Meniere’s disease is a common disorder in middle-aged adults that is characterized by the cluster of the following symptoms in recurrent episodes: spontaneous vertigo, sensorineural hearing loss, tinnitus and a feeling of fullness or pressure in the affected ear. Almost all cases are in one ear only; in about 30 percent of cases, the other ear may develop symptoms. Acute attacks with an onslaught of symptoms may vary in severity and frequency and are often interspersed with frequent remissions that may last several months or longer. Hearing becomes poor and distorted during attacks and may return to near-normal in between. Over time, however, hearing deteriorates in most cases. Eventually, vertigo spells become less frequent and less severe as the sensory cells in the inner ear lose their function.
Meniere’s disease is most common between people of 40 to 60 years of age. In Europe, the incidence is about 50 to 200 out of 100,000 persons a year. In the United States, about 45,000 new cases develop annually. An ear, nose and throat doctor (otolaryngologist) will make the diagnosis of Meniere’s based on patient history and the absence of other similar conditions. We don’t know what causes Meniere’s but the pattern of onset suggests that it is an acquired disorder, perhaps the result of viral infection of the inner ear. In any event, the volume regulation of the inner ear fluid (endolymph) is affected, which leads to increased volume (hydrops). How this change results in the symptoms of Meniere’s disease is also unknown. Specific disorders associated with hydrops (such as temporal bone fracture, syphilis, hypothyroidism, Cogan’s syndrome and Mondini dysplasia) can produce symptoms similar to those of Meniere’s disease. However, some people with hydrops have no symptoms and others with symptoms have no hydrops. The absence of a precise marker for the diagnosis of Meniere’s disease has made researching this disorder very difficult.
Another complication for research is that Meniere’s disease often progresses but fluctuates unpredictably. It is difficult to distinguish natural resolution from the effects of treatment. In clinical trials, researchers see significant improvement in vertigo in both the placebo and treatment groups.
To prevent attacks of Meniere’s disease, medical treatment is used routinely. This includes reducing dietary salt intake, a trial of diuretic therapy, education about the natural history of the disorder and measures to reduce stress. For comfort during acute attacks the patient lies down with eyes closed and often wakes up free of vertigo but feeling wrung out. Drugs to reduce the vertigo, such as valium, are problematic because they can become habit-forming and may interfere with brain remapping, which is an important method of compensating for the disorder.
Changes to Diet
It has been suggested that a low-salt diet reduces endolymphatic volume and would thus be of value in endolymphatic hydrops. Indeed, many patients note a worsening of symptoms with excess sodium in the diet. Most physicians recommend lowering sodium intake to under 2,000 mg daily. Levels below this do not appear to be useful in mitigating Meniere’s.
For people unable to adhere to this program, a diuretic agent may be of value. Several studies from Europe have demonstrated some improvement with this treatment. Not all people benefit, however, and many cannot use diuretics because of sulfa allergy.
Psychological Support
Understanding the natural history of Meniere’s disease provides important information for decision-making. People with Meniere’s need to learn how to gain control over the effects of their symptoms and develop a positive attitude about the disorder. Many physicians have noted that people with Meniere’s are take-charge types whose abilities to organize and manage are potentially threatened by the disruptions imposed by the episodic vertigo. Nonetheless, most patients display great coping skills and use the experiences of other sufferers to good advantage. Many Meniere’s support groups have been formed and provide valuable information sharing.
Meniett Device
For people with vertigo levels of two or more severe attacks a month despite adequate medical management, a trial of Meniett therapy (www.meniett.com) may be warranted. The device provides micropressure pulses to the inner ear through a tube placed in the eardrum. Used three times a day, about 80 percent of patients note relief, whereas those using a placebo device noted significantly less relief over a four-month period. How exactly the Meniett works is not known but may well involve shifting of endolymph fluid through triggering of a natural valve in the inner ear. Use of the Meniett does not preclude surgical therapy later.
Surgery
For people with severe and intractable vertigo, surgical removal (ablation) of the abnormal balance function in the diseased ear provides immediate and usually permanent relief of the vertigo attacks. There are four choices in this regard, each varying in technical complexity and the risk of permanent deafness in the operated ear. However, given that the majority of people have already lost a good deal of their hearing, this risk is not as catastrophic as in people with normal hearing.
Chemical ablation with gentamicin injections in the doctor’s office is the simplest method. Usually more than one injection is necessary and 30 percent of people lose hearing. Surgical decompression of the endolymphatic sac has a very low risk of hearing loss but is only effective long-term in 60 percent of cases. This method is controversial but is useful in people with normal hearing and severe vertigo.
When these simpler methods fail, either labyrinthectomy (removal of the inner ear structures) or vestibular nerve section is an option. Labyrinthectomy always results in deafness and is thus reserved for older people and those with existing loss. The procedure has been used for decades and its effects are reasonably well tolerated.
Surgical cutting of the vestibular nerve section has a very low incidence of hearing loss but it is a technically more demanding and risky procedure. It is generally reserved for younger people in good health with good hearing.
Except in the case of the sac surgery, the goal of surgical treatment is loss of balance function in the operated ear. This produces a mild imbalance that usually resolves in a few weeks as the good ear “takes over.”
If you have suffered vertigo with hearing loss, tinnitus or a feeling of pressure in the ear, consult your physician, and where possible, an otolaryngologist, to learn if your symptoms are associated with Meniere’s disease, and if so, which of these treatments would be best in your case. n
George A. Gates, M.D., is the scientific/medical director of the Deafness Research Foundation and former director/emeritus professor, Virginia Merrill Bloedel Hearing Research Center, Department of Otolaryngology-Head and Neck Surgery, University of Washington School of Medicine.
