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Cognitive skills in children with Usher syndrome type 1 and cochlear implants
Linköping University, The Swedish Institute for Disability Research. Linköping University, Department of Behavioural Sciences and Learning, Disability Research. Linköping University, Faculty of Arts and Sciences. (Forskargruppen kring dövblindhet, HEAD graduate school)
Linköping University, The Swedish Institute for Disability Research. Linköping University, Department of Behavioural Sciences and Learning. Linköping University, Faculty of Arts and Sciences. (Forskargruppen kring dövblindhet)
Örebro Universitetssjukhus, audiologiskt forskningscentrum, Örebro Universitet. (Forskargruppen kring dövblindhet)
Linköping University, Department of Behavioural Sciences and Learning. Linköping University, Faculty of Arts and Sciences.
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2012 (English)In: International Journal of Pediatric Otorhinolaryngology, ISSN 0165-5876, E-ISSN 1872-8464, Vol. 76, no 10, 1449-1457 p.Article in journal (Refereed) Published
Abstract [en]

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 indicate 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.

Place, publisher, year, edition, pages
2012. Vol. 76, no 10, 1449-1457 p.
National Category
Social Sciences Interdisciplinary Psychology
Identifiers
URN: urn:nbn:se:liu:diva-78068DOI: 10.1016/j.ijporl.2012.06.020ISI: 000310048800012OAI: oai:DiVA.org:liu-78068DiVA: diva2:531098
Available from: 2012-06-05 Created: 2012-06-05 Last updated: 2017-12-07
In thesis
1. Cognitive capacities and composite cognitive skills in individuals with Usher syndrome type 1 and 2
Open this publication in new window or tab >>Cognitive capacities and composite cognitive skills in individuals with Usher syndrome type 1 and 2
2015 (English)Doctoral thesis, comprehensive summary (Other academic)
Alternative title[sv]
Kognitiva förmågor och färdigheter hos personer med Usher syndrome typ 1 och 2
Abstract [en]

The present thesis belongs to the research area disability research and deal with specific aspects of cognition in individuals with Usher syndrome type 1 and 2. The subject has been investigated and is discussed within an interdisciplinary framework, though the theories applied and described are derived from the area of cognitive psychology. Usher syndrome is a rare genetic condition causing a combination of visual and hearing impairment: deafblindness. There is a congenital hearing loss that is profound in type 1 and moderate to severe in type 2. During mid-childhood symptoms of visual impairment, e.g. light sensitivity, emerge and a progressive loss of visual field follows as a result of the genetically caused eye disease Retinitis Pigmentosa. The syndrome has previously been well described with respect to the genetical and medical aspects, but there has been very little research with a cognitive perspective on the population. Studies 1 and 2 in the present thesis focused on children with Usher syndrome type 1 with cochlear implants and investigated phonological skills, lexical access, working memory and reading skill in the group. Studies 3 & 4 investigated the same cognitive abilities and theory of mind in adults with Usher syndrome type 2. In study 4 the performance on theory of mind in the adults with Usher syndrome type 2 was also compared to that of another group with genetically caused deafblindness: individuals with Alström syndrome.

The results were that both the children and adults with Usher syndrome had significantly poorer phonological processing than the control groups with normal hearing. There was a large variation on performance on lexical access, especially in the group of children, however several individuals performed at the same level as the control group. Reading skill was found to be at level with the control groups’. There was also great variation in performance on ToM, however the majority of individuals performed similar to the control group with normal hearing and vision. The present project has resulted in some new knowledge on cognitive performance in  individuals with Usher syndrome type 1 and type 2. Performance in the participants with Usher syndrome can to a large extent can be understood by application of the models developed in previous research on populations with hearing impairment or deafness for understanding the impact of hearing with a hearing aid or cochlear implant. However, individuals with Usher syndrome experience additional difficulties in accessing information due to the progressive visual loss and the impact this has on performance is still largely unknown. Hence, the present project would recommend that interventions and support would be designed specifically to each individuals’ needs, with consideration of both the visual impairment and the hearing impairment.

Abstract [sv]

Föreliggande avhandling tillhör ämnet handikappvetenskap och beskriver specifika kognitiva förmågor hos personer med Ushers syndrom typ 1 och 2. Avhandlingens ämne har undersökts utifrån ett tvärvetenskapligt perspektiv, även om de teorier som tillämpas och beskrivs huvudsakligen härrör inom området kognitiv psykologi. Ushers syndrom är en ovanlig genetisk åkomma som leder till kombinationen av syn- och hörselnedsättning: dövblindhet. Individer med typ 1 av syndromet har medfödd dövhet medan individer med typ 2 har en medfödd måttlig till grav hörselnedsättning. Någon gång i åldrarna 6-10 år börjar de första symptomen, till exempel nedsatt mörkerseende, på den genetiskt betingade progressiva synnedsättningen Retinitis Pigmentosa att framträda. Syndromet är väl beskrivet i forskningen med avseende på genetiska och medicinska aspekter, men det finns extremt lite tidigare forskning med kognitivt perspektiv om populationen. Studierna 1 och 2 i föreliggande avhandling fokuserade på barn med Ushers syndrom typ 1 och cochleaimplantat. Dessa studier undersökte fonologisk förmåga, lexikal access, arbetsminne och läsning i gruppen. Studie 3 undersökte samma kognitiva förmågor hos vuxna med typ 2 av syndromet. I studie 4 undersöktes även den sammansatta förmågan Theory of Mind hos de vuxna med typ 2 och deras prestation jämfördes både mot en kontroll grupp med normal hörsel och syn och en kontrollgrupp med annan typ av dövblindhet; Alström syndrom. Resultaten visade att både barnen och de vuxna med Ushers syndrom hade signifikant sämre fonologisk förmåga än kontrollgruppen med normal hörsel. Nivån på prestation varierade stort inom grupperna, särskilt mellan barnen med typ 1, och flera av individerna (barn och vuxna) presterade trots hörselnedsättningen på samma nivå som de normalhörande. Läsfärdigheten befanns vara i nivå med kontrollgrupperna. I den vuxna gruppen var det stor variation i prestation även på Theory of Mind, men de flesta av individerna presterade liknande som kontrollgruppen med normal hörsel och syn. Föreliggande projekt har resulterat i lite mer kunskap om kognitiva färdigheter hos individer med Ushers syndrom typ 1 och 2. De resultat som individerna med Ushers syndrome presterade kan till stor del förstås och tolkas genom tillämpning av teorier och modeller utvecklade för att den inverkan på kognitiva förmågor det har att ha nedsatt hörsel och höra med hjälp av hörselapparat eller cochleaimplantat. Dock tyder fynden i detta projekt även på att individer med Ushers syndrom på grund av den allvarliga synnedsättningen har ytterligare svårigheter att få tillgodogöra sig information, men i vilken utsträckning och på vilket sätt är ännu inte beskrivet. Utifrån fynden i föreliggande studie blev rekommendation att interventioner och stöd till personer med Ushers syndrom utformas specifikt till varje individ, med hänsyn taget både till hens grad av synnedsättning och hörselnedsättning.

Place, publisher, year, edition, pages
Linköping: Linköping University Electronic Press, 2015. 63 p.
Series
Linköping Studies in Arts and Science, ISSN 0282-9800 ; 651Studies from the Swedish Institute for Disability Research, ISSN 1650-1128 ; 72
Keyword
Usher syndrome, Cognition, Phonological skills, Lexical Access, Working memory, Reading, Theory of Mind, Deafblindness, Ushers syndrom, Kognition, Fonologisk förmåga, Lexikal access, Arbetsminne, Läsning, Theory of Mind, Dövblindhet
National Category
Psychology Social Sciences Interdisciplinary
Identifiers
urn:nbn:se:liu:diva-120114 (URN)10.3384/diss.diva-120114 (DOI)978-91-7685-999-5 (ISBN)
Public defence
2015-09-07, Key 1, Hus Key, Campus Valla, Linköpings Universitet, Linköping, 10:00 (Swedish)
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Supervisors
Available from: 2015-07-10 Created: 2015-07-09 Last updated: 2015-09-02Bibliographically approved

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Henricson, CeciliaLyxell, BjörnLidestam, BjörnWass, Malin

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International Journal of Pediatric Otorhinolaryngology
Social Sciences InterdisciplinaryPsychology

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