A comparison of formal and informal methods for assessing language and cognition in children with Rett syndrome

Callie Ward,Shula Chiat,Gillian S Townend

doi:10.1016/j.ridd.2021.103961

Callie Ward, Shula Chiat + Show 1 more

Open Access

https://doi.org/10.1016/j.ridd.2021.103961

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Abstract

BackgroundOpinions about the cognitive and receptive language skills of people with Rett syndrome (RTT) range from severe intellectual impairment to near-normal development. Assessment is challenging because most are non-verbal, with no purposeful hand use. Clarkson et al. (2017) adapted the Mullen Scales of Early Learning for use with eye gaze technology (MSEL-A/ET) for people with RTT. AimsTo investigate and compare the performance of children with RTT on formal and newly-designed informal assessments of language and cognition using eye gaze/tracking technology. Methods and proceduresTen children with RTT aged 4:0–6:8 were assessed on the MSEL-A/ET for Visual Reception (VR) and Receptive Language (RL), and standard MSEL for Expressive Language (EL). Informal assessments of the same skills were embedded in activities such as reading and cake-decorating. Outcomes and resultsStandard scores on MSEL-A/ET VR and RL subtests ranged from ‘very low’ to ‘above average’. All children scored ‘very low’ on standard EL assessment. Informal assessments added information about EL, with children producing 1–3 word utterances and a range of communicative functions through an eye gaze device. Conclusions and implicationsCombining low-tech augmentative and alternative communication, eye gaze technology, informal activities and formal assessment, yields greater insight into children’s abilities. This is important in informing suitable support and education for the individual.

Highlights

Rett syndrome (RTT) is a rare neurodevelopmental disorder typified by near normal development in the first 6–18 months of life followed by a loss of acquired skills
Eye tracking technology in particular, offer an access method that can facilitate delivery of both formal and informal assessment tasks, as well as opening up opportunities to engage in learning and communication
This study demonstrates how assessments can be adapted for eye gaze access, and how assessments delivered using eye gaze and eye tracking technology can reveal a

Summary

Introduction

Rett syndrome (RTT) is a rare neurodevelopmental disorder typified by near normal development in the first 6–18 months of life followed by a loss of acquired skills. Disturbances in motor movement and planning, such as dystonia and dyspraxia (impaired control of voluntary movement, exacerbated by anxiety), affect all individuals with RTT, impacting communication and making accurate assessment challenging (Byiers & Symons, 2013; Djukic & McDermott, 2012). Conclusions and implications: Combining low-tech augmentative and alternative communication, eye gaze technology, informal activities and formal assessment, yields greater insight into chil dren’s abilities. This is important in informing suitable support and education for the individual.

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