Autoimmune Inner Ear Disease (AIED) is a poorly understood etiology of progressive sensorineural hearing loss that is amenable to corticosteroid therapy in approximately 60% of cases.  This response is lost over time, owing to unknown mechanisms.  To date, no effective therapeutic intervention exists for corticosteroid-resistant patients with AIED.  

IL-1ß is an inflammatory cytokine that mediates the innate immune response.  Expression of IL-1ß dictates many of the later responses in other autoimmune and inflammatory disorders.  We have evidence that several IL-1β family members are aberrantly regulated in corticosteroid non-responders with AIED, and may contribute to steroid resistance.  These findings provide the rationale for IL-1 blockade with Anakinra in corticosteroid resistant (non-responder) patients with AIED as a phase I open-label clinical trial to determine whether use of Anakinra can improve audiometric thresholds in these patients.