Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Mar-Dec:32:2058738418808680.
doi: 10.1177/2058738418808680.

Autoimmune inner ear disease (AIED): A diagnostic challenge

Andrea Ciorba  1 , Virginia Corazzi  1 , Chiara Bianchini  1 , Claudia Aimoni  1 , Stefano Pelucchi  1 , Piotr Henryk Skarżyński  2   3   4 , Stavros Hatzopoulos  1
Affiliations

Autoimmune inner ear disease (AIED): A diagnostic challenge

Andrea Ciorba et al. Int J Immunopathol Pharmacol. 2018 Mar-Dec.

Abstract

Autoimmune inner ear disease (AIED) has been defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an 'uncontrolled' immune system response. The inner ear can be the direct target of the immune response, but it can be additionally damaged by a deposition of circulating immune complexes or by systemic immune-mediated diseases. The clinical expression of immune-mediated inner ear disease shows a progressive bilateral and asymmetric SNHL profile, which typically benefits from a steroid and immunosuppressive therapy. The onset of AIED is between 3 and 90 days. Cochlear symptoms can be associated with vestibular disorders and in 15%-30% of cases, AIED occurs in the contest of a systemic autoimmune disease. Currently, the onset of immune-mediated SNHL is not a well-understood process and the pathogenetic mechanisms of AIED remain unclear. Furthermore, there are no standardized diagnostic criteria or reliable diagnostic tests for the diagnosis of AIED. Hence, the definition of immune-mediated cochleovestibular disorders is a challenging diagnosis based on exclusion. A close collaboration between otolaryngologists, audiologists and rheumatologists is recommended, in order to achieve the multidisciplinary management of this rare entity, since an early AIED identification and a prompt medical treatment might result in acceptable hearing outcomes. The paper describes the clinical features of AIED and offers a diagnostic flow-chart to use in the clinical assessment of this condition.

Keywords: AIED; autoimmune disease; hearing loss; immune system; inner ear; steroids.

PubMed Disclaimer

Conflict of interest statement

Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

References

    1. Vambutas A, Pathak S. (2016) AAO: Autoimmune and autoinflammatory (disease) in otology: What is new in immune-mediated hearing loss; Laryngoscope Investigative Otolaryngology 1(5): 110–115. - PMC - PubMed
    1. Mijovic T, Zeitouni A, Colmegna I. (2013) Autoimmune sensorineural hearing loss: The otology-rheumatology interface. Rheumatology 52(5): 780–789. - PubMed
    1. Bovo R, Ciorba A, Martini A. (2009) The diagnosis of autoimmune inner ear disease: Evidence and critical pitfalls. European Archives of Oto-Rhino-Laryngology 266(1): 37–40. - PubMed
    1. Bovo R, Aimoni C, Martini A. (2006) Immune-mediated inner ear disease. Acta Oto-Laryngologica 126(10): 1012–1021. - PubMed
    1. Harris JP, Weisman MH, Derebery JM, et al. (2003) Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: A randomized controlled trial. JAMA 290(14): 1875–1883. - PubMed

MeSH terms