[Skip to Navigation]
Photo Essay
August 2004

Cogan Syndrome With Disfiguring Lipid Keratopathy

Arch Ophthalmol. 2004;122(8):1242-1243. doi:10.1001/archopht.122.8.1242

A 34-year-old healthy man had a history of chronic, recurrent ocularinflammation since childhood and a recently progressive, ring-shaped whiteningof his corneas (Figure 1). He hadan acute episode of vertigo as well as hearing loss and tinnitus at 31 yearsof age, with residual hearing impairment in the right ear. Slitlamp examinationrevealed a 360° deep corneal stromal vascularization with white perivascularexudates (Figure 1). Audiometryshowed marked sensorineural hearing loss in the right ear (Figure 1). Serum lipids and cholesterol level were normal, and resultsof a syphilis serological examination were negative. Cogan syndrome was diagnosed.A right penetrating keratoplasty was performed because of progressive visualdeterioration and darkening of vision. Histopathological examination revealeddeep stromal vascularization with chronic stromal inflammatory infiltrateas well as deposits of lipid-laden macrophages (Figure 1). The right corneal graft remains clear, and the patientawaits contralateral surgery.