title: Outcome of cochlear implantation in children with congenital cytomegalovirus infection or GJB2 mutation.
abstract: CONCLUSIONS
  Outcomes following cochlear implantation in children with congenital cytomegalovirus (CMV) infection were almost equivalent to those of children with GJB2 mutation-related sensorineural hearing loss (SNHL). Although our patients with developmental disorder showed poor auditory performance and speech and language skills after cochlear implantation, SNHL with developmental disorder should not be a contraindication for the procedure.
  
  
  OBJECTIVE
  Congenital CMV infection accounts for approximately 20% of all cases of neonatal hearing loss, while the GJB2 mutation accounts for 30-50% of all cases of profound nonsyndromic hearing loss. Here, outcomes for auditory behavior and speech and language skills were compared in children with congenital CMV infection or GJB2 mutation who received cochlear implantation for profound SNHL.
  
  
  METHODS
  Five children with asymptomatic congenital CMV infection and seven children with GJB2 mutation-related SNHL, with and without developmental disorder, underwent cochlear implantation. Hearing level and speech and language development were evaluated post-implantation using IT-MAIS, MUSS, and S-S method.
  
  
  RESULTS
  The IT-MAIS and MUSS scores of the congenital CMV infection group and the GJB2 mutation group continued to increase for 4 years after implantation. The S-S method score in both groups gradually increased, although the scores for children with mental retardation were low.
authors.affiliation: Department of Otolaryngology, Fukushima Medical University, Fukushima, Japan.
authors.forename: Takamichi
authors.initials: T
authors.lastname: Matsui
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ogawa
authors.forename: Naoko
authors.initials: N
authors.lastname: Yamada
authors.forename: Yoko
authors.initials: Y
authors.lastname: Baba
authors.forename: Yukie
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Mika
authors.initials: M
authors.lastname: Nomoto
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Suzutani
authors.forename: Naoki
authors.initials: N
authors.lastname: Inoue
authors.forename: Koichi
authors.initials: K
authors.lastname: Omori
mesh_terms: D001299:Audiometry; D002675:Child, Preschool; D019929:Cochlear Implantation; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D003587:Cytomegalovirus / Q000235:genetics; D003586:Cytomegalovirus Infections / Q000150:complications / Q000151:congenital*; D004247:DNA / Q000235:genetics*; D004252:DNA Mutational Analysis; D005260:Female; D006309:Hearing / Q000502:physiology*; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000235:genetics* / Q000601:surgery; D006801:Humans; D008297:Male; D009154:Mutation; D011379:Prognosis; D013067:Speech Perception / Q000502:physiology
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26; D004247:DNA