Autoimmune-related hearing loss, described by McCabe in 1979 1 , is characterized by a rapidly progressing, usually bilateral hearing loss, which may fluctuate over a period of weeks to months. The duration and pattern distinguishes this condition from the more gradual hearing loss seen in presbyacusis and the abrupt loss in sudden sensorineural hearing loss. Responsiveness to steroid or immunosuppressant treatment is also considered characteristic of autoimmune inner ear disease (AIED). Vestibular symptoms are common and accompany up to 50% of cases.