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Visual Perception of Trustworthiness in Individuals with Williams Syndrome

Paper Type: Free Essay Subject: Medical
Wordcount: 2007 words Published: 13th Jul 2021

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Visual Perception in Infants and Toddlers with Williams Syndrome

“Visual Search in Typically Developing Toddlers and Toddlers with Fragile X or Williams Syndrome” (Scerif, Cornish, Wilding, Driver, & Karmiloff-Smither, 2004)

Scerif et al. (2004) defined visual selective attention as “the ability to attend to relevant information and ignore irrelevant stimuli.” The investigators established a seminal study in which they conducted two experiments that sought to examine the relationship between visual selective attention and perceptual salience in toddlers. The first experiment aimed to study the implications of manipulations of featural salience on the efficiency of visual search performances in both younger and older groups of typically developing toddlers. In the second experiment, the investigators conducted the same search performance task using toddlers with Fragile X Syndrome (FSX) and toddlers with Williams Syndrome (WS). In order to investigate this objective, all participants were tasked with touching a target while simultaneously ignoring a distractor. Despite conducting two separate experiments, the investigators conducted statistical Group analyses between older toddlers, younger toddlers, participants with FXS and participants with WS. Because a majority of individuals with WS present with visuoconstruction impairments and visuoperceptual deficits, it was predicted that the targets with WS would display issues regarding the visual-perceptual aspects of the visual search task. Additionally, the investigators hypothesized that both the WS and FXS participants would demonstrate repetitive errors in their search performances.

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The frequency of errors made by each participant was calculated and classified into three subgroups: repetitive touches, touches on distractors, and erroneous touches (Scerif et al., 2004). When compared to the typically developing toddlers, the toddlers with WS and FXS made more errors within their visual searches. However, Group statistical analyses revealed insignificant differences between the frequency of visual search errors made by FXS and WS participant groups. One important finding of this study was the distinction between the errors made by FXS and WS participants. The participants with FXS displayed a tendency to engage in repetitive touch whereas the participants with WS engaged with the distractors more than the typically developing participants and the participants with FXS. Collectively, the participants with WS made fewer correct touches than the typically developing toddlers but produced a similar amount responses as the participants with FXS. Additionally, the participants with WS displayed more erroneous errors on the visual search task and under “conditions of low target perceptual salience” (Scerif et al., 2004).

It is important to note that this study was one of the first to examine the visuoperception of toddlers with WS. Therefore, one must exert caution when comparing these results to previously published studies. Compared to the findings of studies including older children and adults with WS, the findings of the present study correspond in that the toddlers with WS displayed deficits in discrimination tasks that required sufficient visual attention. Conversely, the results of the second experiment contradict the findings of previously published studies because the present revealed insignificant discrepancies between the visuoperceptual of participants with WS and the typically developing participants. Furthermore, the findings of this study are significant because they have targeted a seminal population within individuals with WS and suggest a trend of visuoperceptual development. Future studies should consider comparing the visual search performance of individuals with WS across a wider sample of ages.

Visual Perception in Children with Williams Syndrome

“Just Another Face in the Crowd: Evidence for Decreased Detection of Angry Faces in Children with Williams Syndrome” (Santos, Silva, Rosset, & Deruelle, 2010)

Facial expressions are a key component of emotion recognition and provide valuable information that when interpreted appropriately, can be used to guide subsequent interactive behaviors. Individuals with Williams Syndrome (WS), a genetically based disorder characterized by hypersociability and reduced social fear, display tendencies of overusing social engagement devices and overlooking potential social threats (Santos et al., 2010). Previous studies have discerned discrepancies between the visual and cognitive processing of angry faces and happy faces. The “anger superiority effect,” coined by Hansen and Hansen (1998) denotes the increased accuracy and efficiency of processing angry faces in neurologically typical individuals. In considering the implications of the socio-behavioral phenotype of WS, the present study sought to investigate the presence or absence of the “anger superiority effect” in children with WS (Santos et al., 2010).

To investigate this objective, the investigators utilized two visual search tasks in which participants were asked to identify target faces, including angry faces and happy faces, within an array of neutral distractors. The participant sample was comprised of 21 children with WS between the ages of 10 and 17 and 21 typically developing (TD) children between the ages of five and fifteen (Santos et al., 2010). It is important to note that the investigators attempted to achieve experimental control by accounting for the cognitive discrepancies between the two sample groups and matching the TD participants to the participants with WS by both gender and mental age (MA). The participants were tasked with pressing either a green or key to identify the presence or absence of a happy face (Task 1) or an angry face (Task 2) within the facial array (Santos et al., 2010). All participant responses were recorded for both accuracy and latency of response time.

Both the accuracy and latency of responses were analyzed between the WS and TD group, the difference in task expectations, and the number of distractors included within the array. The results of Task 1 demonstrated that collectively, the TD participants detected the angry faces more efficiently than the participants with WS. Conversely, the results of Task 2 revealed no significant discrepancies between the WS and TD participants for Task 2. When compared to the TD participants, the participants with WS were found to detect angry faces in an atypical manner with decreased accuracy and therefore displayed an absence of the anger superiority effect. The findings of this study correspond with those of previously published studies in that children with developmental disabilities frequently present with deficits in socio-emotional processing due to a lack of cognitive resources. Furthermore, the findings of this study signify the importance of targeting the facial detection of angry faces for intervention in children with WS.

Visual Perception in Adults with Williams Syndrome

“Abnormalities in Early Visual Processes are Linked to Hypersociability and Atypical Evaluation of Facial Trustworthiness: An ERP Study with Williams Syndrome” (Shore, Ng, Bellugi, & Mills, 2017)

As evidenced by functional brain imaging, the evaluation of trustworthiness is thought to be automatically processed by specialized neural mechanisms of the human brain within the amygdala (Marzi, Righi, Ottonello, Cincotta, & Viggiano, 2012). These automatic evaluations have adaptive implications in one’s social behavior and emotions. Individuals with Williams Syndrome (WS) typically present with strengths in facial processing but atypical evaluations of potentially dangerous people or situations (Shore et al., 2017). Previously published studies have begun to consider potential connections between the hypersociality of individuals with WS and abnormalities within their visual perception processes. In accordance with these developments, the researchers sought to examine the social approach behavior of WS and typically developing (TD) participants by comparing the neural processing of trustworthiness evaluations made by both participant groups.

The participant sample was comprised of 20 participants with WS aged 19-56 and 21 TD participants aged 18-43. To analyze the neural mechanisms and processes utilized in evaluating the trustworthiness of people’s faces, each participant was asked to wear an EEG cap while determining whether they would talk to a person, based solely on a headshot of a person with a neutral expression. Each participant answered the target question for 100 faces and each photograph was displayed for 1,000 ms. It is important to note that prior to the start of this experiment, each headshot was coded on a 1-7 Likert scale for trustworthiness. Data were recorded to calculate the responses of approach (yes, the participant would talk to the stimulus person) and avoid (no, the participant would not talk to the stimulus person) and the responses were averaged and presented as proportions of approach, maybe, and avoid.

The results of this study demonstrated that the participants with WS agreed to approach the high-trust faces more frequently than the low-trust faces. A between-group analysis revealed no significant discrepancies in the response times but showed that the TD adults agreed to approach high-trust faces less frequently than the participants with WS.

Additionally, the participants with WS did not discriminate between the high- and low trust faces for no responses whereas the TD participants demonstrated the ability to discern between the faces. The atypical approach used by participants with WS to evaluate trustworthiness corresponds with the findings of previously published studies and the socio-behavioral phenotype of WS.

The results of the brainwave monitoring supported the researchers’ hypothesis in that participants with WS performed enhanced structural encoding for high-trust faces and TD participants did not differ between the types of faces (Shore et al., 2017).

As such, the TD participants exhibited an initial bias within toward low-trust faces that were witnessed both in their responses and brainwaves whereas the participants with WS solely discriminated in their responses.

These results are significant in discerning the explicit differences between how adults with WS evaluate trustworthiness compared to TD adults and demonstrating atypical visual-perceptual processing.


Hansen, C. H., & Hansen, R. D. (1988). Finding the face in the crowd: An anger superiority

effect. Journal of Personality and Social Psychology, 54(6), 917–924.

Marzi, T., Righi, S., Ottonello, S., Cincotta, M., & Viggiano, M. P. (2012). Trust at first sight:

Evidence from ERPs. Social Cognitive and Affective Neuroscience, 9, 63–72.

Santos, A., Silva, C., Rosset, D., & Deruelle, C. (2010). Just another face in the crowd: Evidence

for decreased detection of angry faces in children with Williams syndrome. Journal of Neuropsychologia, 48, 1070-1078.

Scerif, G., Cornish, K., Wilding, J., Driver, J., & Karmiloff-Smither, A., 2004). Visual search in

typically developing toddlers and toddlers with Fragile X or Williams syndrome. Developmental Science, 7(1), 116-130.

Shore, D. M., Rowena, N., Bellugi, U., & Mills, D.L. (2017). Abnormalities in early visual

processes are linked to hypersociability and atypical evaluation of facial trustworthiness: An ERP study with Williams syndrome. Cognitive, Affective, & Behavioral Neuroscience, 17, 1002-1017.



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