Study of the Effects of Anakinra in Corticosteroid-resistant Subjects With Sudden Sensorineural Hearing Loss

For patients that experience an acute, sensorineural decline in hearing, timely
corticosteroid administration may result in preservation of some or all of the hearing. For
patients with sudden sensorineural hearing loss who have failed to respond to corticosteroid
therapy, this study will be offered to determine if anakinra is a safe and effective
alternative therapy for clinical hearing restoration. The investigators have previously
demonstrated that IL-1beta inhibition (with anakinra) in a small cohort of patients with
steroid resistant autoimmune inner ear disease was effective in demonstrating audiological
improvements.

Inclusion Criteria:

1. Patients must have Sudden Sensorineural Hearing Loss in one ear, with a no greater
than 25 dB PTA in the contralateral ear.

2. Age and Gender: male and female subjects, age ≥ 18 but ≤ 75, will be recruited.

3. Patients must be capable of understanding and giving informed consent.

4. Patients must have SNHL of greater than or equal to 30dB at three contiguous
frequencies in one ear which evolved in three days or less, with a PTA of 25dB or
less in the contralateral ear.

5. Patients must have previously undergone a trial of high-dose corticosteroid therapy,
at 60 mg daily for a minimum of seven days, with a variable taper thereafter that
consists of a total of 14 consecutive days of corticosteroid use. Patients must have
demonstrated less than a 5 decibel average improvement in their PTA in response to
corticosteroids as measured by their audiogram.

6. NO greater than 30 days may elapse from the discontinuation from the initial course
of steroid treatment to the time of enrollment.

Exclusion Criteria:

1. Patients over 75 years of age, because there is a higher incidence of infections in
the elderly population in general, caution should be used in treating the elderly.

2. Patients with evidence of retrocochlear pathology (vestibular schwannoma) or inner
ear malformation (Mondini Malformation or Enlarged Vestibular Aqueduct) based on
imaging.

3. Patients concurrently receiving methotrexate or TNF-antagonist therapy.

4. Patients with a diagnosis of any immunodeficiency syndrome.

5. Patients with active or chronic infections.

6. Patients currently receiving, or having received treatment for a malignancy in the
past three years.

7. Patients with a diagnosis of chronic renal insufficiency (a creatinine clearance of
<49mL/min) or chronic renal failure.

8. Patients with evidence of neutropenia (an ANC of <1000) prior to treatment with
anakinra.

9. Known hypersensitivity to E. coli derived products.

10. Latex sensitivity.

11. Any patient that received a live vaccine < 3 months prior to enrollment.

12. Any patient with a history of active narcotic abuse, including prescription
narcotics.

13. Pregnant or lactating females.

14. Children, < age 18

15. .Non-English speaking patients, as the word recognition scoring is in English and is
a vital component to the efficacy analysis.

16. Any patient that tests positive for Hepatitis B, C, HIV or tuberculosis on screening,
with the exception of presence of antibodies to Hepatitis B in subjects reporting
prior vaccination, and presence of positive skin testing for TB in subjects who
received BCG in the past.