Approach

There is no cure for meniere disease (MD). The goals of treatment are vertigo control, prevention of further deterioration in hearing whenever possible, amelioration of tinnitus, and balance control. Treatment options, however, do not appear to influence hearing results or the natural history of MD.[48]

Endolymphatic hydrops has been implicated in the pathophysiology or pathogenesis of MD and, therefore, the management of patients with MD has traditionally been targeted toward decreasing endolymphatic pressure. This has been questioned by a study suggesting that such measures aiming at reduction in hydrops would be unlikely to control the disease. Some histopathologic studies of the temporal bones suggest that, although endolymphatic hydrops is a histologic marker for MD, it is not directly responsible for its symptoms.[16] However, studies from 2010 demonstrate through magnetic resonance imaging the central role of endolymphatic hydrops in the pathology of MD.[17] It is important to note that MD presents a research controversy in evaluating the efficacy of different therapies.[49][50][51]

Dietary changes and lifestyle modification

All patients should be educated on dietary changes and lifestyle modification. Patients should be advised to restrict salt intake to 1500 to 2300 mg/day, as this is thought to prevent sodium-related water retention and redistribution into the endolymphatic system.[21][52]​​ Although there are no randomized controlled trials (RCTs) to document the benefits of low-salt diet on the treatment of MD, patients often report exacerbation of their symptoms or even precipitation of an attack after a salty meal.[52][53] 

Limiting caffeine intake, reducing alcohol consumption, ceasing smoking, and managing stress are also advisable, as these may trigger an attack. However, there is no evidence from RCTs to support or refute the restriction of salt, caffeine, or alcohol intake in patients with MD.[53][54]

Such dietary changes may be the only necessary treatment required in the early stages of the disease.

Medical therapy to decrease endolymphatic pressure

Diuretics are believed to reduce the volume of the endolymph and may be offered for maintenance therapy.[21][55]​ The most commonly used diuretics in the treatment of MD are thiazides-with or without potassium-sparing diuretics (e.g., hydrochlorothiazide/triamterene)-and acetazolamide.[21] Thiazide diuretics are thought to act on the sodium/potassium adenosine triphosphatase levels in the stria vascularis in cochlear tissues and to have an effect on the maintenance of endolymph homeostasis.[56] Acetazolamide is thought to act on carbonic anhydrase in dark cells and in the stria vascularis.[52] If the patient remains symptom-free for 6 months, diuretics may be slowly tapered and restarted if required. If there is no response, the patient should be changed to the alternative diuretic. These medications should not be used in patients with a known or suspected reaction to sulfonamides.

The evidence of the efficacy of diuretics on MD is controversial, and direct evidence of its efficacy on disease progression is lacking in the literature.​​​​[2]​ However, diuretics are still considered by many physicians to be first-line treatment in patients with MD. 

Symptomatic treatments

Vertigo

Symptoms of individual and acute vertigo spells can be treated with vestibular suppressants and antiemetics. However, much of the effect is from the sedative action of these drugs. The literature lacks RCTs assessing the effects of these medications for acute attacks of MD. Commonly used treatments include antihistamines (e.g., meclizine, dimenhydrinate, promethazine), benzodiazepines (e.g., diazepam), and phenothiazines (e.g., prochlorperazine).[57] Diazepam should only be used in acute attacks.[21] It should be prescribed at low doses where possible, and long-term prescription avoided due to the risk of dependency. Anticholinergics (e.g., scopolamine and atropine) are not commonly prescribed due to their significant side-effect profile.[21] 

Betahistine is used in some countries to reduce the frequency and severity of the vertigo attacks in patients with MD. However, one Cochrane review did not find enough evidence to show its efficacy in patients with MD and one 2016 RCT found no significant differences in the mean attack rate compared with placebo.[58][59]​​ Betahistine is not approved in the US; however, it may be compounded if necessary.[58][59]

Corticosteroids, whether used orally or as intratympanic injections, may be used to treat acute attacks of vertigo, especially when accompanied by acute hearing loss and tinnitus. They are widely used because of their anti-inflammatory properties, although no RCTs are available to assess their efficacy in MD.[60] 

Tinnitus

Patients with severe, intractable tinnitus can receive relief with a number of modalities, such as tinnitus maskers, tinnitus retraining therapy (TRT), various forms of sound-based therapies such as neuromonics phase-shift tinnitus reduction, amplifications, medication, and biofeedback. Tinnitus questionnaires are helpful in evaluating the severity of the problem and in documenting the effects of various treatment modalities.[61][62][63]

Tinnitus maskers (white noise generators) are devices similar to hearing aids that fit behind or in the ear. They produce an external sound that distracts the patient from the internal tinnitus noise.

TRT is counseling accompanied by white noise generators. TRT is a favored treatment, but it may take up to 18 months before full benefits are achieved.[64][65]

Amplification (hearing aids) may help in masking the tinnitus and achieving residual inhibition.

Biofeedback techniques attempt to decrease the anxiety that is associated with tinnitus. This can be achieved through relaxation techniques, hypnosis, and cognitive behavioral therapy.

Neuromonics uses a customized neural stimulus combined with specific music, delivered through a coordinated program to interact with, interrupt, and desensitize tinnitus disturbance for long-term benefit.[66][67][68]

Medications such as antidepressants (e.g., amitriptyline) and benzodiazepines (e.g., alprazolam) may help patients with intractable tinnitus, but are associated with adverse effects.[69] They should only be used if the above techniques are unsuccessful and debilitating tinnitus persists.

Hearing loss

Sudden hearing loss is treated with corticosteroids (either orally or intratympanically).

Amplification using fully digital hearing aids with variable digitally adjustable circuitry should be evaluated. The traditional view that amplification does not work for patients with MD is not based on experience with modern amplification.

New forms of directional microphones, digital signal processing circuitry, and wireless technology can provide significant benefits in helping MD patients to hear better in environments with competing noise.[70]

Assistive listening devices are a form of amplification for those with situational difficulties in hearing, and who are not yet ready or willing to use personal hearing aids.

Intensive high-quality audiologic counseling is needed for patients with MD in the adjustment to, and acceptance of, amplification.

Intratympanic therapy

In intratympanic therapy, medications are injected into the middle ear and are then absorbed through the round window into the fluid system of the inner ear. This allows targeting of the inner-ear system without exposing the body to the systemic adverse effects of the medication in use.

Two agents can be used intratympanically in patients with MD, depending on the presenting symptoms. Intratympanic corticosteroids are more commonly used in patients with MD presenting with sudden onset of hearing loss.​ Intratympanic gentamicin (an aminoglycoside antibiotic) injections are helpful in the treatment of intractable vertigo.

Intratympanic corticosteroid injections (e.g., dexamethasone or methylprednisolone) are used in patients with MD where systemic corticosteroids are contraindicated, or in patients who do not respond to oral corticosteroids. One double-blind, RCT found no significant difference between intratympanic gentamicin and intratympanic methylprednisolone at controlling attacks of vertigo in patients with refractory, unilateral MD.[71] The reported success rate of intratympanic corticosteroids in other studies has been variable.[60][72][73][74]

When injected into the middle ear, gentamicin preferentially destroys the vestibular labyrinth. This results in chemical labyrinthectomy and is an alternative to surgical labyrinthectomy in patients with intractable vertigo. Hearing loss can be minimized by meticulously titrating the dose of gentamicin to vertigo control, stopping therapy at the earliest signs of hearing loss, and following up closely with repeated hearing tests. This approach has been found to result in complete (81.7%) and effective (96.3%) vertigo control.[75] One meta-analysis on gentamicin injections found complete vertigo control in about 75% of the patients and complete or substantial control in about 93%.[76]​ Hearing level and word recognition did not deteriorate with treatment. None of the trials were double-blind or had a blinded, prospective control, and therefore the level of evidence was insufficient.[76] Overall hearing loss, as a complication of gentamicin injection, has been found in 25% of patients, with a range of 13.1% to 34.7%. In a prospective, double-blind, randomized, placebo-controlled clinical trial, intratympanic gentamicin treatment was found to reduce the score of vertigo severity and perceived aural fullness in the treatment group.[77] Evidence suggests that intratympanic gentamicin injections improve vertigo symptoms, are well tolerated, and have a low incidence of severe hearing loss.[21]​ However, two systematic reviews in 2023 found that evidence for the use of intratympanic gentamicin and corticosteroid injections in the treatment of patients with MD is uncertain.[78][79]​​

Meniett device

The Meniett device is a handheld device that delivers intermittent pressure pulses through the ear canal and is self-administered 3 times per day. A tympanostomy tube is placed in the tympanic membrane and should be kept patent throughout the treatment. It is thought that the pressure treatment induces longitudinal movement of endolymph and improves the hydropic condition. Evidence for the Meniett device for use in MD appears to be mixed. Initial RCTs have shown that the use of the Meniett device significantly reduced vertigo frequency in two-thirds of the patients and that the improvement was maintained long term.[80] Furthermore, no serious adverse effects have been reported in the trials.[81] In contrast, systematic reviews assessing the effectiveness of positive pressure therapy devices (including the Meniett device or similar) have failed to show any benefit of these devices in improving the symptoms of MD.[82][83]​ The American Academy of Otolaryngology 2020 guidelines recommend against the use of positive pressure therapy in patients with MD.[21]

Surgical therapy

The surgical management of patients with MD has changed as a result of the introduction of less invasive office procedures, including intratympanic therapy and the Meniett device.

Surgical approaches are used in patients with intractable vertigo who are refractory to medical therapy. The choice between these procedures depends on the severity of the vertigo spells, degree of serviceable hearing, age and physical condition of the patient, condition of the opposite ear, and the patient's choice.

Surgical procedures are divided into nondestructive procedures that reverse the pathophysiologic hydrops and preserve hearing, such as endolymphatic sac surgery (ELS), and destructive procedures that abolish the vestibular response either by destroying the inner ear (as in labyrinthectomy) or by cutting the vestibular nerve (as in vestibular neurectomy).

ELS is a procedure that consists of decompression of the endolymphatic sac from the overlying bone and drainage of its endolymph. Its role in MD is controversial, with studies that show 90% resolution of vertigo, and others that demonstrate it is no more effective than placebo, or that there is insufficient evidence of the beneficial effect of ELS in MD.[7][84][85][86]​​[87][88][89]​ A recent systematic literature search and meta-analysis revealed a paucity of studies on this surgical procedure, indicating ELS may be a beneficial treatment for patients with MD.[89]​ However, further studies are needed to attain a better understanding of the efficacy of ELS for treating MD.

Labyrinthectomy results in loss of residual hearing and therefore is indicated in patients who have no serviceable hearing. Vestibular nerve section is aimed at preserving residual hearing and is therefore a choice in patients with serviceable hearing.

Vestibular and balance rehabilitation therapy

Vestibular and balance rehabilitation therapy is recommended for patients who have problems with balance.[90][91] Originally, patients considered for vestibular therapy were the ones who had relief from vertigo by destructive surgery or intratympanic gentamicin injections but who complained of persistent disequilibrium. It has been reported that patients whose vertigo is controlled by medical therapy or intratympanic corticosteroid injections and who complain of disequilibrium may benefit from vestibular therapy.[92] Vestibular therapy should not be recommended to patients with MD experiencing acute vertigo attacks.[21]

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