The Language Profile of Preschool Children With 22q11.2 Deletion Syndrome and the Relationship With Speech Intelligibility
Everaert, Emma; Selten, Iris; Boerma, Tessel; Houben, Michiel; Vorstman, Jacob; de Wilde, Hester; Derksen, Desiree; Haverkamp, Sarah; Wijnen, Frank; Gerrits, Ellen
(2023) American journal of speech-language pathology, volume 32, issue 1, pp. 128 - 144
(Article)
Abstract
PURPOSE: Young children with 22q11.2 deletion syndrome (22q11DS) often have impaired language development and poor speech intelligibility. Here, we report a comprehensive overview of standardized language assessment in a relatively large sample of preschool-aged children with 22q11DS. We furthermore explored whether speech ability explained variability in language skills. METHOD: Forty-four
... read more
monolingual Dutch preschoolers (3-6 years) with a confirmed genetic 22q11DS diagnosis participated in this prospective cohort study. Standardized tests (Clinical Evaluation of Language Fundamentals Preschool-2-NL and Peabody Picture Vocabulary Test-III-NL) were administered. Speech intelligibility was rated by two expert speech and language therapists using a standardized procedure. RESULTS: Most children had impaired language skills across all tested domains. The composite score for expressive language was significantly lower than that for receptive language, but the two were strongly correlated. Only small differences between the mean scores on the various subtests were observed, with the lowest scores for expressive morphosyntactic skills. Language scores showed a moderate positive relation with speech intelligibility, but language abilities varied greatly among the children with intelligible speech. CONCLUSIONS: We show that the majority of preschool children with 22q11DS have a broad range of language problems. Other than the relatively larger impairment in expressive than in receptive language skills, our results do not show a clearly delineated language profile. As many of the children with intelligible speech still had below-average language scores, we highlight that language problems require a broad assessment and care in all young children with 22q11DS. Future research using spontaneous language and detailed speech analysis is recommended, to provide more in-depth understanding of children's language profile and the relationship between speech and language in 22q11DS.
show less
Download/Full Text
Keywords: Child, Preschool, Communication Disorders, DiGeorge Syndrome, Humans, Prospective Studies, Speech Disorders/diagnosis, Speech Intelligibility, Developmental and Educational Psychology, Speech and Hearing, Otorhinolaryngology, Linguistics and Language, Review, Journal Article
ISSN: 1058-0360
Publisher: American Speech-Language-Hearing Association (ASHA)
Note: Funding Information: This work was supported by the Netherlands Organization for Scientific Research (NWO) [project number 360-89-080]. We would like to thank all the children, parents, professionals, and schools who participated in this study for their cooperation. They would also like to thank Fenna Duijnkerke and Marieke Huls for their support with data collection. They also acknowledge the support of Stichting Steun 22Q11, Aebele Mink van der Molen, Sasja Duijff, Lara Heestermans, and Jelle Homans with participant recruitment. Publisher Copyright: © 2022 The Authors.
(Peer reviewed)