What is Ménière's disease?

Ménière's disease is an abnormality of the inner ear causing a host of symptoms, including vertigo or severe dizziness, tinnitus or a roaring sound in the ears, fluctuating hearing loss, and the sensation of pressure or pain in the affected ear. The disorder usually affects only one ear and is a common cause of hearing loss. Named after French physician Prosper Ménière who first described the syndrome in 1861, Ménière's disease is now also referred to as endolymphatic hydrops.

What causes Ménière's disease?

The symptoms of Ménière's disease are associated with a change in fluid volume within a portion of the inner ear known as the labyrinth. The labyrinth has two parts: the bony labyrinth and the membranous labyrinth. The membranous labyrinth, which is encased by bone, is necessary for hearing and balance and is filled with a fluid called endolymph. When your head moves, endolymph moves, causing nerve receptors in the membranous labyrinth to send signals to the brain about the body's motion. An increase in endolymph, however, can cause the membranous labyrinth to balloon or dilate, a condition known as endolymphatic hydrops.

Many experts on Ménière's disease think that a rupture of the membranous labyrinth allows the endolymph to mix with perilymph, another inner ear fluid that occupies the space between the membranous labyrinth and the bony inner ear. This mixing, scientists believe, can cause the symptoms of Ménière's disease. Scientists are investigating several possible causes of the disease, including environmental factors, such as noise pollution and viral infections, as well as biological factors.

What are the symptoms of Ménière's disease?

The symptoms of Ménière's disease occur suddenly and can arise daily or as infrequently as once a year. Vertigo, often the most debilitating symptom of Ménière's disease, forces the sufferer to lie down. Vertigo attacks can lead to severe nausea, vomiting, and sweating and often come with little or no warning.

Some individuals with Ménière's disease have attacks that start with tinnitus, a loss of hearing, or a full feeling or pressure in the affected ear. It is important to remember that all of these symptoms are unpredictable. Typically, the attack is characterized by a combination of vertigo, tinnitus and hearing loss lasting several hours. But people experience these discomforts at varying frequencies, durations, and intensities. Some may feel slight vertigo a few times a year. Others may be occasionally disturbed by intense, uncontrollable tinnitus while sleeping. And other Ménière's disease sufferers may notice a hearing loss and feel unsteady all day long for prolonged periods. Other occasional symptoms of Ménière's disease include headaches, abdominal discomfort and diarrhea. A person's hearing tends to recover between attacks but over time becomes worse.

How is Ménière's disease treated?

There is no cure for Ménière's disease. Medical and behavioral therapy, however, are often helpful in managing its symptoms. Although many operations have been developed to reverse the disease process, their value has been difficult to establish. And, unfortunately, all operations on the ear carry a risk of hearing loss.

The most commonly performed surgical treatment for Ménière's disease is the insertion of a shunt, a tiny silicone tube that is positioned in the inner ear to drain off excess fluid.

In another more reliable operation, a vestibular neurectomy, the vestibular nerve which serves balance is severed so that it no longer sends distorted messages to the brain. But the balance nerve is very close to the hearing and facial nerves. Thus, the risk of affecting a patient's hearing or facial muscle control increases with this type of surgical treatment. Also, older patients often have difficulty recovering from this type of surgery.

A labyrinthectomy, the removal of the membranous labyrinth, is an irreversible procedure that is often successful in eliminating the dizziness associated with Ménière's disease. This procedure, however, results in a total loss of hearing in the operated ear—an important consideration since the second ear may one day be affected. Also, labyrinthectomies themselves may result in other balance problems.

Some physicians recommend a change of diet to help control Ménière's symptoms. Eliminating caffeine, alcohol and salt may relieve the frequency and intensity of attacks in some people. Eliminating tobacco use and reducing stress levels may lessen the severity of the symptoms. And medications that either control allergies, reduce fluid retention or improve blood circulation in the inner ear may also help.

How is Ménière's disease diagnosed?

Scientists estimate that there are 3 to 5 million people in the United States with Ménière's disease, with nearly 100,000 new cases diagnosed each year. Proper diagnosis of Ménière's disease entails several procedures, including a medical-history interview and a physical examination by a physician; hearing and balance tests; and medical imaging with magnetic resonance imaging (MRI). Accurate measurement and characterization of hearing loss are of critical importance in the diagnosis of Ménière's disease.

Through the use of several types of hearing tests, physicians can characterize hearing loss as being sensory, arising from the inner ear, or neural arising from the hearing nerve. An auditory brain stem response, which measures electrical activity in the hearing nerve and brain stem, is useful in differentiating between these two types of hearing loss. And under certain circumstances, electrocochleography, recording the electrical activity of the inner ear in response to sound, helps confirm the diagnosis.

To test the vestibular or balance system, physicians irrigate the ears with warm and cool water. This flooding of the ears, known as caloric testing, results in nystagmus, rapid eye movements that can help a physician analyze a balance disorder. And because tumor growth can produce symptoms similar to Ménière's disease, magnetic resonance imaging is a useful test to determine whether a tumor is causing the patients vertigo and hearing loss.

What research is being done?

Scientists are investigating environmental and biological factors that may cause Ménière's disease or induce an attack. They are also studying how fluid composition and movement in the labyrinth affect hearing and balance. And by studying hair cells in the inner ear, which are responsible for proper hearing and balance, scientists are learning how the ear converts the mechanical energy of sound waves and motion into nerve impulses. Insights into the mechanisms of Ménière's disease will enable scientists to develop preventive strategies and more effective treatment.
Where can I get additional information?
The NIDCD currently supports research on Ménière's disease in medical centers and universities throughout the nation. For more information about Ménière's disease, you can contact:

Effects of Meniere's Disease

Meniere's Disease is a very disturbing illness, presenting patients with hearing loss, pressure in the ear, tinnitus, severe imbalance and vertigo.

Vertigo is the most dramatic and distressing symptom of Meniere's; it is described as a sudden loss of normal balance or equilibrium. The room may suddenly begin to spin and rotate at high speed. Focusing is difficult, and if the vertigo continues, nausea and vomiting may occur. Vertigo is commonnly caused by acute labyrinthitis (a viral inflammation of the inner ear), benign positional vertigo (a condition due to abnormally floating crystals in the inner ear that stimulate the nerve endings of the inner ear), delayed symptom of head injury, or result of cervical spine problems.

Hearing loss typically fluctuates with hearing being worse some days than others. The hearing loss in Meniere's may lead to severe permanent hearing loss and deafness in the affected ear.

People with Meniere's Disease report that tinnitus may be variable and often worsen before an attack of vertigo. Tinnitus is often described as a motor-like whirring noise present only in the ear with the hearing loss.

Pressure or a sense of fullness in the affected ear are also common.

Meniere's Disease rarely occurs in children. In most cases, it begins in both men and women in the thirties or early middle age. Also, Meniere's is rarely noted for the first time in older people. Ear surgeons see many patients with dizziness. Very few of these patients actually have Meniere's Disease.

Symptoms

Symptoms of Meniere's Disease come in cycles. The patient suffers multiple episodes lasting several months at a time; then, it generally subsides. In some individuals, the symptoms seem to be more severe in spring, fall or when under extra emotional stress.

The most unpredictable and frightening symptom of Meniere's Disease is vertigo. The vertigo in Meniere's Disease is thought to result from an accumulation of excessive fluid in the inner ear. The fluid pressure stretches the membranes, that divide the compartments of the inner ear. As the membranes of the inner ear stretch, hearing diminishes and tinnitus worsens. When the membranes are severely stretched, the fluids of the inner ear may rupture them. This results in mixing of the fluids, one rich in sodium, the other rich in potassium. The mixture of these fluids is thought to bring on the vertigo.

After the membranes rupture, they eventually heal, but some hearing is usually lost. Surprisingly, with salt restriction, careful dietary planning and a mild diuretic, the symptoms of Meniere's Disease will often subside. In some cases, hearing can return to normal.

Classic symptoms of Meniere's aren't always present. Sometimes, hearing loss will precede episodes of vertigo by several years. Tinnitus alone, without associated hearing loss or vertigo, is rarely caused by Meniere's Disease. The only symptom in very early cases of Meniere's may be a sense of fullness or pressure in one ear.

Diagnosis

Other conditions can produce the same symptoms as Meniere's Disease and, thus, have to be ruled out or excluded in order to develop an accurate diagnosis.

For instance, infections of the inner ear, including syphilis and Lyme's Disease, may produce episodes of vertigo and hearing loss quite indistinguishable from Meniere's; these symptoms usually occur in both ears. Tumors of the inner ear nerve (the eighth nerve), especially acoustic neuromas, can also produce similar symptoms. These tumors grow slowly and compress the nerve. Thus, the hearing loss doesn't have periods of improvement. Also, the patient usually experiences imbalance rather than vertigo.

Ten to 15 percent of cases resembling Meniere's Disease may be the result of an immune disorder of the body, the system producing antibodies which attack the inner ear. Cholesteatomas (cystic growths) and other infections of the middle ear can also produce symptoms similar to Meniere's.

How We Diagnose

Initial evaluation is based on a very careful history given to the ear surgeon, as well as an examination of the ears under the operating microscope to rule out obvious infections or visible growths. Then, a comprehensive hearing test (audiogram) is taken. A low frequency upsloping hearing loss of the neural type noted on the hearing test is typical of Meniere's.

Additional testing is performed:

·         electronystagmography, or balance test (ENG),

·         electrocochleography (ECOG),

·         brainstem evoked response audiometry (BSER),

Magnetic Resonance Imaging (MRI) with a contrast dye called Gadolinium can rule out an acoustic neuroma or other brain tumor as a possible source of symptoms. Lab tests should include examination for inner ear immune related infections or conditions.

Once testing is completed, the ear surgeon can evaluate the results, rule out extraneous conditions and confirm the diagnosis of Meniere's Disease. Even after this extensive testing, the test results may not be conclusive.

What the Tests Reveal

ENG (electronystagmography) measures the nerve of balance. Over time, this nerve will lose function in Meniere's Disease. Most patients with Meniere's have a reduced response to stimulation with cold and warm water or air which is used in this test. Electrocholeography (ECOG) measures the excess fluid accumulation in the inner ear; in Meniere's, this test will also confirm increased pressure due to excess fluids in the inner ear. The Brain Stem auditory evoked responses (BSER) will usually be normal despite the hearing loss, unless a central disorder is present.

What the Other Tests Show

The MRI with Gadolinium specifically visualizes the eighth nerve (acoustic and balance nerve). Some older scanners can miss a small acoustic neuroma (tumor). Newer MRIs can actually visualize the structures of the inner ear including the cochlea and semicircular canals. This is most helpful. The eighth nerve can be clearly identified on MRI scan. A nerve that does not show enhancement (increase in brightness), when the dye is given, rules out an acoustic neuroma from the diagnosis.

Laboratory tests are geared to identify other conditions that may be responsible for Meniere's. Syphilis can involve the inner ear even twenty to thirty years after the original infection. Lyme Disease can also produce Meniere's-like symptoms, and symptoms can surface months after the original infection.
Individuals with certain auto immune disorders such as Lupus and severe rheumatoid arthritis, or who suffer from thyroid disorders such as Grave's Disease and Hashimoto's thyroiditis may be at higher risk for developing Meniere's Disease. This sub-group with their potential auto immune cause for the Meniere's can often be successfully treated with medications which slow the immune system's responses: cortisone-containing medications such as Decadron or Prednisone.

Treatment

When the diagnosis of Meniere's Disease is eventually confirmed, treatment is directed at ending or markedly reducing the frequency and severity of attacks. Treatment includes modification of personal habits, diet, stress reduction and regular exercise -- all extremely important in the overall treatment of Meniere's Disease. Medications will be recommended; evaluation of all treatments must be carefully annotated.

Dietary

Dietary restriction of salt intake is primary. Most Americans consume over 10 grams of salt daily. Under normal conditions, the body requires 2 grams or less. The taste for salting food is an acquired one. Most individuals who restrict their salt intake become keenly aware of excess salt added to their food. Over time, salt restriction results in decreased fluid accumulation in the inner ear, reducing excess pressure on the nerve endings of balance and hearing. A daily diuretic, typically Hydrochlorthiazide (combined with Triamterene to retain potassium) help the body to further reduce fluid retention.

Other Lifestyle Modifications

Smoking must stop immediately. Smoking constricts and reduces blood flow to the tiny blood vessels which nourish the inner ear nerve endings. Caffeine in coffee, tea and colas, as well as chocolate, must also be eliminated from the diet since caffeine excessively stimulates nerve endings. Reasonable exercise such as a daily brisk walk will stimulate circulation and help blood flow. A regular exercise program is also helpful.

Vertigo Medications

Use of medications such as Antivert (Meclizine) is usually of no benefit in true Meniere's Disease, even if it helps in other balance disorders. However, Valium (Diazepam) and other Benzodiazepines have a direct effect on the nerve controlling balance and its central connections to the brain. When Valium is given at the onset of a vertigo attack, it can prevent the attack from continuing. (N.B. Valium and similar medications should not be taken daily, because they may be habit forming.)

Dietary Pros and Cons

Diets can include fresh meats, poultry, vegetables and fruits. Processed meats, canned products, monosodium glutamate, table salt and "Lite salt" should be avoided totally. Olives, pickled foods, chips and some cheeses are also very high in sodium and should be avoided. Flavor can be added by using natural herbs and other spices NOT mixed with salt. Many individuals with Meniere's follow a typical low salt diet, similar to those diets used to control high blood pressure. Dieticians, pamphlets, and diet books are sources of further information.

Younger Patients

Young patients may have symptoms which are more severe and resistant to treatment. When recurring bouts of vertigo begin to interfere with daily activities, surgical options are often discussed. Generally, surgery is not to be considered unless attacks of vertigo are severe and do not respond to treatment. Often, patients with Meniere's have consulted a number of physicians who used the aforementioned treatments without success. Combining Cortisone-type medications with diuretics should be tried once again. Dyazide, combined with oral Decadron or Prednisone (cortisone) given over a period of 2 to 3 weeks will be helpful in gauging some form of medical response. If combined cortisone and diuretics plus diet are not effectice in improving clinical symptoms, then surgery is advised.