A new research project explores the mysterious cognitive disparities of Williams Syndrome

With a grant of $59,208 from the March of Dimes Foundation and a three-year grant of $220,523 from the National Science Foundation, University of Delaware psychology professor Barbara Landau, PhD, is forging a cognitive profile of the rare and puzzling genetic disorder, Williams Syndrome.

Her work is the first to scrutinize the way Williams Syndrome affects children?s verbal descriptions of spatial phenomena. children with the disorder are warm and gregarious, often with strong verbal and musical skills.

But due to a genetic defect, they have severe spatial deficits, one of the main reasons why they score in the retarded range on standardized tests.

With fellow psychologist, James Hoffman, PhD, research assistants Andrea Zukowski and Jerome Pagani and other students, Landau is probing their language and spatial development, exploring the way they talk about space, depth and shapes to clarify the relationship between their spatial abilities and language learning.

For example, Landau?s preliminary studies with eight Williams Syndrome children reveal that the children have a hard time explaining whether a dot is above or below a square, indicating that their verbal skills falter when it comes to describing spatial relations.

'It?s generally thought that as a child you have nonlinguistic representations of objects, events and scenes and then, when you learn language, you map it onto pre-existing representations,' says Landau. 'But with Williams kids, that doesn?t seem to happen. Since their pre-existing spatial representations seem to be impaired, our work is exploring their learning and use of spatial language. Do they have problems describing visual representations because their spatial language is impaired? Or is it because their system of spatial representation works differently?'

To test those questions, Landau plans to conduct a series of experiments on their spatial cognition, including tracking the eye movements of 20 Williams Syndrome children and adults as they perform several tasks?including a computerized block task. Then she?ll compare their performance with that of normal participants.

In addition to shedding light on Williams Syndrome?s effects on spatial language, Landau hopes the research will reveal whether Williams Syndrome spares some aspects of children?s spatial function or whether the deficiencies are pervasive.

?B. Murray