to apply for work-related disability pensions than Meniere disease patients without Tumarkin attacks (197). Some patients with Tumarkin attacks can also have syncope (196; 198; 197; 120).

Meniere syndrome is a chronic disabling condition with major negative effects on physical and psychosocial functioning and quality of life (236; 121). Anxiety and depression are common in patients with Meniere disease (269; 121). Indeed, in a meta-analysis of 15 studies from eight countries involving 6587 patients, the prevalence of depression in patients with Meniere disease was nearly 50% (185).

Cluster analysis has been used to define subgroups of patients with unilateral Meniere disease (68):

	Group 1 (Sporadic, classic Meniere disease), affecting approximately half of patients (53%), without migraine or autoimmune disorder
	Group 2 (Delayed Meniere disease), affecting 8% of patients, is defined by hearing loss, which antedates the vertigo episodes by months or years, without migraine or autoimmune disorder “in most cases”
	Group 3 (Familial Meniere disease), affecting 13% of patients
	Group 4 (Meniere disease with comorbid migraine), affecting 15% of patients
	Group 5 (Meniere disease with comorbid autoimmune disease), affecting 11% of patients

There can be some misclassification between groups 2, 4, and 5, as the group 2 definition focuses on a symptom—hearing loss—which antedates the episodes of vertigo, ignoring comorbid conditions. As a result, some individuals with migraine or autoimmune disorders can be included in group 2 rather than in groups 4 or 5, which focus on comorbid conditions irrespective of the presenting symptom.

Meniere disease may also occur with comorbid benign paroxysmal positioning vertigo (BPPV). A meta-analysis found that the pooled frequency of BPPV in Meniere disease is 14% (95% CI 9% to 18%), but the pooled frequency was lower in cross-sectional studies (8%) than in longitudinal studies (38%) (119).

Prognosis and complications

Endolymphatic hydrops tends to worsen over time (263). Hearing thresholds at low frequencies (125 to 250 Hz) and medium frequencies (500 to 2000 Hz) are proportional to the extent of endolymphatic hydrops in the vestibule and cochlea. However, no significant relationship has been demonstrated between the extent of endolymphatic hydrops and other symptoms and signs (eg, high-frequency hearing loss, subjective tinnitus, and aural fullness).

Remissions and exacerbations are typical of Meniere disease, though the frequency of attacks and the severity of associated vegetative symptoms generally decline over a number of years (171; 152; 191). In at least two thirds of patients, vertigo is successfully controlled with medical management and the natural history of the disease, and many individuals reach a steady-state phase of the disease (40; 191). Still, some patients with Meniere disease can have frequent, severe attacks even 20 years or more after disease onset (97).

Estimates of the frequency of bilateral disease vary widely, in part depending on the sensitivity of the method of identifying contralateral disease (171; 70; 124; 180; 43; 35; 188). Most studies report a frequency of bilateral disease of between 10% and 33%, but a wider range is also reported—from 5% to 50% (69). Some studies suggest that individuals with disabling longstanding unilateral disease are unlikely to develop bilateral disease (187). However, the proportion with contralateral disease does increase with disease duration and may approach 50% after 20 years of follow-up (70; 176). Among individuals with bilateral disease, the metachronous form was seen in 76%, and it took a median of 7 years to become bilateral (188). In cross-sectional studies, the disease onset in patients with bilateral disease is earlier than in patients with unilateral disease (35). Psoriasis and a history of ear infection have been identified as key risk factors for developing bilateral Meniere disease (192).

Attempts have been made to identify subgroups of patients with bilateral Meniere disease. One scheme using cluster analysis defined five subgroups: (1) metachronic (ie, not synchronous) hearing loss, without migraine and without associated autoimmune disorders, representing about half of the cases; (2) synchronous hearing loss, without migraine and without associated autoimmune disorders, representing slightly less than a fifth of cases; (3) familial Meniere disease; (4) with associated migraine; and (5) with associated autoimmune disorders—with the latter three categories each representing about 10% of cases (69).

Staging of Meniere disease is based on the average of pure-tone thresholds at 0.5 kHz, 1 kHz, 2 kHz, and 3 kHz of the worst audiogram in the 6-month period prior to treatment (see Table 3) (05). Staging is only done on cases with certain or definite Meniere disease. For reporting purposes, the stage does not change if hearing changes after treatment.

Table 3. Staging of Meniere Disease

Stage	Pure-tone average (dB)
1 2 3 4	< 26 26 to 40 41 to 70 > 70

Disability, according to the Committee on Hearing and Equilibrium (05), is best measured by standard functional scale, with the patient making the final selection of disability level (see Table 4). The scale assesses the current state of overall function, not just function during attacks.

Table 4. Functional Level Scale for Meniere Disease

Level	Description
1	Dizziness has no effect on activities.
2	Episodic dizziness requires briefly stopping activities, but patient continues to work, drive, and participate in activities without restriction and has not changed plans or activities to accommodate dizziness.
3	Episodic dizziness requires briefly stopping activities, but patient continues to work, drive, and participate in activities without restriction, although he or she has had to change plans or activities to accommodate dizziness.
4	Patient is able to work, drive, care for family, and participate in most essential activities but must exert considerable effort to do so and must constantly make adjustments in activities and budget energy.
5	Patient is unable to work, drive, care for family, or participate in most activities previously engaged in. Even essential activities are limited.
6	Patient has been disabled for at least 1 year or receives disability payments because of dizziness or disequilibrium.

At each office visit, the following should be recorded: the frequency of definitive episodes for the previous 6 months, the pure-tone average (for thresholds at 0.5 kHz, 1 kHz, 2 kHz, and 3 kHz), word recognition scores, and the functional level (05).

Meniere disease is independently associated with an increased risk of injuries (262); medical or surgical treatment for Meniere disease does not reduce the risk of injury in these patients.

Biological basis

	• Endolymph is normally absorbed in the endolymphatic duct and sac.
	• Endolymphatic hydrops is thought to be the pathological substrate of Meniere syndrome, resulting from defects in endolymph absorption.
	• Endolymphatic hydrops refers to increased hydraulic pressure within the endolymphatic system of the inner ear, causing pathologic dilation of the endolymphatic system and an increased volume of endolymph.
	• Not all patients who have endolymphatic hydrops pathologically had clinical Meniere syndrome during life.

Etiology and pathogenesis

Endolymphatic hydrops. Endolymph is normally absorbed in the endolymphatic duct and sac. Endolymphatic hydrops is thought to be the pathological substrate of Meniere syndrome, resulting from defects in endolymph absorption. Endolymphatic hydrops refers to increased hydraulic pressure within the endolymphatic system of the inner ear, causing pathologic dilation of the endolymphatic system and an increased volume of endolymph. However, although endolymphatic hydrops is almost universally present on pathological examination of temporal bones from patients with Meniere syndrome who die, not all patients who have endolymphatic hydrops pathologically had clinical Meniere syndrome during life (203).

A critical review concluded that (1) the preponderance of evidence suggests that endolymphatic hydrops is responsible for some of the auditory symptoms of Meniere disease; (2) Meniere disease should be considered part of a larger spectrum of "hydropic inner ear disease" that also includes some cases without vertigo; and (3) endolymphatic hydrops is a hallmark defining feature of Meniere disease and a sensible target for diagnostic detection (76).

Etiologic factors. The etiology is probably multifactorial in most cases, with a genetic predisposition and environmental triggers, such as infection, cranial or otic trauma, otitis media, viral infection, syphilis, otosclerosis, etc. (81; 175; 72; 99; 146; 61).

Genetics. Most cases are sporadic, but familial aggregation has been recognized in European and Korean populations (138). The presence of familial cases suggests that shared environmental exposures or genetic factors are important in the etiology (99). A systematic investigation of the family histories of a large set of patients affected with Meniere disease found that a family history of Meniere disease was convincing (confirmed or probable) in nearly a quarter (23.4%) of the patients (99).

Genetic studies suggest that Meniere disease may be transmitted as an autosomal dominant trait with reduced penetrance and anticipation (07); a candidate region has been located on chromosome 5 (07). Genes associated with familial Meniere disease include COCH, FAM136A, DTNA, PRKCB, SEMA3D, and DPT (138). Rare deletions in the ERBB3 gene or large duplications covering the AP4M1, COPS6, MCM7, TAF6, MIR106B, MIR25, and MIR93 genes contribute to genetic variation in patients with severe tinnitus and Meniere disease (61).

In a study of the genetic etiology of Meniere disease using next-generation sequencing in three families, seven rare variants were identified to cosegregate with Meniere disease: one variant in the CYP2B6 gene in family I, one variant in GUSB and EPB42 in family II, and one variant in each of the SLC6A, ASPM, KNTC1, and OVCH1 genes in family III (229): dysfunction of CYP2B6 and SLC6A may predispose to Meniere disease via the oxidative stress pathway, where dysfunction of ASPM and KNTC1 may produce dysregulation of mitotic spindle formation.

The finding of rare mutations in TECTA, MYO7A, and OTOG genes and other genes (eg, CDH23, PCDH15, and ADGRV1) in the same families suggest that the integrity of the stereocilia and their interaction with the tectorial and otolithic membranes may be involved in the pathophysiology of familial Meniere disease (48; 67; 182; 183). A heterozygous likely pathogenic variant in the OTOG gene (rs552304627) was found in two unrelated families (206; 141).

Immunology. Immunologic contributions have also been proposed based on higher rates of autoimmune disorders (eg, rheumatoid arthritis, systemic lupus erythematosis, ankylosing spondylitis, atopic dermatitis, vitiligo) in cases than controls (53; 74; 88; 142). At least two inflammatory mechanisms have been involved in Meniere disease: (1) a proinflammatory immune response mediated by interleukin-1 beta, tumor necrosis factor alpha, and IL-6; and (2) a nuclear factor-kappa B-mediated inflammation in the carriers of the single-nucleotide variant rs4947296 (138).

Membrane rupture theory. In the 1960s and later, Schuknecht proposed the “membrane rupture theory” (216; 217; 218), which continues to be popular and has many adherents (201). According to this theory, episodes of vertigo in Meniere syndrome result from breaks in the pathologically dilated membrane that separates the potassium-poor perilymph from the potassium-rich endolymph. The resultant mixture of fluids bathes the vestibular receptors, causing acute depolarization and transient increased function, followed by depolarization blockade and transient loss of function. Symptoms gradually improve as the membrane is repaired or stabilized and sodium and potassium concentrations return to normal.

Problems have been noted with the “membrane rupture theory”: (1) careful pathologic studies have found no evidence of membrane rupture in a significant proportion of patients (176); (2) the initial symptoms and progression of symptoms do not correlate well with pathologically identified ruptures (176); (3) electronystagmographic monitoring has demonstrated that the initial nystagmus during an attack of Meniere syndrome is a paralytic nystagmus beating away from the affected ear, followed by an “irritative-like” secondary nystagmus beating toward the affected ear (154; 153; 33; 181), whereas the initial nystagmus according to the “membrane rupture theory” should be an irritative nystagmus beating toward the affected ear with later development of a paralytic nystagmus as the lesion heals (59). However, the problem is that the irritative nystagmus is brief and typically missed, and an additional phenomenon is not considered, ie, recovery nystagmus.

Irritative nystagmus. Although other labyrinthine disorders typically produce a "paralytic" nystagmus beating toward the "good" ear, in Meniere disease, there can be an excitatory phase with so-called "irritative" nystagmus beating toward the "bad" ear, followed by an inhibitory phase beating toward the "good" ear (14; 27). Meniere disease may, thus, present with nystagmus that reverses direction over a period of minutes, reflecting initial irritative nystagmus giving way to paralytic nystagmus.

Bery and Chang presented a case report with video documentation to document the serial changes in nystagmus characteristics in a patient with Meniere disease during and after an acute attack (27). A 65-year-old woman with right-sided Meniere disease had frequent vertigo attacks associated with fluctuating right-sided tinnitus, hearing loss, and vomiting for a period of 6 months. The attacks were spontaneous, occurred every 1 to 2 days, and lasted around 2 hours. She experienced one of these attacks during eye movement recording. She initially demonstrated right-beating nystagmus (that is, beating toward the affected ear), but after 2 minutes, the nystagmus gradually reversed and became left-beating (that is, beating toward the good ear). During the reversal, she had transient downbeat nystagmus. The left-beating nystagmus persisted for 2 hours. The case, including the excellent video documentation and supplementary materials, provides an excellent example of the changing character of nystagmus that can be seen in Meniere disease.

The very transient nature of the "irritative" nystagmus during the excitatory phase is one reason many clinicians doubted that an irritative component exists as part of Meniere disease.

Recovery nystagmus. So-called "recovery" nystagmus may also occur after a period of several days, again beating toward the "bad" ear as in the initial "irritative" nystagmus (154; 153). Recovery nystagmus occurs after a unilateral peripheral vestibular lesion, when nystagmus transiently reverses direction as some function is restored in the damaged labyrinth (154; 153). This reflects the effects of a restoration or partial restoration of peripheral vestibular inputs after compensatory central rebalancing of the vestibular nuclei has already occured.

Alternative theories. A number of alternative theories have been proposed, including Tonndorf’s concept that attacks are precipitated by mechanical membranous distortions resulting from sudden increases in endolymphatic pressure (243). In patients with endolymphatic hydrops, pressure fluctuations in the dilated endolymphatic system stress the nerve-rich structures of the inner ear, causing Meniere syndrome. The observed diphasic pattern of nystagmus is explained by (1) an initial disruption of ipsilateral vestibular function causing a paralytic nystagmus beating away from the affected side, (2) subsequent central adaptation dampening or resolving the initial paralytic nystagmus, (3) restoration of vestibular function with decline in endolymphatic pressure and resolution of mechanical membrane distortion, and (4) development of secondary “recovery nystagmus” beating toward the affected side as a result of the improved ipsilateral vestibular dysfunction after central compensatory mechanisms had partially or fully rebalanced the neural discharges from both sides (154; 153; 33; 181).

There is some evidence for the involvement of ion homeostatic or blood-labyrinthine barrier dysfunction in human temporal bones: immunohistochemistry staining to detect known protein expression related to ionic homeostasis and barrier function in the cochlea showed that expression of KCNJ10 was significantly reduced in all cochlear regions, including the stria vascularis, and CLDN11 and CLU expression was significantly reduced in spiral ganglion neurons (110).

Chamber configuration and progression of endolymphatic hydrops. Chamber configuration may be a factor controlling the progression of endolymphatic hydrops in Meniere disease (186). Considerable disparities in pressure-induced membrane stress exist among the vestibular chambers due to variations in membrane shape, size, and thickness (186). The thick and highly curved membranes in the semicircular ducts produce low stress levels, whereas the thin and relatively flat membranes in the saccule produce high stress levels (186).

Familial Meniere disease. Familial Meniere disease has been reported in 6% to 9% of sporadic cases, and mutations in various genes (eg, FAM136A, DTNA, PRKCB, SEMA3D, and DPT) and involvement of different structural proteins in the cochlear hair cell stereocilia have been implicated in single families, suggesting genetic heterogeneity (206; 182). One heterozygous likely pathogenic variant in the OTOG gene (rs552304627) was found in two unrelated families (206).

Mammalian cochlear hair cell stereocilia, showing proteins and their coding genes for different components (schematic)

Proteins and their coding genes are listed for the stereocilia links (tip links, horizontal top connectors, shaft connectors, and ankle links) and between the stereocilia and the tectorial membrane. In addition, proteins that a...

Epidemiology

• Meniere syndrome and Meniere disease are common disorders that are estimated to affect more than 2 million people in the United States.

Meniere syndrome and Meniere disease are common disorders that are estimated to affect more than 2 million people in the United States. According to some studies, males and females are affected about equally (171), whereas other studies reported that females are two or three times more likely than males to develop Meniere disease (35; 03; 115). Age of onset varies widely from 7 to 75 years of age, with onset typically from 30 to 60 years of age (171; 35; 252). Meniere disease is reported to have a prevalence of 200 to 500 per 100,000 (86). Although the reported prevalence of Meniere syndrome varies widely in different studies, the prevalence of Meniere syndrome generally increases with increasing age (03).

In South Korea, annual incidence rates increased rapidly from 2013 to 2017 and showed a seasonal pattern with higher incidence rates in summer and autumn than in winter and spring (115).

Putative underlying etiologies and attack triggers have been mostly identified anecdotally, or presumptively determined in case series, but a few analytic epidemiological studies have been performed. Emotional stress increases the probability of an attack during the next hour and possibly during the next 3 hours (230).

The frequency of migraine in Meniere disease is higher than in normal subjects (211). Moreover, the high frequency of migraine symptoms on the same side as the ear affected by Meniere disease suggests a shared pathophysiology in both Meniere disease and migraine (239).

A systematic review and meta-analysis of observational case-control studies using molecular-diagnostics assessed the role of viral infections in the etiology of Meniere disease: 14 studies involving 611 cases with Meniere disease and 373 controls were included (50). Most of the included data concerned the human cytomegalovirus, and meta-analysis of eligible studies showed that evidence of cytomegalovirus infection was associated with an approximately 3-fold increased risk of Meniere disease, although the timing of the infections relative to Meniere disease onset was indeterminate. No association was found for herpes simplex virus 1 or 2 (HSV-1, HSV-2), varicella zoster virus, or Epstein-Barr virus.

In a retrospective population-based case-control cohort study using data from the Korean National Health Insurance Service database, subjects with Meniere disease were compared with a matched cohort without Meniere disease who were enrolled randomly and matched for sex, age, year of diagnosis, income level, and residential area (114): no significant differences were observed between the groups in the incidence of autoimmune diseases, cerebrovascular disease, or cardiovascular disease.

Differential diagnosis

Confusing conditions

The diagnosis of Meniere disease depends on careful application of the clinical diagnostic criteria (05). Meniere disease should not be confused with recurrent vestibulopathy without auditory symptoms, vestibular neuritis (typically with a single episode of vertigo lasting days followed by recovery, even with persistent hearing loss and tinnitus), benign paroxysmal positioning (or positional) vertigo (with brief episodes of positioning-induced vertigo lasting less than 1 minute without associated auditory symptoms), or stuttering progression of sensorineural hearing loss (with or without tinnitus) in the absence of vestibular symptoms.

Meniere disease can be confused with vestibular migraine (58; 201; 136; 163; 265), and current diagnostic criteria may not differentiate the two adequately in all patients (201; 136). Episodic vertigo lasting hours and associated headache may indeed occur with both conditions. Many cases of so-called vestibular Meniere disease probably have migraine-associated vertigo instead. Vestibular symptoms during attacks combined with the results of vestibular function tests may be used to differentiate between vestibular migraine and Meniere disease (265). For example, a reduced vestibular response with caloric testing is supportive of Meniere disease rather than migraine-associated vertigo (58; 265). Some analytic epidemiologic studies have suggested an association between migraine and Meniere disease (201), but it is still unclear whether migraine can cause inner ear damage that progresses to development of endolymphatic hydrops, or whether migraine can in some cases mimic Meniere syndrome. Furthermore, other studies have not found an elevated incidence of migraine headache in patients with Meniere syndrome compared to the general population (78).

Associated or underlying disorders

Meniere syndrome may occur with a variety of conditions that interfere with normal production or resorption of endolymph and might result in endolymphatic hydrops, including head trauma, ear surgery, infections (eg, mumps, syphilis), endocrine abnormalities (eg, thyroid disease), autoimmune-mediated conditions, and possibly impaired venous drainage resulting from internal jugular vein stenosis or occlusion (82; 177; 223; 57; 209; 31; 64). Otosyphilis may have clinical features that include Meniere syndrome and interstitial keratitis, and pathologic findings of endolymphatic hydrops and vasculitis. Classic Cogan syndrome includes Meniere syndrome and interstitial keratitis but without serologic evidence of syphilis, whereas variant Cogan syndrome includes Meniere syndrome and uveitis (or other ocular inflammation) without serologic evidence of syphilis.

Despite repeated suggestions in the medical literature that thyroid dysfunction predisposes to Meniere disease, there is no definitive evidence for this (105). However, a case-control study using health insurance records found significant associations of goiter, hypothyroidism, and hyperthyroidism with Meniere disease (116).

Diagnostic workup

	• Diagnostic evaluation of patients with Meniere syndrome should include audiograms and various tests to exclude potential underlying causes.
	• Audiometry is essential for diagnosis, staging, and monitoring.
	• Caloric tests and either video nystagmography or electronystagmography are recommended.
	• Electrophysiological tests proposed for initial diagnosis or follow-up of patients with Meniere disease include (1) electrocochleography, (2) otoacoustic emissions, and (3) vestibular evoked myogenic potentials.
	• Brain imaging is generally not necessary unless symptoms and signs support a central nervous system basis for episodic vertigo, but the clinical utility of magnetic resonance imaging for Meniere disease is evolving.
	• Endolymphatic hydrops is not pathognomic of Meniere disease, and detecting hydrops on MRI does not directly indicate a diagnosis of Meniere disease.

Diagnostic evaluation of patients with Meniere syndrome should include audiograms and various other tests to exclude various potential causes. Laboratory tests advocated for this purpose include urinalysis and various blood tests, including a complete blood count, electrolytes, serum glucose and hemoglobin A1c, thyroid stimulating hormone, erythrocyte sedimentation rate, antinuclear antibody, serologic tests for syphilis, and Lyme disease antibodies (213; 142).

Audiometry. Audiometry is essential for diagnosis, staging, and monitoring. Audiometric changes do not directly correspond with the vestibular ones, so for therapy planning and diagnosis, both categories of tests are necessary (148).

This should include an audiogram with pure-tone and speech discrimination tests and acoustic reflex decay. Low-frequency or mixed low- and high-frequency hearing loss may be observed, but typically the lower frequencies are most severely affected, giving an upward-sloping configuration (22). Patients with unilateral disease, in particular, demonstrate a characteristic low-frequency hearing loss, even in advanced stages of the disease (22).

Sensorineural hearing loss, low-frequency trough pattern

Audiogram showing a low-frequency trough pattern of sensorineural hearing loss, commonly seen in Meniere disease. The left ear is shown. (Contributed by James Lanska.)

Later in the disease, particularly with bilateral disease, a flat loss (ie, affecting all frequencies) or uncommonly a dome pattern (loss in both high and low frequencies, but minimal loss between 500 Hz and 2000 Hz) (171; 22).

Sensorineural hearing loss, flat pattern

Audiogram showing flat sensorineural hearing loss. (Contributed by James Lanska.)

Recruitment can be demonstrated by deafness for sounds with weak or medium intensity, but normal hearing or possibly hyperacusis for sounds with greater intensity. Serial audiograms may document fluctuating hearing loss, as well as progressive hearing loss over periods of years (22). Immittance testing demonstrates a normal tympanogram, confirming normal external auditory canal and middle ear function.

Low-frequency air-bone gaps may occur in Meniere disease, a phenomenon that had been puzzling because air-bone gaps are commonly found in patients with conductive or mixed hearing loss due to outer- or middle-ear diseases. Analysis of delayed gadolinium-enhanced MRI suggests that direct contact between the distended saccule and the inner surface of the stapes footplate is the likely underlying pathophysiological mechanism (172).

Caloric tests and video- or electro-nystagmography. Caloric tests and either video nystagmography or electronystagmography are recommended (89; 30; 234), particularly before and after surgical treatment or aminoglycoside therapy (05). Horizontal vestibulo-ocular reflex evaluation with caloric testing (a low-frequency test) is more often abnormal than video-head impulse testing (a high-frequency test) in patients with Meniere disease, but both tests can be used to provide complementary information on peripheral vestibular function (30; 162).

Caloric testing and electronystagmography/video nystagmography should be performed on an empty stomach after discontinuing vestibular sedatives for at least 2 weeks. Canal paresis in Meniere syndrome is thought to result from ampullary distortion and resultant disruption of the cupulary attachment of the horizontal semicircular duct (205). Unilateral canal paresis supports symptomatic dysfunction of the paretic ear. Asymmetric caloric function in the presence of normal horizontal head impulse tests is most commonly associated with Meniere disease, compared with other neuro-otological conditions (96; 170); this dissociation may serve as a useful diagnostic marker (96). Like audiometric test results, caloric response reduction may also fluctuate in patients with Meniere syndrome (04; 152). The caloric test can detect abnormalities of vestibular function better than video head impulse tests in all stages of Meniere disease (133).

Caloric responses fluctuate during and between the attacks of Meniere disease (127). During the irritative and recovery phase, less than half of patients show a caloric asymmetry of more than 25%, whereas patients usually show a caloric asymmetry of more than 25% during the paretic phase, with a decreased response almost always in the affected ear. Between attacks, caloric asymmetry is found in less than half of patients, with a decreased response almost always in the affected ear when observed.

A systematic review and meta-analysis (12 articles, published between 2014 and 2021, with 708 patients evaluated) found that the frequency of an abnormal caloric response in patients with Meniere's disease was 64%, whereas the prevalence of altered vHIT was only 28% (237).

A histopathological study using temporal bone specimens and their corresponding clinical testing data found otolith membrane herniation into the horizontal semicircular canal in 69% of Meniere disease ears, with 90% of these ears demonstrating a diminished caloric response, whereas no ears with a normal response had this herniation (224). In addition, there was no evidence of semicircular canals duct dilation/hydrops. The authors concluded that otolith membrane herniation, not semicircular canal duct dilation, is associated with decreased caloric responses in Meniere disease.

Spontaneous or positional nystagmus is detected on electronystagmography/video nystagmography in about 20% of cases (with some studies reporting values as high as 50%) (152). Although spontaneous nystagmus is supportive of active Meniere syndrome, it does not reliably indicate the side of the lesion (154; 153; 33; 181). Electronystagmographic or video nystagmographic monitoring may be helpful in verifying the affected side by identification of recovery nystagmus (154; 153; 33; 181). Such verification is particularly important when planning surgical procedures for treatment. Ideally, monitoring should include direct eye observation or video nystagmography because pure rotatory nystagmus can occur and will not be detected by electronystagmography (181).

Electrophysiological tests. Electrophysiological tests proposed for initial diagnosis or follow-up of patients with Meniere disease include (1) electrocochleography, (2) otoacoustic emissions, and (3) vestibular evoked myogenic potentials (39). Available data are still limited, and the recommended procedures have not reached an international consensus (39). None of the electrophysiological tests can be considered pathognomonic for the diagnosis of Meniere disease; thus, they need to be considered as having a supportive role in clinical diagnosis (39).

Electrocochleography and otoacoustic emissions may be helpful, though supportive data are limited (149), but the data are not incorporated into current diagnostic guidelines because these tests are not universally available or standardized (05). The presence of endolymphatic hydrops is generally indicated by either an elevated summating/action potential ratio with click stimulation or a depressed summating potential amplitude with tone burst stimulation (266). Suggested thresholds are a summating/action potential ratio with click stimulation greater than 0.35 using transtympanic electrocochleography, a summating/action potential ratio greater than 0.42 using extratympanic electrocochleography, or a summating potential amplitude of less than 2 microvolts for at least one frequency within the range of 0.5 to 0.8 kHz using transtympanic electrocochleography with tone burst stimulation set at a repetition rate of 30 to 40 stimuli per second (266). Electrocochleography may be particularly helpful in excluding contralateral disease before proceeding with destructive surgical procedures; it appears to be more sensitive for detecting contralateral disease than relying on the presence of auditory dysfunction, tinnitus, and aural fullness (43).

Cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP, respectively) are being studied but have yet to prove to be important in the diagnosis of Meniere disease (259; 277; 250; 63; 122; 147). Preliminary results suggest that cVEMP thresholds can help distinguish Meniere disease from vestibular migraine, and serial cVEMP testing may be a sensitive marker of disease progression in Meniere disease (250). However, according to a Practice Guideline from the American Academy of Neurology, “Evidence is insufficient to determine […] whether vVEMP or oVEMP is useful in diagnosing […] Meniere disease” (63).

Imaging. Brain imaging is generally not necessary unless symptoms and signs support a CNS basis for episodic vertigo, but the clinical utility of MRI for Meniere disease is evolving (15; 86; 220; 12; 83; 233; 25; 137; 143).

Delayed gadolinium-enhanced MRI after intratympanic contrast administration. Endolymphatic hydrops can be demonstrated in vivo by MRI with intratympanic gadolinium contrast administration, and affected ears show variable degrees of impaired perilymphatic enhancement (65; 66; 263; 244; 104). Prior contrast-enhanced MRI techniques used a 3-dimensional fluid-attenuated inversion recovery (FLAIR) sequence after intratympanic gadolinium administration (sometimes after a 4 to 24 hours) (25; 104). Contrast-enhanced MRI after intratympanic gadolinium can reliably and accurately detect and grade Meniere disease (25). However, the need for intratympanic contrast administration has been obviated in some more recent studies.

Delayed gadolinium-enhanced MRI after intravenous contrast administration. Endolymphatic hydrops can now be detected and graded using 3T MR images of the inner ear acquired (1) at least 2 to 4 hours after intravenous contrast administration with a 3D inversion-recovery sequence (15; 25; 108; 132; 173; 184; 249; 16; 93), (2) 6 hours after double-dose intravenous contrast administration with 3D-FLAIR (129), or (3) 24 hours after intravenous contrast administration with a 3D real inversion recovery (3D-real IR) sequence (52). Some reports have emphasized the need for delaying MRI more than 3 hours after intravenous contrast administration (36).

Most imaging studies have been done on a 3-T MRI scanner, and it had been thought that endolymphatic hydrops could only be demonstrated on MRI with a 3-T magnet. However, multiple studies have shown that endolymphatic hydrops can be confidently demonstrated with the current standard 4-hour delayed intravenous gadolinium-enhanced 3D-FLAIR sequence on MRI with a 1.5-T magnet (94; 113).

Sensitivity and specificity. Studies have reported 10% to 33% of patients with Meniere disease do not have MRI-demonstrable changes of hydrops (199; 15; 174). In one study of 54 patients, endolymphatic hydrops was demonstrated in 90% on the clinically affected side and in 22% on the clinically silent side with high levels of interobserver agreement (15).

The combination of perilymphatic enhancement and endolymphatic hydrops in patients suspected of having Meniere disease increases the positive predictive value in diagnosing definite Meniere disease (249).

In the absence of access to MRI with 3D reconstruction of the endolymphatic space, measurements of the saccule on routine nonenhanced 3D-T2 MRI can show hydrops and help diagnose Meniere disease (228; Lopez-Escamez and Attye 2019).

Evaluation of the vestibular endolymphatic space contacting the oval window has high specificity and positive predictive value in differentiating Meniere disease ears from other ears (45). Obliteration of the inferior portion of the vestibule and the contact with the stapes footplate by the vestibular endolymphatic space are reliable markers for diagnosing Meniere disease (45).

MRI may be helpful in the differentiation of Meniere disease and "menieriform diseases" with clinical overlap, including vestibular migraine and vestibular schwannoma (233; 129b; 271). In addition, MRI may reveal brain lesions, semicircular canal abnormalities, and perilymphatic fistulae in patients with clinical Meniere disease (12).

MRI does not yet seem helpful for diagnosing cases of Meniere disease with early symptoms and the reproducibility of the hydrops protocols with various MRI scan manufacturers is debatable, limiting widespread adoption of these techniques into clinical practice (137).

Endolymphatic hydrops is detectable in a high proportion of Meniere disease cases (99%), but it is not specific for Meniere disease. For example, it is present in about half of cases with vestibular schwannoma (48%) and is also present in "healthy" temporal bones (13%) (246). Therefore, endolymphatic hydrops is not a pathognomonic phenomenon, and detecting this on MRI does not directly indicate a diagnosis of Meniere disease.

Staging. Various approaches have been used to stage endolymphatic hydrops overall (159) and to grade cochlear and vestibular components separately (129) while also assessing cochlear and vestibular perilymphatic enhancement (129).

Endolymphatic hydrops after intravenous injection of gadolinium contrast agent

Structural images of the inner ear showing no, mild, and significant endolymphatic hydrops, after intravenous injection of an ordinary dose of gadolinium (Gd) contrast agent with a 3T MRI. Arrows and arrowheads indicate endolym...
Cochlear and vestibular endolymphatic hydrops with different degrees

High-resolution 3D-FLAIR images were obtained 6 hours after intravenous gadolinium injection. (a) Cochlear hydrops grade II: a 45-year-old man with right Meniere disease. Axial 3D-FLAIR image showed that the scala vestibuli was...
Left versus right vestibular schwannoma comparison (MRI)

Comparison of MRI from a 50-year-old woman with left vestibular schwannoma (a, b) and a 49-year-old man with right vestibular schwannoma (c) in the internal auditory canal. The inner auditory canal schwannoma presented (a, c) r...
Meniere disease and posterior semicircular canal dysplasia

A 62-year-old woman with left Meniere disease and posterior semicircular canal dysplasia. 3D-FLAIR (a) and 3D-T2WI (b) showed left posterior semicircular canal dysplasia (arrowheads). The endolymph compartment occupies more tha...

MRI results can be used to classify the degree of endolymphatic hydrops into four stages (84; 26; 104; 129):

	Stage 0, no hydrops: no enlargement of the endolymphatic space in the cochlea and vestibule, and the perilymphatic space is clearly visible
	Stage 1, mild hydrops: the endolymphatic space is enlarged with hypointensity bulging into the perilymphatic space with hyperintensity
	Stage 2, marked hydrops: the endolymphatic space is enlarged, the scala media is convex to the scala vestibuli, and the perilymphatic space is semicircular
	Stage 3, extreme hydrops: the endolymphatic space is severely dilated, the scala media continues to be convex to the scala vestibuli, and the perilymphatic space is compressed into a flattened line (84; 104).

Degree of cochlear endolymphatic hydrops

(1a) Right cochlear endolymphatic hydrops grade 0. (1b) Right cochlear endolymphatic hydrops grade 1. (1c) Right cochlear endolymphatic hydrops grade 2. (1d) Right cochlear endolymphatic hydrops grade 3. Orange arrow shows the ...

Cochlear hydrops can be classified into three degrees or grades from 0 to 2 (15):

	Grade 0, no cochlear hydrops
	Grade 1, cochlear hydrops
		(a) irregular dilation and partial obstruction of the scala vestibuli (b) dilation of the endolymphatic space encompasses more than 50% of the vestibulum (c) a circular perilymphatic space remains visible


	Grade 2, cochlear hydrops
		(a) total obliteration of the scala vestibuli (b) dilation of the endolymphatic space leads to effacement of the perilymphatic space.

Mid-modiolar area of the cochlea and correlated axial cryosections

Cropped axial delayed gadolinium-enhanced 3D FLAIR images at the mid-modiolar area of the cochlea and correlated axial cryosections with hematoxylin and eosin staining (magnification, × 7) and color overlay. (a) Normal cochlea ...

Vestibular hydrops can be classified into four degrees or grades from 0 to 3 (26) by a modification of an earlier three-level grading system (15), with the four-grade system having greater sensitivity to detect Meniere disease while preserving specificity (26):

	Grade 0, no vestibular hydrops
	Grade 1, vestibular hydrops: the saccule, normally the smallest of the two vestibular sacs, is equal or larger than the utricle but is not yet confluent with the utricle
	Grade 2, vestibular hydrops: dilation of the endolymphatic space encompasses more than 50% of the vestibulum
	Grade 3, vestibular hydrops: dilation of the endolymphatic space leads to effacement of the perilymphatic space.

Inferior part of the vestibulum and correlated axial cryosections

Cropped axial delayed gadolinium-enhanced 3D FLAIR images at the inferior part of the vestibulum and correlated axial cryosections with hematoxylin and eosin staining (magnification, × 7) and color overlay. (a) Normal vestibule...

In another approach, vestibular hydrops can be classified into three degrees or grades from 0 to 2 by calculating the ratio of the area of the endolymphatic space to the total area of the endolymphatic and perilymphatic spaces ("R-value") (104):

Grade 0, no vestibular hydrops: R ≤ 1/3
Grade 1, vestibular hydrops: 1/3 < R <
Grade 2, vestibular hydrops: R ≥ ½

Grading of vestibular endolymphatic hydrops

(2a) Right vestibular endolymphatic hydrops grade 0. (2b) Right vestibular endolymphatic hydrops grade 1. (2c) Right vestibular endolymphatic hydrops grade 2. Orange arrow shows the area of endolymphatic hydrops. (Source: Huang...

The degrees of cochlear and vestibular hydrops are positively correlated (103).

Perilymphatic enhancement is one signature of blood-perilymph barrier impairment (26).

Perilymphatic enhancement is the signature of blood-perilymph barrier impairment

Axial delayed gadolinium-enhanced 3D FLAIR images at the level of the inner ear in a 77-year-old woman with unilateral left-sided definite Meniere disease. There is cochlear hydrops grade 1 (small arrowhead) and vestibular hydr...

Assessment of both endolymphatic hydrops and perilymphatic enhancement improves the diagnostic accuracy for probable and definite Meniere disease (129). In a meta-analysis, the combination of increased perilymphatic enhancement and endolymphatic hydrops (three studies, 122 Meniere disease ears) achieved the highest sensitivity (87%) whilst maintaining high specificity (91%) (44).

Another marker of the blood-labyrinth barrier in Meniere disease is the signal intensity ratio (SIR) of the cochlear basal turn on 3D-FLAIR MRI (276). The SIR of the affected ear in Meniere disease was significantly increased compared to that of the unaffected ear (276). The SIR of the unaffected side in Meniere disease was higher than that of both ears in healthy controls (276). The SIR in the affected ear of Meniere disease was positively correlated with both cochlear and vestibular hydrops (276).

Vestibular aqueduct abnormalities on CT and MRI can be classified into three degrees or grades from 0 to 2 (the original article used A to C, but a numbered approach is used here for overall consistency with grading of cochlear and vestibular hydrops) (11; 270):

	Grade 0: Normal with a continuous vestibular aqueduct Grade 1: A discontinuous vestibular aqueduct Grade 2: No visible vestibular aqueduct

Vestibular aqueduct grading on the Pöschl planes

Vestibular aqueduct grading on the Pöschl planes on 3D sampling perfection with application-optimized contrasts using different flip angle evolutions (3D-SPACE). (a) Grade A with a continuous vestibular aqueduct (arrow). (b) Gr...
Visibility of vestibular aqueduct grading on the Pöschl planes on temporal bone CT

(a) Grade 0 with a continuous vestibular aqueduct (arrow). (b) Grade I with a discontinuous vestibular aqueduct (arrow). (c) Grade II with a complete ossification of vestibular aqueduct. Note: The Pöschl projection is used in i...

The morphology of the vestibular aqueduct in the 45° oblique (Poschl) plane can be classified into funnel, tubular, filiform, hollow, and obliterated types (273; 270).

Various types of vestibular aqueduct

(a) Funnel type. (b) Tubular type. (c) Filiform type. (d) Hollow type. (e) Obliterated type. (Source: Xia K, Lei P, Liu Y, et al. Comparison of vestibular aqueduct visualization on computed tomography and magnetic resonance ima...

Peri-aqueductal pneumatization was classified into three levels or grades from 1 to 3:

	Grade 1: large-cell peri-aqueductal pneumatization Grade 2: small-cell peri-aqueductal pneumatization Grade 3: absence of air cells (232; 270)

Types of peri-aqueductal pneumatization

(a) Large-cell pneumatization (arrow) in the vicinity of the aqueduct. (b) Small-cell pneumatization (arrow) in the vicinity of the aqueduct. (c) Absence of air cell. (Source: Xia K, Lei P, Liu Y, et al. Comparison of vestibula...

In patients with Meniere disease, visualization of the vestibular aqueduct on 3D-SPACE MRI is poorer than that observed on CT and may be affected by its osseous configuration (270). MRI vestibular aqueduct visualization has a weak positive correlation with ipsilateral CT vestibular aqueduct visualization (270). For the affected side, vestibular aqueduct visualization with MRI was negatively correlated with the frequency of obliterated morphology on CT and positively correlated with that of tubular morphology on CT (270). MRI vestibular aqueduct visualization was not affected by periaqueductal pneumatization on CT (270).

Clinical-radiologic correlation. Symptomatic endolymphatic hydrops visualized on MRI is reliably on the affected side, and asymptomatic endolymphatic hydrops is detected on the unaffected contralateral side in as many as one fifth of cases (132). Analysis of the vestibular aqueduct by MRI detects symmetric abnormalities in both ears in many patients with unilateral Meniere disease (11). Resolution of endolymphatic hydrops on delayed intravenous contrast-enhanced 3D-FLAIR MRI has been demonstrated following acetazolamide therapy (220) and other medical treatments (274).

Pure tone averages and endolymphatic staging are higher for combined vestibular and cochlear endolymphatic hydrops than for isolated cochlear or vestibular hydrops (104). The degree of cochlear hydrops was positively correlated with pure tone averages, low-frequency hearing thresholds, mid-frequency hearing thresholds, and the stage of endolymphatic hydrops (104).

Relationship between the degree of cochlear endolymphatic hydrops and pure-tone average

According to grading criteria of the 1997 World Health Group, the pure tone average hearing threshold was calculated as the average pure tone audiometry thresholds of binaural air conduction at 500, 1000, 2000, and 4000 Hz (PTA...
Relationship between the degree of cochlear endolymphatic hydrops and disease staging

Meniere disease staging was obtained by calculating the pure tone average hearing thresholds at 500, 1000, 2000, and 3000 Hz (Committee on Hearing and Equilibrium, American Academy of Otolaryngology-Head and Neck Foundation. Co...

There was no significant correlation between the grade of vestibular endolymphatic hydrops and pure tone averages or the stage of endolymphatic hydrops (104), although other studies have reported associations between vestibular endolymphatic hydrops on MRI and hearing loss (103).

Endolymphatic hydrops detected by MRI shows limited and inconsistent correlation with caloric stimulation results (103; 71).

Bone mineral density assessment. Dual-energy x-ray absorptiometry of the hip and spine (not the heel) is recommended to assess bone mineral density in patients with Meniere disease. Meniere disease is associated with low bone mineral density (osteopenia or osteoporosis) as measured by dual-energy x-ray absorptiometry at the femoral neck (226), and the vertigo and imbalance associated with the disease present a significantly increased risk of falls and falls with injury. To minimize potential fall-related fractures, dual-energy x-ray absorptiometry and monitoring of 25-hydroxy vitamin D levels are recommended.

Other tests. Some authors advocate provocative glycerol or furosemide tests (157); however, the outcome of these tests is highly dependent on patient and provider expectations and suggestion (241).

Management

	• The aims of management of Meniere disease are to (1) reduce the number and severity of acute vertiginous attacks and their associated symptoms (eg, hearing loss and tinnitus), (2) alleviate chronic symptoms (eg, hearing loss and tinnitus), and (3) prevent progression of the disease and its associated symptoms (eg, hearing loss and tinnitus).
	• Current medical management can control vertiginous attacks in approximately 75% to 80% of patients but does not appear to alter the long-term progression of hearing loss.
	• Initial management of Meniere syndrome involves diagnosis and treatment of the primary disease (eg, thyroid disease).
	• Chronic management of Meniere disease includes the use of hearing aids and masking devices, lifestyle modification, salt restriction, and diuretic therapy.
	• The major focus of lifestyle modification is avoiding potential disease triggers, including stress, fatigue, high-salt foods, and other potential trigger substances, although evidence supporting this approach is limited and of low quality.
	• In patients with Meniere syndrome or Meniere disease, vestibular sedatives, particularly those with anti-emetic or anxiolytic properties, and intravenous fluids may be helpful in decreasing symptoms acutely during an attack.
	• A low-sodium diet and diuretics are considered the mainstays of treatment for Meniere disease, although evidence supporting this approach is limited and of low quality.
	• Ablative therapy is a second-line management approach for failures of medical therapy, although evidence supporting this approach is limited and of low quality.
	• Surgical treatment of Meniere syndrome and Meniere disease is reserved for medical treatment failures but remains controversial as it based on limited poor-quality evidence.
	• Vestibular rehabilitation is generally recommended after aminoglycoside treatment or surgery, and may be helpful in minimizing disability, particularly in older individuals.
	• Certain tasks requiring balance and having a high risk of injury with falls (eg, climbing ladders) should generally be avoided.

The management goals of Meniere disease are to (1) reduce the number and severity of acute vertiginous attacks and their associated symptoms (eg, hearing loss and tinnitus); (2) alleviate chronic symptoms (eg, hearing loss, tinnitus, disequilibrium); and (3) prevent progression of the disease and its associated symptoms (eg, hearing loss, tinnitus, disequilibrium) (40). The treatment plan should be individualized based on the clinical features, including hearing stage, disease duration, vertigo attack profile, and comorbidities (62). Many patients with Meniere disease can be managed using nonablative therapy (eg, medical management, intratympanic steroids, and endolymphatic shunt surgery) before ablative techniques (eg, intratympanic gentamicin and vestibular neurectomy) (38).

A major problem in the management of Meniere disease is the overall poor quality of supporting studies for various treatments (247; 123; 254; 255; 256; 257; 258). Even many systematic reviews and meta-analyses in this field show a lack of rigor. Few existing clinical guidelines meet the standards of quality established by the AGREE II appraisal instrument (17). In addition, the placebo effect in Meniere disease trials is associated with some symptomatic improvement in subjective outcomes, such as patient-reported vertigo episodes (238).

Treatment of Meniere disease can be considered in terms of acute phase management and management of recurrent intermittent presentations (159).

Treatment options for acute and intermittent phases of Meniere disease

(Source: Mohseni-Dargah M, Falahati Z, Pastras C, et al. Meniere's disease: Pathogenesis, treatments, and emerging approaches for an idiopathic bioenvironmental disorder. Environ Res. 2023;238[Pt 1]:116972. Creative Commons Att...

Acute-phase management is pharmacologic. Long-term measures should begin with conservative (nondestructive) measures with "adjuvant" measures (eg, vestibular rehabilitation) as necessary. Low vertigo severity, low comorbidity burden, and absence of hearing loss are factors associated with a conservative treatment response in Meniere's disease (155). Destructive measures are used if other approaches are unsuccessful.

Medical management. Current medical management can control vertiginous attacks in approximately 75% to 80% of patients but does not appear to alter the long-term progression of hearing loss (02). Initial management of Meniere syndrome involves diagnosing and treating the primary disease (eg, thyroid disease). Chronic management of Meniere disease should include therapeutic counseling, the use of hearing aids and masking devices, lifestyle modification, salt restriction, and diuretic therapy (62). A diary of vertigo attacks with symptoms during the episodes should be encouraged to improve phenotyping (62).

The use of hearing aids can help with hearing impairment and, in many cases, benefits the subjective tinnitus that accompanies the disorder. Masking devices are also helpful for the management of subjective tinnitus.

The major focus of lifestyle modification is avoiding potential disease triggers, including stress, fatigue, high-salt foods (eg, pizza, processed meats, canned soups, smoked fish, potato chips, peanuts, nuts, beer, etc.), and other potential trigger substances (eg, caffeine, nicotine, alcohol, monosodium glutamate, carbohydrate-rich foods, and foods high in cholesterol and triglycerides).

In patients with Meniere syndrome or Meniere disease, vestibular sedatives (eg, meclizine, dimenhydrinate, prochlorperazine, promethazine, prochlorperazine, benzodiazepines), particularly those with anti-emetic or anxiolytic properties, and intravenous fluids may help decrease symptoms acutely during an attack. Although some authors advocate intratympanic or systemic dexamethasone treatment, no definite treatment effect has been demonstrated (100).

A low-sodium diet and diuretics are considered the mainstays of chronic treatment for Meniere disease (260; 79; 264), and sodium restriction has been a foundation of disease management since the 1930s (40; 264; 225). Evidence of their validity remains weak (40; 51; 225; 254). Clinical experience and expert consensus suggest that a 1.0- to 1.5-gram low-sodium diet and diuretics (eg, hydrochlorothiazide and triamterene) may help to decrease the fluid pressure or volume within the inner ear and decrease the frequency of attacks, although these measures do not help once an acute attack has developed. Vestibular complaints decrease with diuretic therapy, but diuretics have no significant effect on hearing or tinnitus (245; 40). Acetazolamide was recommended for many years, but most authors have become discouraged with its use, and it has not been shown to prevent long-term deterioration in hearing (32; 47; 202; 42). A meta-analysis of the utility of diuretics for Meniere disease identified three systematic reviews that collectively included 19 studies, of which four were randomized trials (207). The authors concluded that it is unclear whether diuretics lead to a symptomatic improvement of vertigo or an objective decrease in hearing loss in patients with Meniere disease.

A small randomized controlled trial (25 subjects in each arm) found that a low-sodium diet with adequate water intake improved the hearing and alleviated vertigo and tinnitus in subjects with Meniere disease (275).

A longitudinal treatment study using a historical control found that a daily intake of 35 ml of water per kilogram of body weight dramatically relieved vertigo and significantly improved hearing over a 2-year period, whereas in a historical control group, patients had some improvement in vertigo but generally had progressive hearing loss over a similar time period (166). The authors concluded that the “deliberate modulation of the intake of water may be the simplest and most cost-effective medical treatment for patients with Meniere disease” (166).

The use of corticosteroids is controversial and largely empiric (40). It may help improve vertigo but does not significantly improve hearing or tinnitus.

Preliminary work has suggested a potential role for betahistine in improving hearing function in Meniere disease (160; 221; 264; 135), but this will have to be substantiated with well-designed randomized controlled trials, which are presently lacking (54; 247; 248). An umbrella systematic review concluded that betahistine probably does not reduce Meniere disease symptoms when compared with placebo (248).

Cochrane reviews concluded that the evidence for lifestyle or dietary interventions and for systemic pharmacological interventions for Meniere disease is very uncertain (254; 257); few randomized controlled trials have compared these interventions to placebo or no treatment, and the evidence that is currently available from these studies is of low or very low certainty.

Intratympanic steroids. The evidence comparing intratympanic corticosteroids to placebo or no treatment is of low or very low certainty (255). Limited evidence, restricted to one trial, supports the effectiveness of intratympanic steroids for improving vertigo in patients with Meniere disease (193). In an open-label, uncontrolled trial, there was a strong correlation between hearing improvement and dexamethasone treatment (161). Despite the limited evidence, a systematic review suggested that to achieve both hearing preservation and vertigo control, intratympanic steroids plus high-dose betahistine may be superior to intratympanic gentamicin because of the risk of hearing loss with intratympanic gentamicin despite good control of vertigo (01). The utility of this therapy will have to be substantiated with well-designed randomized controlled trials (248).

A Cochrane review found that the evidence for intratympanic corticosteroids in the treatment of Meniere disease is uncertain because of the relatively few published randomized trials and the concerns about publication bias in this area, given the identification of two large randomized trials that remain unpublished (255).

Ablative aminoglycoside therapies. Ablative therapy is a second-line management approach for failures of medical therapy. Among the various forms of ablative therapy, the most common are intratympanic aminoglycoside administration and vestibular neurectomy (02). Limited evidence from a single trial supports the effectiveness of intratympanic steroids for improving vertigo in patients with Meniere disease (193). The utility of this therapy will have to be substantiated with well-designed randomized controlled trials (248).

Oral aminoglycosides were used historically to treat Meniere syndrome and Meniere disease, but the resulting bilateral vestibular hypofunction was (and remains) extremely debilitating. Schuknecht described middle-ear perfusion with aminoglycosides for treatment of Meniere disease in the late 1950s (215), but transtympanic administration of aminoglycosides did not become popular as a treatment for Meniere disease until the late 1990s, providing an alternative to the disabling bilateral vestibular effects of systemic administration of aminoglycosides. Low-dose intratympanic application of gentamicin can provide a graduated destruction of sensory and epithelial cells in the cochlea in association with the generation of reactive oxygen species and reactive nitrogen species, including oxidation product of nitric oxide in the lateral wall and the organ of Corti (98; 167). Aminoglycosides are now generally applied unilaterally within the middle ear cavity, where they are absorbed into the inner ear through the round window membrane. Various methods of delivering medication to the middle ear have been employed, including injection through tympanostomy tubes or catheters through the tympanic membrane, needle injections through the tympanic membrane, and less commonly with a Eustachian tube catheter (29). No particular combination of method of administration, single versus multiple dosing, or concentration of aminoglycoside injected has yet been established as the most effective (29).

Transtympanic administration of aminoglycosides generally carries relatively low risks, has minimal systemic effects, and can be performed in patients whose comorbidities would preclude surgery (208; 41). Gentamicin is the aminoglycoside most frequently used for this purpose in the United States. Intratympanic gentamicin appears to be an effective treatment for vertigo in patients with Meniere disease but carries a risk of hearing loss (194). Studies indicate approximately 90% to 95% efficacy (complete elimination of vertigo in approximately 80% to 85% and more than 50% reduction in frequency in 10% to 15%), with clinically significant (ie, 10 dB or greater decrement in pure-tone average) worsening of hearing in the treated ear in 10% to 30% of patients (46; 10; 195; 29; 101; 112; 20; 56; 41). Extreme worsening (ie, 30 dB or greater decrement in pure-tone average) of hearing in the treated ear is rare (10; 41), and a significant fraction of treated ears (as much as a quarter in some studies) have a significant improvement in hearing (10). However, many of the available studies were poorly designed, and further controlled studies are needed (41).

Patients selected for transtympanic administration of aminoglycosides should be carefully selected and should generally undergo titrated therapy with low-dose gentamicin (41). Transtympanic gentamicin should be used cautiously: given that approximately 20% of patients ultimately develop bilateral disease, there is a significant risk of developing bilateral vestibular hypofunction, either from the disease itself or from the treatment, with incapacitating and irreversible disequilibrium and oscillopsia (40). Titration methods or multiple daily injections may be used in patients with profound or nonserviceable hearing (268). Treatment protocols with an injection interval of a week or more or those with “as needed” monthly injections provide the same level of vertigo control with better hearing preservation and should be used in patients with serviceable hearing (268). Vestibular-evoked myogenic potentials and the head thrust test are more reliable than other vestibular tests for the follow-up of patients undergoing gentamicin treatment (268).

A systematic review showed some benefit of intratympanic gentamicin over intratympanic corticosteroids for subjective outcomes but no difference regarding objective outcomes or complication rate (267).

Low-dose intratympanic gentamicin administration based on clinical symptomatology can produce satisfactory control of vertigo attacks while avoiding cochlear damage (214).

A Cochrane review concluded that the evidence for the use of intratympanic gentamicin in the treatment of Meniere disease is very uncertain because there are few published randomized trials, and all these enrolled a very small number of participants (256); because the studies assessed different outcomes, using different methods, and reported at different time points, pooling the results to obtain more reliable estimates of the efficacy of this treatment was not possible.

Surgical treatment. Surgical treatment of Meniere syndrome and Meniere disease is reserved for medical treatment failures but remains controversial (79; 02). The goal of surgery is to control episodic vertigo while maintaining preoperative hearing levels, though practically, surgical treatment typically has a severe impact on hearing function (144). Destructive procedures are generally reserved for severe cases, with associated significant disability, but are contraindicated with bilateral disease (which variously is reported to occur in from 7% to 50% of cases). Provided the contralateral vestibular apparatus functions adequately, the brain eventually compensates for the loss of a single labyrinth.

A Cochrane review was unable to draw clear conclusions about the efficacy of surgical interventions for Meniere disease because limited evidence was identified, and all of it was of low or very low certainty (123).

Endolymphatic sac decompression with or without shunt is thought to decrease endolymph pressure by removing the petrous bone overlying the sac; a drainage tube from the sac to either the mastoid space or subarachnoid space can be inserted to further reduce endolymph pressure. The success of endolymphatic sac decompression in controlling vertigo and stabilizing hearing has been reported at between 60% to 94% and 80% to 88%, regardless of whether a shunt is also performed (260; 23). Additionally, endolymphatic sac decompression is associated with a significantly improved perception of health in patients (60), and it has been reported that this may decrease the incidence of developing Meniere disease in contralateral ears with silent endolymphatic hydrops within the first 5 postoperative years (117). The procedure does not directly destroy or damage hearing or balance organs or pathways, and it can usually be done as an outpatient procedure (260). However, even retrospective comparative studies have suggested that endolymphatic sac decompression does not alter the long-term natural course of vertigo (227), and a critical Danish randomized, double-blind study that included a sham surgical procedure found that endolymphatic sac decompression was no more effective than sham surgery (240). Furthermore, the procedure is associated with worsened hearing in about one third of cases, and about one quarter subsequently have another secondary treatment after endolymphatic sac decompression (102). A systematic review of endolymphatic sac surgery for Meniere disease concluded that "there is still a lack of high-quality research, suggesting that endolymphatic sac surgery provides a significant amount of symptomatic relief for Meniere patients" (55); a subsequent systematic review came to the same conclusion (248). Nevertheless, the procedure remains popular among otolaryngologists (231), and some have concluded, based on longitudinal follow-up of a case series, that endolymphatic shunt surgery provides successful vertigo control at least as well as intratympanic gentamicin injections, with a lower incidence of audiovestibular complications (75).

For patients with useful hearing in the affected ear (ie, better than 80 dB pure-tone average and 20% speech discrimination) and who have failed other treatments, vestibular neurectomy through a retrosigmoid or middle fossa approach has a high rate of vertigo control (over 95%) with a high rate of hearing preservation (approximately 90% to 95%) (180; 208). Adverse outcomes are uncommon but may include facial nerve damage, hearing loss, exacerbation of tinnitus or dizziness, CSF leakage, meningitis, and hemorrhage. A minimally invasive vestibular neurotomy has been advocated as a safe and effective surgical treatment for these individuals, with reported benefits including improved dizziness and anxiety (156).

Labyrinthectomy also has a high rate of vertigo control (over 95%) and is useful for patients with unilateral disease whose hearing on the affected side has already been destroyed by the disease process (212; 128). This procedure is less invasive than vestibular nerve section and is accomplished using either a transcanal approach (cochleovestibular neurectomy) through the external ear canal or a transmastoid approach with extension of a mastoidectomy by drilling through the semicircular canals. Compared to vestibular nerve section, risk of CSF leakage or meningitis is lower because craniotomy is not required. Patients affected by end-stage Meniere disease with vertigo and severe hearing loss can successfully undergo labyrinthectomy and cochlear implantation, although in elderly patients, there is a significant risk of persistent instability (235).

Triple semicircular canal occlusion is an emerging surgical technique that holds promise as a partially ablative procedure with a high vertigo control rate in Meniere's disease (145). Triple semicircular canal occlusion has a high rate of vertigo control (97% in one retrospective study of 90 cases) with a high rate of preserved hearing (77% in the same retrospective study) (131). Combined triple semicircular canal occlusion and cochlear implantation is a viable treatment option that is effective for vertigo control and for restoring hearing in patients with Meniere disease (272).

Management of otolithic crises. Some authorities advocate conservative management of otolithic crises, particularly given that many cases have a flurry of episodes followed by remission (Janzen and Russel 1988; 13). However, the events can be associated with significant injuries, and for some patients, the attacks are so dangerous or intractable that surgery is indicated (13). Otolithic crises have been treated successfully with either surgical ablation (labyrinthectomy) or trans-tympanic gentamicin therapy in the absence of serviceable hearing, or middle fossa vestibular nerve section to preserve serviceable hearing (28; 169; 107; 251). Even older patients can be successfully treated with ablative surgery with excellent compensation and no vertigo or falls up to 10 years after surgery (107). Vestibular nerve section is usually recommended only for patients who have had symptoms for at least 5 years unless the patient is severely incapacitated or is in danger of severe bodily harm because of otolithic crises (28). Intratympanic gentamicin treatment appears to be a long-lasting and effective treatment for vestibular drop attacks in patients with Meniere disease (251).

Positive-pressure therapy. Because patients with Meniere disease have symptoms that can fluctuate with ambient air pressure, in the late 1980s, Densert and colleagues evaluated the utility of positive-pressure pulses applied to the middle ear and demonstrated that such pulses improved electrocochleographic parameters in these patients (40). Since then, portable low-amplitude, alternating-pressure generators (eg, the Meniett device from Medtronic Xomed, Inc.) have been developed to be worn in the external auditory canal and have been shown to be efficacious and safe in patients with Meniere disease (73; 40; 134). The device requires the placement of a long-term tympanostomy tube. Most patients note gradual improvement in vertigo when using the Meniett device; consequently, this device had been considered a useful option for patients with Meniere disease who had failed medical treatment (40). However, hearing does not improve with use of the device, and the device is expensive and rarely covered by insurers. Moreover, a randomized, double-blind, placebo-controlled, multicenter trial found no difference between the portable Meniett low-pressure pulse generator (Medtronic Xomed, Jacksonville, FL) and the placebo device groups in the treatment of Meniere disease (210).

A Cochrane review concluded that the evidence for positive pressure therapy for Meniere disease is very uncertain (258); few randomized controlled trials have compared this intervention to placebo or no treatment, and the evidence that is currently available from these studies is of low or very low certainty. A separate umbrella systematic review concluded that positive pressure therapy probably does not reduce Meniere disease symptoms when compared with placebo (248).

Cochlear implantation. Cochlear implantation in advanced Meniere disease is a valid option that can partially restore hearing, regardless of the disease duration, uni- or bilaterality, age at implantation, previous therapeutic procedures, and stage of activity of Meniere disease (24; 118; 151; 253; 111). It is helpful in improving hearing ability and suppressing tinnitus (253). Although cochlear implantation and labyrinthectomy in adult patients with Meniere disease can improve speech recognition and sound source localization for some patients, observed performance may be poorer than with traditional cochlear implantation candidates (219; 37).

Vestibular rehabilitation. Vestibular rehabilitation is generally recommended after aminoglycoside treatment or surgery and may be helpful in minimizing disability, particularly in older individuals (164; 40; 135; 204). However, available studies had a high risk of bias, and none had long-term follow-ups. Therefore, further studies are required to establish the efficacy of vestibular rehabilitation in Meniere disease, whether managed medically or surgically (204).

Certain tasks requiring balance and having a high risk of injury with falls (eg, climbing ladders) should generally be avoided.

Media

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Patient Profile

Age range of presentation: 6 to 65+ years
Sex preponderance: female>male, >2:1
Heredity: heredity may be a factor
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

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Meniere syndrome

Differential Diagnosis

recurrent vestibulopathy without auditory symptoms
vestibular neuritis
benign paroxysmal positioning (or positional) vertigo
stuttering progression of sensorineural hearing loss
vestibular migraine
- episodic vertigo
- migraine-associated vertigo

Also Relevant

Autoimmune sensorineural hearing loss
Benign paroxysmal positional vertigo
Dizziness
Inner ear concussion
Noise-induced hearing loss
- Subjective tinnitus
- Sudden deafness
- Superior semicircular canal dehiscence syndrome
- Vertigo

Meniere syndrome …

Meniere syndrome

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Table 1. Diagnostic Criteria for Definite Meniere Disease

Table 2. Diagnostic Criteria for Probable Meniere Disease

Prognosis and complications

Table 3. Staging of Meniere Disease

Table 4. Functional Level Scale for Meniere Disease

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Media

References

Contributors

Author

Patient Profile

ICD & OMIM codes

This is an article preview.
Start a Free Account
to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Common maculopathies

Toxic and nutritional deficiency optic neuropathies

Third nerve palsy

Nystagmus

Chronic progressive external ophthalmoplegia

Von Hippel-Lindau disease

Giant cell arteritis

Meniere syndrome

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Table 1. Diagnostic Criteria for Definite Meniere Disease

Table 2. Diagnostic Criteria for Probable Meniere Disease

Prognosis and complications

Table 3. Staging of Meniere Disease

Table 4. Functional Level Scale for Meniere Disease

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Media

References

Contributors

Author

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Common maculopathies

Toxic and nutritional deficiency optic neuropathies

Third nerve palsy

Nystagmus

Chronic progressive external ophthalmoplegia

Von Hippel-Lindau disease

Giant cell arteritis

This is an article preview.
Start a Free Account
to access the full version.