title: Cochlear implantation and congenital deafness:  perceptive and lexical results in 2 genetically pediatric identified population.
abstract: OBJECTIVE
  To evaluate the perceptive and linguistic results in 2 predefined genetic population of children with implants.
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  Otolaryngology department of a tertiary referral hospital.
  
  
  METHODS
  Among 336 children with prelingual deafness who underwent implantation in our department between 1997 and 2007, 85 with GJB2 gene-related (Cx) deafness and 30 patients with Waardenburg syndrome (WaardS) were included. Mean age at implantation was 4.7 years (range, 15 mo to 16.5 yr) in the Cx group and 4.8 years (range, 16 mo to 16 yr) in the WaardS group. The mean follow-up period was 6.5 years (range, 19 mo to 12 yr) in the Cx group and 7.1 years (range, 27 mo to 13.5 yr) in the WaardS group. More than 75% of the families in both groups scored 4 and 5 when using the Mary Pat Moeller rating (p > 0.05). A psychoneurological evaluation was performed in one third of the patients in both groups.
  
  
  METHODS
  Patients underwent linguistic and perceptive evaluations at 12, 24, 36, and 48 months: speech perception with closed-set and open-set words, speech production (Level 1 = vocalizations to Level 5 = complex sentences), and lexical comprehension with EVIP/GaelP (Peabody Picture Vocabulary Test) tests that ranged in 5 levels (-2 SD to +2 SD).
  
  
  RESULTS
  Score results for open-set words were 38.5%, 57.5%, 69%, and 75% in the Cx group and 30.5%, 59%, 67.5%, and 78% in the WaardS group (p > 0.05) at 12, 24, 36, and 48 months. The proportion of children in Levels 4 and 5 of speech production was 27%, 42%, 57.5%, and 58% in the Cx group and 23%, 33%, 55%, and 66% in the WaardS group (p > 0.05). Lexical result at Level -2 SD at 1 year was 90% in the Cx group and 85% in the WaardS group and that at 4 years was 70% in the Cx group and 65% in the WaardS group (p > 0.05).
  
  
  CONCLUSIONS
  Perceptive and linguistic evolutions for both populations were of good quality, but lexical evaluation showed residual language difficulties in both groups.
authors.affiliation: Service d'Otorhinolaryngologie et de Chirurgie Cervico-Faciale, Hôpital d'Enfants Armand-Trousseau, AP-HP, Paris, France.
authors.forename: Wissame
authors.initials: W
authors.lastname: El Bakkouri
authors.forename: Natalie
authors.initials: N
authors.lastname: Loundon
authors.forename: Briac
authors.initials: B
authors.lastname: Thierry
authors.forename: Jerome
authors.initials: J
authors.lastname: Nevoux
authors.forename: Sandrine
authors.initials: S
authors.lastname: Marlin
authors.forename: Isabelle
authors.initials: I
authors.lastname: Rouillon
authors.forename: Erea Noel
authors.initials: EN
authors.lastname: Garabédian
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D015331:Cohort Studies; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D003638:Deafness / Q000151:congenital* / Q000235:genetics / Q000601:surgery*; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006319:Hearing Loss, Sensorineural / Q000235:genetics / Q000601:surgery; D006801:Humans; D007223:Infant; D012189:Retrospective Studies; D013067:Speech Perception; D014849:Waardenburg Syndrome / Q000235:genetics
chemical_list: D017630:Connexins; C000607042:GJB2 protein, human; D000072259:Connexin 26