Sudden deafness

The author explains the clinical presentation, pathophysiology, diagnostic work-up, and management of sudden deafness. "Sudden" deafness is defined as sensorineural hearing loss of 30 decibels or more in at least 3 contiguous frequencies occurring over less than 3 days. The pathophysiology of sudden deafness is poorly understood. Various theories have been proposed, including those attributing sudden deafness to vascular insults, infectious (especially viral) agents, autoimmune or inflammatory mechanisms, or disruption of labyrinthine membranes. Despite extensive investigation, most cases remain idiopathic. Systemic steroids, or a combination of systemic and intratympanic steroids, are commonly recommended, but some employ intratympanic steroid therapy as a first-line therapy because systemic and transtympanic administration of corticosteroids has been found to result in similar clinical outcomes. Intratympanic steroid perfusion should be offered in patients with incomplete recovery from idiopathic sudden sensorineural hearing loss after failure of initial management, and when used as salvage therapy, intratympanic steroids can result in significant gains in hearing. The overall prognosis depends on the underlying etiology, but a high rate of spontaneous resolution occurs overall (ie, about two thirds of cases). For those who do not recover from idiopathic sudden deafness in their only hearing ear (ie, producing bilateral deafness), cochlear implantation can be considered as early as 3 months after initiating treatment of sudden deafness.