Microangiopathy of the inner ear, deafness, and cochlear implantation in a patient with Susac syndrome.

CONCLUSION: The cochlear implant was beneficial as an attempt to restore hearing and improve communication abilities in this patient with profound sensorineural hearing loss secondary to Susac syndrome.

OBJECTIVE: To report the audiological outcomes of cochlear implantation (CI) in a young woman with Susac syndrome after a 6-month follow-up period. Susac syndrome is a rare disorder. It is clinically characterized by a typical triad of sensorineural deafness, encephalopathy, and visual defect, due to microangiopathy involving the brain, inner ear, and retina.

METHODS: This was a retrospective review of a case at a tertiary referral center. After diagnosis, the patient was evaluated by a multidisciplinary team and received a cochlear implant in her right ear.

RESULTS: The patient achieved 100% open-set sentence recognition in noise conditions and 92% monosyllable and 68% medial consonant recognition in quiet conditions after 6 months of implant use. She reported the use of the telephone 3 months after activation.