Autoimmune inner ear disease (AIED) is an inflammatory condition of the inner ear. It occurs when the body’s immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occurring in less than one percent of the 28 million Americans with a hearing loss.
The symptoms of AIED are sudden hearing loss in one ear progressing rapidly to the second ear. The hearing loss can progress over weeks or months. Patients may feel fullness in the ear and experience vertigo. In addition, a ringing, hissing or roaring sound in the ear may be experienced. Diagnosis of AIED is difficult and is often mistaken for otitis media until the patient develops a loss in the second ear. One diagnostic test that is promising is the Western Blot Immunoassay.
Most patients with AIED respond to the initial treatment of steroids, prednisone and methotrexate (a chemotherapy agent). Some patients may benefit from the use of hearing aids. If patients are unresponsive to drug therapy and hearing loss persists, a cochlear implant maybe considered.