REVIEW article
Front. Audiol. Otol.
Sec. Otology and Neurotology
Volume 3 - 2025 | doi: 10.3389/fauot.2025.1624303
Advances in Immune Mediated Inner Ear Disease
Provisionally accepted
- 1Stanford University, Stanford, United States
- 2University of Pittsburgh, Pittsburgh, United States
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Immune-mediated inner ear disease (IMIED) is emerging in our understanding as a cause of sensorineural hearing loss (SNHL). It poses a considerable diagnostic challenge due to the lack of specific tests and diagnostic biomarkers as well as clinical features that overlap with other audiovestibular disorders. Cases may involve isolated inner ear dysfunction or occur in the context of systemic autoimmune diseases. IMIED pathogenesis involves the interplay of autoantibodies, cytotoxic T cells, and innate immune mechanisms. Corticosteroid responsiveness is a defining feature of IMIED, but refractory cases may require alternative immunosuppressive agents. Although emerging immunosuppressive regimens demonstrate potential efficacy, larger trials are warranted to establish diagnostic and therapeutic guidelines. While symptomatic treatments including hearing aids and cochlear implants are beneficial in patients with IMIED associated hearing loss, future strategies focus on preventing irreversible inner ear damage and preserving hearing by developing innovative immunomodulatory strategies.
Keywords: Hearing Loss, Immunity, Macrophages, Autoimmunity, inner ear Reviewer 1. Comment 2
Received: 07 May 2025; Accepted: 18 Jul 2025.
Copyright: © 2025 Kullar and Santa Maria. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Peter Kullar, Stanford University, Stanford, United States
Peter Santa Maria, University of Pittsburgh, Pittsburgh, United States
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