williams syndrome: language development

In this task, children were asked to place one of several pictures into a scene based on the researchers instructions. Watch on What is Williams syndrome? with Williams-Beuren syndrome. Phonological memory refers to immediate memory for the sounds of language, usually measured by verbatim recall of nonsense words or syllables that follow the phonotactics rules of the language in which the child is being assessed. Participants were at ceiling on the rhyming measures. For the remaining children, full-scale IQ ranged from 40 89, with a mean of 54.5. 8600 Rockville Pike Williams syndrome is a neurodevelopmental genetic disorder characterized by a unique phenotype, including mild to moderate intellectual disability and an uneven neuropsychological profile of relative strengths and weaknesses. Language and Williams syndrome: How intact is intact? (2004), and Garayzabal Heinze and Cuetos Vega (2008) included measures of phonological awareness. Williams syndrome is a neurodevelopmental disorder characterized by hypersociability and unique neurocognitive abnormalities. This chapter begins with a brief description of Williams syndrome (WS). As such, it requires not only the ability to recognize words rapidly but also the ability to appropriately group words into grammatical units to provide the basis for reading with expression. The few studies of these topics involving individuals with Williams syndrome are described below. Davis BL, Velleman SL. Grammatical ability is more strongly correlated with verbal working memory ability for children with WS than for typically developing children (Mervis & John, 2010a). The chromosomal alteration usually occurs as a random event during the formation of reproductive cells (eggs or sperm) in a parent of an affected individual. Ongoing intervention emphasizing relational language, pragmatics, grammar, phonics, and reading comprehension will maximize the ability of children with WS to participate in and benefit from educational opportunities. Epub 2014 Jun 11. General Motor Control was most often most affected (85% severe), probably due to low muscle tone. Contact a health care provider if you have questions about your health. In: Alloway TP, Gathercole SE, editors. Forty seven participants scored above basal on the WORD. E, Bhatt AB, Pober BR. The authors argue that for all participants, the higher of the two reading standard scores was above the persons full-scale IQ, therefore exceeding expectations based on IQ. Metalinguistics refers to the ability to consciously manipulate components of language, whether phonemes, words, or sentences (syntactic structures). Grant J, Karmiloff-Smith A, Gathercole SA, Paterson S, Howlin P, Davies M, Udwin O. Phonological short-term memory and its relationship to language in Williams syndrome. Peregrine E, Rowe ML, Mervis CB. The Differential Ability Scales-II (DAS-II; Elliott, 2007) includes a supplemental subtest, Phonological Processing, normed for ages 5 12 years, that measures phonological awareness. Preparation of this manuscript was supported by grant # R37 HD29957 from the National Institute of Child Health and Human Development (C. B. Mervis, PI). In this method, children spend most of their time listening to other children read rather than practicing reading; good readers averaged 6 minutes a day of oral reading and poor readers averaged 2 minutes or less. The two groups differed significantly on all 10 scales (p < .001). Fourteen participants attained at least a basal score on the Neale; 13 of them plus 4 additional participants obtained at least a basal score on the WORD. Del Campo M, Antonell A, Magano LF, Munoz FJ, Flores R, Bayes M, Perez Jurado Mervis CB. Leyfer OT, Woodruff-Borden J, Klein-Tasman BP, Fricke JS, Mervis CB. (2004) included a measure of reading comprehension in their studies. Department of Psychological and Brain Sciences, 317 Life Sciences Building, University of Louisville, Louisville, KY 40292, 502-852-3604; Department of Psychological and Brain Sciences, 317 Life Sciences Building, University of Louisville, Louisville, KY 40292, 502-852-3604. Verbal Motor Production Assessment for Children. As a group, children with Williams syndrome evidence relative strengths in receptive concrete vocabulary and phonological processing, with mean level of performance in the low average range for the general population. (1996). The Williams syndrome group was matched to a group of typically developing children whose CA (7.9 years) matched the Williams syndrome groups mean age equivalent on the Spanish version of the PPVT (7.8 years). Findings from studies that have related the phonological awareness abilities of individuals with Williams syndrome to their reading abilities are discussed in the Reading section. Synthetic phonics programs, which involve teaching children to break a word into its constituent phonemes and then blend them together to form the word, have been most effective in teaching children in the general population to read. In striking contrast to their performance on concrete vocabulary measures, the performance of children with Williams syndrome on relational vocabulary measures is very low. Results indicated large and significant differences as a function of group for single word-reading, nonword reading, and reading comprehension. People with Williams syndrome (WS), a rare neurodevelopmental disorder that is caused by a deletion on the long arm of chromosome 7, often show an uneven cognitive profile with participants performing better on language and face recognition tasks, in contrast to visuospatial and number tasks. Symposium on Research in Child Language Disorders; Madison, WI. Vocabulary and comprehension instruction: Summary and implications of the National Reading Panel findings. 1993) and associated with atypical social and cognitive profiles.Perhaps the most noted, albeit relatively little systematically investigated aspect of the WS behavioral profile is "sociability," a hallmark feature described as . polymorphism found more frequently in parents of children with Williams syndrome. Language Development in Williams Syndrome | The Oxford Handbook of Sensory modulation problems, including difficulty with auditory filtering and hypersensitivity to sound, also are very common (John & Mervis, 2010). Mervis CB, Becerra AM. What does it mean if a disorder seems to run in my family? In: Abbeduto L, editor. Journal of Clinical and Experimental Neuropsychology. For example, when preschoolers with WS who had limited language were administered the Autism Diagnostic Observation Schedule-Generic (ADOS-G; Lord et al., 2000), 48% were classified by the ADOS-G algorithm as autism spectrum disorder or autism (Klein-Tasman et al., 2007). Volterra et al. Klein BP, Mervis CB. In the following paragraphs, the results of the studies are compared and contrasted. Early communication therapy is vital for getting children started in the right direction. 2007;13(1):3-15. doi: Federal government websites often end in .gov or .mil. HHS Vulnerability Disclosure, Help This point will need to be taught explicitly to most children with Williams syndrome. How are genetic conditions treated or managed? In a study of 6 prelinguistic children with WS followed longitudinally for varied periods of time (age range 939 months), Velleman, Currier, Caron, Curley, and Mervis (2006) also found delays in vocal development. Available from http://www.ncbi.nlm.nih.gov/books/NBK1249/. Nevertheless, as a group, children with this syndrome show a consistent pattern characterized by relative strengths in concrete vocabulary and phonological processing (language skills strongly related to single-word reading) and relative weaknesses in relational concepts, receptive grammar, verbal working memory, comprehension monitoring, and discourse (language skills strongly related to reading comprehension). Meyer-Lindenberg A, Mervis CB, Berman KF. This variability is apparent both as a function of type of ability and, within a particular ability, as a function of the individuals tested. Peregrine E, Rowe ML, Mervis CB. It then summarizes the findings from research on intellectual ability as measured by intelligence tests and considers research on early language acquisition, followed by findings from research on the language abilities of school-aged children and adolescents with WS. In: Pickering SJ, editor. Elevated calcium level in the blood. This therapy is critical but in addition, the child needs to receive language therapy at the same rate and intensity as he/she would have received it had feeding therapy not been needed. Williams Syndrome. syndrome. Language and communicative development in Williams In: Bishop D, Mogford K, editors. This pattern suggests that there is likely to be considerable variability among children with Williams syndrome in both single-word reading ability and reading comprehension ability. Meta-analysis results indicated that systematic phonics instruction is most effective if it is the first reading method to which a child is exposed. Department of Psychological and Brain Sciences, University of Louisville. All children could read at least a few of the real words, but 8 (18%) could not read any nonwords. Typical errors included using intonation rather than grammar to mark a question, using BE rather than DO (e.g., Is the bug want cookies?), or double-marking (e.g., Is the bug is hungry?). Age-associated memory changes in adults with Williams syndrome. Signaling noncomprehension of language: A comparison of Fragile X syndrome and Down syndrome. Given this pattern, it is not surprising that reading ability is also highly variable. Mervis CB, Morris CA, Klein-Tasman BP, Bertrand J, Kwitny S, Appelbaum LG, Rice CE. Most of the recent studies of the reading abilities of individuals with Williams syndrome have focused on single-word reading and factors affecting this ability. The participants mean T score was 30.25 [SD = 10.56, range: 10 (lowest possible) 48], indicating a mean level of performance at the 2nd percentile for the general population, with as much variability as for the general population (Mervis, unpublished data). In: Bishop D, Mogford K, editors. Nevertheless, as a group, children with Williams syndrome show a consistent pattern of relative strengths and weaknesses in broad language abilities. On this supplemental subtest, the child is instructed to repeat strings of numbers presented by the examiner in reverse order. Comprehensive Test of Phonological Processing. 3302 Accesses Part of the Autism and Child Psychopathology Series book series (ACPS) Abstract This chapter aims to provide a summary of the neurobiological perspective of language evolution as the central thread and to show how language, brain, and genetic studies are linked. (2004) argued that children with Williams syndrome seemed likely to benefit from the whole word approach. The descriptions provided are typically paradoxical; individuals with WS are often described as evidencing near-normal language abilities in the face of severe intellectual disability and as being extremely sociable at the same time as evidencing limited understanding of social norms (Mervis & John, 2010a, 2010b). One of the strongest findings to emerge from meta-analyses of studies of reading development is the importance of early, explicit, and systematic instruction in phonemic awareness and phonics for children in the general population. Mervis CB, John AE. Williams syndrome: MedlinePlus Genetics and the Williams syndrome (WS) is a rare genetic disorder characterized by heart disease, failure to thrive, hearing loss, intellectual or learning disability, speech and language delay, gregariousness, and non-social anxiety. Laing et al. tend to have a very social and engaging personality, statistical vocabulary learning-word-object associations, delay in the appearance of first words and language acquisition, higher frequency of hesitations and repetitions in speech, because individuals with Williams syndrome have a strong working memory, provide, Introduction to Language and Communication, https://williams-syndrome.org/what-is-ws/general-information, https://williams-syndrome.org/featured-education-resources, https://medlineplus.gov/genetics/condition/williams-syndrome/, https://www.ncbi.nlm.nih.gov/books/NBK1249/. American Journal of Medical Genetics Part C. John AE, Rowe ML, Mervis CB. In: Morris CA, Lenhoff HM, Wang PP, editors. Although language is typically considered a relative strength for children with WS, there is still a clear pattern of relative strengths and weaknesses within that domain. However, Robinson et al. [Google Scholar] Mervis CB, Bertrand J. Developmental relations between cognition and language: Evidence from Williams syndrome. Jrvinen-Parsley A, Bellugi U, Reilly J, Mills DL, Galaburda A, Reiss AL, Korenberg JR. Genes and Cognition. For the 11 readers, phoneme deletion and expressive vocabulary were significantly correlated with single-word reading, and phoneme identification and single-word reading were significantly correlated with nonword reading. Fluent reading is important for freeing working memory space for mental model construction, but it is not sufficient. 10.1016/j.jpeds.2016.08.027. Garayzabal Heinze & Cuetos Vega (2008) studied 12 Spanish children with Williams syndrome (mean CA 12. Once again, the majority of participants (82%) earned standard scores 70; 10% earned standard scores 100. Robinson et al. Mervis CB, John AE. Department of Psychological and Brain Sciences, University of Louisville; intellectual disability, language development, literacy, neurodevelopmental disorders, phonics, reading comprehension, Williams syndrome. Although the mean General Conceptual Ability (GCA; similar to IQ) is in the mild intellectual disability range (65, range: 31 96), the mean Verbal Cluster SS (74, range: 30 111) and the mean Nonverbal Reasoning Cluster SS (79, range: 37 118) are in the borderline to low average range as is mean performance on verbal short-term memory (72, range: 40 102). Comprehensive Test of Phonological Processing. This score is considerably lower than the mean of 69.32 (SD = 15.36) reported by Mervis and Becerra (2007) for 306 individuals with Williams syndrome. Factors beyond IQ that may have affected reading were typically not addressed. More recently, the WSPP has been shown to characterize >90% of an additional sample of 100 individuals with WS aged 5 20 years (Mervis & John, 2010b). 4;310(5749):802-4. doi: 10.1126/science.310.5749.802. 10.1038/s41572-021-00276-z. More recent research has confirmed that the grammatical abilities of children with WS are consistently above those of matched children with DS. Palacios-Verdu MG, Segura-Puimedon M, Borralleras C, Flores R, Del Campo M, Nevertheless, these areas are critical for both academic performance and peer relationships. Despite this lower-than-expected mean IQ, the participants in Levy et al. WS also is associated with specific cognitive and personality/behavioral phenotypes, as described below. Kamil ML. Careers, Unable to load your collection due to an error. At the group level, weaknesses for children with Williams syndrome include relational vocabulary, grammatical comprehension, verbal working memory, comprehension monitoring, and discourse-level processing. Performance on the Test of Relational Concepts (TRC: Edmonston & Litchfield-Thane, 1988), which measures receptive knowledge of spatial, quantitative, temporal, and dimensional concepts) averages ~30 points lower than performance on the PPVT (Mervis & John, 2008) for children with WS aged 5 7 years. Over the past two decades, WS has been featured in the public media on several occasions. Working memory and neurodevelopmental conditions. Williams-Beuren syndrome: Research, evaluation, and treatment. Most children start to acquire a rich vocabulary and pick up more words after mastering crawling or walking. Three children who have Williams syndrome, aged 3 years, 6 years, and 10 years. This pattern does not hold for children with WS; for most children with WS, the onset of referential language precedes the onset of referential pointing by several months (Mervis & Bertrand, 1997). The performance of the Williams syndrome and control groups on nonword reading was compared in three of the four studies, with contrasting findings. Distinctive personality characteristics of 8-, 9-, and 10-year-old children with Williams syndrome. Language abilities in Williams syndrome: A critical review In fact, for children with IQs < 100, predicted reading standard scores are well above IQ. International Professional Meeting of the Williams Syndrome Association; Richmond, VA. 2006. Genes: The gene expression approach. Losh et al. also used a paired associate procedure in which real words which varied in imageability were paired with consonant trigrams that varied in their phonetic similarity to the real word to address the importance of phonological and semantic processes in learning to read single words. Science. Four types of skills are assessed: rhyming, blending, deletion, and phoneme identification & word segmentation. The voice of evidence in reading research. As age increases, children with WS are more likely to be diagnosed with ADHD-Predominantly Inattentive and less likely to be diagnosed with ADHD-Combined. More extreme versions of this position were taken by several academicians who had not worked directly with individuals with Williams syndrome. Matisoff AJ, Olivieri L, Schwartz JM, Deutsch N. Risk assessment and Levy Y, Hermon S. Morphological abilities of Hebrew-speaking adolescents. Laing E, Hulme C, Grant J, Karmiloff-Smith A. Bellugi U, Wang P, Jernigan TL. Obesity or diabetes can develop in adulthood. Robinson, Mervis, and Robinson (2003) considered the performance of 39 children with Williams syndrome aged 4 16 years and found that variance in backward digit recall ability accounted for the largest amount of variance in receptive grammatical ability as measured by the TROG. Cognitive and behavioral characteristics of children with Williams syndrome: Implications for intervention approaches. Although there was a wide range of GCAs in each group, mean GCA was significantly higher for the Phonics group (67.42, range: 49 98) than for the Whole Word group (58.00, range: 39 80). The site is secure. Skin findings in Williams syndrome. Performance is measured by a T score (general population mean = 50, SD = 10). Most of the articles discussed in the previous two sections ended with brief statements regarding the implications of the reported research for reading instruction for children with Williams syndrome. Comprehension strategy instruction should begin at the same time as reading instruction begins, rather than being delayed until the child is reading single words well. Fraser J, Conti-Ramsden G. Contribution of phonological and broader language skills to literacy. Bethesda, MD 20894, Web Policies What is Williams syndrome? Catts HW, Adlof SM, Ellis Weismer S. Language deficits in poor comprehenders: A case for the simple view of reading. Seattle (WA): University of Washington, Language abilities in Williams syndrome: a critical review Prevalence of Williams syndrome is approximately 1 in every 25,000 live births ( Bellugi, Lichtenberger, Jones, Lai, & St. George, 2000 ). argued, based on clinical experience, that the DSM-IV criteria for GAD often do not capture the nature of worrying in children with WS. In: Bartke S, Siegmller J, editors. Four research groups have compared the performance of individuals with Williams syndrome to that of considerably younger typically developing children.

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