Autoimmune sensorineural hearing loss (ASNHL) remains without an adequate therapy other than corticosteroids. Here we report 2 patients who had a favorable response to cyclosporine (CSA).

Patient 1. A 58-year-old white male was diagnosed with ASNHL following an upper respiratory tract infection. He responded well to high dose prednisone but attempts at tapering were unsuccessful. CSA was started at 4 mg/kg/day with a dramatic response, which allowed tapering the prednisone to 20 mg/day. When CSA was tapered down, he had a prompt and significant worsening of his hearing and his word recognition dropped to 44%. Resuming the original dose of CSA brought his hearing back to baseline and his word recognition to 92% within a week. Patient 2. A 57-year-old white female underwent a left cochlear implant for longstanding hearing loss. One month following surgery, however, the hearing in both ears started to deteriorate. ASNHL was diagnosed. Despite continued high dose prednisone, the hearing in her right ear continued to deteriorate. CSA was started at 4 mg/kg/day. Her hearing improved markedly with word recognition improving to 86% with the hearing aid. 8 weeks later CSA was stopped because of incapacitating tremors. Her hearing then worsened significantly despite treatment with steroids.

ASNHL is a well-established autoimmune disease with poor response to most immunosuppressants. CSA has been successfully used in rheumatoid arthritis and, at low doses, in autoimmune urticaria. These two patients suggest that low dose CSA can be an invaluable tool in the treatment of ASNHL and, perhaps, other autoimmune disorders.