Williams Syndrome: UD Research Pinpoints Language And Learning Traits Of Those With The Disorder

November 06, 1998

People with Williams Syndrome, a genetic disorder affecting an estimated one among every 25,000 individuals, are frequently described as having extraordinary musical and verbal skills, despite a profound inability to conceptualize spatial information.

Yet, research at the University of Delaware--to be presented Nov. 6 in Boston, Mass.--shows that language use by children with Williams Syndrome may, in fact, be directly affected by their cognitive deficits related to spatial events.

And, studies of eye movement among Williams Syndrome children, scheduled for presentation Nov. 20 in Dallas, Texas, suggest that some of this spatial deficit may result, at least in part, from "their tendency to allocate attention to smaller regions of space than normal children, as well as their difficulties encoding an object's properties and location," says Barbara Landau, a professor of psychology and director of UD's Language and Cognition Laboratory.

The UD research, directed by Landau, in collaboration with research specialist Andrea L. Zukowski, Prof. James Hoffman and others, is shedding light on how Williams Syndrome affects the brain and cognitive development. Ultimately, she says, a better understanding of the disorder "may suggest strategies to help people with Williams Syndrome organize their world and learn more effectively, by emphasizing their strengths."

Individuals with Williams Syndrome might be unable to solve simple mathematical problems, and they can't draw a circle that is half red and half green. Surprisingly, however, some adults with the disorder have been known to memorize thousands of songs in many different languages. Understanding such "uneven cognitive profiles" is at the heart of Landau's research.

At the Boston University Conference on Language Development on Nov. 6, Zukowski will describe a study of spatial language skills among eight children with Williams Syndrome (ages 7 to 14), who were compared with 12 non-affected children (ages 4 to 7) and 12 adults. To learn how children with Williams Syndrome use "motion verbs" and other spatial language, Zukowski explains, subjects were asked to view 80 short, videotaped events. After each event, researchers asked, "What happened?"

When describing such events as a doll falling into a bowl, children with Williams Syndrome, like other subjects, correctly identified the moving object. But they were less likely to mention the bowl or other stable, "reference" object in the scenes--especially when the main object moved away from the reference object, Zukowski reports. Their overall use of verbs was "essentially uncompromised," Landau says, though some verb differences were observed among the subject groups.

Children with Williams Syndrome demonstrated the greatest difficulty using prepositions to describe the "path" of the moving object, Landau says. Specifically, they rarely used such words as "off," "through" or "away from," according to Zukowski.

Such language traits may be closely related to the way that children with Williams Syndrome process spatial information, Landau says.

"Children with Williams Syndrome don't seem to have much trouble describing a scene in which one object is moving toward another object--in other words, going "into" or "onto" the object," she reports. "But when the children look at scenes in which one object is moving away-"out of" or "off of" another object--then they often fail to mention the stable object, and they also fail to describe the path of the moving object."

The problem, she says, is that children with Williams Syndrome have difficulty grasping the spatial relationships between objects. "If something is "on top of" something, and the two objects are close together, that's easy," she notes. "But if they're far apart, the child may be less likely to be able to conceptualize it, less likely to store it in memory, and possibly even less likely to mention it."

Children with Williams Syndrome don't seem to have widespread, major, spatial vocabulary problems, she says. Where they do show clear difficulties, however, "these cases may be a consequence of their impaired, non-linguistic knowledge." On Nov. 20 during the Psychonomic Society's 39th annual meeting, Hoffman and Landau, with graduate student Barney (Jerome) Pagani, will discuss their investigations of eye movement among those with Williams Syndrome, a project funded by the March of Dimes and the National Science Foundation.

Using a special, eye-tracking device that uses a tiny camera hidden within the visor of a cap placed on subjects' heads, UD researchers studied children's eye movements as they attempted to solve a set of block construction problems. The subjects with Williams Syndrome "made many more errors than controls, particularly on complex problems composed of multi-part blocks," Landau reports. Eye recordings "suggest that at least part of their spatial deficit is due to their allocation of attention to single blocks, rather than to configurations."

The goal of the UD research, Landau says, is to pinpoint which aspects of language and learning are "uncompromised" among those with Williams Syndrome. Such knowledge might make it possible to improve educational opportunities for people who have the disorder.

University of Delaware

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