Cognitive skills in children with Usher syndrome type 1 and cochlear implants

Cecilia Henricson*, Malin Wass, Björn Lidestam, Claes Möller, Björn Lyxell

*Corresponding author for this work

    Research output: Contribution to journalArticlepeer-review

    10 Citations (Scopus)

    Abstract

    Introduction: Usher syndrome is a genetic condition causing deaf-blindness and is one of the most common causes of syndromic deafness. Individuals with USH1 in Sweden born during the last 15 years have typically received cochlear implants (CI) as treatment for their congenital, profound hearing loss. Recent research in genetics indicates that the cause of deafness in individuals with Usher type 1 (USH1) could be beneficial for the outcome with cochlear implants (CI). This population has not previously been the focus of cognitive research. Objective: The present study aims to examine the phonological and lexical skills and working memory capacity (WMC) in children with USH1 and CI and to compare their performance with children with NH, children with hearing-impairment using hearing-aids and to children with non-USH1 deafness using CI. The participants were 7 children aged 7-16 years with USH1 and CI. Methods: The participants performed 10 sets of tasks measuring phonological and lexical skills and working memory capacity. Conclusions: The results indicate that children with USH1 and CI as a group in general have a similar level of performance on the cognitive tasks as children with hearing impairment and hearing aids. The group with USH1 and CI has a different performance profile on the tests of working memory, phonological skill and lexical skill than children with non-USH1 deafness using CI, on tasks of phonological working memory and phonological skill.

    Original languageEnglish
    Pages (from-to)1449-1457
    Number of pages9
    JournalInternational Journal of Pediatric Otorhinolaryngology
    Volume76
    Issue number10
    DOIs
    Publication statusPublished - Oct 2012

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