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Cogan's syndrome

E William St Clair, MD
Rex McCallum, MD
Section Editor
Eric L Matteson, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Cogan's syndrome (CS) is a chronic inflammatory disorder that most commonly affects young adults. Clinical hallmarks are interstitial keratitis (IK) and vestibuloauditory dysfunction [1]. Associations between CS and systemic vasculitis, as well as aortitis, also exist [2-4].

The pathology, pathogenesis, clinical manifestations, and treatment of CS will be presented here.


Limited autopsy data derived from temporal bone specimens of patients with Cogan's syndrome (CS) reveal a range of findings, including [5-7]:

Lymphocyte and plasma cell infiltration of the spiral ligament

Endolymphatic hydrops

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Literature review current through: Nov 2017. | This topic last updated: Aug 28, 2017.
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