Autoimmune inner ear disease: a retrospective review of forty-seven patients.

The purpose of this retrospective study was to characterize and further define autoimmune inner ear disease (AIED) using the Harris AIED classification. A retrospective review was conducted at two tertiary medical centers for 47 patients who were diagnosed with AIED. The overall patient response rate to oral prednisone treatment was 69.7%. The sensitivity of the test for a serum antibody against heat-shock protein 70 (HSP70) was 54.5% and the specificity was 42.9%. Therefore, the clinical utility of the HSP70 antibody test appeared to be limited with respect to the diagnosis of AIED. Vertigo, tinnitus and aural fullness improved significantly with both of the newly developed adalimumab (Humira®) and rituximab (Rituxan®). However, hearing loss did not improve in the present study.