Objectives Children and young people with intellectual disability (ID) face an extraordinary number of obstacles to accessing healthcare services. By their nature, clinical settings are highly sensory environments which can be adapted with adequate clinician understanding of patient need. For many people with ID associated with a rare genetic syndrome, stimulating environments can be aversive due to sensory processing differences (SPD) and increased rates of autism and anxiety (Edwards et al, 2022; Glod, Riby & Rodgers. 2019; Uljarevic et al. 2018). However, no profile of SPD among groups with ID currently exists and standardised sensory adaptations to clinical settings are limited. A systematic review was therefore conducted to profile existing literature on SPD among people with syndromic ID and suggest improvements to healthcare accessibility.

Methods Prior to review completion, methodology and analysis plans were registered and documented in a protocol (CRD42022369003). Search terms and inclusion criteria were developed based on a scoping search and with advice from The Cerebra Network for Neurodevelopmental Disorders. Included papers were original studies examining sensory processing in people of any age with one of 8 genetic syndromes: Fragile X, Down’s, Prader-Willi, William’s, Cornelia-de-Lange, Angelman, Smith-Magenis, or Wolf-Hisrchhorn. These groups were chosen based on their documented association with SPD or autism.

Results 71 papers were included from an initial pooled sample of 1,361. 42% of these included papers assessed SPD in William’s syndrome, 28% in Down’s syndrome, and 25% in Fragile X syndrome (table 1). A full sensory profile for these groups is presented in figure 1. Fragile X and William’s syndrome groups more commonly reported emotional and avoidance behaviours, whilst Down’s syndrome was associated with seeking behaviours and distraction. Some papers highlighted direct positive correlation between SPD and repetitive and challenging behaviours in William’s and Fragile X syndromes. Emotional and avoidance behaviours included crying, covering ears, and panicking, and challenging behaviours included self-injury, destruction of property, and aggression.

Conclusion In summary, the findings of this review highlight the variability of sensory phenotypes seen in syndromic ID. As the first systematic review on this topic, it highlights the need for care guidelines specific to people with SPD and the importance of assessing the individual sensory requirements of paediatric inpatients. There is further demand for a large-cohort longitudinal analysis allowing cross-group phenotypic comparisons and analysis of correlation between chronological age and SPD.

References

1. Edwards G, Jones C, Pearson E, Royston R, Oliver C, Tarver J, Crawford H, Shelley L, Waite J. (2022) Prevalence of anxiety symptomatology and diagnosis in syndromic intellectual disability: a systematic review and meta-analysis.

2. Glod M, Riby DM, Rodgers J. (2019) Short report: Relationships between sensory processing, repetitive behaviors, anxiety, and intolerance of uncertainty in autism spectrum disorder and Williams syndrome.

3. Uljarevic M, Labuschagne I, Bobin R, Atkinson A, Hocking DR. (2018) Brief Report: The Impact of Sensory Hypersensitivity and Intolerance of Uncertainty on Anxiety in Williams Syndrome.