Autoimmune Sensorineural Hearing Loss

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. In this chapter, we will summarize many of the current studies that have established the idea of ASNHL being autoimmune in many cases. Also, we will address the need for specific diagnostic tools to better classify/diagnose this entity. These tools may lead to the development and application of immunosuppressive therapies to prevent the deterioration of hearing loss and could possibly prevent the requirement for cochlear implantation.