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

Ward, C., Chiat, S. ORCID: 0000-0002-8981-8153 and Townend, G. S. (2021). A comparison of formal and informal methods for assessing language and cognition in children with Rett syndrome.. Research in Developmental Disabilities, 114, 103961. doi: 10.1016/j.ridd.2021.103961


BACKGROUND: Opinions 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.

AIMS: To 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 PROCEDURES: Ten 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 RESULTS: Standard 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 IMPLICATIONS: Combining 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.

Publication Type: Article
Additional Information: © 2021 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY license DOI:
Publisher Keywords: Rett syndrome, Eye tracking, Cognitive assessment, Receptive and expressive language assessment, Augmentative and alternative communication, Neurodevelopmental disorder
Subjects: R Medicine > RJ Pediatrics > RJ101 Child Health. Child health services
T Technology
Departments: School of Health Sciences > Language & Communication Science
Date available in CRO: 08 Jun 2021 09:17
Date deposited: 8 June 2021
Date of acceptance: 20 April 2021
Date of first online publication: 27 April 2021
Text - Published Version
Available under License Creative Commons Attribution.

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