A 43-year-old man presented for neurologic evaluation with acute bilateral hearing loss. Two weeks before presentation, he had bilateral eye pain diagnosed as scleritis. He also noted a subacute history of fevers, night sweats, and diarrhea. Neurologic examination revealed bilateral sensorineural hearing loss, confirmed with audiometry. MRI demonstrated enhancement of the cochlea bilaterally (figure). Extensive evaluations for rheumatologic and infectious etiologies were negative.
Figure Bilateral cochlear enhancement
Coronal T1-weighted brain MRI with contrast highlighting bilateral cochlear enhancement (A, white arrows). Coronal T1 image with contrast for comparison shows an unaffected patient demonstrating no enhancement (B).
Cogan syndrome is a rare systemic disease classified among the vasculitides characterized by audiovestibular and ocular involvement.1 The patient stabilized with oral prednisone. If refractory, treatment with steroid-sparing immunosuppressants or cochlear implants can be considered.1
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Disclosure: The authors report no disclosures.
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