Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant

Objective To report a fatal case of Susac syndrome in a congenitally deaf patient with a cochlear implant and a history of migraines, emphasizing the diagnostic challenges in patients with preexisting conditions. Patient A 33-year-old male with congenital hearing loss, a cochlear implant, and chronic migraines who presented with mild subacute auditory disturbance and headaches that later progressed to severe encephalopathy. Intervention Explantation of a non–magnetic resonance imaging (MRI) compatible cochlear implant followed by MRI, fundoscopy, and the administration of immunosuppressive medications. Main Outcome Measures Diagnosis was confirmed by characteristic MRI appearance and the presence of a hemi-retinal artery occlusion. Results After weeks of immunosuppressive treatment, the patient died of a global cerebral ischemic event of unknown origin. Conclusions For patients with preexisting sensorineural hearing loss and cochlear implants, Susac syndrome poses a diagnostic challenge. Auditory disturbances in the absence of cochlear implant failure should prompt further evaluation for visual disturbances and encephalopathy. MRI and fundoscopy should be performed to detect other features of the disease.