AIED was first described by Lehnhardt in 1958. Since then, investigations have demonstrated that the inner ear is capable of participating in the immune response and that this immune response can cause autoimmune injury to the inner ear. AIED affects all ages but most commonly presents in the 45- to 55-year range. It has an equal male-to-female involvement. AIED presents as progressive bilateral sensorineural hearing loss over weeks to months, with or without vestibulopathy. In some cases, the presentation may be unilateral loss with substantial delay before contralateral involvement occurs. The most specific laboratory test remains a Western blot for antibodies to a 68 kDa antigen found in inner ear tissues, and, if positive, is predictive of a high likelihood of steroid responsive disease. Treatment is with high-dose corticosteroids for at least a month, as lower doses or shorter therapy durations are associated with a high incidence of disease exacerbation. Although significant improvements in the pathogenesis and treatment of AIED have occurred, it still remains a disease that is difficult to diagnose and treat.
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