title: Specific language impairment in children with velocardiofacial syndrome: four case studies.
abstract: OBJECTIVE
  To describe specific language impairment in four children with velocardiofacial syndrome (VCFS).
  
  
  METHODS
  A descriptive, retrospective study of four cases.
  
  
  METHODS
  University Hospital Groningen, tertiary clinical care.
  
  
  METHODS
  Of 350 patients with cleft plate, 18 children were diagnosed with VCFS. Four children are described.
  
  
  METHODS
  In all children, cardiac and plastic surgery was carried out in the first year of life. Afterward, interventions consisted of hearing improvement, pharyngoplasty, and speech therapy.
  
  
  RESULTS
  Inadequate and uncharacteristic development of articulation and expressive language in four children with VCFS were observed. They differed from the majority in two ways: their nonverbal IQ was in the normal range, and their language skills were below expectations for their IQ.
  
  
  RESULTS
  Four of 18 patients with VCFS (22%) showed poor response to therapy and did not develop language in accordance with their normal learning abilities (nonverbal learning capacities and language comprehension). Persistent hypernasal resonance and severe articulation problems remained in all four children. In two children the expressive language profile was also not in agreement with the nonverbal profile: they produced only two- and three-word utterances at the age of 6.0 and 5.3 years. The other two children at the age of 6.8 and 6.4 years produced very long sentences, but they were unintelligible.
  
  
  CONCLUSIONS
  The speech and language impairment of the four children may be characterized as a phonological or verbal programming deficit syndrome and as such can be described as a specific language impairment in conjunction with VCFS.
authors.affiliation: Department of Otorhinolaryngology, University Hospital Groningen, Groningen, the Netherlands. s.m.goorhuis.brouwer@kno.azg.nl
authors.forename: Siena M
authors.initials: SM
authors.lastname: Goorhuis-Brouwer
authors.forename: Frederik G
authors.initials: FG
authors.lastname: Dikkers
authors.forename: Peter H
authors.initials: PH
authors.lastname: Robinson
authors.forename: Wilhelmina S
authors.initials: WS
authors.lastname: Kerstjens-Frederikse
mesh_terms: D002648:Child; D002675:Child, Preschool; D002971:Cleft Lip / Q000150:complications; D002972:Cleft Palate / Q000150:complications; D019066:Facies; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007805:Language Development Disorders / Q000209:etiology*; D008297:Male; D009633:Nonverbal Communication; D013064:Speech Disorders / Q000209:etiology; D013577:Syndrome; D014069:Tonsillitis / Q000150:complications; D014681:Velopharyngeal Insufficiency / Q000150:complications*