MÉNIÈRE’S DISEASE

MÉNIÈRE’S DISEASE

Current Diagnosis

• Ménière’s disease is characterized by recurrent attacks of severe vertigo, nausea, and vomiting typically lasting 1 to 6 hours.

• Ménière’s disease causes tinnitus on the side of the affected ear; the tinnitus can change in pitch and loudness during attacks of vertigo.

• Attacks of vertigo are associated with reduced or muffled hearing on the side of the affected ear.

• Fluctuating unilateral hearing loss and low-frequency hearing loss on the side of the affected ear is characteristic.

Current Therapy

•   Initial therapy includes a sodium-restricted diet (preferably <1500 mg of sodium daily) and a diuretic agent (e.g., thiazide diuretics).

• Betahistine,1 a medication widely used in Europe that may be obtained at compounding pharmacies in the United States, may be helpful in selected patients at a dosage of 8 to 24 mg three times daily.

• Transtympanic infusion of gentamicin1 or corticosteroids and endolymphatic sac surgery may be considered as minimally invasive options in patients with serviceable hearing. Gentamicin poses a moderate risk of inducing hearing loss.

• Vestibular nerve sectioning is highly effective in stopping vertigo attacks with a reasonably low risk of causing further hearing loss, but it is a more invasive procedure.

• Labyrinthectomy is highly effective in stopping vertigo attacks but causes complete hearing loss, so it is only an option for patients who have already lost all useful hearing. Patients who have undergone procedures that cause acute loss of vestibular function often have vertigo for a while after the surgery; the vertigo improves more quickly with vestibular rehabilitative therapy.1

1 Not FDA approved for this  indication.

Ménière’s disease is a disorder of the inner ear that results in recurrent, spontaneous episodes of vertigo, hearing loss, ear fullness, and tinnitus affecting the same ear. The hearing loss often fluctuates early in Ménière’s disease, but fluctuating hearing is not always present. Eventually permanent hearing loss occurs, affecting the low frequencies initially but ultimately affecting all frequencies.

Epidemiology

The reported prevalence of Ménière’s disease varies from about 50 to 200 per 100,000. Females are somewhat more prone to Meniere’s than males with a female:male ratio of about 1.9:1.

Risk Factors

Possible risk factors for later development of Ménière’s syndrome include autoimmune disorders, migraine, syphilitic otitis and viral infection of the inner ear, head trauma, and a family history of Ménière’s disease.

Pathophysiology

The underlying mechanism is generally thought to be due to endolymphatic hydrops, a type of swelling of the endolymphatic compartment that ultimately leads to permanent damage of the inner ear structures. Possible reasons for this periodic swelling of the endolymph compartment include mechanical obstruction of endolymph flow or at least dysregulation of the electrochemical membrane potential between endolymph and perilymph compartments.

Endolymphatic hydrops appears to be the mechanism for a number of conditions that can lead to Ménière’s syndrome. When no primary cause is identified, the idiopathic form of the syndrome is called Ménière’s disease. When an underlying cause is found, it is referred to as Ménière’s syndrome or secondary Ménière’s or secondary endolymphatic hydrops. Secondary Ménière’s syndrome can result from delayed endolymphatic hydrops in which symptoms come on years after a prior disorder or viral injury of the labyrinth, syphilitic otitis, autoimmune inner ear disease, and trauma.

Prevention

There is no known way of preventing the development of Ménière’s disease because it occurs in many people with no risk factors.

Clinical Manifestations

Ménière’s can manifest with periodic unilateral (or, less commonly, bilateral) hearing loss or isolated attacks of vertigo. Not uncommonly, Ménière’s has both elements present at its onset, but the vertigo is usually the symptom that gets the most attention. Vertigo usually lasts 1 to 6 hours but can last as little at 30 minutes or as long as all day.

Patients prone to motion sickness often report that their dizziness lasts longer because the aftereffect of vertigo lingers longer in those who are motion sensitive. Vertigo attacks in Ménière’s disease are often quite severe and generally render patients unable to move around owing to the vertigo, nausea, and recurrent vomiting.

The hearing loss is usually unilateral, and patients might notice fluctuation in hearing accompanied by ear fullness and a low-pitched roaring tinnitus either before or coincident with the vertigo. If audiometry can be performed during this time, low-frequency (250– 1000 Hz) hearing loss may be documented and might improve after the attack ceases. This signature fluctuating low-frequency sensorineural hearing loss is very helpful (Figure 1) when found, but getting patients with vertigo attacks in for testing while they are in the acute stage of vertigo is usually not feasible.

FIGURE 1    Audiogram showing typical low-frequency hearing  trough of Ménière’s disease affecting the right ear.

Occasionally, patients with Ménière’s have sudden random drop attacks in which they abruptly fall without loss of consciousness.

These spells, referred to as otolithic crises of Tumarkin, can lead to serious injury and should prompt aggressive treatment. The mechanism is presumed to be related to sudden mechanical deformation or sudden neural discharges related to the otolith structures of the inner ear. Because there is no specific treatment for Tumarkin crises, treatment entails the standard treatments for Ménière’s disease in general.

As Ménière’s disease progresses, usually over months to years, permanent low-frequency hearing loss develops that gives way to hearing loss at all frequencies. Eventually, the patient can lose all or most hearing on the affected side. Meanwhile, with each vertigo attack, some vestibular function is lost, and so as vertigo attacks continue, vestibular loss ensues, often paralleling the hearing loss.

Ménière’s occasionally burns out, meaning that enough vestibular function has been lost that acute hydrops no longer produces vertigo. Patients might report less severe dizziness or just a vague feeling of unsteadiness. This usually indicates advanced Ménière’s, and unilateral hearing and vestibular loss should be expected.

Bilateral Ménière’s is present at the time of initial diagnosis in about 10% of cases and by 10 years of symptoms, about 35% have bilateral involvement. Bilateral Ménière’s has treatment implications because treating one side alone will be unlikely to stop the vertigo attacks, which could be emanating from the untreated side. Surgical treatment of both sides is also problematic because it could leave the patient with bilateral hearing loss, vestibular loss, or both.

Diagnosis

The diagnosis of Ménière’s disease is made clinically based on unilateral hearing loss, tinnitus, ear fullness, and episodic vertigo typically lasting 1 to 6 hours. The criteria for definite, probable, and possible Ménière’s are listed in Box 1. Several recent studies using three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) 24 hours following administration of bilateral intratympanic gadolinium (Gd) injection and similar techniques have demonstrated abnormalities consistent with endolymphatic hydrops. MRI at this point, however, is not yet established as useful in affirming the diagnosis of Ménière’s.

Box 1
Diagnostic Criteria for Ménière’s  Disease
Definite Ménière’s Disease

• Two or more spontaneous (non-positional) episodes of vertigo, each lasting 20 minutes to 12 hours

• Audiometrically documented low- to medium-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during or after one of the episodes of vertigo.

•   Fluctuating hearing, tinnitus or ear fullness in the affected ear.

•   Not better accounted for by another vestibular diagnosis.

Probable Ménière’s Disease

• Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours

•   Fluctuating hearing, tinnitus or ear fullness in the affected ear.

•   Not better accounted for by another vestibular diagnosis.

From Lopez-Escamez JA, et al. Diagnostic criteria for Menière’s disease. J Vestib   Res.  2015;25(1):1–7.

Differential Diagnosis

Vestibular neuritis causes a single attack of vertigo, sometimes with acute hearing loss (labyrinthitis), but it can usually be distinguished from Ménière’s because vestibular neuritis has a lifetime recurrence rate of only 2%, whereas Ménière’s vertigo attacks recur multiple times and cause fluctuating and gradual decline in hearing.

When a patient reports episodic vertigo attacks in the absence of unilateral tinnitus, ear fullness, and hearing loss, one must be cautious in diagnosing Ménière’s disease because vestibular migraine (basilar- type migraine) can produce a similar pattern. Diagnostic criteria have been published for vestibular migraine (Lempert, 2013).

Benign paroxysmal positional vertigo causes brief recurrent spells of spinning without hearing loss, so it is rarely confused with Ménière’s disease.

Vertebrobasilar insufficiency can cause isolated vertigo, but the duration is usually only several minutes and without hearing loss, whereas Ménière’s attacks last hours and are associated with unilateral auditory symptoms.

Acoustic neuroma (vestibular schwannoma) typically leads to slowly progressive unilateral sensorineural hearing loss, but vertigo is infrequently a prominent feature because patients compensate gradually as their vestibular function wanes due to compressive effects of the tumor.

Fluctuating unilateral hearing loss, ear fullness, and tinnitus can occur without vertigo and is referred to as cochlear hydrops. Such symptoms can precede the development of vertigo but may be treated as Ménière’s nonetheless. Lermoyez’s syndrome refers to transiently improved hearing and tinnitus during attacks of vertigo. More commonly, however, hearing declines around and during vertigo spells.

Treatment

Medical Management

Treatment of Ménière’s disease is mainly aimed at preventing the vertigo attacks, but no treatments are known that reliably restore or arrest hearing loss or tinnitus. It is presumed that if attacks of vertigo, ear fullness, fluctuating hearing, and tinnitus are all stopped, hearing should stabilize, but this is still a supposition. The management of Ménière’s is hierarchical, starting with a low-sodium diet and a diuretic and proceeding to more-invasive or destructive surgical procedures only when initial medical treatment fails. When a patient has very frequent vertigo attacks and is disabled, more-aggressive treatment may be considered sooner.

Dietary sodium should be restricted to 1000 to 1500 mg daily. The average person consumes about 4000 mg daily, and the maximum recommended daily intake of sodium is 2300 mg. Because more than 70% of the daily sodium comes from processed foods, this dietary restriction generally requires more than simply stopping the use of table salt, which generally accounts for only about 10% of daily sodium intake for most people.

A diuretic is usually added to the sodium restriction. The most commonly prescribed diuretic is hydrochlorothiazide 25 mg combined with triamterene 37.5 mg (Dyazide)1 because this combination usually obviates the need for potassium supplementation. Acetazolamide (Diamox)1 125 to 250 mg bid or furosemide (Lasix)1 10 to 20 mg daily are also options. Patients with severe sulfa allergy can use low-dose ethacrynic acid (Edecrin)1  12.5 to 25 mg daily.

An acute attack of vertigo from Ménière’s disease can be managed with vestibular suppressants such as dimenhydrinate (Dramamine)1 50 mg, meclizine (Antivert) 25 to 50 mg, diazepam (Valium)1  2 to 5 mg, or promethazine (Phenergen)1 12.5 to 50 mg. Scopolamine in patch form (Transderm-Scōp)1 is too slow to be useful because it takes hours to be absorbed transdermally. Scopace has been discontinued and is no longer being manufactured. Scopolamine tablets may be available in certain compounding pharmacies.2,6 Nausea can additionally be managed with prochlorperazine (Compazine) 10 mg orally or 25 mg by suppository, with oral or sublingual ondansetron (Zofran)1 4 to 8 mg, or with other antiemetics. Generally the vertigo attacks subside in several hours even without treatment.

Betahistine (Serc)1 may also be helpful in Ménière’s, though it is not commonly used in the United States. The role of betahistine is not firmly established, although it is widely prescribed for vertigo throughout the world. Betahistine may be helpful when successful medical management with diet and diuretics has helped but is inadequate to stop attacks. Betahistine is a vasodilator, a modest H1 histamine agonist, and a powerful H3  histamine receptor antagonist. Its method of action in Ménière’s is unknown, though it increases blood flow and might also influence endolymphatic fluid regulation and receptors in the endolymphatic sac. Doses of betahistine may start at 8 mg PO three times daily and may be increased to 16 mg three times daily. It is available outside the United States in the form of Serc (Solvay Pharmaceuticals, Belgium). Betahistine may be made within the United States at compounding pharmacies with a physician prescription and may also be imported from outside the United States for individual use. Side effects are few, though occasionally patients report some headache. Betahistine is not histamine, and we have not found it to cause or aggravate urticaria.

Oral corticosteroids are often used but rarely seem effective except in cases of bilateral Ménière’s resulting from autoimmune inner ear disease (AIED). Oral corticosteroids rarely seem to be effective in typical Ménière’s disease.

Ménière’s attacks have been said to be triggered by caffeine, chocolate, stress, visual stimuli, and dropping barometric pressure. Such triggers should be avoided when possible, but strong associations with these triggers can also occur in migrainous vertigo. Vestibular rehabilitative therapy is not generally helpful in Ménière’s because most vertigo attacks remit and the patients have few vestibular symptoms between attacks. However, when vestibular loss has occurred and the patient appears to have not compensated well for it, vestibular rehabilitation can be helpful.

Bilateral Ménière’s Disease

The estimated incidence of bilateral Ménière’s varies in the literature but is estimated to occur in about 15% of cases and bilateral involvement increases over time. The possibility of bilateral involvement weighs on the decision making for any procedures that sacrifice hearing or vestibular function because it leaves the patient with only one functioning labyrinth.

Serviceability of Hearing

Serviceable hearing is residual hearing that can be useful to the patient by wearing hearing aids. In general, because hearing loss is permanent, one should avoid sacrificing any serviceable hearing.

Injections and Surgical Treatments

Patients who continue to have disabling vertigo despite a low-salt diet, diuretics, and possibly betahistine are considered to have failed conservative medical therapy. The next step in treatment depends on the age, health status, and residual hearing and vestibular function of the patient and also on the surgeon’s opinion and experience with the various options. If spells are infrequent, symptomatic management of vertigo attacks may be the best option. Table 1 outlines some of the additional interventional options.

Table 1

Procedures Used in the Management of Ménière’s Disease

 

1  Not FDA approved for this indication.

Intratympanic Gentamicin

Transtympanic gentamicin (Garamycin)1 administration has become increasingly used as an effective treatment of Ménière’s. This is partly due to the ease of administration of gentamicin and its relative safety. Gentamicin is a commonly used aminoglycoside antimicrobial agent with activity against gram-negative bacteria that also happens to be ototoxic. It damages hair cells of the labyrinth and preferentially affects hair cells of the vestibular neuroepithelium over that of the cochlea. Even so, transtympanic administration still has some risk of causing hearing loss. Gentamicin is administered by injecting a small amount (usually about 0.5 mL) of gentamicin sulfate (80 mg/2 mL) solution through the tympanic membrane into the middle ear space. To avoid rapid drainage through the eustachian tube, the injection is done with the patient supine and kept in that position for an hour or so to allow the gentamicin to absorb through the round window into the inner ear. There are many protocols, but commonly an injection is given once, and additional injections can be considered every 3 to 4 weeks until improvement in vertigo attacks is realized. Studies suggest that intratympanic gentamicin is an effective treatment for vertigo complaints in Ménière’s disease but there is some risk of hearing loss.

Endolymphatic Sac Surgery

Another option for patients with residual functional hearing is endolymphatic sac shunt or decompression. This procedure eliminates vertigo in 50% to 75% of those treated. Nevertheless, its effectiveness compared to placebo has remained a point of contention based on a number of studies. It has been suggested that the procedure’s effectiveness depends on the operative technique and experience of the surgeon.

Intratympanic Steroids

Sometimes, a trial of intratympanic corticosteroids may be tried, though its effectiveness in controlled trials is still not compelling. Even so, a trial of corticosteroids administered in this manner poses little risk.

Vestibular Neurectomy

The most definitive procedure for stopping vertigo attacks in Ménière’s when the goal is to preserve hearing is vestibular neurectomy. This procedure involves craniotomy and severing the vestibular nerve while preserving the cochlear nerve. This procedure requires overnight hospitalization and general anesthesia, and it does pose some risk to facial nerve function and hearing on the affected side.

Labyrinthectomy

For patients who have unilateral Ménière’s and no serviceable hearing, labyrinthectomy is a commonly used procedure. This procedure entails the removal of the membranous labyrinth and is highly effective in stopping recurrent vertigo attacks from that ear. In elderly patients or those who are poor surgical candidates, a more limited transtympanic cochleosacculotomy may be performed.

Meniett Treatment

The Meniett device is a portable low-frequency pressure-wave delivery system that administers a wave of pressure of about 12 cm H2O to the middle ear via a tympanostomy tube for about 0.6 seconds pulsed at 6 Hz for 5 minutes about three to five times daily. Quality randomized trials demonstrating the effectiveness of this treatment are lacking, however. This method has few risks and only requires myringotomy and a pressure equalization tube. A practical limitation is the cost of the device, which is not often paid for by many health insurance companies.

Monitoring

Following patients with Ménière’s disease should include monitoring of the frequency and severity of vertigo spells and the fluctuation in hearing, ear fullness, and tinnitus. Periodic audiometry is probably the most sensitive measure of stabilization of the condition. Vestibular testing may be considered when changes might alter the treatment strategy. Brain imaging is only helpful in excluding other disorders, but does not yet have a role in the management of Ménière’s disease.

Complications

Known complications include the progression of unilateral and occasionally bilateral hearing and vestibular function. There may also be complications associated with some of the treatments as described earlier. Owing to the unpredictability of the vertigo attacks, many patients avoid certain activities and might even develop agoraphobic features because the attacks are so severe and disruptive. In most cases, these avoidance behaviors improve if Ménière’s can be controlled.

Conclusion

The management of Ménière’s disease is still part art and part science. Treatment options are many, but patients should be reassured that, in most cases, something can be offered to help improve their vertigo and quality of life.

References

1.     Ahsan S.F., Standring R., Wang Y. Systematic review and meta- analysis of Meniett therapy for Meniere’s disease. Laryngoscope. 2015;125(1):203–208.

2.    Lempert T. Vestibular migraine. Semin Neurol. 2013;33:212–218.

3.     Lopez-Escamez J.A., et al. Diagnostic criteria for Ménière’s disease. J Vestib Res. 2015;25(1):1–7.

4.    Nevoux J., Franco-Vidal V., Bouccara D., et al. Diagnostic and therapeutic strategy in Ménière’s disease. Guidelines of the French Otorhinolaryngology-Head and Neck Surgery Society (SFORL). Eur Ann Otorhinolaryngol Head Neck Dis. 2017 Jan 3 (Epub ahead of print).

5.     Phillips J.S., Westerberg B. Intratympanic steroids for Ménière’s disease or syndrome. Cochrane Database Syst Rev. (7):2011;doi:10.1002/14651858.CD008514.pub2 CD008514.

6.      Pullens B., van Benthem P.P. Intratympanic gentamicin for Ménière’s disease or syndrome. Cochrane Database Syst Rev. (3):2011;doi:10.1002/14651858.CD008234.pub2 CD008234.

7.    Pullens B., Verschuur H.P., van Benthem P.P. Surgery for Ménière’s disease. Cochrane Database Syst Rev. 2013;28(2) CD005395.

1  Not FDA approved for this  indication.

2  Not available in the United  States.

6  May be compounded by  pharmacists.

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