A 24-year-old man presents with bilateral progressive sensorineural hearing loss, episodic vertigo mimicking Meniere's disease, and ocular examination revealing bilateral interstitial keratitis with photophobia and eye pain. Systemic evaluation shows large vessel arteritis. The most likely diagnosis is:
- A Susac syndrome
- B Behcet disease
- C Cogan syndrome ✓
- D Autoimmune inner ear disease without systemic involvement
Correct answer: C. Cogan syndrome
Explanation
Cogan syndrome is defined by the combination of interstitial keratitis and vestibulocochlear dysfunction that closely mimics Meniere's disease, occurring typically in young adults. Up to a third of patients develop systemic vasculitis, which can involve large vessels and cause aortitis with aortic insufficiency. Susac syndrome features encephalopathy, retinal artery occlusion, and hearing loss without keratitis. Behcet disease causes uveitis rather than interstitial keratitis. Treatment is systemic corticosteroids.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.