The overall objective of the proposed research is to delineate, and make sense of, the process of lexical development and its relations to socio-communicative development, grammatical development, and both general and specific aspects of nonlinguistic cognitive development. The research will focus on the language and cognitive development of children with Williams syndrome and children with Down syndrome. Williams syndrome is associated with relative strengths in language (especially concrete vocabulary and grammar) and verbal short-term memory and severe weakness in visuospatial cognition. In contrast, Down syndrome is associated with severe weaknesses in expressive language (especially grammar) and in verbal short-term memory and relative strength in nonlinguistic aspects of cognition. Because of these differences in the general nature of the relations between language and nonlinguistic cognition, inclusion of these two groups of children in the same studies provides a unique opportunity to distinguish factors that are likely to be universal and necessary to language development from those that are specific to particular rates or paths to language development. The proposed research consists of a longitudinal study of early language and cognitive development beginning in late infancy, a longitudinal study of cognitive and language development of school-age children as measured by standardized assessments, and cross-sectional studies of language development, especially socio-communicative development. Both observational and experimental methods will be used. There are three specific aims. The first is to delineate the different types of early lexical growth curves and examinetheir relations to later patterns of language and cognitive development. The second is to examine socio-communicative development and its role in language and cognitive development. The third is to determine the longitudinal trajectories of language and cognitive abilities of children with Williams syndrome and factors that influence these trajectories. The researchwill have implications both for theoretical models of the relations between language and cognitive development and for the design of cognitive, language, and reading intervention for children with developmental or intellectual disabilities.

Public Health Relevance

The results of the proposed research will provide a basis for recommendations regarding speech/language and developmental intervention for children with developmental delay in birth - 3 and preschool programs. The results also will increase the research foundation for the design of language, social skills, and reading intervention for school age children with developmental or intellectual disabilities.

National Institute of Health (NIH)
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Method to Extend Research in Time (MERIT) Award (R37)
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Special Emphasis Panel (NSS)
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Oster-Granite, Mary Lou
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University of Louisville
Schools of Arts and Sciences
United States
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Klein-Tasman, Bonita P; van der Fluit, Faye; Mervis, Carolyn B (2018) Autism Spectrum Symptomatology in Children with Williams Syndrome Who Have Phrase Speech or Fluent Language. J Autism Dev Disord 48:3037-3050
Gregory, Michael D; Kolachana, Bhaskar; Yao, Yin et al. (2018) A method for determining haploid and triploid genotypes and their association with vascular phenotypes in Williams syndrome and 7q11.23 duplication syndrome. BMC Med Genet 19:53
Esbensen, Anna J; Hooper, Stephen R; Fidler, Deborah et al. (2017) Outcome Measures for Clinical Trials in Down Syndrome. Am J Intellect Dev Disabil 122:247-281
Pérez-García, Débora; Brun-Gasca, Carme; Pérez-Jurado, Luis A et al. (2017) Behavioral Profiles of Children With Williams Syndrome From Spain and the United States: Cross-Cultural Similarities and Differences. Am J Intellect Dev Disabil 122:156-172
Pitts, C Holley; Mervis, Carolyn B (2016) Performance on the Kaufman Brief Intelligence Test-2 by Children With Williams Syndrome. Am J Intellect Dev Disabil 121:33-47
Pitts, C H; Klein-Tasman, B P; Osborne, J W et al. (2016) Predictors of specific phobia in children with Williams syndrome. J Intellect Disabil Res 60:1031-42
Cashon, Cara H; Ha, Oh-Ryeong; Graf Estes, Katharine et al. (2016) Infants with Williams syndrome detect statistical regularities in continuous speech. Cognition 154:165-168
Mervis, Carolyn B; Klein-Tasman, Bonita P; Huffman, Myra J et al. (2015) Children with 7q11.23 duplication syndrome: psychological characteristics. Am J Med Genet A 167:1436-50
Strong, Emma; Butcher, Darci T; Singhania, Rajat et al. (2015) Symmetrical Dose-Dependent DNA-Methylation Profiles in Children with Deletion or Duplication of 7q11.23. Am J Hum Genet 97:216-27
Morris, Colleen A; Mervis, Carolyn B; Paciorkowski, Alex P et al. (2015) 7q11.23 Duplication syndrome: Physical characteristics and natural history. Am J Med Genet A 167A:2916-35

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