Nonsyphilitic Interstitial Keratitis and Bilateral Deafness (Cogan's Syndrome) Associated with Cardiovascular Disease

Nonsyphilitic Interstitial Keratitis With Vestibuloauditory Symptoms

“…Although approximately 25 clinical cases have been reported in the past 20 years the cause of the disease is still unknown.1 Most of the cases have subsided leaving permanent deafness and variable, but usually mild, corneal scarring. Histologic studies including the three previous autopsies [2][3][4] have shown systemic vascular disease sometimes interpreted as polyarteritis.…”

Isolation of Chlamydia psittaci from a patient with interstitial keratitis and uveitis associated with otological and cardiovascular lesions.

“…The chronic fluctuating stromal keratitis and vascularisation of the cornea associated with deafness and cardiovascular abnormalities are highly suggestive of Cogan's syndrome (Eisentein and Taubenhass, 1958;Duke-Elder, 1965;Seel, 1972), though it is difficult to exclude the diagnosis of Reiter's disease because of the associated sacroiliac arthritis. The persistently raised levels of the ESR and IgM in the blood indicated that an infection might be responsible for her various signs.…”

“…The results of these reports indicate that C. psittaci potentially though infrequently is a pathogen for the cardiovascular system and may cause myocarditis as well as valvular lesions (Eisentein and Taubenhass, 1958;Gelfand et al, 1972).…”

Cogan’s Syndrome

“…Aortitis may occur soon after disease onset or years later, and be associated with aortic valve regurgitation ( Fig. 2) (Eisenstein and Taubenhaus 1958;Fisher and Hellstrom 1961;Grasland et al 2004;Gluth et al 2006). Arteritis of the coronary arteries and premature coronary artery disease may occur, and rarely, central nervous system vasculitis (Albayram et al 2001).…”