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Autoimmune ear disease: clinical and diagnostic relevance in Cogan’s sydrome

Luigi Maiolino, Salvatore Cocuzza, Angelo Conti, Luisa Licciardello, Agostino Serra, Salvatore Gallina
  • Salvatore Cocuzza
    ENT Department, University of Catania, Italy
  • Angelo Conti
    ENT Department, University of Catania, Italy
  • Luisa Licciardello
    ENT Department, University of Catania, Italy
  • Agostino Serra
    ENT Department, University of Catania, Italy
  • Salvatore Gallina
    ENT Department, University of Palermo, Italy

Abstract

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan’s syndrome. Cogan’s syndrome is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis, vestibular and auditory dysfunction. Associations between Cogan’s syndrome and systemic vasculitis, as well as aortitis, also exist. We report a case of a young woman who presented audiological and systemic characteristics attributable to Cogan’s syndrome. In the description of the case we illustrate how the appearance and evolution of the disease presented.

Keywords

Autoimmune ear disorder; Cogan’s syndrome; Hearing loss.

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Submitted: 2016-08-11 11:03:14
Published: 2017-03-30 15:12:27
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Copyright (c) 2017 Luigi Maiolino, Salvatore Cocuzza, Angelo Conti, Luisa Licciardello, Agostino Serra, Salvatore Gallina

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