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Autoimmune inner ear disease (AIED) was first defined by Brian McCabe in a landmark paper describing an autoimmune loss of hearing which could not be easily categorized.[1] The disease results in progressive sensorineural hearing loss (SNHL) that acts bilaterally and asymmetrically, and sometimes affects an individual's vestibular system. AIED is used to describe any disorder in which the inner ear is damaged as a result of an autoimmune response. [2] Research has come to the consensus that AIED is the result of antibodies or other immune cells that cause damage to structure of the inner ear such as the cochlea and vestibular system. Of note, AIED is the only known SNHL that responds to medical treatment, but withholding treatment for longer than three months may result in permanent hearing loss and the need for cochlear implant installation. [3]

Although AIED has been studied extensively over the past 25 years, no clear mechanism of pathogenesis has emerged. A recent paper[4] performed a literature review of all relevant articles dating back to 1980, and proposed a mechanism of pathogenesis which includes an inflammatory response and immune cell attack on inner ear structures. This response leads to an over-activation of other immune cells such as T helper cells, resulting in vascular changes and cochlear harm. AIED appears to be a consequence of damaged sensorineural hearing due to electrochemical disturbances, microthrombosis, and immune cell deposition. Additionally, self-reactive antibodies and T-cells contribute to the aforementioned damage. Research has suggested a valuable next step in uncovering AIED pathogenesis is inquiry into the role of interleukin-1β (IL-1β).[5][6]

Several medical therapies have proven beneficial in the treatment of AIED, with corticosteroid therapy being the most effective. However, corticosteroid therapy has shown to only be effective in 70% of patients, suggesting that more research into novel treatment methods is required. The goal of most AIED treatments is to administer corticosteroids over a certain period of time, re-evaluate hearing at each appointment, and eventually taper off corticosteroid administration. Ideally, patients can be tapered off with hearing fully recovered, though this is the least likely outcome. Often, tumor necrosis factor-α (TNF-α) inhibitors must be administered alongside corticosteroids to achieve a favorable outcome and eventually end to corticosteroid treatment. Recent papers have indicated that the TNF-α inhibitor, infliximab, has the potential to allow for sustained patient improvement and alleviation of symptoms. [7][8]

  1. ^ McCabe, Brian (September 1979). "Autoimmune sensorineural hearing loss". Annals of Otology, Rhinology, and Laryngology. PMID 496191.
  2. ^ "Autoimmune Inner Ear Disease | Vestibular Disorders Association". vestibular.org. Retrieved 2016-02-11.
  3. ^ "Autoimmune Inner Ear Disease (AIED) | American Hearing Research Foundation". american-hearing.org. Retrieved 2016-02-11.
  4. ^ Goodall, A. F.; Siddiq, M. A. (2015-10-01). "Current understanding of the pathogenesis of autoimmune inner ear disease: a review". Clinical otolaryngology: official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery. 40 (5): 412–419. doi:10.1111/coa.12432. ISSN 1749-4486. PMID 25847404.
  5. ^ Rauch, Steven D. (2014-09-01). "IL-1β inhibition in autoimmune inner ear disease: can you hear me now?". The Journal of Clinical Investigation. 124 (9): 3685–3687. doi:10.1172/JCI77197. ISSN 1558-8238. PMC 4151210. PMID 25133418.
  6. ^ Zhao, Ruijuan; Zhou, Hongyan; Su, Shao Bo (2013-11-01). "A critical role for interleukin-1β in the progression of autoimmune diseases". International Immunopharmacology. 17 (3): 658–669. doi:10.1016/j.intimp.2013.08.012. ISSN 1878-1705. PMID 24012439.
  7. ^ Heywood, R. L.; Hadavi, S.; Donnelly, S.; Patel, N. (2013-11-01). "Infliximab for autoimmune inner ear disease: case report and literature review". The Journal of Laryngology and Otology. 127 (11): 1145–1147. doi:10.1017/S002221511300217X. ISSN 1748-5460. PMID 24125068.
  8. ^ Gazeau, Pierre; Saraux, Alain; Devauchelle-Pensec, Valérie; Cornec, Divi (2014-09-01). "Long-term efficacy of infliximab in autoimmune sensorineural hearing loss associated with rheumatoid arthritis". Rheumatology (Oxford, England). 53 (9): 1715–1716. doi:10.1093/rheumatology/keu025. ISSN 1462-0332. PMID 24625506.