title: The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.
abstract: Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Toranomon Hospital, Tokyo 105-8470, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Koyama
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Kashio
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Aki
authors.initials: A
authors.lastname: Sakata
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Tsutsumiuchi
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Yu
authors.initials: Y
authors.lastname: Matsumoto
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Shotaro
authors.initials: S
authors.lastname: Karino
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Akinobu
authors.initials: A
authors.lastname: Kakigi
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Shinichi
authors.initials: S
authors.lastname: Iwasaki
authors.affiliation: Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Yamasoba
mesh_terms: D001309:Auditory Threshold / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D006309:Hearing / Q000502:physiology*; D006801:Humans; D011182:Postoperative Care; D013060:Speech / Q000502:physiology; D016896:Treatment Outcome; D014849:Waardenburg Syndrome / Q000503:physiopathology* / Q000601:surgery