Immune-Mediated Inner Ear Disease

Claudio Lunardi, Antonio Puccetti

Research output: Chapter in Book/Report/Conference proceedingChapter


Inner ear damage can be caused by viral infections, trauma, vascular damage, and immune mechanisms, and is responsible for sensorineural hearing loss, a syndrome characterized by rapid progression of hearing impairment that is frequently non-responsive to medical treatment. Immune-mediated inner ear disease can be of autoimmune origin either as an isolated process affecting only the ear, to be considered as an organ-specific autoimmune disease, or as part of a systemic autoimmune disorder in approximately 30% of cases. The incidence and prevalence of autoimmune inner ear disease is unknown due to the lack of reliable diagnostic tests and to the limited access to the site of autoimmune aggression. Since the rapid administration of steroids and/or other immunosuppressive agents may result in hearing preservation, a great effort is needed in order to find both clinical and immunological features able to identify these patients. Animal models of autoimmune inner ear disease may help in this effort to clarify the largely unknown pathophysiology of the disease and the response to therapy.

Original languageEnglish
Title of host publicationThe Autoimmune Diseases: Fifth Edition
PublisherElsevier Inc.
Number of pages12
ISBN (Print)9780123849298
Publication statusPublished - Dec 2013


  • Autoimmune inner ear disease
  • Cogan's syndrome
  • Inner ear antigens
  • Sensorineural hearing loss
  • Systemic autoimmune diseases

ASJC Scopus subject areas

  • Immunology and Microbiology(all)


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