Diagnosis and treatment of sensorineural hearing deficits of autoimmune origin

: Sensorineural hearing loss (SHL) of autoimmune origin is characterized by a rapidly progressive onset. Diagnosis is difficult and the condition responds to corticoids or immunosuppressant agents. Two cases with an onset over weeks and progressive hearing loss are reported. Both patients had SHL associated with another autoimmune disease. Laboratory findings were abnormal (immunoglobulins and lymphocyte populations), suggesting an immune disorder. Treatment with 1 mg/kg/day of deflazacort restored hearing to normal. Both patients are currently asymptomatic. The clinical and laboratory response of patients to deflazacort supported the diagnosis of autoimmune SHL.