title: Cochlear implantation in children with internal ear malformations.
abstract: OBJECTIVE
  To evaluate surgical aspects and results of cochlear implantation in inner ear malformations.
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  Ear, nose, and throat department of a tertiary referral hospital.
  
  
  METHODS
  Out of 260 implanted children, 18 (6.9%) had inner ear malformations: complex cochleovestibular malformation (n = 11), common cavity (n = 1), and enlarged vestibular aqueduct (EVA) (n = 6). Deafness was progressive in 12 cases (G1) and congenital in 6 cases (G2). Genetics lead to diagnosis in 12 of 13 cases: PSD mutation (n = 11), Waardenburg syndrome (n = 1), negative (1). Mean age at implant was 7.8 years. Mean follow-up period was 48 months.
  
  
  METHODS
  Medical and surgical outcomes were reported. Closed (CSW) and open (OSW) set word perception and level of speech production were evaluated each year. The results were compared pre- and postoperatively and between the two groups.
  
  
  RESULTS
  Gusher at surgery was observed in 50% of cases, with a persistent leak in one case. No facial injury or infectious complications were observed. At 12 months, 83% of the population had achieved more than 75% recognition in CSW, versus 16% before implant (p = 0.001). After 2 years, 64% of patients had more than 50% recognition in OSW. Good oral language was seen in 76% at 2 years and 100% at 3 years, versus 55% before implant (respectively, p > 0.05 and p = 0.03). At 1 year after implant, 83% of the G1 and 20% of the G2 achieved more than 50% recognition in OSW (p = 0.02). After 24 months, 83% of G1 and 40% of G2 had more than 50% in OSW (p > 0.05). Before implant, 75% in G1 and 0% in G2 had good oral language (p = 0.01). At 1 year, 83% in G1 and 16% in G2 had good oral language (p = 0.02). At 2 years, 100% in G1 and 20% in G2 had good oral language (p = 0.02). One child in G1 had no improvement after implantation.
  
  
  CONCLUSIONS
  No major complication was seen. Perceptive and linguistic results were variable and depended on the type of the deafness. In progressive deafness, the perceptive and linguistic result are expected to be good. In congenital deafness, the results are more variable.
authors: ENT Department, Armand Trousseau Children's Hospital, Paris, France. natalie.loundon@trs.ap-hop-paris.fr
authors: N
authors: N
authors: Loundon
authors: I
authors: I
authors: Rouillon
authors: N
authors: N
authors: Munier
authors: S
authors: S
authors: Marlin
authors: G
authors: G
authors: Roger
authors: E N
authors: EN
authors: Garabedian
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D015331:Cohort Studies; D003638:Deafness / Q000151:congenital / Q000503:physiopathology / Q000601:surgery*; D018450:Disease Progression; D007758:Ear, Inner / Q000002:abnormalities*; D006801:Humans; D012189:Retrospective Studies; D013067:Speech Perception; D013068:Speech Production Measurement; D016896:Treatment Outcome