The study replicates findings from a previous study with an international sample and confirms the importance of self-determination to enhance QOL. Subsequent research should examine the direction of the relationship between self-determination and QOL and examine the relationship of essential characteristics of self-determined behaviour and core domains of QOL in greater detail.

The aim of this study was to assess wayfinding abilities in Down syndrome (DS). The ability to learn routes though a virtual environment (VE) and to make a novel shortcut between two locations was assessed in individuals with DS (N=10) and control participants individually matched on mental age (MA) or chronological age (CA). The results showed that most of the participants with DS was able to learn routes through VE, even though they needed more trials than the CA controls to reach the learning criterion. However, they did not have flexible wayfinding behaviour since they were not able to find a shortcut between two known locations (unlike the CA controls). The results suggested that most individuals with DS could acquire knowledge about specific routes without being able to integrate that knowledge into a configurational understanding.

The ability to navigate new environments has a significant impact on the daily life and independence of people with learning difficulties. The aims of this study were to investigate the development of route learning in Down syndrome (N = 50), Williams syndrome (N = 19), and typically developing children between 5 and 11 years old (N = 108); to investigate use of landmarks; and to relate cognitive functions to route-learning ability in these groups. Overall, measures of attention and long-term memory were strongly associated with route learning, even once non-verbal ability was controlled for. All of the groups, including 5-to 6-year-old TD children, demonstrated the ability to make use of all landmark types to aid route learning; those near junctions, those further from junctions, and also distant landmarks (e.g. church spire, radio mast). Individuals with WS performed better than a matched subset of TD children on more difficult routes; we suggest that this is supported by relatively strong visual feature recognition in the disorder. Participants with DS who had relatively high levels of non-verbal ability performed at a similar level to TD participants. Research highlights• Individuals with Williams syndrome performed better than a matched subset of typically developing children on more difficult routes.• Measures of attention and long-term memory were strongly associated with route learning.

The ability to learn a route through a virtual environment was assessed in 19 older children and adults with Williams syndrome (WS) and 40 typically developing (TD) children aged 6-9 years. In addition to comparing route-learning ability across groups, we were interested in whether participants show an adult-like differentiation between "useful" and "less useful" landmarks when learning a route and the relative salience of landmark position versus landmark identity. Each virtual environment consisted of a brick wall maze with six junctions. There were 16 landmarks in the maze, half of which were on the correct path and half on incorrect paths. Results showed that both groups could learn each route to criterion (two successful completions of a route without error). During the learning phase, the WS group produced more errors than the TD group and took longer to reach criterion. This was predominantly due to the large number of perseverative errors (i.e., errors that were made at the same choice point on consecutive learning trials) made by the WS group relative to the TD children. We suggest that this reflects a difficulty in inhibiting erroneous responses in WS. During the test phase, the TD group showed stronger recall of landmarks adjacent to junctions (more useful landmarks) than of landmarks along path sections (less useful landmarks) independent of each individual's level of nonverbal ability. This pattern was also evident in the WS group but was related to level of nonverbal maturation; the differentiation between recall of junction and path landmarks increased as nonverbal ability increased across WS participants. Overall, the results demonstrate that individuals with WS can learn a route but that the development of this ability is atypical.

BackgroundIndividuals with Down syndrome (DS) and individuals with Williams syndrome (WS) have poor navigation skills, which impact their potential to become independent. Two aspects of navigation were investigated in these groups, using virtual environments (VE): route knowledge (the ability to learn the way from A to B by following a fixed sequence of turns) and configural knowledge (knowledge of the spatial relationships between places within an environment).MethodsTypically developing (TD) children aged 5 to 11 years (N = 93), individuals with DS (N = 29) and individuals with WS (N = 20) were presented with a sparse and a rich VE grid maze. Within each maze, participants were asked to learn a route from A to B and a route from A to C before being asked to find a novel shortcut from B to C.ResultsPerformance was broadly similar across sparse and rich mazes. The majority of participants were able to learn novel routes, with poorest performance in the DS group, but the ability to find a shortcut, our measure of configural knowledge, was limited for all three groups. That is, 59 % TD participants successfully found a shortcut, compared to 10 % participants with DS and 35 % participants with WS. Differences in the underlying mechanisms associated with route knowledge and configural knowledge and in the developmental trajectories of performance across groups were observed. Only the TD participants walked a shorter distance in the last shortcut trial compared to the first, indicative of increased configural knowledge across trials. The DS group often used an alternative strategy to get from B to C, summing the two taught routes together.ConclusionsOur findings demonstrate impaired configural knowledge in DS and in WS, with the strongest deficit in DS. This suggests that these groups rely on a rigid route knowledge based method for navigating and as a result are likely to get lost easily. Route knowledge was also impaired in both DS and WS groups and was related to different underlying processes across all three groups. These are discussed with reference to limitations in attention and/or visuo-spatial processing in the atypical groups.