Autoimmune inner ear disease is a rare condition where the immune system attacks cells in the inner ear, causing symptoms such as hearing loss, tinnitus, and dizziness. Diagnosis is difficult, and treatment involves corticosteroids or cytotoxic drugs. Cochlear implants may be an option for those with permanent hearing loss.
Autoimmune inner ear disease, also called AIED, is a disease in which the body’s immune system begins to attack cells in the inner ear. The disease can cause symptoms such as dizziness, hearing loss and tinnitus. About 28 million Americans have ear problems that cause hearing loss; of these less than 1% have AIED.
Symptoms of autoimmune inner ear disease are often rapid in onset. Usually, sudden hearing loss in one ear progresses rapidly to affect both ears. Over the course of several weeks to months, the affected individual will lose partial or complete hearing in both ears. In addition to hearing loss, a person with AIED may also experience dizziness, ringing in the ears, tinnitus, dizziness and loss of balance, and a feeling of fullness in one or both ears.
These symptoms occur because AIED is an autoimmune disease, a class of disorders in which the immune system is activated abnormally. In this case, the immune system responds to certain cells in the body as if they are invading organisms such as bacteria or viruses. When autoimmune inner ear disease is triggered, immune system cells and antibodies attack certain proteins found in the cells of the inner ear, killing the cells and ultimately destroying the function of the ear.
Diagnosing AIED is often difficult because symptoms are relatively nonspecific. Autoimmune inner ear disease is often mistaken for otitis media, a more common and less serious inner ear infection that is treated with antibiotics. In many cases, a correct diagnosis of AIED is made only when the disease begins to affect both ears.
As with many other types of autoimmune disease, the primary treatment of autoimmune inner ear disease is corticosteroid medication. Steroids are prescribed because they have an immunosuppressive effect, which means they suppress the immune system so that the inner ear is no longer threatened. The problem with steroid treatment, however, is that it’s not specific. Steroids will help treat AIED by suppressing the immune system’s attack on the inner ear, but steroids will also suppress the entire immune system, making the patient more vulnerable to infection.
Steroids are generally considered unsuitable as a long-term treatment due to their side effects. For people who do not benefit from steroids, cytotoxic drugs such as cyclophosphamide and methotrexate can be used. Cytotoxic drugs are usually used as cancer treatments, but they are also useful here because these drugs kill rapidly dividing cells, a category to which both cancer cells and immune cells belong.
People who develop permanent hearing loss in both ears may be candidates for a cochlear implant. The cochlear implant is an electronic device that is surgically implanted inside the inner ear. Implants work by stimulating the auditory nerves in the inner ear to produce sound.
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