Children with Usher syndrome: mental and behavioral disorders

Dammeyer, Jesper

doi:10.1186/1744-9081-8-16

Cited by 28 publications

(22 citation statements)

References 38 publications

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“…There were two studies where there was uncertainty over their inclusion. One was a study of children with Usher syndrome with complex disabilities in addition to hearing loss [ 73 ]. An additional issue with this paper was that SDQ scores were not reported for six children with diagnosed “mental or behavioural disorder”.…”

Section: Methodsmentioning

confidence: 99%

Emotional and behavioural difficulties in children and adolescents with hearing impairment: a systematic review and meta-analysis

Stevenson

Kreppner

Pimperton

et al. 2015

Eur Child Adolesc Psychiatry

173

105

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The aim of this study is to estimate the extent to which children and adolescents with hearing impairment (HI) show higher rates of emotional and behavioural difficulties compared to normally hearing children. Studies of emotional and behavioural difficulties in children and adolescents were traced from computerized systematic searches supplemented, where appropriate, by studies referenced in previous narrative reviews. Effect sizes (Hedges’ g) were calculated for all studies. Meta-analyses were conducted on the weighted effect sizes obtained for studies adopting the Strength and Difficulties Questionnaire (SDQ) and on the unweighted effect sizes for non-SDQ studies. 33 non-SDQ studies were identified in which emotional and behavioural difficulties in children with HI could be compared to normally hearing children. The unweighted average g for these studies was 0.36. The meta-analysis of the 12 SDQ studies gave estimated effect sizes of 0.23 (95 % CI 0.07, 0.40), 0.34 (95 % CI 0.19, 0.49) and −0.01 (95 % CI −0.32, 0.13) for Parent, Teacher and Self-ratings of Total Difficulties, respectively. The SDQ sub-scale showing consistent differences across raters between groups with HI and those with normal hearing was Peer Problems. Children and adolescents with HI have scores on emotional and behavioural difficulties measures about a quarter to a third of a standard deviation higher than hearing children. Children and adolescents with HI are in need of support to help their social relationships particularly with their peers.

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Section: Methodsmentioning

confidence: 99%

Emotional and behavioural difficulties in children and adolescents with hearing impairment: a systematic review and meta-analysis

Stevenson

Kreppner

Pimperton

et al. 2015

Eur Child Adolesc Psychiatry

173

105

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“…Many case reports on USH patients showing mental disorders exist in the literature [199–211]. Two recent reports also imply that usherin and whirlin may be associated with other sensory modalities, touch and pain [164, 167].…”

Section: Current Gaps In Understanding and Treating Ushmentioning

confidence: 99%

Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities

Mathur

Yang

2015

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Diseas

285

301

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Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH.

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“…Our understanding of etiologies of hearing loss that put children at a higher risk of an ASD is fairly limited. However, small case reports suggest that children with CHARGE syndrome (coloboma, heart defect, atresia choanae or choanal atresia, retarded growth and development, genital abnormality, and ear abnormality), 15 children with congenital cytomegalovirus, 16 and children with Usher syndrome 17 may have a higher risk of having an ASD. Children with extreme prematurity have higher rates of positive autism screening; however, definitively higher rates of autism spectrum diagnoses have not been confirmed.…”

Section: Medical Considerationsmentioning

confidence: 99%

A Summary of Current Understanding Regarding Children with Autism Spectrum Disorder Who Are Deaf or Hard of Hearing

et al. 2014

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This article provides a consensus perspective based on the authors' expertise and the limited available literature regarding our understanding of children with an autism spectrum disorder (ASD) who are deaf or hard of hearing (D/HH). The challenges in the accurate identification of an ASD in children who are D/HH, including red flags for a potential ASD and screening and assessment for ASD, are described in this article. Additionally, strategies to guide professionals in their communication about a possible ASD with families and to frame the need for expanding aspects of communication important for this group of children are suggested.

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Children with Usher syndrome: mental and behavioral disorders

Cited by 28 publications

References 38 publications

Emotional and behavioural difficulties in children and adolescents with hearing impairment: a systematic review and meta-analysis

Emotional and behavioural difficulties in children and adolescents with hearing impairment: a systematic review and meta-analysis

Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities

A Summary of Current Understanding Regarding Children with Autism Spectrum Disorder Who Are Deaf or Hard of Hearing

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