Sudden sensorineural hearing loss: supporting the immunologic theory

Sudden deafness constitutes a diagnostic challenge. Classically, 2 causes, viral and vascular, are considered in the origin of idiopathic sudden hearing loss. More recently added to the list of possibilities are rupture of the membranous labyrinth and immune-mediated sensorineural hearing loss. The latter can be either primary and localized to the inner ear or, in perhaps fewer than one third of cases, secondary to generalized systemic autoimmune disease. The purpose of the present review is to define immune-mediated sudden sensorineural hearing loss as a distinctive entity, on the basis of clinical, immunologic, and pathological findings, and suggest a profile of the typical patient.