title: [Cochlear implant in patients with congenital malformation of inner ear].
abstract: OBJECTIVE
  To study surgical difficulty and key of the cochlear implant in patients with congenital malformation of inner ear.
  
  
  METHODS
  The cochlear implantations were performed in our department from Jan. 2001 to Apr. 2003 for 18 patients with the malformation of inner ear.
  
  
  RESULTS
  In this series, there were 11 cases of large vestibular aqueduct syndrome (LVAS), 3 cases of Waardenberg syndrome, 3 cases of Mondini malformation, and 1 case of Usher syndrome. All 18 patients accepted the Nucleus 24-channel cochlear implantations, including Nucleus straight electrode in 13 cases but Contour implantation in 5 cases of LVAS. During operations, leakage of perilymph but not cerebrospinal fluid (CSF) from the open of scala tympani occurred in 11 cases of LVAS, however, the electrode was inserted successfully. The abnormalities of round window occurred in one of 3 cases of Waardenberg syndrome and 3 cases of Mondini malformation, respectively.
  
  
  CONCLUSIONS
  The cochlear implant could be conducted successfully for the LVAS, and the postoperative effect was same as the ones for the deafness persons with normal development of inner ear. However, for the patients with Mondini syndrome and common cavity, it is important to accurately assess the extent of abnormalities in the inner ear and accompanied malformation before operation, and to evaluate the full extent of difficulties of the operation in order to minimize the risk of CSF leakage and meningitis.
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, PLA General Hospital, Beijing 100853, China. hdy301@263.net
authors.forename: Dong-yi
authors.initials: DY
authors.lastname: Han
authors.forename: Wen-ming
authors.initials: WM
authors.lastname: Wu
authors.forename: Xin
authors.initials: X
authors.lastname: Xi
authors.forename: De-liang
authors.initials: DL
authors.lastname: Huang
authors.forename: Wei-yan
authors.initials: WY
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D000328:Adult; D002558:Cerebrospinal Fluid Otorrhea / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities* / Q000601:surgery*; D019929:Cochlear Implantation / Q000009:adverse effects; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D013067:Speech Perception; D016896:Treatment Outcome; D014723:Vestibular Aqueduct / Q000002:abnormalities