A woman in her 20 s with a remote history of chronic iritis and bilateral sensorineural hearing loss presented with proptosis, eye pain, blurred vision and elevated intraocular pressures. Her medical history was significant for end-stage renal disease and congestive heart failure. Imaging was limited due to cochlear implants and a cardioverter defibrillator. Infectious and autoimmune investigations were negative. After initial management with topical pressure-lowering drops, she was started on systemic corticosteroids with rapid improvement in visual acuity and resolution of symptoms. The diagnosis of atypical Cogan syndrome was made by exclusion, based on her exam findings, ocular and auditory history, systemic features and positive clinical response to treatment. This case highlights the diagnostic challenges of atypical Cogan syndrome, particularly in the setting of delayed symptom onset and systemic involvement. Early recognition is essential, as timely corticosteroid therapy may prevent irreversible vision and hearing loss.
Amiri et al. (Fri,) studied this question.