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Cogan syndrome

Disease definition

Cogan syndrome (CS) is a rare autoimmune disorder of unknown origin characterized by inflammatory ocular disease (mainly interstitial keratitis) and vestibulo-auditory manifestations (mainly acute onset hearing loss, tinnitus and vertigo), in the setting of a negative work-up for syphilis, with a variable risk of developing into a systemic disease. Systemic manifestations may occur in more than 70% of cases.

ORPHA:1467

  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Childhood, Adolescent, Adult, Elderly
  • ICD-10: H16.3
  • OMIM: -
  • UMLS: -
  • MeSH: D055952
  • GARD: 1421
  • MedDRA: 10056667

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