A Clinical Trial of Anakinra for Steroid-Resistant Autoimmune Inner Ear Disease

Phase 1

Completed

Brief Summary: 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.

Detailed Description: 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.

Recruitment & Eligibility

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

Arm && Interventions

Group	Intervention	Description
Single Arm-Open Label	Anakinra	Single Arm-Open Label use of Anakinra

Primary Outcome Measures

Name	Time	Method
To Assess the Potential Efficacy of Anakinra in Improving Hearing Thresholds in Corticosteroid-resistant Patients With Autoimmune Inner Ear Disease	180 days	The primary endpoint is to determine whether those treated with anakinra for 84 days demonstrate an improved hearing threshold compared with their pre-anakinra-treatment threshold. Audiometric thresholds will be compared to those treated with a prolonged corticosteroid taper and those that elect for no further treatment. The durability of the response will be measured over a total of 180 days.

Secondary Outcome Measures

Name	Time	Method
Number of Serious Adverse Events Reported	84 days	To assess the number of Serious Adverse Events reported in any subject that received at least one injection dose of anakinra