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Williams syndrome (WS) is a genetic disorder associated with severe visuocognitive impairment. Individuals with WS also report difficulties with everyday wayfinding. To study the development of body-, environment-, and object-based spatial frames of reference in WS, we tested 45 children and adults with WS on a search task in which the participant and a spatial array are moved with respect to each other. Although individuals with WS showed a marked delay, like young controls they demonstrated independent, additive use of body- and environment-based frames of reference. Crucially, object-based (intrinsic) representations based on local landmarks within the array were only marginally used even by adults with WS, whereas in typical development these emerge at 5 years. Deficits in landmark use are consistent with wayfinding difficulties in WS, and may also contribute to problems with basic localization, since in typical development landmark-based representations supplement those based on the body and on self-motion. Difficulties with inhibition or mental rotation may be further components in the impaired ability to use the correct reference frame in WS.

Original publication

DOI

10.1111/j.1467-7687.2007.00662.x

Type

Journal article

Journal

Dev Sci

Publication Date

07/2008

Volume

11

Pages

583 - 595

Keywords

Adolescent, Adult, Child, Child Development, Child, Preschool, Female, Form Perception, Humans, Male, Motion Perception, Perceptual Disorders, Space Perception, Vision, Ocular, Visual Perception, Williams Syndrome