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    • Children with congenital VI have been found to

    2018-10-25

    Children with congenital VI have been found to deviate from the typical trajectory of social development observed in typically-sighted children. Firstly, children with VI pass social cognitive Theory of Mind tests at a later age compared to typically-sighted children (Brambring and Asbrock, 2010; Peterson et al., 2000; Minter et al., 1998; Green et al., 2004; McAlpine and Moore, 1995). Secondly, a relatively large proportion of children with VI display behaviours that are commonly associated with autism, like stereotyped movement, echolalic speech, and lack of engagement with caregivers and peers (Preisler, 1991; Brown et al., 1997; Hobson and Bishop, 2003; Tadic et al., 2009). Difficulties in social processing persist in mid-childhood and potentially into adolescence. For instance, a study in a specialist secondary school indicated that 22% of students with VI met clinical criteria for ASD (Mukkades et al., 2007, also see Jure et al., 2016). Analyses of clinically referred samples suggest an even higher prevalence (Absoud et al., 2010; Williams et al., 2013; Rogers and Newhart-Larson, 2008). However, Hobson et al. (2010) provided some evidence of later ‘recovery’ from early symptomatology This potential change in the trajectory of social processing makes mid-childhood a particularly interesting buy Imatinib to investigate if qualitative differences in the processing of social stimuli can be detected. Another potential limitation of previous studies concerns the behavioural assessment methods. Standard assessments of social cognition and Theory of Mind for sighted children rely predominantly on visually presented materials, e.g. recognising facial expressions or inferring the mental state of actors or puppets. Some attempts have been made to adapt materials for children with VI (Brambring and Asbrock, 2010), but these assessments have not been sufficiently tested and standardised in a representative sample − probably due to the rarity of isolated VI disorders. Other studies rely on verbally presented materials (Pijnacker et al., 2012) that are potentially confounded by differences in language development in children with VI, such as linguistic delays and difficulties in semantic and pragmatic comprehension (Andersen et al., 1993; Mulford, 1988; Wakefield et al., 2006). Therefore, the current study used a more direct measurement of auditory social stimulus processing in an electrophysiological experiment, which was not dependent on vision or language ability or verbal working memory. We hypothesised that differences in the processing of social stimuli in children with VI would be apparent in responses to auditory social stimuli. The ‘subjects-own-name’ (SON) paradigm was considered particularly suitable as it assesses responses to a basic social stimulus in the auditory domain, ie. hearing one’s own name spoken. Own-name stimuli have been shown to be processed preferentially, automatically, and without conscious control in the typically-sighted child from as early as 5 months of age (Alexopoulos et al., 2012; Parise et al., 2010; Perrin et al., 2006; Pfister et al., 2012). Differences in processing of own-name stimuli, compared with typically developing children, have been associated with deficits in mentalizing ability in children with high-functioning autism (Cygan et al., 2014; Lombardo et al., 2009). In the event-related response, own-name stimuli consistently elicit an early negative deflection with a maximum over frontal channels followed by later positive deflection similar to the orienting-P3 (P3a) in typical adults (Tateuchi et al., 2012, 2015). Manipulations of presentation frequency indicate that the early SON-related negativity is distinct from the orienting P3 (Eichenlaub et al., 2012). Further, comparisons of responses to names of close family members indicate that the SON-related negativity is specific to the subject’s own name rather than personally familiar names in general (Tateuchi et al., 2015). Neural generators of SON-related processing are likely to involve a network of areas, including the right superior temporal sulcus (STS), medial prefrontal cortex (PFC), and inferior parietal sulcus (IPS) (Holeckova et al., 2006; Perrin et al., 2005; Kampe et al., 2003). Using the event-related response in the SON paradigm, we anticipated that there may be a reduction in amplitude of the SON-related response in children with VI similar to effects observed in other groups with suspected deficits in social processing (Cygan et al., 2014; Nadig et al., 2007).