Overview
A Phase II randomized, placebo controlled study design of anakinra (Kineret) in corticosteroid-resistant or intolerant Meniere's disease (CR-MD)and corticosteroid-resistant or intolerant autoimmune inner ear disease (CR-AIED) patients. Patients will be randomized by a 2:1 allocation to anakinra or placebo for 42 continuous days. After day 42, a second placebo-controlled period will begin for an additional 42 days. This will be followed by a 264 day observation period, during which, hearing declines may be re-treated with anakinra after 30 days following the initial drug.
Description
This study will be evaluating whether or not anakinra (kineret) is superior to placebo for improving sustained hearing thresholds in patients with CR or intolerant MD and CR or intolerant AIED. Final enrollment is contingent on an acute decline in hearing in the active ear and 2 sequential audiograms greater than or equal to 28 days apart that shows a stable or declining hearing threshold and word recognition score not improved with corticosteroid therapy. Any patient that attempted to be treated with steroid therapy and was deemed intolerant will also be considered for the trial. Anakinra (kineret) 100mg/0.67ml prefilled syringes or placebo injection will be randomized at 2:1 allocation and will be self-administered by the patient daily for 42 days. After day 42, a second placebo-controlled period will begin for an additional 42 days (all patients will receive anakinra for either 42 or 84 days depending on the randomization assignment). A 264 day observation period will begin after day 84, during which the option to retreat with anakinra will be offered to those patients that responded to the therapy in the first 84 day period.
Eligibility
Inclusion Criteria:
* meet diagnostic criteria for Autoimmune Inner Ear Disease (AIED) with active deterioration in one ear as defined below
* SNHL of greater than 30dB at one or more frequencies in the active ear. The contralateral ear may have any hearing threshold that is either stable or worsening, but may not exhibit improvement in response to corticosteroids even if the more active ear declines. Active deterioration is defined as a 15dB decline at one frequency (excluding 250 or 8kHz as a sole indicator) on their audiogram, or 10dB at 2+ frequencies developing in \> or equal 3 days but \< or equal 90 days, or if the hearing loss evolved in less than 3 days, the patient displayed features suggestive of an autoimmune disorder
* Capable of understanding and giving informed consent
* Have 2 sequential audiograms following the decline in hearing \>or equal 28 days apart with no demonstrable improvement in hearing following corticosteroid therapy (\
