Rilonacept for Treatment of Autoimmune Neurosensory Hearing Loss

Summary

This study is an open label proof of concept study of rilonacept for patients with ANSHL

Timeline

Start

2017-02-01

Primary completion

2018-03

Completion

2018-03

Publications

• Background McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol. 1979 Sep-Oct;88(5 Pt 1):585-9. doi: 10.1177/000348947908800501.
• Background Harris JP, Sharp PA. Inner ear autoantibodies in patients with rapidly progressive sensorineural hearing loss. Laryngoscope. 1990 May;100(5):516-24. doi: 10.1288/00005537-199005000-00015.
• Background Moscicki RA, San Martin JE, Quintero CH, Rauch SD, Nadol JB Jr, Bloch KJ. Serum antibody to inner ear proteins in patients with progressive hearing loss. Correlation with disease activity and response to corticosteroid treatment. JAMA. 1994 Aug 24-31;272(8):611-6.
• Background Tebo AE, Szankasi P, Hillman TA, Litwin CM, Hill HR. Antibody reactivity to heat shock protein 70 and inner ear-specific proteins in patients with idiopathic sensorineural hearing loss. Clin Exp Immunol. 2006 Dec;146(3):427-32. doi: 10.1111/j.1365-2249.2006.03227.x.
• Background Hirose K, Wener MH, Duckert LG. Utility of laboratory testing in autoimmune inner ear disease. Laryngoscope. 1999 Nov;109(11):1749-54. doi: 10.1097/00005537-199911000-00005.
• Background Bouman H, Klis SF, Meeuwsen F, de Groot JC, Smoorenburg GF, Veldman JE. Experimental autoimmune inner ear disease: an electrocochleographic and histophysiologic study. Ann Otol Rhinol Laryngol. 2000 May;109(5):457-66. doi: 10.1177/000348940010900504.
• Background McCabe BF. Autoimmune inner ear disease: therapy. Am J Otol. 1989 May;10(3):196-7.
• Background Saracaydin A, Katircioglu S, Katircioglu S, Karatay MC. Azathioprine in combination with steroids in the treatment of autoimmune inner-ear disease. J Int Med Res. 1993 Jul-Aug;21(4):192-6. doi: 10.1177/030006059302100404.
• Background Sismanis A, Wise CM, Johnson GD. Methotrexate management of immune-mediated cochleovestibular disorders. Otolaryngol Head Neck Surg. 1997 Feb;116(2):146-52. doi: 10.1016/S0194-59989770316-4.
• Background Luetje CM. Theoretical and practical implications for plasmapheresis in autoimmune inner ear disease. Laryngoscope. 1989 Nov;99(11):1137-46. doi: 10.1288/00005537-198911000-00006.
• Background Kitashara M, Yazawa.Y, Uchida K. Immunoglobulin treatment for advance cases of bilateral Meniere's disease. In: Nadol JB ed. Meniere's disease: pathogenesis, pathophysiology, diagnosis and treatment. Berkeley, Ca: Kugler Publications; 1989; 411-419.
• Background Harris JP, Weisman MH, Derebery JM, Espeland MA, Gantz BJ, Gulya AJ, Hammerschlag PE, Hannley M, Hughes GB, Moscicki R, Nelson RA, Niparko JK, Rauch SD, Telian SA, Brookhouser PE. Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. JAMA. 2003 Oct 8;290(14):1875-83. doi: 10.1001/jama.290.14.1875.
• Background Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum. 2004 Oct;34(2):544-8. doi: 10.1016/j.semarthrit.2004.07.001.
• Background Cohen S, Shoup A, Weisman MH, Harris J. Etanercept treatment for autoimmune inner ear disease: results of a pilot placebo-controlled study. Otol Neurotol. 2005 Sep;26(5):903-7. doi: 10.1097/01.mao.0000185082.28598.87.
• Background Rahman MU, Poe DS, Choi HK. Etanercept therapy for immune-mediated cochleovestibular disorders: preliminary results in a pilot study. Otol Neurotol. 2001 Sep;22(5):619-24. doi: 10.1097/00129492-200109000-00010.
• Background Cohen S, Roland P, Shoup A, Lowenstein M, Silverstein H, Kavanaugh A, Harris J. A pilot study of rituximab in immune-mediated inner ear disease. Audiol Neurootol. 2011;16(4):214-21. doi: 10.1159/000320606. Epub 2010 Oct 27.
• Background Rynne M, Maclean C, Bybee A, McDermott MF, Emery P. Hearing improvement in a patient with variant Muckle-Wells syndrome in response to interleukin 1 receptor antagonism. Ann Rheum Dis. 2006 Apr;65(4):533-4. doi: 10.1136/ard.2005.038091.
• Background Yamazaki T, Masumoto J, Agematsu K, Sawai N, Kobayashi S, Shigemura T, Yasui K, Koike K. Anakinra improves sensory deafness in a Japanese patient with Muckle-Wells syndrome, possibly by inhibiting the cryopyrin inflammasome. Arthritis Rheum. 2008 Mar;58(3):864-8. doi: 10.1002/art.23261.
• Background Pathak S, Goldofsky E, Vivas EX, Bonagura VR, Vambutas A. IL-1beta is overexpressed and aberrantly regulated in corticosteroid nonresponders with autoimmune inner ear disease. J Immunol. 2011 Feb 1;186(3):1870-9. doi: 10.4049/jimmunol.1002275. Epub 2011 Jan 3.
• Background Vambutas A, Lesser M, Mullooly V, Pathak S, Zahtz G, Rosen L, Goldofsky E. Early efficacy trial of anakinra in corticosteroid-resistant autoimmune inner ear disease. J Clin Invest. 2014 Sep;124(9):4115-22. doi: 10.1172/JCI76503. Epub 2014 Aug 18.

Drugs

Indications