Specific language impairment in children with velocardiofacial syndrome: four case studies.

OBJECTIVE

To describe specific language impairment in four children with velocardiofacial syndrome (VCFS).

DESIGN

A descriptive, retrospective study of four cases.

SETTING

University Hospital Groningen, tertiary clinical care.

PATIENTS: Of 350 patients with cleft plate, 18 children were diagnosed with VCFS. Four children are described.

INTERVENTIONS: 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.

MAIN OUTCOME: 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.