Role of cochlear implantation in susac’s syndrome

Abstract

Objectives: Susac’s syndrome is characterized by the triad of branched retinal artery occlusions, cochlear microangiopathy, and encephalopathy. The underlying process is thought to be a small vessel vasculitis causing microinfarcts in the retina, cochlea, and brain. The purpose of this report is to present the applicability of cochlear implantation in Susac’s syndrome with associated auditory neuropathy and encephalopathy. Methods: A clinical case review of a 39-year-old female with Susac’s syndrome is presented. The work-up and treatment is discussed together with the post-cochlear implant audiologic assessment. The patient developed bilateral profound sensorineural hearing loss (SNHL), encephalopathy, and retinopathy secondary to Susac’s syndrome. After the patient’s neurological symptoms stabilized, an otolaryngology consult was obtained. The preoperative audiologic evaluation demonstrated absent ABRs bilaterally and intact OAEs at 2 frequencies in one ear suggesting mixed cochlear damage with additional auditory neuropathy. Results: Despite the confounding features of residual cognitive deficit and auditory neuropathy, the patient’s hearing loss was managed successfully with cochlear implantation. After 6 months with her cochlear implant, the patient had markedly improved hearing in noise testing from 0% to 94% correct and word recognition from 0% to 76% at 60 dB. Conclusion: Susac’s syndrome is a rare disorder resulting in SNHL, blindness, and encephalopathy. Early angiography to identify branched retinal artery occlusion lends a clue to the diagnosis. Immunosuppressant therapy is necessary to minimize progression of the disease. Cochlear implantation is a viable option for those patients who develop profound sensorineural hearing loss, even with evidence of auditory neuropathy and limited cognitive deficits.