Introduction
Autoimmune 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.