Version May 2024
INTRODUCTION — Meniere disease (MD), named for French physician Prosper Menière, is a condition characterized by episodic vertigo, tinnitus, and hearing loss [1].
This topic will present an overview of the diagnosis and treatment of MD. More detailed topics addressing the differential diagnosis of vertigo, tinnitus, and hearing loss are presented separately. (See "Evaluation of the patient with vertigo" and "Causes of vertigo" and "Etiology and diagnosis of tinnitus" and "Etiology of hearing loss in adults" and "Sudden sensorineural hearing loss in adults: Evaluation and management".)
DEFINITIONS
●Meniere disease – The classic triad of symptoms (episodic vertigo, tinnitus, and hearing loss), likely caused by endolymphatic hydrops of the labyrinthine system of the inner ear. (See 'Pathogenesis' below.)
●Meniere syndrome – The symptomatic triad of episodic vertigo, tinnitus, and hearing loss occurring secondary to other inner ear disorders.
EPIDEMIOLOGY — Meniere disease (MD) may occur at any age, but symptoms typically begin between the ages of 20 and 40 years.
Meniere syndrome in children is most often associated with congenital malformations of the inner ear [2,3], and in adults it may be caused by acquired otologic disease (table 1). (See 'Differential diagnosis' below.)
The incidence of MD is difficult to determine because of the lack of widely accepted diagnostic criteria but ranges from 10 to 150 per 100,000 persons [4]. Among patients with MD, bilateral disease occurs in 10 to 50 percent of patients [5-7].
PATHOGENESIS — The classic pathologic lesion of Meniere disease (MD) is termed endolymphatic hydrops, which can only be definitively diagnosed by postmortem histopathologic analysis of the temporal bone. However, while endolymphatic hydrops is present in all patients with MD, not all patients with endolymphatic hydrops are symptomatic. In some studies, the degree of hearing loss, but not other clinical features of MD (eg, vertigo, tinnitus), may correlate with the severity of endolymphatic hydrops [8]; thus, the exact mechanism responsible for all of the symptoms of MD is unknown.
Endolymphatic hydrops can cause distortion and distention of the membranous, endolymph-containing portions of the labyrinthine system (figure 1). Although most patients with endolymphatic hydrops have no identifiable additional otologic disease, multiple potential causes have been proposed (table 1). In affected patients, is unclear why excess fluid builds up in the endolymphatic spaces of the inner ear. Lack of a single etiologic cause may reflect underlying clinical and genetic heterogeneity in MD [9].
Proposed etiologies include:
●One theory postulates an abnormality in the flow of endolymph or resorption of endolymph at the endolymphatic sac. Endolymphatic hydrops has been experimentally induced in animals by blocking the entrance to the endolymphatic sac [10]. In another hypothesis, endolymphatic hydrops may begin as a result of detached saccular otoconia (calcium deposits), which may block the normal flow of endolymph. This mechanism has also been postulated as the cause of benign paroxysmal positional vertigo [11]. (See "Benign paroxysmal positional vertigo", section on 'Pathogenesis'.)
●Hypoplasia of the vestibular aqueduct [12].
●An immunologic mechanism, with autoantibodies against some component of the endolymphatic sac [13-15].
●An autosomal dominant inheritance pattern has been observed in 8 to 15 percent of patients with MD [16,17], raising the possibility of a genetic etiology. Among patients with a family history, earlier age of onset and more severe symptoms were observed in successive generations [16]. No specific gene has been identified, however.
●Migraine occurs more commonly in patients with MD than in the general population, leading to the postulation of a common vascular pathophysiology for the two disorders [18,19].
DIAGNOSIS
Clinical presentation — The course of Meniere disease (MD) is variable. Some patients experience progressive hearing loss with infrequent vestibular symptoms; some have severe and frequent vertigo with only mild auditory symptoms; and some manifest both auditory and vestibular symptoms in equal measure. Approximately two-thirds of patients experience vertigo attacks in clusters, while one-third have sporadic attacks. The frequency of vertigo episodes may decline over time [20]. Most patients tend to cycle from active symptoms to prolonged remissions.
●Vertigo – The vertigo is typically described as a rotatory spinning or rocking, may be associated with nausea and vomiting, and persists from 20 minutes to 24 hours [21]. Additional sensations of disequilibrium or other patterns of dizziness are seen in about 15 percent of cases; the sense of disequilibrium may persist between acute vertigo attacks. (See "Evaluation of the patient with vertigo".)
●Hearing loss – Early in the presentation, hearing loss usually fluctuates and often initially affects only the lower frequencies [22]. Hearing loss typically progresses and often results in permanent hearing loss at all frequencies in the affected ear over the subsequent 8 to 10 years. Episodes of hearing loss are typically associated with intense ipsilateral aural fullness or head pressure [21]. (See "Etiology of hearing loss in adults".)
●Tinnitus – Tinnitus may be fluctuating or constant, with variable pitch and intensity. Tinnitus may occur concurrent or independent of auditory symptoms or vertigo. (See "Etiology and diagnosis of tinnitus".)
Diagnostic criteria and testing — A clinical diagnosis of MD is made based upon the following criteria [23-25]:
●Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
●Audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear
●Fluctuating aural symptoms (reduced or distorted hearing, tinnitus, or fullness) in the affected ear
●Symptoms not better accounted for by another vestibular diagnosis
Patients typically have auditory and/or vestibular symptoms for three to five years before they meet the diagnostic criteria for MD; a large time gap between episodes may preclude a more rapid diagnosis.
Although audiometric testing is a required part of the diagnostic evaluation, there is no specific diagnostic test for MD. Other tests commonly used in the evaluation of patients with symptoms of MD include imaging and vestibular testing. Imaging, although not required for diagnosis of MD, is frequently performed to exclude important disorders that can present with similar symptoms (eg, vestibular schwannoma). (See 'Differential diagnosis' below.)
A review of the testing and test results in patients with MD are as follows:
●Audiometry – Audiometry is a necessary part of the evaluation in all patients with suspected MD [26]. The most common audiometric pattern in early MD is a low-frequency or combined low- and high-frequency sensorineural hearing loss with normal hearing in the mid-frequencies. Over time, the hearing loss "flattens out" with hearing loss in all frequency ranges.
●Vestibular testing – Vestibular testing, if performed, may be normal early in the course of the disease but will eventually become abnormal on the affected side. Vestibular testing is not mandatory; it is primarily useful in determining candidacy for interventional treatments in patients with more severe or persistent symptoms.
Vestibular evaluation includes electronystagmography (ENG), rotary chair testing, and computerized dynamic posturography. With progression of MD, both the ENG and rotary chair test will likely show declining peripheral vestibular function in the affected ear. The ENG (particularly the caloric test, in which the ear canals are irrigated with warm and cool water to stimulate the inner ear) is more sensitive for inner balance dysfunction, but the rotary chair test is more specific. (See "Evaluation of the patient with vertigo", section on 'Electronystagmography and video nystagmography'.)
●Imaging – Magnetic resonance imaging (MRI) of the temporal bone can identify features that support a diagnosis of MD (eg, less prominent endolymphatic duct), but the findings are not diagnostic [27]. Nevertheless, MRI (or computed tomography [CT] if MRI is contraindicated) is typically done to rule out central nervous system (CNS) lesions that can mimic MD, including tumors, aneurysms or stenosis of the posterior circulation, Arnold-Chiari malformations, and multiple sclerosis. (See "Principles of magnetic resonance imaging", section on 'Precautions' and 'Differential diagnosis' below.)
●Other tests – Other testing may be used as part of the evaluation of MD, but these tests are not standardized and are not widely available for clinical use.
•Tests for antibodies against inner ear antigens exist [28-30], but they are not considered to be clinically useful and are not part of a routine evaluation for MD.
•There are tests for endolymphatic hydrops, which include glycerine, urea, or sorbitol "stress" tests [31] and electrocochleography [32]. However, these tests have low sensitivity and specificity, and their role in the diagnosis and management of MD is controversial.
•The vestibular evoked myogenic potential (VEMP) is a test that shows promise for diagnosis and monitoring [33]. Cervical VEMP (cVEMP) is an inhibitory sacculocolic reflex test that shows characteristic changes in symptomatic ears of patients with MD [33] and may detect early saccular hydrops before the onset of classic Meniere symptoms [34]. Ocular VEMP (oVEMP) engages both utricular and saccular afferent nerve fibers and may also be useful in assessment of Meniere patients [35]. In addition to diagnosis, VEMP may be useful for monitoring patients for disease progression and to identify the active ear in patients with bilateral disease. However, VEMP is an emerging technology that has not yet been standardized or fully validated clinically. (See "Evaluation of the patient with vertigo", section on 'Vestibular evoked myogenic potentials'.)
DIFFERENTIAL DIAGNOSIS — A variety of other conditions can present with symptoms similar to Meniere disease (MD) and are often considered in the differential diagnosis (table 2). Such conditions include:
●Migraine (migrainous vertigo or vestibular migraine) – The prevalence of migrainous vertigo, or vestibular migraine, is approximately 3 to 5 percent in the general population, higher than the 0.2 percent prevalence of MD in the United States population [36]. Migraine-associated vertigo is an important consideration, especially among those with a history of migraine headache, and in young patients with new onset of episodic vertigo [37]. Thus, among those with vertigo, migraine should always be considered as an alternative diagnosis or coexisting condition.
In cases of vestibular migraine, headache may occur during or following the vertigo (in which case vertigo is considered the migraine aura). Vestibular migraine is often accompanied by photophobia or phonophobia, symptoms not seen in vertigo attacks associated with MD. Diagnostic criteria for vestibular migraine include episodic vestibular symptoms and at least two migraine symptoms (migrainous headache, photophobia, phonophobia, or visual or other aura) occurring during at least two vertiginous episodes [38]. (See "Vestibular migraine", section on 'Clinical features'.)
Successful treatment with triptans during an attack is generally diagnostic, although the vertigo symptom is less responsive than the headache. The evaluation and diagnosis of vestibular migraine is discussed in detail elsewhere. (See "Vestibular migraine", section on 'Diagnosis'.)
●Vestibular schwannoma – Patients with vestibular schwannoma (acoustic neuroma) typically present with progressive asymmetric hearing loss but may occasionally have fluctuating hearing loss. These patients rarely have true vertigo but may complain of imbalance or disequilibrium. Occasional patients with vestibular schwannoma will have tinnitus and imbalance but no hearing loss. Among patients with vestibular schwannoma, auditory brainstem response (auditory evoked response) testing and magnetic resonance imaging (MRI) show abnormalities indicating compression of the eighth nerve complex. (See "Vestibular schwannoma (acoustic neuroma)".)
●Multiple sclerosis – Multiple sclerosis (MS) can present with symptoms identical to those of MD. However, during an attack of MS with vertigo, the observed nystagmus is typically more severe and longer lasting than in patients with MD, and patients with MS may have additional neurologic complaints. On electronystagmogram (ENG) testing, central abnormalities are often seen in patients with MS but are typically absent in early MD. In MS, white matter lesions may be seen on brain MRI and cerebrospinal fluid abnormalities may be detected; these findings are absent in patients with MD. (See "Evaluation and diagnosis of multiple sclerosis in adults".)
●Transient ischemic attack – Although transient ischemic attacks (TIAs) may present with similar symptoms, there are differences which distinguish them from MD attacks. Symptoms due to TIAs are typically shorter in duration than attacks of MD. Patients with TIAs rarely experience simultaneous vestibular and cochlear symptoms, while these commonly occur together in MD. TIAs do not cause persistent or recurrent tinnitus or objective hearing loss, while these are common in MD. (See "Initial evaluation and management of transient ischemic attack and minor ischemic stroke".)
●Benign paroxysmal positional vertigo – Patients with benign paroxysmal positional vertigo (BPPV) also have recurrent attacks of vertigo, but unlike that in MD, the vertigo is typically precipitated by head movements and brief in duration. Additionally, in BPPV, hearing symptoms are absent. (See "Benign paroxysmal positional vertigo", section on 'Symptoms'.)
●Cogan syndrome – Cogan syndrome is a chronic inflammatory condition seen most commonly in young adults, and is characterized by ocular disease, vasculitis, and vestibuloauditory symptoms similar to those seen in MD. The clinical features, evaluation, and diagnosis of Cogan syndrome is reviewed in detail elsewhere. (See "Cogan syndrome".)
●Other conditions – Chronic conditions such as diabetes, anemia, and thyroid disease may cause symptoms that have some overlap with features of MD, including tinnitus and chronic disequilibrium, although true vertigo is not typical. In addition, these comorbid conditions may affect the severity of MD.
TREATMENT — Determining the optimal treatment regimen for Meniere disease (MD) is limited by the lack of high-quality data [9,39]. The relapsing and remitting nature of the disorder makes comparisons of various treatments difficult.
Goals and patient expectations — Treatment of MD may improve or relieve symptoms but does not correct the underlying abnormal pathophysiology. Patient education is an important part of management and includes an explanation of the disease and its natural history, review of treatment options, and expectations regarding response to treatment.
The goals of treatment are to improve the patient's quality of life by [9]:
●Reducing the frequency and severity of vertigo attacks
●Reducing or eliminating the tinnitus and hearing loss associated with attacks
●Alleviating chronic symptoms of tinnitus and disequilibrium
●Minimizing disability associated with MD
●Preventing progressive hearing loss
With noninvasive management (including dietary modification/lifestyle adjustment, medications, and vestibular rehabilitation), up to 90 percent of patients with MD are able to maintain normal daily activities. Vertigo attacks can be controlled in 90 to 95 percent of patients with treatment; patients with suspected MD should be referred at a relatively early stage to an otolaryngologist to control attacks and minimize progression of hearing loss.
Initial diet and lifestyle adjustment for all patients — Initial therapy for all patients with MD includes dietary and lifestyle adjustments.
Patients with MD are more vulnerable to dietary and environmental factors, and triggers may include high salt intake, caffeine, alcohol, nicotine, stress, monosodium glutamate (MSG), and allergies (food and environmental) [40]. Among patients with identified triggers, avoidance or minimization of exposure to these substances, as well as treatment for allergies if appropriate, may alleviate or ameliorate symptoms.
We suggest salt restriction as part of initial therapy for all patients [23,41,42], although there is a lack of high-quality data supporting the efficacy of this approach [9,40]. An appropriate salt-restricted diet will include a maximum of 2 to 3 g of sodium daily, with the daily sodium intake evenly spread across meals to avoid a large bolus at any time. (See "Patient education: Low-sodium diet (Beyond the Basics)".)
In addition, we suggest limiting caffeine and alcohol consumption to one caffeinated beverage (coffee, tea, or cola) and one alcoholic drink daily, particularly if these are triggers for attacks. Caffeine and nicotine are vasoconstrictors that may reduce microvascular flow in the labyrinthine system. Similarly, alcohol causes fluid and electrolyte shifts that may stress a vulnerable ear.
We advise that patients with MD continue the above dietary and lifestyle adjustments indefinitely.
Vestibular rehabilitation for residual disequilibrium between attacks — We suggest referring patients with MD and persistent disequilibrium symptoms between attacks for vestibular rehabilitation therapy. Vestibular rehabilitation uses exercise activities to maximize balance and central nervous system (CNS) compensation for disequilibrium symptoms.
Although vestibular rehabilitation has been used primarily in patients whose vertigo has responded to medical or surgical treatment but who have residual disequilibrium [43], it may also be used for any patient with MD who experiences significant balance and disequilibrium symptoms between acute vertigo attacks [9]. Participation in vestibular rehabilitation therapy does not reduce the frequency or severity of vertigo attacks.
Further, vestibular rehabilitation has no role in the management of acute vertigo due to MD [23].
Pharmacotherapy for patients with refractory symptoms — A number of medications have been used to treat MD, both to reduce the intensity and severity of attacks and to treat acute vertigo. Daily vasodilator or diuretic therapy with as-needed vestibular suppressants and antiemetics are typically used when dietary and lifestyle adjustments alone do not adequately control the episodes. In the majority of patients, combinations of these agents can control episodes of vertigo or decrease their severity, although they have not been shown to prevent hearing loss [44].
Chronic management with chronic betahistine or diuretic therapy — In patients with MD with a poor quality of life due to refractory symptoms (ie, recurrent vertigo) despite dietary and lifestyle interventions, we suggest the use of pharmacotherapy rather than no pharmacotherapy.
Betahistine (a vasodilator available in Europe, Central and South America, and Canada, and as a compounded preparation in the United States), and diuretics are the two pharmacologic options to reduce the severity and intensity of attacks. We suggest treatment with betahistine rather than diuretics when available because it is well tolerated and does not require monitoring of metabolic effects, including blood pressure, kidney function, and electrolytes. However, when betahistine is unavailable, we treat these patients with chronic diuretic therapy.
If betahistine is selected, the typical maintenance dose is 8 to 16 mg orally three times daily. We generally begin with once-daily dosing and evaluate the patient's response at –two- to four-week intervals; if there is insufficient control of symptoms, we increase to twice daily, and then to three times daily if needed. Maintaining patients on twice-daily dosing is also an option [45].
If a diuretic is selected, we typically prescribe hydrochlorothiazide-triamterene 25 mg/37.5 mg orally once daily. Other diuretic options include furosemide 20 mg orally once daily or acetazolamide 250 to 500 mg orally twice daily.
Concomitant treatment with both betahistine and a diuretic is also an option [23], although this is less frequently used. Some experts add a second agent if there is insufficient control of symptoms on a single agent, although there are no high-quality data to support this approach.
For patients who respond to treatment with betahistine or a diuretic, treatment is continued for approximately six months; if symptoms remain well controlled, the medication can be tapered (for betahistine) and discontinued. Pharmacotherapy can be resumed if troublesome symptoms recur.
There are no high-quality data on the efficacy of these medications. However, these medications are widely used due to possible benefit and the low risk of adverse effects. Although early trials demonstrated efficacy of both betahistine and diuretics in the control of vertigo symptoms [46], two subsequent systematic reviews found methodologic flaws in the included studies and determined that there was insufficient evidence to establish efficacy [47,48]. In addition, in a subsequent randomized trial comparing placebo with low- and high-dose betahistine, there was no difference in the incidence of attacks between the three groups [49].
Theoretically, betahistine and diuretics may reduce the degree of endolymphatic hydrops. Betahistine is thought to act by improving microvascular circulation in the stria vascularis of the cochlea or by inhibiting vestibular nuclei activity [41,46,50,51]. Diuretics are thought to improve endolymph resorption and also maintain a balanced fluid dynamic in the endolymphatic system, particularly when used in conjunction with sodium restriction.
Appropriate monitoring for renal function, electrolytes, and blood pressure should be performed for any patient on chronic diuretic therapy, with particular cautions about the risks of orthostasis in older adults. (See "Diuretic-induced hyponatremia" and "Time course of loop and thiazide diuretic-induced electrolyte complications" and "Mechanisms, causes, and evaluation of orthostatic hypotension", section on 'Medications'.)
Acute treatment with vestibular suppressants and antiemetics — Acute episodes of vertigo should be managed with vestibular suppressants and antiemetics if necessary (table 3). The lowest effective medication dose should be used, and the potential adverse effects (including the risk of sedation) should be considered. (See "Treatment of vertigo".)
●For patients with MD (managed with dietary and lifestyle adjustments with or without chronic betahistine or diuretic pharmacotherapy), we use benzodiazepine vestibular suppressants to treat episodic acute vertigo attacks. We use clonazepam 0.25 mg to 0.5 mg orally two to three times daily as needed, or diazepam 1 to 5 mg orally twice daily as needed.
●For patients with severe nausea or vomiting associated with acute vertigo attacks, we treat with promethazine 12.5 to 25 mg orally every six to eight hours as needed, or ondansetron 4 mg orally two to three times daily as needed.
For patients not able to tolerate oral medications, promethazine may be administered rectally or intramuscularly (IM); intravenous (IV) administration is also an option, although is generally not preferred due to risk of extravasation injury. Ondansetron may be administered IV.
For the patient with acute vertigo and persistent vomiting, in addition to antiemetic treatment, assessment of volume status and with appropriate volume repletion is appropriate.
Refractory, disabling symptoms despite initial pharmacotherapy — Approximately 10 percent of patients with MD have intractable, unremitting, or progressive symptoms that significantly impair their quality of life despite the addition of initial pharmacotherapy (betahistine or diuretic) to dietary and lifestyle adjustments. Patients with a continued poor quality of life due to MD symptoms despite three months of pharmacotherapy are candidates for additional treatments.
Among patients with severe symptoms, there are criteria available to quantify the severity of their functional impairment. As an example, there is a self-assessment tool, the Meniere Disease Functional Level Scale (table 4), which is useful in evaluating the severity of MD symptoms before treatment as well as assessing the efficacy of therapy.
Separate criteria for evaluating occupational disability due to MD also exist [52]:
●Mild – Intermittent or continuous dizziness/unsteadiness that precludes working in a hazardous environment
●Moderate – Intermittent or continuous dizziness that results in a sedentary occupation
●Severe disability – Symptoms so severe as to exclude gainful employment
For patients with symptoms severe enough to require further treatment, there is no widely accepted agreement on which treatment is preferred. Our approach is as follows:
Continue pharmacotherapy with betahistine or diuretcs if beneficial — Regarding the continuation of pharmacotherapy in patients with refractory symptoms:
●For patients who experience any degree of symptomatic improvement with betahistine or diuretic therapy, the medication is continued.
●For those patients who experience no improvement in symptoms with such pharmacotherapy, the medication is discontinued.
Glucocorticoid therapy for all patients with refractory, disabling symptoms — For all patients with refractory MD, (including those who continue to take betahistine or diuretics and those in whom such pharmacotherapy is discontinued), we suggest treatment with glucocorticoids rather than other therapies. Glucocorticoid treatment options include systemic or intratympanic administration. (See 'Systemic glucocorticoids' below and 'Intratympanic glucocorticoids' below.)
Although intratympanic administration of glucocorticoids may have slightly better efficacy, oral administration is generally better tolerated and preferred by most patients.
In addition to considering the patient's preferences, we evaluate their medical comorbidities, considering any possible contraindications to a short course of high dose oral glucocorticoids. For those patients in whom systemic glucocorticoid therapy is contraindicated, we treat with intratympanic glucocorticoids.
Systemic glucocorticoids — For the majority of patients with MD, hearing loss, and refractory, disabling vertigo symptoms despite first-line treatments, we treat with a limited course of oral glucocorticoids [53].
We typically treat with prednisone 1 mg/kg daily, for a maximum dose of 60 mg orally once daily, for 7 to 14 days.
The use of systemic glucocorticoid therapy for MD is based upon the possible immunologic etiology of the disease and the efficacy of steroid therapy in the treatment of sudden sensorineural hearing loss [9]. This noninvasive treatment is often preferred by patients to invasive treatment options.
For those patients in whom high-dose glucocorticoid therapy may present a medical management issue (eg, insulin dependent diabetes), we would prescribe in consultation with the patient's primary care clinician or appropriate specialist. Additionally, we would not use this treatment in those patients for whom systemic glucocorticoids are contraindicated due to intolerance or comorbidity. (See "Major adverse effects of systemic glucocorticoids".)
Intratympanic glucocorticoids — For patients with MD with disabling vertigo symptoms despite first-line treatments, and in whom oral glucocorticoid therapy is contraindicated, or who through shared decision making prefer intratympanic therapy, we offer treatment with intratympanic glucocorticoids.
With this technique, a glucocorticoid solution is delivered into the middle ear space by injection or cannula. We administer an initial intratympanic dexamethasone injection, approximately 0.3 mL of a 10 to 24 mg/mL solution (available preparations vary from 4 to 10 mg/mL for a traditional solution to 24 to 40 mg/mL for the compounded preparation) into the affected ear, and we may repeat injections at once weekly intervals for an additional one to two injections based upon the patient's response. If there is no improvement in vertigo after three injections, we discontinue this treatment and move on to other available therapies for patients with preserved hearing. We routinely check an audiogram after completion of the injection protocol or at any point if the patient reports an increase in tinnitus or decrease in hearing.
Treatment with intratympanic glucocorticoids is based upon the possible immunologic basis of MD. As in systemic therapy, there is no ototoxicity associated with this treatment [9].
In prospective studies of patients with MD, there were improvements in vertigo and functional scores following intratympanic dexamethasone injections [54,55]. The benefits may be maintained over time (6 to 24 months), although patients may require repeat injections and the efficacy of subsequent injections may decrease [55-57].
Although intratympanic dexamethasone may not be as effective as intratympanic gentamicin for control of vertigo, there may be a slight improvement in hearing in some treated patients. As an example, in a trial comparing low-dose intratympanic gentamicin and intratympanic dexamethasone, control of vertigo was greater for the gentamicin group, but among those in the dexamethasone group with complete vertigo control, there was no loss, and even slight improvement in hearing [58].
Studies of intratympanic dexamethasone have used different dosing and frequency, and optimal regimens for intratympanic glucocorticoids have not been established.
Refractory, disabling symptoms despite glucocorticoid therapy — For patients with refractory MD symptoms and continued poor quality of life despite treatment with systemic or intratympanic glucocorticoids, we offer additional treatments. For all patients, and in particular those patients who have not experienced any improvement with any previous therapy, we also reconsider alternative diagnoses as the cause of their symptoms. (See 'Differential diagnosis' above.)
When considering further treatment for MD, we generally use the degree of labyrinthine function (severity of vertigo attacks and the degree of disequilibrium between attacks) and the level of hearing loss to determine the most appropriate management for an individual patient.
Treatments include destructive therapies (eg, intratympanic gentamicin, surgical labyrinthectomy, and vestibular neurectomy) which reduce or eliminate signals from the affected labyrinthine system to the brain, and nondestructive procedures (including endolymphatic sac procedures [decompression or shunting or both], sacculotomy) which may reduce the accumulation of fluid in the endolymphatic spaces or otherwise alter fluid and electrolyte physiology in the inner ear; the exact mechanism of their effect is unknown.
As an example, for patients with preserved hearing in the affected ear, in whom maintenance of hearing is a primary concern, we avoid therapies which may potentially result in hearing loss. Conversely, for patients with complete hearing loss in the affected ear, avoidance of therapies which may cause hearing damage in the treated ear is less of a concern.
Additionally, treatments that permanently and completely eliminate the labyrinthine signals to the brain are used as a last option for these patients. Although these treatments eliminate the disabling vertigo, the potential future development of MD in the contralateral ear is a concern; in such cases, the patient may be left with no labyrinthine function.
Patients with preserved hearing in affected ear
●For MD patients with preserved hearing, refractory vertigo symptoms, and continued poor quality of life despite treatment with systemic or intratympanic glucocorticoids, we offer treatment with endolymphatic sac procedures (including decompression and/or shunting) or sacculotomy.
●For patients with preserved hearing who have no or inadequate symptomatic improvement from an endolymphatic sac procedure or sacculotomy, we typically then offer treatment with intratympanic gentamycin. In addition, particularly for patients with no response to any previous therapy, we consider an alternative diagnosis as the cause for their symptoms. (See 'Differential diagnosis' above.)
●If treatment with intratympanic gentamycin is unsuccessful in adequately improving symptoms, we then offer ablative surgery with vestibular neurectomy or labyrinthectomy as a last option.
•Endolymphatic sac procedures (decompression and/or shunting) and sacculotomy – The nondestructive surgical procedures (including endolymphatic sac procedures [decompression or shunting or both] and sacculotomy) are other treatment options in patients with MD and preserved hearing. Although they are associated with a low risk of sensorineural hearing loss in patients with intact hearing, there are concerns that their efficacy may be due to placebo effect [59].
These procedures expose the endolymphatic sac and duct, with the aim to improve drainage of endolymph. However, anatomic studies of the endolymphatic sac indicate that such drainage is not plausible [60].
Two trials have examined the efficacy of these procedures. In one trial of endolymphatic sac decompression, installation of steroids into the sac following drainage did not relieve vertigo symptoms but increased the percent of patients with some hearing improvement [61]. In a trial of endolymphatic shunt surgery using sham surgery as a control, there was no difference in efficacy between groups [62]. However, in subsequent data reanalysis, endolymphatic shunt surgery was superior to sham surgery for control of vertigo, nausea, vomiting, and tinnitus [63].
In multiple case series, control of vertigo was reported in 75 to 80 percent of patients undergoing these procedures [61,64-67]. Symptomatic improvement in hearing and tinnitus have also been reported with apparent long-term maintenance of these benefits [61,67,68].
•Intratympanic gentamicin – Aminoglycosides are toxic to the sensory neuroepithelium of the inner ear; gentamicin is more vestibulotoxic than cochleotoxic and is the preferred aminoglycoside for intratympanic administration. With this technique, gentamicin is delivered into the middle ear space (by injection or cannula), allowing the drug to locally penetrate the labyrinth through the round window membrane. It destroys hair cells in the semicircular canals, ablating labyrinthine function, theoretically without causing any systemic effects. (See "Pathogenesis and prevention of aminoglycoside nephrotoxicity and ototoxicity".)
We administer an initial intratympanic gentamicin injection (approximately 0.3 mL of a 26 to 40 mg/mL buffered solution, concentration depends upon availability) into the affected ear and may repeat at one week if there is no response to the first injection. If there is some improvement in symptoms, we administer another injection one to two weeks later. We routinely check an audiogram after the second injection or at any point if the patient reports an increase in tinnitus or decrease in hearing.
In multiple prospective studies, intratympanic gentamicin was associated with improved vertigo in 80 to 90 percent of patients with MD [69-73]. However, treatment with intratympanic aminoglycoside is itself associated with a risk of irreversible sensorineural hearing loss in up to 30 percent of patients [74].
Minimizing the dose of gentamicin administered may impact vestibular function without affecting cochlear (hearing) function. As examples:
-In one prospective study, a single, low-dose transtympanic gentamicin injection resulted in good control of vertigo in 76 percent of patients followed for four or more years, with minimal hearing loss [75]. A second injection was required to control vertigo symptoms in 15 to 20 percent of patients.
-In a randomized trial comparing low-dose intratympanic gentamicin with intratympanic dexamethasone, complete vertigo control was greater in the gentamicin group compared with the dexamethasone group (81 versus 43 percent) [58]. Significant hearing loss (>10 dB) occurred in 13 percent of patients in the gentamicin group.
There is no consensus regarding the optimal protocol for intratympanic gentamicin administration, however. As an example, in one meta-analysis, titrating repeated gentamicin doses to vestibular response was superior to other treatment regimens [76]. In another meta-analysis, there was no difference in efficacy between fixed dose and titration regimens, but there were significant quality issues in all included trials [77].
Despite alterations in regimens meant to mitigate the ototoxicity associated with this treatment, there is no way to completely eliminate the risk of hearing loss associated with IT gentamicin. Thus, although effective in controlling vertigo, it must be used with caution in patients with preserved hearing.
Additionally, despite the low risk of systemic effects (eg, nephrotoxicity) with intratympanic administration, we do not offer intratympanic gentamicin therapy in patients with significantly impaired kidney function or a solitary functioning kidney.
•Labyrinthectomy – Labyrinthectomy involves surgical destruction of the bony and membranous labyrinth by removal of all of the neuroepithelium from the affected side; this relieves vertigo in virtually all patients but also causes irreversible unilateral hearing loss in all treated patients [78]. Thus, this procedure is only indicated in individuals with significant or complete hearing loss on the affected side, and those who have intractable symptoms despite medical therapy.
The concern of many clinicians is that patients may have subclinical disease in the opposite ear that will ultimately progress, and for such patients this may ultimately result in total deafness. While labyrinthectomy is an effective procedure, we avoid this treatment if there is any evidence of MD in the contralateral ear. If the decision is made to proceed with labyrinthectomy, patients should be made aware of the potential risks of future loss of bilateral hearing and vestibular function should MD develop in the contralateral ear.
•Vestibular neurectomy – Vestibular neurectomy involves surgical lysis of the vestibular nerve bundle as it enters the internal auditory canal. It relieves vertigo in 90 to 95 percent of patients and is associated with a low risk (10 to 20 percent) of sensorineural hearing loss [78].
However, vestibular neurectomy requires craniotomy with general anesthesia, requiring overnight intensive care unit monitoring.
Concerns over side effects may limit use. However, in a cohort study of patients with unilateral disabling MD treated with vestibular neurotomy, 90.5 percent no longer had vertiginous attacks and there were no drop attacks after an average follow-up of over 12 years. [79]. Furthermore, a separate case series indicated resolution or improvement of vertigo postoperatively in all 12 patients with MD who underwent the procedure with the possibility of using the endoscope as an adjunct in some procedures [80].
Vestibular neurectomy is reserved for those patients with persistent symptoms despite other treatments, who have relatively preserved of hearing in the affected ear, and who are unwilling to risk the hearing loss associated with intratympanic gentamicin.
Patients with complete hearing loss in affected ear
●For MD patients with complete hearing loss in the affected hear, refractory vertigo symptoms, and continued poor quality of life despite treatment with systemic or intratympanic glucocorticoids, we suggest intratympanic gentamycin treatment.
●If treatment with intratympanic gentamycin is unsuccessful in adequately improving symptoms, we offer ablative surgery with vestibular neurectomy or labyrinthectomy. However, for patients in whom there is no symptomatic response to gentamycin treatment, we consider an alternative diagnosis as the cause for their symptoms before proceeding to surgery. (See 'Differential diagnosis' above.)
HEARING AMPLIFICATION FOR HEARING LOSS — Hearing aids should be considered for patients with significant binaural hearing loss due to Meniere disease (MD). However, among these patients (particularly early in their disease), hearing fluctuation often leads to frustration with amplification devices and poor patient compliance. Hearing amplification is reviewed in detail elsewhere. (See "Hearing amplification in adults".)
THERAPIES THAT WE DO NOT USE — There are several therapies used in the treatment of Meniere disease (MD) that we do not routinely use or advise due the lack of efficacy and possible evidence of harm.
●Positive pressure pulse generator – Positive pressure ("overpressure") applied to the middle ear may improve fluid exchange in the inner ear. Overpressure treatment, in which a device applies pulses of pressure to the middle ear via a ventilation tube, has been used in patients who failed medical therapy or as an adjunct to medical therapy in patients with functional level 3 or greater (table 4). Maintenance of a patent tympanostomy tube is required for overpressure treatment.
Meta-analyses including prospective studies and randomized trials using overpressure in MD are mixed, with one showing improved vertigo symptoms and hearing [81] and another showing no improvement in vertigo and a possible adverse effect on hearing [82]. In addition, the long-term efficacy of overpressure in the control of vertigo is uncertain, and hearing conservation should not be expected in all patients using this therapy.
In addition, the pulse generator device is expensive and insurance coverage for this procedure is variable. Thus, out-of-pocket costs may be an additional consideration for use of this therapy.
●Vitamins and herbal therapies – Some patients have reported symptomatic improvement with vitamin regimens and herbal remedies. However, these treatments are not supported by evidence, and we do not advise that patients use these treatments to manage MD [9].
●Systemic immunotherapy – There are no high-quality data supporting the use of immunosuppressive therapy with low-dose methotrexate [83] or etanercept [84], and we do not use these medications to treat MD.
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Hearing loss and hearing disorders in adults".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, “The Basics” and “Beyond the Basics.” The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on “patient info” and the keyword(s) of interest.)
●Basics topic (see "Patient education: Meniere disease (The Basics)")
●Beyond the Basics topic (see "Patient education: Vertigo (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
●Pathophysiology – Meniere disease (MD), characterized by the classic triad of symptoms (episodic vertigo, tinnitus, and hearing loss), is likely caused by endolymphatic hydrops of the labyrinthine system of the inner ear (figure 1). (See 'Definitions' above.)
●Clinical presentation – The course of MD is variable. Some patients experience progressive hearing loss with infrequent vestibular symptoms; some have severe and frequent vertigo with only mild auditory symptoms; and some manifest both auditory and vestibular symptoms in equal measure. Most patients tend to cycle from active symptoms to prolonged remissions. (See 'Clinical presentation' above.)
●Diagnostic criteria – A clinical diagnosis of MD is made based upon the following criteria:
•Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
•Audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear
•Fluctuating aural symptoms (reduced or distorted hearing, tinnitus, or fullness) in the affected ear
•Symptoms not better accounted for by another vestibular diagnosis
Although audiometric testing is a required part of the diagnostic evaluation, there is no specific diagnostic test for MD. Imaging, although not required for diagnosis of MD, is frequently performed to exclude important disorders that can present with similar symptoms. (See 'Diagnostic criteria and testing' above.)
●Differential diagnosis – A variety of other conditions can present with symptoms similar to MD and are often considered in the differential diagnosis. The conditions include vestibular migraine, vestibular schwannoma, multiple sclerosis (MS), transient ischemic attacks (TIAs), benign paroxysmal positional vertigo, and Cogan syndrome (table 2). (See 'Differential diagnosis' above.)
●Dietary and lifestyle modifications for all patients – As initial therapy for all patients with MD, we suggest lifestyle modification, including salt restriction, and limiting caffeine and alcohol consumption (to a maximum of one of each beverage daily) (Grade 2C). If other triggers are identified (eg, nicotine, stress, monosodium glutamate [MSG]), these should also be avoided. Dietary and lifestyle modifications should be continued indefinitely. (See 'Initial diet and lifestyle adjustment for all patients' above.)
●Vestibular rehabilitation for persistent disequilibrium – For patients with MD and persistent disequilibrium symptoms between attacks, we suggest referral for vestibular rehabilitation therapy (Grade 2C). Although vestibular rehabilitation does not reduce the frequency of vertigo attacks, the exercise activities maximize balance and central nervous system (CNS) compensation for disequilibrium symptoms. Vestibular rehabilitation has no role in the treatment of acute vertigo due to MD. (See 'Vestibular rehabilitation for residual disequilibrium between attacks' above.)
●Pharmacotherapy for refractory symptoms – For all patients with MD with refractory symptoms and poor quality of life despite dietary and lifestyle interventions, we suggest the use of pharmacotherapy rather than no pharmacotherapy (Grade 2C). Betahistine and diuretics are the two options for pharmacologic therapy to reduce the severity and intensity of MD attacks. We suggest treatment with betahistine rather than diuretics, when available (Grade 2C). Betahistine is well tolerated and, unlike diuretics, does not require monitoring of adverse effects such as hypotension, altered kidney function, and electrolyte abnormalities. (See 'Chronic management with chronic betahistine or diuretic therapy' above.)
For patients who respond to treatment with betahistine or a diuretic, treatment is continued for approximately six months; if symptoms remain well controlled, the medication can be tapered (for betahistine) and discontinued. Pharmacotherapy can be resumed if troublesome symptoms recur.
Acute episodes of vertigo should be managed with vestibular suppressants and antiemetics if necessary (table 3). (See 'Acute treatment with vestibular suppressants and antiemetics' above.)
●Glucocorticoid therapy (systemic or intratympanic) for persistent symptoms – Among all patients with refractory symptoms severe enough to require further treatment beyond dietary changes, lifestyle adjustment, and first-line pharmacotherapy, there is no widely accepted agreement upon which treatment is preferred. However, we suggest treatment with glucocorticoids rather than other therapies for these patients (Grade 2C). (See 'Glucocorticoid therapy for all patients with refractory, disabling symptoms' above.)
•For the majority of patients with MD and refractory, disabling vertigo symptoms despite first-line treatments, we treat with a limited course of oral glucocorticoids.
•For patients with MD with disabling vertigo symptoms despite first-line treatments, and in whom oral glucocorticoid therapy is contraindicated, or who through shared decision-making prefer intratympanic therapy, we offer treatment with intratympanic glucocorticoids.
●Additional treatment options for patients refractory to glucocorticoid therapy – For patients with refractory MD symptoms and continued poor quality of life despite treatment with glucocorticoids (systemic or intratympanic), we offer additional treatments. We generally use the degree of labyrinthine function (severity of vertigo attacks and the degree of disequilibrium between attacks) and the level of hearing loss to determine the most appropriate management for an individual patient. (See 'Refractory, disabling symptoms despite glucocorticoid therapy' above.)
•For MD patients with preserved hearing, we offer treatment with endolymphatic sac procedures (including decompression and/or shunting) or sacculotomy; if this is unsuccessful, we typically then offer treatment with intratympanic gentamycin.
•For patients with MD with complete hearing loss in the affected ear, we suggest treatment with IT gentamycin rather than labyrinthectomy (Grade 2C). Labyrinthectomy is generally reserved for those patients who have disabling symptoms that persist despite treatment with IT gentamicin. (See 'Patients with complete hearing loss in affected ear' above.)
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Steven Rauch, MD, who contributed to an earlier version of this topic review.
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