Autoimmune inner ear disease (AIED) is a clinical syndrome of bilateral sensorineural hearing loss (SNHL) >30dB at one or more frequencies progressing over a period of 3-90 days. Progression of SNLH >15dB at one frequency or >10dB in two frequencies in at least one ear should be demonstrated. Vestibular symptoms may be present in 50% of patients and systemic autoimmune disease (SAD) coexists in 30% of patients. In some cases with personal or familial history of SAD, AIED begins as sudden unilateral SNHL progressing rapidly to the second ear.