Meniere Disease (Idiopathic Endolymphatic Hydrops) Treatment & Management

Medical therapy can be directed toward treatment of the actual symptoms of the acute attack or directed toward prophylactic prevention of the attacks. Most care in the emergency department (ED) is based on symptomatic relief of the clinical findings.

Aside from acute control of vertigo and associated effects (eg, dehydration from protracted vomiting), inpatient care is generally unnecessary. Patients with Ménière disease require admission only if the symptoms are severe and refractory to medical management.

If endolymphatic hydrops is attributable to a given disease process—that is, if it is Ménière syndrome rather than Ménière disease—the first-line management is diagnosis and treatment of the primary disease (eg, syphilis, thyroid disease).

Surgical therapy for Ménière disease is reserved for medical treatment failures.

All patients should have rapid follow up with their primary care provider. Some patients may require referral to an otolaryngologist or neurologist. Salt-restricted diet, steroids, and the use of diuretics are often first-line therapies. ^[10]

Medical treatment of Ménière’s disease is aimed at symptomatic relief. In an acutely vertiginous patient, management is directed toward vertigo control. ^[29] Intravenous (IV) or intramuscular (IM) diazepam provides excellent vestibular suppression and antinausea effects. Steroids can be given for anti-inflammatory effects in the inner ear. IV fluid support can help prevent dehydration and replaces electrolytes.

Typically, vestibulosuppressants and antinausea medications (eg, meclizine, prochlorperazine) are prescribed for prn use. Note that frequent and long-term use of these medications is not recommended. Long-term use of vestibulosuppressants can lead to bad vestibular compensation skills and result in poor balance function. Sedative effects can affect patient productivity. Furthermore, long-term tachyphylaxis may result.

During the quiescent phase, medical treatment of Ménière disease is tailored to each patient. Lifestyle and dietary changes are usually the first step. Avoiding trigger substances (eg, salt, chocolate, caffeine) alone may be sufficient. Smoking cessation also is recommended. If medications are required, a 3-month trial of a diuretic (eg, hydrochlorothiazide/triamterene) and dietary management are prescribed.

Vestibulosuppressants

In general, medications that decrease symptoms (eg, meclizine [Antivert], droperidol [Inapsine], prochlorperazine [Compazine], diazepam [Valium], lorazepam [Ativan], alprazolam [Xanax]) only mask the vertigo. These masking agents are vestibulosuppressants and work by dulling the brain’s response to signals from the inner ear.

Diuretics and diureticlike medications

Some diuretics or medications with diuretic-like properties (eg, hydrochlorothiazide and triamterene [Dyazide], hydrochlorothiazide [Aquazide], acetazolamide [Diamox], methazolamide [Neptazane]) decrease fluid pressure in the inner ear. These medications help prevent attacks but do not help after the attack is triggered.

Although diuretics are often used, their efficacy has not been established with appropriate clinical trials. Loop diuretics should be used with caution due to the potential for ototoxicity.

Steroids

Steroids have also been helpful in treating endolymphatic hydrops because of their anti-inflammatory properties. Steroids can reverse vertigo, tinnitus, and hearing loss, probably by reducing endolymphatic pressure. Steroids can be given orally, intramuscularly, or even transtympanically. A trial of IM steroid injection followed by a tapering dose of oral steroids has been recommended. ^[23] No trials evaluating the efficacy of systemic steroids for the treatment of Ménière disease have been done. ^[30]

Although the transtympanic route is controversial, it is gaining wider acceptance throughout the otologic community. Transtympanic steroid injection has been shown to be beneficial in controlling loss of hearing and the number of vertigo attacks. ^[31]

Aminoglycosides

Aminoglycosides are reserved for end-stage intractable Ménière disease. They are a class of antibiotics that were serendipitously discovered to be preferentially toxic to the vestibular (balance) end organ. Destruction of the vestibular end organ renders the brain insensitive to fluctuations in inner ear pressure brought on by Ménière disease.

When given systemically, aminoglycosides affect both ears. Although aminoglycosides can be used to treat extremely severe bilateral Ménière disease, such treatment leaves the patient with little or no balance function. The resulting complete loss of inner ear function (ie, Dandy syndrome) can be debilitating.

Currently, the preferred method of giving aminoglycosides is through transtympanic injections. This concentrates the medication in the affected ear, with little systemic or contralateral adverse effects. It has been very effective, as has been shown in many studies.

Histamine agonists

Histamine agonists such as betahistine (Serc) are widely used in Europe and South America for the treatment of Ménière disease. Betahistine’s mechanism of action has not been established with certainty, but it is thought to act by increasing circulatory flow to the cochlear stria vascularis ^[32] or through inhibition of vestibular nuclei activity. ^[33]

Many have reported success with the use of betahistine to mitigate symptoms of Ménière disease. Unfortunately, because this agent has not been approved by the US Food and Drug Administration (FDA), it is not discussed much in the United States.

One innovation in the treatment of Ménière disease is the Meniett device. ^{[34, 35, 36, 37]} Its use is not precisely a medical treatment, and the device itself does not require surgical installation. It does, however, require insertion of a tympanostomy tube so that the device can work; therefore, its use may qualify as a surgical treatment.

The Meniett device delivers pulses of pressure to the inner ear via the tympanostomy tube. Although no one knows exactly why this approach works, some patients have symptomatic relief when the device is used on a daily basis. Because it is relatively new, long-term results have not been fully evaluated.

Surgical therapy for Ménière disease is reserved for medical treatment failures and is otherwise controversial. ^[38] Historically, several surgical procedures have been invented, tested, and discarded. Surgical procedures are divided into 2 major classifications: destructive and nondestructive surgical procedures.

Endolymphatic hydrops causes fluid pressure accumulation within the inner ear, which causes temporary malfunction and misfiring of the vestibular nerve. These abnormal signals cause vertigo. Destruction of the inner ear, the vestibular nerve, or both prevents these abnormal signals from reaching the brain. As long as the opposite inner ear and vestibular apparatus function normally, the brain eventually will compensate for the loss of 1 labyrinth over the following weeks to months.

Destruction of one inner ear depends on the adequate function of the opposite ear. Unfortunately, Ménière disease can be bilateral (7-50%), in which case this method is contraindicated. Since balance and hearing are closely intertwined within the labyrinth, destruction of the balance portion carries a high risk of hearing loss. Note that destructive procedures are irreversible and reserved for severe cases.

Nondestructive surgical procedures are directed toward improving the state of the inner ear. They are less invasive than destructive procedures and do not preclude the use of other treatment modalities. Discussion here is limited to the 4 most generally accepted management options, as follows:

• Endolymphatic sac decompression or shunt placement ^[39]

• Vestibular nerve section

• Labyrinthectomy

• Intratympanic injection of medications such as gentamicin ^{[40, 41, 42]} or steroids ^[43]

Go to Surgical Treatment of Meniere Disease for complete information on this topic.

In theory, the endolymphatic sac procedure decreases endolymph pressure accumulation by removing some of the mastoid bone, which encases the endolymph reservoir. This procedure allows the reservoir sac to expand more freely, thus dissipating pressure. A drain or valve from the endolymphatic space to either the mastoid or subarachnoid space can be inserted as another means of further reducing pressure.

Success rates (in terms of controlling vertigo and stabilizing hearing acuity) with this procedure are reported at 60-80%. These success rates do not seem to differ significantly from those of endolymphatic sac decompression alone versus those that are coupled with shunt procedures. Morbidity and mortality rates of endolymphatic sac decompression are relatively low. The risk of hearing loss and facial nerve damage is minimal in experienced hands. Severe postoperative pain is unusual; in most cases the recovery period is typically short and uneventful.

The endolymphatic sac procedure is one of the most controversial issues in neuro-otology. ^{[44, 45, 46]} Citing the famous Danish study, critics argue that endolymphatic surgery is no more effective than sham surgery and that any benefit is due to placebo effect. A Cochrane review article concluded that there is insufficient evidence to support endolymphatic sac surgery for the treatment of Ménière disease. ^[47]

Proponents argue that patients indeed improve and, compared to destructive procedures, the endolymphatic sac procedures have low risk and morbidity rate. These procedures may provide relief in patients who have not responded to medical therapy.

Exposing the endolymphatic sac is essentially an extended mastoidectomy. Special care is taken to skeletonize the sigmoid sinus, posterior fossa dura, and posterior semicircular canal. The thin, egg-shelled bone is removed from the posterior fossa dura and sigmoid sinus.

The endolymphatic sac is distinguished from dura by color and texture differences. The sac is thicker and whiter than the surrounding tissue. The location of the sac can vary, but it is generally immediately posterior or posterior inferior to the posterior semicircular canal.

In endolymphatic sac decompression, the procedure is terminated after all of the bone overlying the sac is removed. The shunt procedures involve incising the lateral leaf of the sac and inserting a drainage tube into the internal lumen of the endolymphatic duct. As already mentioned, the shunt can drain into either the subarachnoid space or the mastoid space.

According to a recent study, endolymphatic sac surgery (sac decompression or mastoid shunt) was found to be effective at controlling vertigo in the short term (>1 yr of follow-up) and long term (>24 mo) in at least 75% of patients with Ménière disease in whom medical therapy had failed. Sac decompression and mastoid shunting techniques provided similar vertigo control rates. In addition, mastoid shunting, with and without silastic, also provided similar vertigo control rates. Nonuse of silastic, however, seemed to maintain stable or improved hearing in more patients, as compared to silastic sheet placement. The data suggested that once the sac is opened, placing silastic does not add benefit and may be deleterious.

Go to Surgical Treatment of Meniere Disease for complete information on this topic.

For patients with useful hearing in the affected ear, sectioning the diseased vestibular nerve can be the ultimate solution. Although the hearing and balance functions are housed in a single common chamber within the inner ear, their neural connections to the brain separate into distinct nerve bundles as they course through the internal auditory canal. This anatomic separation allows balance function to be isolated and ablated without affecting hearing function.

Because this surgical procedure opens the internal auditory canal, it is similar to the approaches for an acoustic neuroma. Vestibular nerve sections typically are done through either a retrosigmoid or a middle fossa approach. The translabyrinthine approach does not spare hearing. It is used only for added insurance when labyrinthectomy is intended.

Most agree that the retrosigmoid approach (accomplished through a small craniotomy, posterior to the sigmoid sinus) is technically less difficult than the middle fossa approach. Exposure of the cerebellopontine angle allows visualization of the eighth cranial nerve.

Correct identification of the vestibular nerve is mandatory to avoid severing the facial nerve and cochlear nerve. Proper anatomic relationships must be confirmed. Because the nerve bundle rotates as it exits the internal auditory canal, the vestibular nerve moves from its lateral position to a more superior location. The vestibular nerves are closest to the tentorium. Facial nerve and cochlear nerve monitoring via intraoperative audiometric brainstem responses is essential.

The middle fossa approach for vestibular nerve section is more technically complex. The procedure creates a 5 X 5-cm middle fossa craniotomy just superior to the temporal line above the external ear canal. The middle fossa dura is retracted superiorly to expose the bone.

After proper anatomic landmarks are identified, the search for the internal auditory canal begins. Once the internal auditory canal is found and opened, the vestibular nerve is sectioned as laterally as possible. Fat is packed into the internal auditory canal and held in position by the temporal lobe dura. Finally, the craniotomy is closed.

The advantage of the middle fossa approach is that it achieves a slightly more complete ablation of vestibular function than the retrosigmoid approach. Because the vestibular fibers are cut immediately as they exit the vestibular end organ, the chance of stray vestibular fibers crossing over to travel along the facial and cochlear nerves is minimal. The retrosigmoid approach sections the vestibular nerve more medially, potentially after stray vestibular fibers have already crossed. Although unusual, failure of the retrosigmoid approach may be due to these crossed fibers.

The disadvantages of the middle fossa approach include the difficulty in finding the internal canal and the limited exposure within the canal because of the facial nerve’s position. Cutting the vestibular nerve without exerting pressure on the cochlear and facial nerves is difficult, in that the vestibular bundle lies deep within the canal. The risk of damaging the cochlear artery, with resulting hearing loss, is significant.

Vestibular nerve section has the advantage of a high rate of vertigo control (95-98%) with a high rate of hearing preservation in the operated ear (95%). The risks of this procedure, although rare, are similar to those of craniotomy and acoustic neuroma surgery and include the following: facial nerve damage, hearing loss, exacerbation of tinnitus and dizziness, cerebrospinal fluid leakage, headaches, hemorrhage, and infection (eg, meningitis).

Postoperatively, patients generally require 3-5 days of inpatient care. Accommodation to the surgical loss of 1 vestibular apparatus usually takes weeks to months. Vestibular rehabilitation during this period is often helpful.

Go to Surgical Treatment of Meniere Disease for complete information on this topic.

Labyrinthectomy for Ménière disease has the advantage of a high cure rate (>95%) and is useful in patients whose hearing on the diseased side has been destroyed already by Ménière disease. Labyrinthectomy involves ablation of the diseased inner ear organs. It is a less complex procedure than vestibular nerve section, in that does not require entry into the cranial cavity. In addition, it is less invasive than vestibular nerve section.

In one study, endolymphatic sac surgery (sac decompression or mastoid shunt) was shown to be effective at controlling vertigo in the short term (>1 yr of follow-up) and long term (>24 mo) in at least 75% of patients with Ménière disease in whom medical therapy had failed. Sac decompression and mastoid shunting techniques provided similar vertigo control rates. Mastoid shunting, with and without silastic, also provided similar vertigo control rates. Nonuse of silastic, however, seemed to maintain stable or improved hearing in more patients, as compared to silastic sheet placement. The data suggested that once the sac was opened, placing silastic did not add benefit and may have been deleterious. ^[48]

Labyrinthectomy carries less danger of cerebrospinal fluid (CSF) leak and meningitis because craniotomy is not required. Like those who undergo vestibular nerve section, labyrinthectomy patients require a few days of inpatient care. Accommodation to the surgical loss of 1 vestibular apparatus usually takes weeks or months. Vestibular rehabilitation during this period is also helpful.

The transcanal approach proceeds through the external ear canal. A tympanomeatal flap is first elevated. Next, a right-angle pick is inserted through the oval window and maneuvered to disrupt and scramble the nerve tissues of the labyrinth. A drill can be used to connect the round window and oval window to obtain better exposure to the neuroepithelium.

The basic mastoidectomy approach involves extending the mastoidectomy by drilling through the semicircular canals; this allows a more complete ablation of the labyrinthine neuroepithelium than can be achieved via the transcanal approach.

Intraoperative view of the left ear treated with labyrinthectomy; endolymphatic sac can be seen in this view.

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Ten patients with Ménière disease who underwent a cochlear implant achieved substantial receptive communication improvement, according to the authors of the study. In addition, they noted that implantation seems to neither adversely alter the natural history of vestibular function nor notably exacerbate auditory symptoms. ^[49]

In 10 patients with severe hearing loss (single-sided deafness) and recalcitrant vertigo attacks because of Ménière disease, simultaneous labyrinthectomy and cochlear implantation effectively relieved vertigo attacks and improved auditory function. According to the authors of this study, the cochlear implant restored auditory function to the deafened ear, and the binaural input appears to improve sound localization for most patients. ^[50]

Go to Surgical Treatment of Meniere Disease for complete information on this topic.

Transtympanic perfusion of medication was popularized by Dr. John Shea in 1995. ^[51] The procedure is still evolving and, although variations have been devised, the concept remains the same.

In transtympanic perfusion, medications for Ménière disease are applied through a myringotomy within the middle ear cavity, where they presumably are absorbed through the round window membrane into the inner ear (see the image below). This is a relatively low-risk, simple procedure that applies a high concentration of medicine with minimal systemic effects. It is similar to the placement of tympanostomy tubes, which can be done in the office or in an outpatient setting.

If steroids are administered with this transtympanic technique, the procedure is classified as nondestructive. Transtympanic steroid application is useful, particularly when patients have poor tolerance for the systemic adverse effects of steroids. A higher inner ear concentration can be obtained with transtympanic steroids over oral or intramuscular steroids. Success rates seem favorable, although long-term studies are being gathered.

If aminoglycosides are administered, the surgery is classified as destructive. When given transtympanically, aminoglycosides can concentrate their effects in the affected ear.

Because streptomycin is difficult to obtain in the United States, owing to US Food and Drug Administration (FDA) restrictions, gentamicin ^[52] is used more widely. Early studies show about 90% efficacy. Some authors report significant worsening of hearing in 5-15% of patients.

Vestibular therapy is a physical therapy and occupational therapy modality that helps habituate patients to their vestibular loss. It helps recalibrate a patient's balance by helping them compensate for the effects of the inner ear disorder. It is performed by repetitive balance exercises.

Because of the fluctuating nature of Ménière disease, vestibular therapy is not particularly useful as a primary treatment. However, it is useful in the rehabilitation of patients who have undergone vestibular ablation. In fact, vestibular rehabilitation is strongly recommended in those who have undergone aminoglycoside perfusion, labyrinthectomy, or vestibular nerve section. It can be helpful in teaching patients to cope with vertigo and imbalance.

Dietary measures

Dietary management is appropriate in patients not severely affected; patients avoid substances that may trigger or exacerbate fluid pressure buildup in the inner ear. For Ménière disease, much as for systemic hypertension, the goal is to reduce the total body fluid volume. This, in turn, may reduce the inner ear fluid volume.

Because sodium seems to play a major role in fluid retention within the inner ear, avoiding foods with high sodium content (eg, pizza, preserved foods, smoked fish) is paramount. Note that many preserved and smoked foods contain sodium nitrite, which can contribute to high sodium content. Consult with a nutritionist to establish a rigid salt-restricted diet (1.5 g sodium/d).

Activity restriction

Endolymphatic hydrops does not preclude regular activity. Exercise is recommended in moderation. However, because of the unpredictable nature of the disease, balance-intensive, dangerous tasks (eg, especially climbing ladders) should be avoided.

Quality evidence is lacking regarding deterrence and prevention of acute attacks of Ménière disease; however, a salt restricted diet, as described elsewhere (see Diet and Activity), is often suggested. In addition, avoidance of trigger substances may help prevent acute episodes. The following substances should be avoided:

• Caffeine

• Nicotine

• Chocolate, which has shown to be a potent trigger substance

• Tobacco

• Alcohol, particularly red wine and beer

• Foods with high cholesterol or triglyceride content

• Foods with high carbohydrate content

• Excessive sweets and candy

Finally, it is often suggested that patients try to avoid loud noises and to make use of stress-reduction techniques.

Because the differential diagnosis is vast, many specialists are often consulted, including the following:

• Otolaryngologist

• Neurologist

• Cardiologist

• Endocrinologist

• Internist

• Physical medicine/rehabilitation specialist (especially in the postoperative period)

This can be a drain on financial resources. “Shotgun” multispecialty consultations are not recommended.

Typically, the specialist most suited to care for Ménière disease patients is a neurotologist (otolaryngologist or neurologist who is interested in dizziness). Strategic consultation with these physicians first provides cost-effective triage.