Autoimmune sensorineural hearing loss (ASNHL) is typically presented by a bilateral rapidly progressive hearing loss that may also occur suddenly. Both autoantibodies and autoreactive T cells have been implicated in the etiopathogenesis of ASNHL. However, the identity of a specific or highly relevant inner-ear self-antigen is still required. A definite diagnosis of ASNHL is usually made by excluding ototoxicity, and other factors that mimic ASNHL but most importantly by demonstrating a good therapeutic response to corticosteroid treatment.