A case of atypical Cogan's syndrome with uncommon corneal findings.
OBJECTIVE
We report a case of atypical bilateral interstitial keratitis associated with Cogan's syndrome.


METHODS
A 28-year-old man presented with a 2-year history of recurrent bilateral keratitis. Bilateral hearing loss preceded the ocular symptoms by 2 years. The patient also complained of skin nodules, headache, back pain, and arthritis. Corneal finding were consistent with superior stromal keratitis with stromal neovascularization and lipid deposition in the stroma. The patient's audiogram revealed cochlear pathology compatible with Cogan's syndrome (sensorineural deafness).


RESULTS
The patient was treated with topical steroids but eventually required corneal transplantation in the right eye as a consequence of progressive loss of vision secondary to progressive lipid keratopathy. Visual acuity at the patient's most recent follow-up evaluation was 20/40.


CONCLUSIONS
This case represents an unusual type of interstitial keratitis associated with Cogan's disease. The absence of ocular symptoms at the time of initial ear involvement and the atypical presentation of the keratitis were responsible for the delay in diagnosis in this patient, resulting in hearing impairment.
Miserocchi
E
E
Ocular Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
Baltatzis
S
S
Foster
C S
CS
D000328:Adult; D001299:Audiometry; D016510:Corneal Neovascularization / Q000175:diagnosis* / Q000601:surgery; D003319:Corneal Stroma / Q000098:blood supply / Q000473:pathology; D003638:Deafness / Q000175:diagnosis*; D006312:Hearing Loss, Bilateral / Q000175:diagnosis*; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis*; D006801:Humans; D007634:Keratitis / Q000175:diagnosis* / Q000601:surgery; D015948:Keratoplasty, Penetrating; D008064:Lipidoses / Q000175:diagnosis; D008297:Male; D013577:Syndrome