Abstract

Visuospatial difficulties in Williams syndrome (WS) are well documented. Recently, research has shown that spatial difficulties in WS extend to large-scale space, particularly in coding space using an allocentric frame of reference. Typically developing (TD) children and adults predominantly rely on the use of a sequential egocentric strategy to navigate a large-scale route (retracing a sequence of left–right body turns). The aim of this study was to examine whether individuals with WS are able to employ a sequential egocentric strategy to guide learning and the retracing of a route. Forty-eight TD children, aged 5, 7, and 9 years and 18 participants with WS were examined on their ability to learn and retrace routes in two (6-turn) virtual environment mazes (with and without landmarks). The ability to successfully retrace a route following the removal of landmarks (use of sequential egocentric coding) was also examined. Although in line with TD 5-year-olds when learning a route with landmarks, individuals with WS showed significantly greater detriment when these landmarks were removed, relative to all TD groups. Moreover, the WS group made significantly more errors than all TD groups when learning a route that never contained landmarks. On a perceptual view-matching task, results revealed a high level of performance across groups, indicative of an ability to use this visual information to potentially aid navigation. These findings suggest that individuals with WS rely on landmarks to a greater extent than TD children, both for learning a route and for retracing a recently learned route. TD children, but not individuals with WS, were able to fall back on the use of a sequential egocentric strategy to navigate when landmarks were not present. Only TD children therefore coded sequential route information simultaneously with landmark information. The results are discussed in relation to known atypical cortical development and perceptual-matching abilities in WS.

Highlights

  • Williams syndrome (WS) is a neurodevelopmental disorder arising from a deletion of around 27 genes on chromosome 7q11.23 (Koehler et al, 2014)

  • No significant differences were found across Typically developing (TD) groups (p > 0.05 for all)

  • Individuals with WS demonstrate a reliance on visual landmarks for route-learning and way-finding, to a greater extent than TD children of comparable non-verbal ability

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Summary

Introduction

Williams syndrome (WS) is a neurodevelopmental disorder arising from a deletion of around 27 genes on chromosome 7q11.23 (Koehler et al, 2014). The use of an allocentric spatial frame of reference to guide navigation (coding spatial relationships between objects external to the self) is problematic for individuals with WS as evidenced on both on small- (Nardini et al, 2008; Bernardino et al, 2013) and large-scale tasks (Farran et al, 2010; Broadbent et al, 2014) It is currently unclear whether such spatial difficulties in WS are evident in the use of an egocentric frame of reference (coding spatial relationships between the self and environmental objects) during large-scale navigation. Some authors have reported the importance of environmental landmarks as navigational aids (e.g., Jansen-Osmann, 2002), while others have found little benefit of the presence of landmarks, when other strategies, such as recalling a sequence of left–right turns, are readily available to the navigator (e.g., Tlauka and Wilson, 1994)

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