The purpose of this study is to determine if Anakinra (an interleukin-1 receptor antagonist) can improve hearing thresholds in those patients with Autoimmune Inner Ear Disease (AIED) that did not respond to oral steroid therapy for a sudden decline in hearing. The patients to be enrolled will have recently completed a course of oral steroids and demonstrated no change in their audiometric thresholds following corticosteroid therapy.

Patients with immune mediated hearing loss (also known as autoimmune hearing loss) are typically treated with corticosteroids. Of those treated, approximately 60% respond, however, that response may be lost over time. Other therapies use to date have proven largely ineffectual in improving hearing. This study proposes a phase I open label clinical trial of Anakinra for corticosteroid-resistant patients to determine if this therapy is efficacious in hearing restoration.

Inclusion Criteria:

• Bilateral sensorineural hearing loss with an active decline in hearing in one ear
• No audiometric improvement with 28-30 days of oral prednisone or other corticosteroid, including an initial dose of 60mg per day for 14 days
• Enrollment within 14 days of completion of corticosteroid therapy
• Age 13 years and older
• No evidence of neutropenia (low white blood cell count)
• No evidence of retrocochlear pathology (ie. acoustic neuroma/vestibular schwannoma)
• May have concurrent, systemic autoimmune disease

Exclusion Criteria:

• Age over 75, or less than 13
• Neutropenia
• Renal insufficiency
• Pregnant females
• Unilateral hearing loss
• Patients with any immunodeficiency syndrome
• Patients receiving methotrexate or any TNF (tumor necrosis factor) antagonist therapy
• Patients with chronic infections
• Patients treated for a malignancy within the past 3 years
• Patients with a latex allergy
• Patients with an inner ear anomaly
• Patients with retrocochlear pathology

Corticosteroid-resistant AIED patients.