Sudden sensorineural hearing loss: an autoimmune disease?

OBJECTIVES: To review our current knowledge of the pathogenesis of sudden sensorineural hearing loss, including viral infection, vascular occlusion and immune system-mediated mechanisms, and to discuss the pathogenesis as it relates to pharmacotherapy.

SYSTEMATIC REVIEW METHODOLOGY: Relevant publications on the pathogenesis of sudden sensorineural hearing loss from 1944 to 2010 were analysed.

RESULTS AND CONCLUSIONS: Sudden sensorineural hearing loss is defined as hearing loss of 30 dB in three sequential frequencies over 3 days or less. It can be an isolated symptom or the presenting symptom of a systemic disease. The aetiology and pathogenesis remain unknown. Detailed investigation typically reveals a specific cause in about 10% of patients. Proposed theories of causation include viral infections, vascular occlusion and immune system-mediated mechanisms. A variety of therapies have been proposed based on the various proposed aetiologies.