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- Henricson, Cecilia
(författare)
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Cognitive capacities and composite cognitive skills in individuals with Usher syndrome type 1 and 2
- 2015
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- 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.
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| 2. |
- Henricson, Cecilia, 1980-, et al.
(författare)
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Cognitive skills in children with Usher syndrome typ 1 and cochlear implants
- 2012
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Ingår i: International Journal of Pediatric Otorhinolaryngology. - Clare, Ireland : Elsevier. - 0165-5876 .- 1872-8464. ; 76:10, s. 1449-1457
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Tidskriftsartikel (refereegranskat)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.
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| 5. |
- Henricson, Cecilia, 1980-, et al.
(författare)
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Phonological skills and working memory in children with C1 and Usher typ 1
- 2012
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Konferensbidrag (refereegranskat)abstract
- Bakgrund: Ushers syndrom (USH) innebär hörselnedsättning/dövhet i kombination med Retinitis Pigmentosa och, i två av de tre kliniska typer som finns, ingen eller gradvis förlust av balansfunktion. Det är den vanligaste orsaken till dövblindhet och har varit i fokus i många studier med molekylära, fysiologiska och funktionella konsekvenser av de genetiska mutationerna. Det finns dock få studier med inriktning mot kognitiva aspekter. Föreliggande studie har undersökt kognition med tydlig koppling till hörsel och talspråk hos barn med Ushers syndrom typ 1 och cochleaimplantat.Metod: Sju barn i åldrarna 7.5-16 år, med bekräftad USH1-diagnos och CI, deltog i studien. Deltagarnas prestation på 10 test, riktade mot fonologiska och lexikala färdigheter samt arbetsminne, jämfördes mot tre kontrollgrupper: barn med normal hörsel, barn med hörselnedsättning och hörapparat, samt barn med icke-syndromal dövhet och cochleaimplantat.Resultat och slutsats: Liksom i tidigare studier av barn med CI når gruppen med USH1 och CI inte samma nivå avseende fonologiska och lexikala färdigheter, samt presterar signifikant lägre avseende fonologiskt arbetsminne än barn med normal hörsel. Resultaten från föreliggande studie visar dock att deltagarna med USH1 generellt har högre prestationsnivå än andra barn med CI, och presterar på en liknande nivå som barn med hörselnedsättning och hörapparat.
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| 6. |
- Henricson, Cecilia, 1980-, et al.
(författare)
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Phonological skills and working memory in children with CI and Usher type I
- 2011
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Konferensbidrag (refereegranskat)abstract
- Children with CI have a distinctive pattern of development of phonological skills (Wass, 2010). Previous research (Spencer & Tomblin, 2009; Wass et al., 2009) has shown that deaf children with CI perform on par with children with normal hearing on some cognitive tasks, whereas they have a significantly lower performance level than children with normal hearing on tasks with high demands on phonological processing. The present project aims to examine phonological skills and working memory capacity in children with Usher type 1 and compare their performance with deaf children with CI and nonsyndromal deafness, with children with normal hearing and children with hearing impairment. Few studies have focused on cognitive development in children with Usher syndrome type I and there is a lack of knowledge on whether the deafness and the co-existing visual condition have an impact on cognitive development and the spoken language development which is an outcome of the CI. Six children with Usher syndrome type I, in ages 6-14 years participated in the study. Preliminary results revealed that children with Usher syndrome type I have a similar pattern of results on the tests as the comparison group of children with CI.
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| 9. |
- Henricson, Cecilia, 1980-, et al.
(författare)
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Reading skill in five children with Usher Syndrome type 1 and Cochlear implants
- 2015
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Annan publikation (övrigt vetenskapligt/konstnärligt)abstract
- Objective: The aim of this study was to explore and describe reading skill in children with Usher syndrome type 1 and who have cochlear implants (USH1+CI), and to position their performance in relation to that of three control groups: children with normal hearing (NH), children with hearing impairment and hearing aids (HI+HA), and children with other types of deafness and CI (other CI).Method: Reading comprehension and decoding was measured in five children with USH1+CI in the ages 7.5–16 years. The children participated during a test session of 2–2.5 hours and performed tests including reading skill, WM, phonological skills, and lexical skills.Results: Four of the children with USH1+CI achieved results similar to those of the control group with NH on the measures of reading skill. One child with USH1+CI performed below all control groups. Three of the children with USH1+CI had high performance on both the measures of phonological skill and on the tests of reading skill. The groups perform similar results on the tests of reading skill.Conclusions: Three of the children with USH1+CI decode non-words with a phonological decoding strategy, similar to the strategy applied by the control group with NH. Two of the children with USH1+CI relied on an orthographic decoding strategy, possibly relying on other cognitive skills than the phonological strategy.
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| 10. |
- Henricson, Cecilia, 1980-, et al.
(författare)
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Theory-of-mind and cognitive function in adults with Usher and Alström syndromes
- 2016
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Ingår i: Journal of Visual Impairment & Blindness. - : AMER FOUNDATION BLIND. - 0145-482X .- 1559-1476. ; 110:5, s. 349-366
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Theory-of-Mind (ToM) refers to the ability to impute mental states to one self and to others. ToM was investigated in adults with Usher syndrome type II (USH2) and Alström syndrome (AS) - two syndromes causing acquired deafblindness. The syndromes differ with regard to onset and degree of sensory loss. Individuals with AS in contrast to individuals with USH2 display a high incidence of additional physical diseases. Cognitive shortcomings are generally not observed in USH2 or in AS, but cognitive delay and a delay in receptive language have been reported in AS. The results were compared to adults with normal hearing and vision (NHV).Methods: Thirteen persons with USH2, 12 persons with AS, and 33 persons with NHV participated. All participants performed a test of working memory capacity and verbal ability. ToM was tested with Happe´s Strange Stories test, taxing the ability to understand the emotions and actions of story characters, comprising a mental condition. The test also include a section of matched stories, tapping verbal problem solving ability in a physical condition, and a set of tasks tapping the ability to recall verbal material.Results: There were no differences between the three groups in the ability to recall verbal material. Significant differences were however established on working memory, and on verbal problem solving in a physical condition, with higher results for the NHV group. The two groups with deafblindness also displayed poorer ToM performance than the NHV group, by producing fewer correct mental references. The two groups with deafblindness differed from each other also in the ability to produce mental inferences as such, where the USH group outperformed the AS group. Intra-group variability in this ability was also observed within the two syndromal groups. Differences were related to verbal ability, complex working memory capacity, visual status, and to a minor extent auditory capacity. The prevalence and severity of additional physical diseases in AS was not related to ToM performance.Conclusions: A limited access to information as a function of sensory loss could influence degree of experience of the physical world, but also of social situations and of communication, affecting ToM development negatively. Early loss of visual field and visual acuity was related to ToM performance in individuals with USH2 and AS. Access to information also requires processing skills promoted by effective cognitive skills. Working memory capacity was related to ToM in USH. This relation also points to the contribution of hearing in development of ToM. Differences between the two groups could be a function of genetic conditions, where the gene causing USH2 only affects the ear and the eye, while AS in addition has a multi-systemic pathology with varying onset and degree. Differences in ToM performance in the AS group could however not be directly attributed to health conditions.
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