» What are the Cognitive and Behavioural Characteristics of Cornelia de Lange Syndrome

Sensory Processing

Sensory processing is a term that describes how the brain takes in and manages input from all the senses. In sensory processing, the brain deals with information from the five traditional senses (touch, taste, smell, sound and sight) and two other senses which contribute to a person’s balance (movement and awareness of where their body parts are in space).

Difficulties in sensory processing can lead to hyposensitivity and hypersensitivity (141,142,143). Hyposensitivity occurs when an individual is under-sensitive to stimuli and has trouble processing information from the senses. Hypersensitivity occurs when an individual is over-sensitive to stimuli, for example, common sounds could be painful or overwhelming. Individuals with hypersensitivity usually have low sensory thresholds. This means that the sensory system is activated by very little sensory input.

Individuals with CdLS usually experience difficulties in sensory processing (145), regardless of their level of intellectual disability (146).  In addition to hypo- and hypersensitivity, there can be confusion or fixation on sensory stimuli. For example, gastrointestinal problems and other organ issues can lead to anxiety, mood disorders and self-injury. Individuals with CdLS who also have an autism spectrum disorder (ASD) may have low sensory thresholds (141) and defensive responses towards sensory stimuli (147). Hyper- and hyposensitivity and other sensory processing difficulties should be assessed, and support strategies should be implemented in individuals with CdLS throughout their lifespan (R50). Interventions should address individual’s sensory needs in order to enhance development and participation in daily living (146).

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