The clinical picture of autoimmue inner ear disease consists of bilateral progressive sensorineural hearing loss. Although sensorineural hearing loss usually develops over weeks or months, sudden hearing loss may occur in some cases. A 56 year-old woman with autoimmune inner ear disease presented with sudden bilateral sensorineural hearing loss, dizziness and tinnitus. An auditory brainstem response test revealed bilateral cochlear lesions. The anti-nuclear antibody test was positive at a titer of 1 : 5120, and the rheumatoid factor test was positive at a titer of 1: 20480. Autoimmune inner ear disease was diagnosed from patient’s clinical manifestations, positive immune laboratory tests, and beneficial treatment response to prednisolone 1 mg/kg/day for one week. This patient was discharged one week after admission and no recurrence or complications were noted on OPD follow-up after 3 years.