Affiliative behavior in Williams syndrome: social perception and real-life social behavior.

A frequently noted but largely anecdotal behavioral observation in Williams syndrome (WS) is an increased tendency to approach strangers, yet the basis for this behavior remains unknown. We examined the relationship between affect identification ability and affiliative behavior in participants with WS relative to a neurotypical comparison group. We quantified social behavior from self-judgments of approachability for faces, and from parent/other evaluations of real life. Relative to typical individuals, participants with WS were perceived as more sociable by others, exhibited perceptual deficits in affect identification, and judged faces of strangers as more approachable. In WS, high self-rated willingness to approach strangers was correlated with poor affect identification ability, suggesting that these two findings may be causally related. We suggest that the real-life hypersociability in WS may arise at least in part from abnormal perceptual processing of other people's faces, rather than from an overall bias at the level of behavior. While this did not achieve statistical significance, it provides preliminary evidence to suggest that impaired social-perceptual ability may play a role in increased approachability in WS.

Cross-modal influences of affect across social and non-social domains in individuals with Williams syndrome.

The Williams syndrome (WS) cognitive profile is characterized by relative strengths in face processing, an attentional bias towards social stimuli, and an increased affinity and emotional reactivity to music. An audio-visual integration study examined the effects of auditory emotion on visual (social/non-social) affect identification in individuals with WS and typically developing (TD) and developmentally delayed (DD) controls. The social bias in WS was hypothesized to manifest as an increased ability to process social than non-social affect, and a reduced auditory influence in social contexts. The control groups were hypothesized to perform similarly across conditions. The results showed that while participants with WS exhibited indistinguishable performance to TD controls in identifying facial affect, DD controls performed significantly more poorly. The TD group outperformed the WS and DD groups in identifying non-social affect. The results suggest that emotionally evocative music facilitated the ability of participants with WS to process emotional facial expressions. These surprisingly strong facial-processing skills in individuals with WS may have been due to the effects of combining social and music stimuli and to a reduction in anxiety due to the music in particular. Several directions for future research are suggested.

Atypical hemispheric asymmetry in the perception of negative human vocalizations in individuals with Williams syndrome.

Williams syndrome is a neurological condition associated with high levels of auditory reactivity and emotional expression combined with impaired perception of prosody. Yet, little is currently known about the neural organization of affective auditory processing in individuals with this disorder. The current study examines auditory emotion processing in individuals with Williams syndrome. Hemispheric organization for positive and negative human non-linguistic sound processing was compared in participants with and without the disorder using a dichotic listening paradigm. While controls exhibited an expected right cerebral hemisphere advantage for processing negative sounds, those with Williams syndrome showed the opposite pattern. No differences between the groups emerged for the positive stimuli. The results suggest aberrant processing of negative auditory information in Williams syndrome.

The relationship between form and function level receptive prosodic abilities in autism.

Prosody can be conceived as having form (auditory-perceptual characteristics) and function (pragmatic/linguistic meaning). No known studies have examined the relationship between form- and function-level prosodic skills in relation to the effects of stimulus length and/or complexity upon such abilities in autism. Research in this area is both insubstantial and inconclusive. Children with autism and controls completed the receptive tasks of the Profiling Elements of Prosodic Systems in Children (PEPS-C) test, which examines both form- and function-level skills, and a sentence-level task assessing the understanding of intonation. While children with autism were unimpaired in both form and function tasks at the single-word level, they showed significantly poorer performance in the corresponding sentence-level tasks than controls. Implications for future research are discussed.

Enhanced perceptual processing of speech in autism.

Theories of autism have proposed that a bias towards low-level perceptual information, or a featural/surface-biased information-processing style, may compromise higher-level language processing in such individuals. Two experiments, utilizing linguistic stimuli with competing low-level/perceptual and high-level/semantic information, tested processing biases in children with autism and matched controls. Whereas children with autism exhibited superior perceptual processing of speech relative to controls, and showed no evidence of either a perceptual or semantic processing bias, controls showed a tendency to process speech semantically. The data provide partial support to the perceptual theories of autism. It is additionally proposed that the pattern of results may reflect different patterns of attentional focusing towards single or multiple stimulus cues in speech between children with autism and controls.

Defining the social phenotype in Williams syndrome: a model for linking gene, the brain, and behavior.

Research into phenotype-genotype correlations in neurodevelopmental disorders has greatly elucidated the contribution of genetic and neurobiological factors to variations in typical and atypical development. Etiologically relatively homogeneous disorders, such as Williams syndrome (WS), provide unique opportunities for elucidating gene-brain-behavior relationships. WS is a neurogenetic disorder caused by a hemizygous deletion of approximately 25 genes on chromosome 7q11.23. This results in a cascade of physical, cognitive-behavioral, affective, and neurobiological aberrations. WS is associated with a markedly uneven neurocognitive profile, and the mature state cognitive profile of WS is relatively well developed. Although anecdotally, individuals with WS have been frequently described as unusually friendly and sociable, personality remains a considerably less well studied area. This paper investigates genetic influences, cognitive-behavioral characteristics, aberrations in brain structure and function, and environmental and biological variables that influence the social outcomes of individuals with WS. We bring together a series of findings across multiple levels of scientific enquiry to examine the social phenotype in WS, reflecting the journey from gene to the brain to behavior. Understanding the complex multilevel scientific perspective in WS has implications for understanding typical social development by identifying important developmental events and markers, as well as helping to define the boundaries of psychopathology.

Is the linguistic content of speech less salient than its perceptual features in autism?

Open-ended tasks are rarely used to investigate cognition in autism. No known studies have directly examined whether increased attention to the perceptual level of speech in autism might contribute to a reduced tendency to process language meaningfully. The present study investigated linguistic versus perceptual speech processing preferences. Children with autism and controls were tested on a quasi-open-format paradigm, in which speech stimuli contained competing linguistic and perceptual information, and could be processed at either level. Relative to controls, children with autism exhibited superior perceptual processing of speech. However, whilst their tendency to preferentially process linguistic rather than perceptual information was weaker than that of controls, it was nevertheless their primary processing mode. Implications for language acquisition in autism are discussed.

Evidence for reduced domain-specificity in auditory processing in autism.

Neurological and behavioral findings indicate that atypical auditory processing characterizes autism. The present study tested the hypothesis that auditory processing is less domain-specific in autism than in typical development. Participants with autism and controls completed a pitch sequence discrimination task in which same/different judgments of music and/or speech stimulus pairs were made. A signal detection analysis showed no difference in pitch sensitivity across conditions in the autism group, while controls exhibited significantly poorer performance in conditions incorporating speech. The results are largely consistent with perceptual theories of autism, which propose that a processing bias towards featural/low-level information characterizes the disorder, as well as supporting the notion that such individuals exhibit selective attention to a limited number of simultaneously presented cues.