Cogan Syndrome

Synonyms

Nonsyphilitic interstitial keratitis

Definition

Rare multisystem disease that affects the cornea by causing stromal interstitial keratitis and the vestibuloauditory system by causing vertigo and deafness.

Etiology

An autoimmune disorder but the exact etiology is unknown, possible immune reaction to a common autoantigen in the cornea and middle ear (Kanski and Bowling 2011). The disease also shares some clinicopathologic features with polyarteritis nodosa (Skuta et al. 2011–2012).

Clinical Presentation

Usually affect young adults. The majority of the patients report an upper respiratory tract infection few weeks before the onset of ocular or vestibuloauditory symptoms (Snow and Wackym 2009). The most common eye finding is a chronic bilateral interstitial keratitis; it begins with faint paralimbal (peripheral) subepithelial infiltrate of the cornea, and the patient may complain of irritation, photophobia, redness, and decreased vision. Later those lesions may progress centrally...