Specific language impairment in children with velocardiofacial syndrome: four case studies.
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.
Goorhuis-Brouwer
Siena M
SM
Department of Otorhinolaryngology, University Hospital Groningen, Groningen, the Netherlands. s.m.goorhuis.brouwer@kno.azg.nl
Dikkers
Frederik G
FG
Robinson
Peter H
PH
Kerstjens-Frederikse
Wilhelmina S
WS
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*