Autoimmune Inner Ear Disease (AIED)

Autoimmune inner ear disease was first described in 1979. Patients with autoimmune inner ear disease have rapidly, progressive bilateral sensorineural hearing loss. The diagnosis of AIED is based primarily on clinical presentation. Serologic blood testing is done to look for systemic immunologic dysfunction. The treatment of AIED is primarily immunosuppressive therapy with high-dose corticosteroids. Patterns of response to corticosteroid therapy are variable. Alternatives to systemic corticosteroids include intratympanic steroid therapy as well as other immunomodulators which have been tried on an experimental basis.