Intratympanic Injection for Autoimmune Inner Ear Disease

Study Overview
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Brief Summary
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The investigators plan to conduct an open-label intratympanic injection proof-of-concept trial of golimumab, a TNF-alpha inhibitor, assessing for hearing loss progression in patients with autoimmune inner ear disease (AIED). This specific aim will be achieved using a two-arm approach. First, the investigators propose to dose 3 individual subjects with a single intratympanic injection of golimumab and follow each for 30 days, closely examining them for adverse events. If there are no serious adverse events, with FDA approval, the investigators propose to dose 14 subjects, each with 4 intratympanic injections of golimumab.

Official Title
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Safety and Efficacy of Intratympanically Delivered Golimumab for Stabilization of Hearing in Patients With Autoimmune Inner Ear Disease: An Open-label Proof-of-concept Clinical Trial

Conditions
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Autoimmune Inner Ear Disease

Intervention / Treatment
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* Drug: Golimumab


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of autoimmune inner ear disease (AIED) by the principal investigator Idiopathic, bilateral sensorineural hearing loss History of, or audiograms showing, rapid progression of hearing loss Bilateral loss of at least 30 dB in one or more frequencies (0.25 - 6 kHz) Hearing responsive on high-dose oral steroids and steroid-dependent, as determined by history and the principal investigator. The subject can be taking oral steroid, at the maintenance level, if enrolled in the First Arm and, if enrolled in the Second Arm, will begin taper after the first injection. Provided written informed consent for participation in the clinical study Exclusion Criteria: Positive MRI for vestibular schwannoma Positive FTA (syphilis) Significant middle ear disease (e.g., otitis media) Positive blood test for Lyme disease Positive tuberculosis test Concurrent or past treatment or use of medications and/or substances known to cause ototoxicity(for example, aminoglycosides [e.g., gentamicin], cisplatin, loop diuretics, Yorgason et al., 2006) Known adverse reaction to golimumab, adalimumab, etanercept, infliximab, rituximab, ustekinumab, or other biologic immunomodulators Concurrent (within the past 3 months prior to enrollment) live viral intranasal vaccine (flu) Positive test for HIV Positive test for Hepatitis B and C Presence of a demyelinating disease, such as multiple sclerosis Women of childbearing potential only: Positive serum pregnancy test prior to the only/first injection Active infections

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: First Arm<br>Determine safety of intratympanic injection | Drug: Golimumab<br>* Intratympanic injection 0.3ml First Arm: 1 injection Second Arm: 4 injections<br>* Other names: Simponi;|
| Experimental: Second Arm<br>Efficacy evaluation of 4 intratympanic injections | Drug: Golimumab<br>* Intratympanic injection 0.3ml First Arm: 1 injection Second Arm: 4 injections<br>* Other names: Simponi;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Serious Adverse Events | Serious Adverse Events | 30 days | 
| Pure-tone threshold change | Change in pure-tone threshold from baseline to 6 week after initiation of treatment | 6 weeks | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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autoimmune, hearing loss, bilateral hearing loss, steroid-dependent