This is a relative new term to encompass any sensorineural hearing loss that responds to an immunomodulatory drug. The most commonly used of these are corticosteroids, of which prednisone, methylprednisolone and dexamethasone are common examples. Immunosuppressive drugs include methotrexate, mycophenolate, cyclosporin, cyclophosphamide among others are classically used in auto-immune disorders and organ transplantation. Biologic agents mooted in the treatment of inner ear disease include those that target TNF-alpha, and Interleukin-1beta.
According to this definition Sudden Sensorineural Hearing Loss (SSNHL), Autoimmune Inner Ear Disease (AIED) and Meniere’s Disease can be included.
Other even rarer syndromes include Cogan’s Syndrome, Otosyphilis and Muckle Wells Disease.