Ménière's disease

The underlying cause of Ménière's disease is unknown, although it probably results from a combination of environmental and genetic factors. Possible causes that have been studied include viral infections; trauma to the middle ear; middle ear infection (otitis media); head injury; a genetic predisposition; syphilis; allergies; abnormal immune system response; migraines; and noise pollution.

The symptoms of Ménière's disease are thought to relate to changes in fluid volume in the inner ear, which contains structures necessary for normal hearing and balance. Changes in fluid volume may disrupt signals sent from the inner ear to the brain, or may lead to tears or ruptures of the structures that affect hearing and balance.

More detailed information about the causes of symptoms associated with Ménière's disease is available on NIDCD's Web site.

A diagnosis of Ménière's disease is often suspected based on the presence of characteristic signs and symptoms. These may include:

• Two or more episodes of vertigo lasting at least 20 min each
• Tinnitus
• Temporary hearing loss
• A feeling of fullness in the ear

Proper diagnosis of Ménière's disease entails several procedures, including a medical history interview; a physical examination; 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). Recording the 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. 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 or air. This procedure, known as caloric testing, results in nystagmus, rapid eye movements that can help a physician analyze a balance disorder.

Since tumor growth can produce symptoms similar to Ménière's disease, an MRI is a useful test to determine whether a tumor is causing the patient's vertigo and hearing loss.

While some people with Ménière's disease have identified factors that may trigger an attack, triggers often differ among affected people. Additionally, some people have triggers that have not been reported in others. While exercise and physical activity are not typically listed as a common triggers in affected people, the authors of a 2012 article stated that some of their affected participants reported physiological factors as triggers of attacks. While exercise was not specifically referred to, factors that were mentioned included physical factors such as tiredness and physical exertion or tension; as well as postural factors such as head position (e.g. bending), getting up too quickly, and quick head movements.

In an article from 2010, the author wrote that perspiring and subsequent fluid replacement is a potential source of inner ear stress that could trigger Ménière's attacks. It was noted that in their clinic, they stress that when affected people perspire, it is important that they actively replace their fluid loss with a sports drink or other electrolyte solution. They are also advised to sip the replacement solution frequently as they perspire, rather than dehydrating for an hour and then replacing with a single large amount of fluid.

People with Ménière’s disease can be more vulnerable to dietary and environmental factors that can impact hearing and balance. Some affected people find that certain events and situations (triggers) can set off attacks. While triggers differ among affected people, possible triggers often mentioned may include high salt intake, caffeine, alcohol, nicotine, stress, allergies, overwork, tiredness (fatigue), emotional distress, additional illnesses, pressure changes, and/or certain foods. People with identified triggers may be able to avoid or alleviate some attacks or symptoms by avoiding their trigger(s).

Ménière's disease is an abnormality of the inner ear. Signs and symptoms may include disabling vertigo or severe dizziness lasting from minutes to hours; tinnitus or a roaring sound in the ears; fluctuating hearing loss; and the sensation of pressure or pain in the affected ear. A small percentage of people have drop attacks. The disorder usually affects only one ear, but some people develop symptoms in both ears many years after their initial diagnosis. Although the exact cause of Ménière's disease is unknown, the symptoms are thought to be associated with a change in fluid volume within a portion of the inner ear known as the labyrinth. Treatment may include medications or surgery depending on the severity of the condition.

Family studies have shown that genetic predisposition plays a notable role in Meniere’s disease in 8% to 15% of people with this condition. When the disorder is familial, it most often has an autosomal dominant pattern of inheritance. This means that a parent with Meniere’s disease has a 50% chance with each pregnancy to pass their genetic susceptibility for Meniere’s disease to their child. However not everyone who inherits the genetic susceptibility goes on to develop symptoms. A single study found that 40% of people who inherit genetic susceptibility for Meniere's disease never develop symptoms of this condition. To date, no specific genetic risk factors (e.g., gene mutations) have been found in association with Meniere's disease.

Other factors that have been investigated as risk factors for Menieres disease include viral infections, trauma to the middle ear, middle ear infection (otitis media), head injury, syphilis, allergies, abnormal immune system responses, migraines and noise pollution.

If you are concerned about your family history of Meniere's disease and what it may mean for you, you may find it helpful to speak with a genetics professional. Genetics professionals are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about genetic consultations is available from Genetics Home Reference at http://ghr.nlm.nih.gov/handbook/consult. To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral.

The following online resources can help you find a genetics professional in your community:

• The National Society of Genetic Counselors provides a searchable directory of US and international genetic counseling services.
• The American College of Medical Genetics has a searchable database of US genetics clinics.
• The University of Kansas Medical Center provides a list of US and international genetic centers, clinics, and departments.
• The American Society of Human Genetics maintains a database of its members, which includes individuals who live outside of the United States. Visit the link to obtain a list of the geneticists in your country, some of whom may be researchers that do not provide medical care.

In addition, our search identified the following researcher at Oregon Health & Sciences University whose research interests including finding genetic risk factors for Meniere's disease.

Anh Nguyen-Huynh, M.D., Ph.D.
Oregon Health & Sciences University
Department of Otolaryngology/Head and Neck Surgery
Research: http://www.ohsu.edu/xd/education/schools/school-of- medicine/about/spotlight/nguyen-huynh.cfm
Bio: http://www.ohsu.edu/xd/health/services/providers/index.cfm?personid=56

The diagnostic criteria for Ménière's disease proposed by the American Academy of Otolaryngology and Head and Neck Surgery stipulate that a "definite" diagnosis of Ménière's disease requires the following:

• Two spontaneous episodes of rotational vertigo lasting at least 20 minutes
• Audiometric confirmation of sensorineural hearing loss
• Tinnitus and/or a perception of aural fullness

Based on these widely-used diagnostic criteria, the condition is only diagnosed if vertigo is present; therefore, it likely follows that all affected individuals experience this symptom.

The course of Ménière's disease can vary widely among affected people. The condition is often most bothersome during the early stages. During progression to later stages, vertigo spells often disappear. Acute attacks are typically replaced by constant imbalance and progressive hearing loss.

The long-term outlook (prognosis) for people with Ménière's disease also varies. Periods of remission punctuated by exacerbations of symptoms are typical. Some people have minimal symptoms, whereas others have severe attacks. Episodes may occur as infrequently as once or twice a year or they may occur on a regular basis. The pattern of exacerbation and remission makes evaluation of prognosis difficult. In general, the condition tends to spontaneously stabilize over time and it is said to "burn out.” The spontaneous remission rate is high with over 50% experiencing this within 2 years, and over 70% after 8 years. However, most people are left with poor balance and poor hearing. Those whose condition does not stabilize are generally well-managed with medications but surgical treatment is necessary in about 5-10% of cases.

At the present time there is no cure for Ménière's disease, but lifestyle modifications and some treatments can help affected people cope with the symptoms. The symptoms of the disease are often controlled successfully by reducing the body’s retention of fluids through dietary changes (such as a low-salt or salt-free diet and no caffeine or alcohol). Medications such as antihistamines, anticholinergics, and diuretics may lower endolymphatic pressure by reducing the amount of endolymphatic fluid. Eliminating tobacco use and reducing stress levels may also help lessen the severity of symptoms.

Symptoms such as dizziness, vertigo, and associated nausea and vomiting may respond to certain medications. The U.S. Food and Drug Administration (FDA) recently approved a device for Ménière’s disease that fits into the outer ear and delivers intermittent air pressure pulses to the middle ear (pressure pulse treatment). The air pressure pulses appear to act on endolymph fluid to prevent dizziness.

Different surgical procedures are an option for people with persistent, debilitating vertigo. Labyrinthectomy (removal of the inner ear sense organ) can effectively control vertigo, but sacrifices hearing and is reserved for patients with nonfunctional hearing in the affected ear. Vestibular neurectomy, selectively severing a nerve from the affected inner ear organ, usually controls the vertigo while preserving hearing but carries surgical risks. Recently, the administration of the ototoxic antibiotic gentamycin directly into the middle ear space has gained popularity worldwide for the control of vertigo associated with Ménière's disease.

The Hearing Health Foundation (HHF), a non-profit funder of hearing and balance research, aims to prevent and cure hearing loss, tinnitus, and other hearing disorders through groundbreaking research, and to promote hearing health. Their Ménière's Disease Grants (MDG) program funds research focused on advancing the understanding of disease mechanisms, or diagnosis and treatment, of Ménière's disease.

Hearing Health Foundation
363 Seventh Avenue, 10th Floor
New York, NY 10001
Phone: (212) 257-6140
TTY: (888) 435-6104
Fax: (212) 257-6139
Email: info@hhf.org
Website: http://www.hhf.org

The National Institute on Deafness and Other Communication Disorders (NIDCD) conducts and supports biomedical and behavioral research and research training in the normal and disordered processes of hearing, balance, smell, taste, voice, speech, and language. Click on the link to view information on this topic.