A case of atypical Cogan's syndrome with uncommon corneal findings

Purpose. We report a case of atypical bilateral interstitial keratitis asso ciated with Cogan's syndrome. Methods. A 28-year-old man presented with a 2 -year history of recurrent bilateral keratitis. Bilateral hearing loss prec eded the ocular symptoms by 2 years. The patient also complained of skin no dules, headache, back pain, and arthritis. Corneal finding were consistent with superior stromal keratitis with stromal neovascularization and lipid d eposition in the stroma. The patient's audiogram revealed cochlear patholog y compatible with Cogan's syndrome (sensorineural deafness). Results. The p atient was treated with topical steroids but eventually required corneal tr ansplantation in the right eye as a consequence of progressive loss of visi on secondary to progressive lipid keratopathy. Visual acuity at the patient 's most recent follow-up evaluation was 20/40. Conclusion. This case repres ents an unusual type of interstitial keratitis associated with Cogan's dise ase. The absence of ocular symptoms at the time of initial ear involvement and the atypical presentation of the keratitis were responsible for the del ay in diagnosis in this patient, resulting in hearing impairment.