title: Cochlear Implantation in Patients With CHARGE Syndrome.
abstract: OBJECTIVE
  To determine the optimal surgical approach for cochlear implantation (CI) preoperatively based on the spatial relation of a displaced facial nerve (FN) and middle ear structures and to analyze clinical outcomes of CHARGE syndrome.
  
  
  METHODS
  Facial nerve displacement and associated deviation of inner ear structures were analyzed in 13 patients (17 ears) with CHARGE syndrome who underwent CI. Surgical accessibility through the facial recess was assessed based on anatomical landmarks. Postoperative speech performance and associated clinical characteristics were analyzed.
  
  
  RESULTS
  The most consistently identified ear anomalies were semicircular canal aplasia (100%), ossicular anomaly (100%), and vestibular hypoplasia (88%). Facial nerve displacement was found in 77% of cases (anteroinferior: 47%, anterior: 24%, inferior: 6%). The width of available surgical space around facial recess was significantly greater in cases of facial recess approach (2.85 ± 0.9 mm) than those of alternative approach (0.12 ± 0.29 mm, P = .02). Postoperatively, 53% achieved better than category 4 on the categories of auditory perception (CAP) scale. The CAP category was significantly correlated with internal auditory canal diameter (P = .025) and did not differ according to the applied surgical approach.
  
  
  CONCLUSIONS
  Preoperative determination of surgical accessibility through facial recess would be useful for safe surgical approach, and successful hearing rehabilitation was achievable by applying appropriate surgical approaches.
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea Department of Otorhinolaryngology, Korea University College of Medicine, Korea University Ansan Hospital, Ansan, Korea.
authors.forename: Yoon Chan
authors.initials: YC
authors.lastname: Rah
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Ji Young
authors.initials: JY
authors.lastname: Lee
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Myung-Whan
authors.initials: MW
authors.lastname: Suh
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Moo Kyun
authors.initials: MK
authors.lastname: Park
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jun Ho
authors.initials: JH
authors.lastname: Lee
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea Department of Otorhinolaryngology, Sungkyunkwan University School of Medicine, Kangbuk Samsung Hospital, Seoul, Republic of Korea.
authors.forename: Sun O
authors.initials: SO
authors.lastname: Chang
authors.affiliation: Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea shaoh@snu.ac.kr.
authors.forename: Seung-Ha
authors.initials: SH
authors.lastname: Oh
mesh_terms: D058747:CHARGE Syndrome / Q000150:complications / Q000534:rehabilitation*; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000379:methods*; D003638:Deafness / Q000209:etiology / Q000534:rehabilitation*; D004429:Ear Ossicles / Q000002:abnormalities*; D007758:Ear, Inner / Q000002:abnormalities; D005154:Facial Nerve / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012665:Semicircular Canals / Q000002:abnormalities*; D014723:Vestibular Aqueduct / Q000002:abnormalities
keywords: CHARGE syndrome; anomaly; cochlear implantation; facial nerve; facial recess