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The characteristics McCabe (13) described continue to be relevant even today, including asymmetric sensorineural hearing loss, occasionally involvement of balance organs, worsening over time, and responsiveness to steroids and other immunosuppressants such as cyclophosphamide. There is a spectrum of disease encompassed by the current terms autoimmune inner ear disease (AIED) and immune-mediated cochleovestibular disorders. The presentations include rapidly progressive sensorineural hearing loss, sudden deafness, and Meniere's syndrome (14). One of the authors (R.T.S.) has treated cases with virtually all audiometric patterns, including total deafness and normal hearing with vestibular symptoms only.