Autoimmune inner ear disease (AIED) is a syndrome of progressive hearing loss and/or dizziness that is caused by antibodies or immune cells attacking the inner ear. In most cases, there is reduction of hearing accompanied by tinnitus, as a ringing, hissing, roaring noise, which occurs over a few months.
AIED occurs when the body's immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. It can happen in isolation or as part of other systemic autoimmune disorders. Twenty percent of patients have other autoimmune diseases, such as rheumatoid arthritis or lupus.
The exact clinical description can vary somewhat; however, most agree that the syndrome follows a fairly typical course. AIED will manifest as rapidly progressing, nerve related hearing loss that is most commonly bilateral. Typically, it begins unilaterally and ultimately affects the other ear, and it can occur suddenly. Word recognition is usually disproportionately poor.
Diagnosing an autoimmune disorder as the cause of inner ear symptoms can be difficult. To succeed, a physician must have training and experience in these disorders. An early diagnosis is important because treatment cannot only stop the disease progression but in some cases can reverse the damage.