The reliability of Hebb repetition learning and its association with language and reading in adolescents with intellectual disabilities.

Hebb repetition learning (HRL) refers to neurodevelopmental processes characterised by repeated stimulus exposure without feedback, which result in changes in behaviour and/or responses, e.g., long-term learning of serial order. Here, we investigate effects of HRL on serial order memory. The present research aimed to assess the reliability of new HRL measures and investigate their relationships with language and reading skills (vocabulary, grammar, word reading) in adolescents with intellectual disability (ID). A comparison group of children of similar mental age with typical development (TD) was also assessed. ID and TD groups were tested on HRL tasks, evaluating test-retest and split-half reliability. The relationship between HRL and language and reading was analysed after accounting for the influence of mental age and verbal short-term memory. The HRL tasks displayed moderate test-retest (and split-half) reliability, HRL tasks with different stimuli (verbal, visual) were related, and we identified issues with one method of HRL scoring. The planned regression analyses failed to show relationships between HRL and language/reading skills in both groups when mental age, a very strong predictor, was included. However, further exploratory regression analyses without mental age revealed HRL's predictive capabilities for vocabulary in the ID group and reading in the TD group, results which need further investigation and replication. HRL displays promise as a moderately reliable metric and exhibits varied and interpretable predictive capabilities for language and reading skills across groups.

Translation, cross-cultural adaptation, and preliminary validation of a patient-reported outcome measure for genetic counseling outcomes in Sweden.

Genetic counseling is key for understanding the consequences of hereditary and genetic diseases and, therefore, crucial for patients, their families, and healthcare providers. Genetic counseling facilitates individuals' comprehension, decision-making, and adaptation to hereditary diseases. This study focuses on the Swedish adaptation of the Genetic Counseling Outcome Scale-24 (GCOS-24), an internationally validated, patient-reported outcome measure (PROM) for quantifying patient empowerment in genetic counseling. This study aimed to translate and cross-culturally adapt the GCOS-24 to measure patient-reported outcome from genetic counseling in Sweden. The adaptation process was meticulously conducted, adhering to international guidelines, with cross-cultural adaptation, translation, and back translation, to ensure semantic, conceptual, and idiomatic equivalence with the original English version. Face validity and understandability was assured using qualitative cognitive interviews conducted with patient representatives, and by a committee of experts in the field. The psychometric properties of the Swedish version of GCOS-24 (GCOS-24swe) were evaluated using a robust sample of 374 patients. These individuals received genetic counseling by telephone or video, necessitated by the constraints of the COVID-19 pandemic. Participants responded to GCOS-24swe both before and after genetic counseling. The GCOS-24swe demonstrated face validity, good internal consistency (Cronbach's alpha = 0.86), significant responsiveness (Cohen's d = 0.65, p < 0.001), and good construct validity. The study's findings underscore the GCOS-24swe's potential as an effective instrument in both clinical practice and research within Sweden. It offers a valuable means for assessing patient empowerment, a key goal of genetic counseling. Additional psychometric assessment of test-retest reliability and interpretability would further enhance the utility of GCOS-24swe.

Relationship between self-reported listening and communication difficulties and executive function: a protocol for a systematic review and meta-analysis.

INTRODUCTION: Listening and communication difficulties can limit people's participation in activity and adversely affect their quality of life. Hearing, as well as listening and communication difficulties, can be measured either by using behavioural tests or self-report measures, and the outcomes are not always closely linked. The association between behaviourally measured and self-reported hearing is strong, whereas the association between behavioural and self-reported measures of listening and communication difficulties is much weaker, suggesting they assess different aspects of listening. While behavioural measures of listening and communication difficulties have been associated with poorer cognitive performance including executive functions, the same association has not always been shown for self-report measures. The objective of this systematic review and meta-analysis is to understand the relationship between executive function and self-reported listening and communication difficulties in adults with hearing loss, and where possible, potential covariates of age and pure-tone audiometric thresholds. METHODS AND ANALYSIS: Studies will be eligible for inclusion if they report data from both a self-report measure of listening difficulties and a behavioural measure of executive function. Eight databases are to be searched: MEDLINE (via Ovid SP), EMBASE (via Ovid SP), PsycINFO (via Ovid SP), ASSIA (via ProQuest), Cumulative Index to Nursing and Allied Health Literature or CINAHL (via EBSCO Host), Scopus, PubMed and Web of Science (Science and Social Science Citation Index). The JBI critical appraisal tool will be used to assess risk of bias for included studies. Results will be synthesised primarily using a meta-analysis, and where sufficient quantitative data are not available, a narrative synthesis will be carried out to describe key results. ETHICS AND DISSEMINATION: No ethical issues are foreseen. Data will be disseminated via academic publication and conference presentations. Findings may also be published in scientific newsletters and magazines. PROSPERO REGISTRATION NUMBER: CRD42022293546.

The Perfect Match! A Review and Tutorial on Issues Related to Matching Groups in Investigations of Children with Neurodevelopmental Conditions.

Research concerned with children and young people who have neurodevelopmental disabilities (ND) in relation to early language acquisition usually involves comparisons with matched group(s) of typically developing individuals. In these studies, several important and complex issues need to be addressed. Three major issues are related to: (1) the choice of a variables on which to carry out group matching; (2) recruiting children into the study; and (3) the statistical analysis of the data. To assist future research on this topic, we discuss each of these three issues and provide recommendations about what we believe to be the best course of action. To provide a comprehensive review of the methodological issues, we draw on research beyond the topic of early language acquisition. Our overall aim is to contribute to research that considers questions about delay or differences in development patterns of development and about identifying potentially causal variables.

Effects of a phonics intervention in a randomized controlled study in Swedish second-grade students at risk of reading difficulties.

Teaching phoneme awareness to children at risk for early reading difficulties has been recognized as successful in several studies. In this randomized controlled trial (RCT)-study, we add to this research by optimizing core procedural as well as teaching components in a phonics-directed intervention and extend the RCT reading intervention research into a semi-transparent language context. The aim of the present study was to evaluate the effects of a novel Swedish intensive phonics program. This randomized controlled pre-test and post-test intervention study targeted second-grade students with early reading difficulties. Students were identified by a repeated screening procedure and allocated to intervention (n = 34) and control (n = 34) conditions. A 9-week intensive phonics-based program was administrated one-to-one, by special education teachers in Swedish mainstream elementary schools. Results show an improvement in the intervention group, compared with the controls on all outcome measures. Findings indicate that the supplementary phonics program, delivered with high intensity, can significantly increase word reading skills and reading comprehension in second-grade students with early reading difficulties.

Reliability of FUNDES-Child-SE - measuring participation and independence of children and youths with disabilities.

BACKGROUND: There is a need for an instrument to measure participation and independence in children with disabilities. FUNDES-Child-SE has its origin in the participation questionnaire Child and Adolescent Scale of Participation. AIMS: Test the psychometric properties of internal consistency and test-retest reliability. MATERIAL AND METHODS: This cross-sectional study included caregivers of 163 children with disability aged 6-18 years, 59 of whom were also included in the test-retest study. Descriptive statistics were used to evaluate the proportions of valid ratings. Internal consistency and test-retest reliability were tested through Cronbach's alpha and the intra-class correlation coefficient. RESULTS: The amount of not relevant/not applicable ratings was substantial but varied between items and subdomains. Internal consistency was acceptable (0.8-0.95), and the test-retest was marginal to excellent (0.73-0.95). CONCLUSIONS: The reliability together with the content validity support the use of the FUNDES-Child-SE to measure participation and independence in children with disabilities. However, results should be interpreted with caution due to the small sample size and possible selection bias. Modifications to reduce the not relevant/not applicable responses should be investigated together with the instrument's responsiveness. SIGNIFICANCE: FUNDES-Child-SE can be used to facilitate a discussion of participation and independence and to plan interventions in a habilitation setting.

A structural equation mediation model captures the predictions amongst the parameters of the ease of language understanding model.

Objective: The aim of the present study was to assess the validity of the Ease of Language Understanding (ELU) model through a statistical assessment of the relationships among its main parameters: processing speed, phonology, working memory (WM), and dB Speech Noise Ratio (SNR) for a given Speech Recognition Threshold (SRT) in a sample of hearing aid users from the n200 database. Methods: Hearing aid users were assessed on several hearing and cognitive tests. Latent Structural Equation Models (SEMs) were applied to investigate the relationship between the main parameters of the ELU model while controlling for age and PTA. Several competing models were assessed. Results: Analyses indicated that a mediating SEM was the best fit for the data. The results showed that (i) phonology independently predicted speech recognition threshold in both easy and adverse listening conditions and (ii) WM was not predictive of dB SNR for a given SRT in the easier listening conditions (iii) processing speed was predictive of dB SNR for a given SRT mediated via WM in the more adverse conditions. Conclusion: The results were in line with the predictions of the ELU model: (i) phonology contributed to dB SNR for a given SRT in all listening conditions, (ii) WM is only invoked when listening conditions are adverse, (iii) better WM capacity aids the understanding of what has been said in adverse listening conditions, and finally (iv) the results highlight the importance and optimization of processing speed in conditions when listening conditions are adverse and WM is activated.

Structural validity and internal consistency of the Strengths and Stressors in Parenting (SSF) Questionnaire in parents of children with developmental disabilities.

The current study investigated the structural validity and internal consistency of the Strengths and Stressors (SSF) questionnaire. The SSF is used in Swedish habilitation services to measure the positive and negative consequences that the fostering of a child with a developmental disability can have on family functioning in six domains: parent's feelings and attitudes, social life, family finances, relationship to the other parent, siblings, and professional support. The proposed six-factor model was tested with confirmatory factor analysis with data collected from 291 parents of children with developmental disabilities. The six-factor model had an acceptable fit according to most fit indices, but two items were non-significant. Overall, the internal consistency was acceptable or good. The SSF, with the proposed six-factor solution, can be a useful tool when assessing parental perspectives on the impacts of having a child with a developmental disability in clinical settings and research.

Aberrant resting-state connectivity of auditory, ventral attention/salience and default-mode networks in adults with attention deficit hyperactivity disorder.

Background: Numerous resting-state studies on attention deficit hyperactivity disorder (ADHD) have reported aberrant functional connectivity (FC) between the default-mode network (DMN) and the ventral attention/salience network (VA/SN). This finding has commonly been interpreted as an index of poorer DMN regulation associated with symptoms of mind wandering in ADHD literature. However, a competing perspective suggests that dysfunctional organization of the DMN and VA/SN may additionally index increased sensitivity to the external environment. The goal of the current study was to test this latter perspective in relation to auditory distraction by investigating whether ADHD-adults exhibit aberrant FC between DMN, VA/SN, and auditory networks. Methods: Twelve minutes of resting-state fMRI data was collected from two adult groups: ADHD (n = 17) and controls (n = 17); from which the FC between predefined regions comprising the DMN, VA/SN, and auditory networks were analyzed. Results: A weaker anticorrelation between the VA/SN and DMN was observed in ADHD. DMN and VA/SN hubs also exhibited aberrant FC with the auditory network in ADHD. Additionally, participants who displayed a stronger anticorrelation between the VA/SN and auditory network at rest, also performed better on a cognitively demanding behavioral task that involved ignoring a distracting auditory stimulus. Conclusion: Results are consistent with the hypothesis that auditory distraction in ADHD is linked to aberrant interactions between DMN, VA/SN, and auditory systems. Our findings support models that implicate dysfunctional organization of the DMN and VA/SN in the disorder and encourage more research into sensory interactions with these major networks.

No learning loss in Sweden during the pandemic evidence from primary school reading assessments.

The COVID-19 pandemic has led to worldwide school closures, with a risk of learning loss. Sweden kept primary schools open, but it is unknown whether student and teacher absence and pandemic-related stress factors affected teaching and student progress negatively. In this study, reading assessment data from 97,073 Swedish primary school students (grades 1-3) were analysed to investigate potential learning loss. Results showed that word decoding and reading comprehension scores were not lower during the pandemic compared to before the pandemic, that students from low socio-economic backgrounds were not especially affected, and that the proportion of students with weak decoding skills did not increase during the pandemic. Study limitations are discussed. We conclude that open schools benefitted Swedish primary school students.

The structure of executive functioning in 11 to 14 year olds with and without special educational needs.

The structure and development of executive functioning (EF) have been intensively studied in typically developing populations, with little attention given to those with Special Educational Needs (SEN). This study addresses this by comparing the EF structure of 132 adolescents (11-14 years-old) with SEN and 138 adolescents not requiring additional support (Non-SEN peers). Participants completed verbal and non-verbal assessments of key components of EF: inhibition, working memory and switching. Confirmatory Factor Analysis on each group tested one-, two- and three-factor models of EF. In both groups, there was statistical support for the fit of one- and two-factor models with no model being clearly better than the others; there was little support for three-factor models. Parsimony suggests that the one-factor model best represents the structure of EF. In light of our results, the implications for the nature of EF in early adolescence in both SEN and Non-SEN groups are discussed.

Hebb repetition learning in adolescents with intellectual disabilities.

BACKGROUND: Hebb repetition learning is a form of long-term serial order learning that can occur when sequences of items in an immediate serial recall task are repeated. Repetition improves performance because of the gradual integration of serial order information from short-term memory into a more stable long-term memory trace. AIMS: The current study assessed whether adolescents with non-specific intellectual disabilities showed Hebb repetition effects, and if their magnitude was equivalent to those of children with typical development, matched for mental age. METHODS: Two immediate serial recall Hebb repetition learning tasks using verbal and visuospatial materials were presented to 47 adolescents with intellectual disabilities (11-15 years) and 47 individually mental age-matched children with typical development (4-10 years). RESULTS: Both groups showed Hebb repetition learning effects of similar magnitude, albeit with some reservations. Evidence for Hebb repetition learning was found for both verbal and visuospatial materials; for our measure of Hebb learning the effects were larger for verbal than visuospatial materials. CONCLUSIONS: The findings suggested that adolescents with intellectual disabilities may show implicit long-term serial-order learning broadly commensurate with mental age level. The benefits of using repetition in educational contexts for adolescents with intellectual disabilities are considered.

Is Having Hearing Loss Fundamentally Different? Multigroup Structural Equation Modeling of the Effect of Cognitive Functioning on Speech Identification.

OBJECTIVES: Previous research suggests that there is a robust relationship between cognitive functioning and speech-in-noise performance for older adults with age-related hearing loss. For normal-hearing adults, on the other hand, the research is not entirely clear. Therefore, the current study aimed to examine the relationship between cognitive functioning, aging, and speech-in-noise, in a group of older normal-hearing persons and older persons with hearing loss who wear hearing aids. DESIGN: We analyzed data from 199 older normal-hearing individuals (mean age = 61.2) and 200 older individuals with hearing loss (mean age = 60.9) using multigroup structural equation modeling. Four cognitively related tasks were used to create a cognitive functioning construct: the reading span task, a visuospatial working memory task, the semantic word-pairs task, and Raven's progressive matrices. Speech-in-noise, on the other hand, was measured using Hagerman sentences. The Hagerman sentences were presented via an experimental hearing aid to both normal hearing and hearing-impaired groups. Furthermore, the sentences were presented with one of the two background noise conditions: the Hagerman original speech-shaped noise or four-talker babble. Each noise condition was also presented with three different hearing processing settings: linear processing, fast compression, and noise reduction. RESULTS: Cognitive functioning was significantly related to speech-in-noise identification. Moreover, aging had a significant effect on both speech-in-noise and cognitive functioning. With regression weights constrained to be equal for the two groups, the final model had the best fit to the data. Importantly, the results showed that the relationship between cognitive functioning and speech-in-noise was not different for the two groups. Furthermore, the same pattern was evident for aging: the effects of aging on cognitive functioning and aging on speech-in-noise were not different between groups. CONCLUSION: Our findings revealed similar cognitive functioning and aging effects on speech-in-noise performance in older normal-hearing and aided hearing-impaired listeners. In conclusion, the findings support the Ease of Language Understanding model as cognitive processes play a critical role in speech-in-noise independent from the hearing status of elderly individuals.

Rapid Implementation of Telegenetic Counseling in the COVID-19 and Swedish Healthcare Context: A Feasibility Study.

This study reports the process and preliminary findings of rapid implementation of telegenetic counseling in the context of Swedish healthcare and COVID-19 pandemic, from both a patient and a provider perspective. Fourty-nine patients and 6 healthcare professionals were included in this feasibility study of telegenetic counseling in a regional Department of Clinical Genetics in Sweden. Telegenetic counseling is here defined as providing genetic counseling to patients by video (n = 30) or telephone (n = 19) appointments. Four specific feasibility aspects were considered: acceptability, demand, implementation, and preliminary efficacy. Several measures were used including the Genetic Counseling Outcome Scale 24 (collected pre- and post-counseling); the Telehealth Usability Questionnaire; a short study specific evaluation and Visiba Care evaluations, all collected post-counseling. The measures were analyzed with descriptive statistics and the preliminary results show a high level of acceptance and demand, from both patients and providers. Results also indicate successful initial implementation in the regional Department of Clinical Genetics and preliminary efficacy, as shown by significant clinically important improvement in patients' empowerment levels.

An interactive digital calendar with mobile phone reminders (RemindMe) for people with cognitive impairment: A pilot randomized controlled trial.

BACKGROUND: People with cognitive impairment often need support to perform everyday-life activities. Interventions are available, but evidence-based interventions are lacking. AIM: This pilot RCT aimed to investigate use of an intervention with an interactive digital calendar with mobile phone reminders (RemindMe) in relation to change in outcomes and impact on occupational performance, independence, health-related quality of life, and psychosocial impact of the support for people with cognitive impairment. METHOD: The study design was a multi-centre parallel-group pilot RCT [ClinicalTrails.gov, identifier: NCT04470219]. Fifteen participants from primary rehabilitation centres in Sweden were recruited and randomly assigned to intervention group (n = 8) receiving the intervention with RemindMe, or control group (n = 7) receiving usual treatment by an occupational therapist. Data were collected at baseline, after two- and four months, and analysed using descriptive and non-parametric statistics. RESULT: The Canadian Occupational Performance Measure (COPM), and the Functional Independence Measure (FIM item n-r) showed significant differences. There were no significant differences in health-related quality of life nor in the psychosocial impact of the used support. CONCLUSION: Plausible changes in outcome measures were found in COPM and FIM (items n-r). These instruments indicate change in outcome measures and impact on occupational performance and independence.

The Effects of Working Memory Load on Auditory Distraction in Adults With Attention Deficit Hyperactivity Disorder.

Cognitive control provides us with the ability to inter alia, regulate the locus of attention and ignore environmental distractions in accordance with our goals. Auditory distraction is a frequently cited symptom in adults with attention deficit hyperactivity disorder (aADHD)-yet few task-based fMRI studies have explored whether deficits in cognitive control (associated with the disorder) impedes on the ability to suppress/compensate for exogenously evoked cortical responses to noise in this population. In the current study, we explored the effects of auditory distraction as function of working memory (WM) load. Participants completed two tasks: an auditory target detection (ATD) task in which the goal was to actively detect salient oddball tones amidst a stream of standard tones in noise, and a visual n-back task consisting of 0-, 1-, and 2-back WM conditions whilst concurrently ignoring the same tonal signal from the ATD task. Results indicated that our sample of young aADHD (n = 17), compared to typically developed controls (n = 17), had difficulty attenuating auditory cortical responses to the task-irrelevant sound when WM demands were high (2-back). Heightened auditory activity to task-irrelevant sound was associated with both poorer WM performance and symptomatic inattentiveness. In the ATD task, we observed a significant increase in functional communications between auditory and salience networks in aADHD. Because performance outcomes were on par with controls for this task, we suggest that this increased functional connectivity in aADHD was likely an adaptive mechanism for suboptimal listening conditions. Taken together, our results indicate that aADHD are more susceptible to noise interference when they are engaged in a primary task. The ability to cope with auditory distraction appears to be related to the WM demands of the task and thus the capacity to deploy cognitive control.

Decoding Abilities in Adolescents with Intellectual Disabilities: The Contribution of Cognition, Language, and Home Literacy.

Decoding abilities in individuals with intellectual disabilities (ID) are substantially lower than for typical readers. The underlying mechanisms of their poor reading remain uncertain. The aim of this study was to investigate the concurrent predictors of decoding ability in 136 adolescents with non-specific ID, and to evaluate the results in relation to previous findings on typical readers. The study included a broad range of cognitive and language measures as predictors of decoding ability. A LASSO regression analysis identified phonological awareness and rapid automatized naming (RAN) as the most important predictors. The predictors explained 57.73% of the variance in decoding abilities. These variables are similar to the ones found in earlier research on typically developing children, hence supporting our hypothesis of a delayed rather than a different reading profile. These results lend some support to the use of interventions and reading instructions, originally developed for typically developing children, for children and adolescents with non-specific ID.

Investigating Reading Comprehension in Adolescents with Intellectual Disabilities: Evaluating the Simple View of Reading.

Reading comprehension difficulties are common in individuals with intellectual disabilities (ID), but the influences of underlying abilities related to reading comprehension in this group have rarely been investigated. One aim of this study was to investigate the Simple View of Reading as a theoretical framework to describe cognitive and linguistic abilities predicting individual differences in reading comprehension in adolescents with non-specific ID. A second aim was to investigate whether predictors of listening comprehension and reading comprehension suggest that individuals with ID have a delayed pattern of development (copying early grade variance in reading comprehension) or a different pattern of development involving a new or an unusual pattern of cognitive and linguistic predictors. A sample of 136 adolescents with non-specific ID was assessed on reading comprehension, decoding, linguistic, and cognitive measures. The hypotheses were evaluated using structural equation models. The results showed that the Simple View of Reading was not applicable in explaining reading comprehension in this group, however, the concurrent predictors of comprehension (vocabulary and phonological executive-loaded working memory) followed a delayed profile.

Definitions and Operationalization of Mental Health Problems, Wellbeing and Participation Constructs in Children with NDD: Distinctions and Clarifications.

Children with impairments are known to experience more restricted participation than other children. It also appears that low levels of participation are related to a higher prevalence of mental health problems in children with neurodevelopmental disorders (NDD). The purpose of this conceptual paper is to describe and define the constructs mental health problems, mental health, and participation to ensure that future research investigating participation as a means to mental health in children and adolescents with NDD is founded on conceptual clarity. We first discuss the difference between two aspects of mental health problems, namely mental disorder and mental illness. This discussion serves to highlight three areas of conceptual difficulty and their consequences for understanding the mental health of children with NDD that we then consider in the article: (1) how to define mental health problems, (2) how to define and assess mental health problems and mental health, i.e., wellbeing as separate constructs, and (3) how to describe the relationship between participation and wellbeing. We then discuss the implications of our propositions for measurement and the use of participation interventions as a means to enhance mental health (defined as wellbeing). Conclusions: Mental disorders include both diagnoses related to impairments in the developmental period, i.e., NDD and diagnoses related to mental illness. These two types of mental disorders must be separated. Children with NDD, just like other people, may exhibit aspects of both mental health problems and wellbeing simultaneously. Measures of wellbeing defined as a continuum from flourishing to languishing for children with NDD need to be designed and evaluated. Wellbeing can lead to further participation and act to protect from mental health problems.

Social interaction and gender as factors affecting the trajectories of children's engagement and hyperactive behaviour in preschool.

BACKGROUND AND AIMS: Social interactions in preschool and a child's gender are, in cross-sectional studies, related to the child's overall levels of hyperactive behaviour and engagement in preschool activities. However, whether social interaction and gender can predict children's engagement and hyperactivity is not thoroughly investigated. This study aims to investigate the longitudinal influence of gender, child-to-child interaction, and teacher responsiveness on the association between trajectories of children's levels of core engagement and hyperactive behaviour. It was hypothesized that peer-to-child interaction and teacher responsiveness in preschool settings are related to positive change in engagement among children with hyperactive behaviour, especially for boys. SAMPLE AND METHODS: Swedish preschool staff completed questionnaires assessing the variables of interest for children aged 1-5 (N = 203). Data were collected on three occasions over a two-year period. Latent growth curve (LGC) models were used to explore whether teacher responsiveness, peer-to-child interaction, and gender predict trajectories of engagement and hyperactivity. RESULTS: The results revealed that high levels of hyperactivity were associated with lower levels of engagement on the first occasion. Positive peer-to-child interactions and responsive teachers were significant predictors of an increased level of engagement and decreased level of hyperactive behaviour, especially for boys. CONCLUSIONS: The findings underscore the need to improve social interactions, especially peer-to-child interactions, to improve engagement in children with hyperactive behaviour, especially boys. Implications for practices and research are discussed.