title: Cochlin in autoimmune inner ear disease: is the search for an inner ear autoantigen over?
abstract: BACKGROUND
  Autoimmune inner ear disease (AIED) is characterised by a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss over a period of weeks to months. It is an uncommon disease accounting for less than 1% of all cases of hearing impairment or dizziness. The diagnosis is often missed and this impacts on the prognosis as the condition responds well to steroids and immunosuppressants if recognised early.
  No useful specific test for autoimmunity affecting the inner ear exists.
  
  
  OBJECTIVE
  To gather evidence regarding cochlin in AIED.
  
  
  METHODS
  Systematic review of human studies and animal experimental studies on inner ear antigens was undertaken.
  
  
  METHODS
  We searched MEDLINE (1965-2012), and Pubmed for relevant studies. A combination of key words for inner ear, autoimmunity (autoimmune, immune mediated) and cochlin were used.
  
  
  RESULTS
  A number of antigens have been implicated in autoimmune inner ear disease. Cochlin is a major component of the extracellular matrix in the inner ear and a promising candidate. We present evidence in literature on the role of this protein in the pathogenesis of autoimmune inner ear disease.
authors.affiliation: Department of Otolaryngology, New Cross Hospital, Wolverhampton WV10 0QP, United Kingdom. Electronic address: paramita.baruah@nhs.net.
authors.forename: Paramita
authors.initials: P
authors.lastname: Baruah
mesh_terms: D000818:Animals; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D005109:Extracellular Matrix / Q000276:immunology; D016326:Extracellular Matrix Proteins / Q000276:immunology*; D006319:Hearing Loss, Sensorineural / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007759:Labyrinth Diseases / Q000188:drug therapy / Q000276:immunology*; D051379:Mice
chemical_list: D001324:Autoantigens; C110668:COCH protein, human; C491729:Coch protein, mouse; D016326:Extracellular Matrix Proteins; D007166:Immunosuppressive Agents
keywords: Autoimmune inner ear disease; Cochlin