Immunosuppressive Therapy for Autoimmune Inner Ear Disease

Abstract

Autoimmune inner ear disease (AIED) is a rare disease that is diagnosed after clinical suspicion and response to corticosteroids. AIED manifests as progressive, bilateral, although often asynchronous, sensorineural hearing loss and can be associated with vestibular symptoms. Since its description as a defined disease entity in 1979, the initial mainstay of treatment remains high-dose corticosteroids. Several animal models have been developed to assist in determining efficacy of immunosuppression in AIED, and several clinical studies have also investigated the role of both steroid and steroid-sparing treatments. Here we discuss the basic science and clinical research surrounding the history of immunosuppressive therapy in AIED.

KEYWORDS::

• autoimmunity
• corticosteroid
• hearing loss
• immunomodulation
• immunosuppression

Financial & competing interests disclosure

This work was supported by NIH grant DC-006422 (awarded to Vincent K Tuohy). Vincent K Tuohy has patent application #60/842,923 entitled: Increased Frequencies of Cochlin Specific T Cells in Patients with Autoimmune Sensorineural Hearing Loss.

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Additional information

Funding

This work was supported by National Institute of Health (NIH) grant DC-006422 (awarded to Vincent K Tuohy). Vincent K Tuohy has patent application #60/842,923 entitled: Increased Frequencies of Cochlin Specific T Cells in Patients with Autoimmune Sensorineural Hearing Loss. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. No writing assistance was utilized in the production of this manuscript.