Demystifying autoimmune inner ear disease

  • Soumyajit DasEmail author
  • Satvinder Singh Bakshi
  • Ramesh Seepana
Review Article



Autoimmune inner disease (AIED) is an uncommon cause of sensorineural hearing loss and poses a diagnostic challenge. The present study aims to review the existing knowledge on the clinicopathological aspects, the diagnostic challenges, and therapeutic interventions in AIED.


The incidence of AIED is less than five cases per 100,000 population. There are no definite seromarkers which make diagnosis of AIED difficult. Even though various markers have been studied, their sensitivity and specificity have not been replicated in the clinical scenario. The treatment of the condition is also an enigma. Corticosteroids are the drug of choice and require long-term use to prevent relapse. Various other therapeutic agents have been studied in a small cohort of patients, but the efficacy of these drugs needs to be validated in a large multicentric trial.


Timely intervention can restore hearing loss in AIED patients, but the clinician has to find a delicate balance between the hearing outcome and the potential side effects resulting from long-term use of the drugs. Treatment of steroid resistant AIED is a challenge and there are no universal guidelines for the same. AIED being an uncommon diagnosis, multicentric trials and collaboration are required to formulate diagnostic criteria and therapeutic guidelines.


Inner ear Autoimmunity Hearing loss Sensorineural Diagnosis Inner ear antigens Circulating antibodies Positron emission tomography Magnetic resonance imaging 




Compliance with ethical standards

Conflict of interest

The author declares that they have no competing interests.

Research involving Human Participants and/or Animals

No human participant and/or animals were used in the study.

Informed consent

Not taken as no human participants were used in the study.


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© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of ENTAll India Institute of Medical SciencesMangalagiriIndia

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