title: Microangiopathy of the inner ear, deafness, and cochlear implantation in a patient with Susac syndrome.
abstract: CONCLUSIONS
  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.
authors: Department of Otolaryngology, University of São Paulo School of Medicine, Brazil. alinebittencourt@hotmail.com
authors: Aline Gomes
authors: AG
authors: Bittencourt
authors: Andrea Felice Dos
authors: AF
authors: Santos
authors: Maria Valeria Schmidt
authors: MV
authors: Goffi-Gomez
authors: Kellen
authors: K
authors: Kutscher
authors: Robinson Koji
authors: RK
authors: Tsuji
authors: Rubens De
authors: RD
authors: Brito
authors: Ricardo Ferreira
authors: RF
authors: Bento
mesh_terms: D019929:Cochlear Implantation; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000601:surgery; D006320:Hearing Tests; D006801:Humans; D055955:Susac Syndrome / Q000150:complications*; D055815:Young Adult