IntroductionAutoimmune inner ear disease (AIED) is a condition first described by McCabe in 1979 based on a diagnostic study on a cohort of patients[1] who presented with bilateral sensorineural hearing loss (SNHL) with a specific clinical pattern that did not fit with existing diagnoses and demonstrated audiometric improvement with corticosteroid and immunosuppressive therapy. The pathogenesis of AIED is not completely understood, though several mechanisms have been proposed, including uncontrolled humoral and cell-mediated reactions against inner ear antigens, [2] resulting in autoantibody development and T-cell responses. [3]AIED accounts for <1% of SNHL cases [2]but may be under-diagnosed due to a lack of standardised diagnostic criteria. [4] The main treatment has been corticosteroids; however, the overall reported response is 60-70%[2] and only 14% remain responsive after 34 months. [5] There is a risk of further decline in hearing in the absence of therapy. Alternative immunosuppressive treatments [3] were tried but these have yielded variable results. There is no standard treatment protocol for AIED following corticosteroids as randomised controlled trials are limited.[6] Here, we report a case of AIED in a patient that responded well to infliximab.