title: Cogan's syndrome: an autoimmune inner ear disease.
abstract: OBJECTIVE
  The objective of our study was to review our current knowledge of the aetiopathogenesis of Cogan's syndrome, including viral infection and autoimmunity, and to discuss disease pathogenesis with relevance to pharmacotherapy.
  
  
  METHODS
  Relevant publications on the aetiopathogenesis and pharmacotherapy of Cogan's syndrome from 1945 to 2012 were analysed.
  
  
  CONCLUSIONS
  Cogan's syndrome is a rare autoimmune vasculitis, and its pathogenesis is unknown. Infection, but primarily autoimmunity, may play contributing roles in the pathogenesis of this disease. It is characterised by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. Approximately 70% of patients have systemic disease, of which vasculitis is considered the pathological mechanism. The immunologic theory is based on the release of auto-antibodies against corneal, inner ear and endothelial antigens, and of anti-nuclear cytoplasmic auto-antibodies (ANCA). Corticosteroids are the first line of treatment, and multiple immunosuppressive drugs have been tried with varying degrees of success. Tumour necrosis factor (TNF)-alpha blockers are a category of immunosuppressive agents representing a recent novel therapeutic option in Cogan's syndrome.
authors: ENT Department, Policlinico Umberto I, University of Rome Sapienza, Italy. jaja1978@hotmail.it
authors: A
authors: A
authors: Greco
authors: A
authors: A
authors: Gallo
authors: M
authors: M
authors: Fusconi
authors: G
authors: G
authors: Magliulo
authors: R
authors: R
authors: Turchetta
authors: C
authors: C
authors: Marinelli
authors: G F
authors: GF
authors: Macri
authors: A
authors: A
authors: De Virgilio
authors: M
authors: M
authors: de Vincentiis
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases; D055952:Cogan Syndrome / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D007759:Labyrinth Diseases
chemical_list: D001323:Autoantibodies