Research gaps in the neurodevelopmental assessment of children with complex congenital heart defects: a scoping review.

Background: Children with congenital heart defects (CHD) are at risk for a range of developmental disabilities that challenge cognition, executive functioning, self-regulation, communication, social-emotional functioning, and motor skills. Ongoing developmental surveillance is therefore key to maximizing neurodevelopmental outcome opportunities. It is crucial that the measures used cover the spectrum of neurodevelopmental domains relevant to capturing possible predictors and malleable factors of child development. Objectives: This work aimed to synthesize the literature on neurodevelopmental measures and the corresponding developmental domains assessed in children aged 1-8 years with complex CHD. Methods: PubMed was searched for terms relating to psycho-social, cognitive and linguistic-communicative outcomes in children with CHD. 1,380 papers with a focus on complex CHD that reported neurodevelopmental assessments were identified; ultimately, data from 78 articles that used standardized neurodevelopmental assessment tools were extracted. Results: Thirty-nine (50%) of these excluded children with syndromes, and 9 (12%) excluded children with disorders of intellectual development. 10% of the studies were longitudinal. The neurodevelopmental domains addressed by the methods used were: 53% cognition, 16% psychosocial functioning, 18% language/communication/speech production, and 13% motor development-associated constructs. Conclusions: Data on social communication, expressive and receptive language, speech motor, and motor function are underrepresented. There is a lack of research into everyday use of language and into measures assessing language and communication early in life. Overall, longitudinal studies are required that include communication measures and their interrelations with other developmental domains.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Call to Action.

This Call to Action is the eighth and final article in this special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. Collectively, these articles highlight evidence-informed actions to enhance family well-being and to optimize developmental outcomes among children who are DHH. This Call to Action outlines actionable steps to advance FCEI-DHH supports provided to children who are DHH and their families. It also urges specific actions to strengthen FCEI-DHH programs/services and systems across the globe, whether newly emerging or long-established. Internationally, supports for children who are DHH are often siloed, provided within various independent sectors such as health/medicine, education, early childhood, and social and disability services. With this Call to Action, we urge invested parties from across relevant sectors to join together to implement and improve FCEI-DHH programs/services and systems, build the capacity of early intervention (EI) Providers and other professionals, extend research regarding FCEI-DHH, and fund EI supports, systems, and research, all with the aim of advancing outcomes for families and their children who are DHH.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Methods.

This is the fourth article in a series of eight that comprise a special issue on family-centered early intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, FCEI-DHH. This article describes the co-production team and the consensus review method used to direct the creation of the 10 Principles described in this special issue. Co-production is increasingly being used to produce evidence that is useful, usable, and used. A draft set of 10 Principles for FCEI-DHH and associated Tables of recommended behaviors were developed using the knowledge creation process. Principles were refined through two rounds of eDelphi review. Results for each round were analyzed using measures of overall group agreement and measures that indicated the extent to which the group members agreed with each other. After Round 2, with strong agreement and low to moderate variation in extent of agreement, consensus was obtained for the 10 Principles for FCEI-DHH presented in this special issue. This work can be used to enhance evolution of FCEI-DHH program/services and systems world-wide and adds to knowledge in improvement science.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Support Principles.

This article is the sixth in a series of eight articles that comprise a special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. The Support Principles article is the second of three articles that describe the 10 Principles of FCEI-DHH, preceded by the Foundation Principles, and followed by the Structure Principles, all in this special issue. The Support Principles are composed of four Principles (Principles 3, 4, 5, and 6) that highlight (a) the importance of a variety of supports for families raising children who are DHH; (b) the need to attend to and ensure the well-being of all children who are DHH; (c) the necessity of building the language and communication abilities of children who are DHH and their family members; and (d) the importance of considering the family's strengths, needs, and values in decision-making.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Cultural & Global Implications.

This article is the third in a series of eight articles that comprise this special issue on family-centered early intervention for children who are deaf or hard of hearing and their families (FCEI-DHH). It highlights the origins of FCEI-DHH in Western contexts and well-resourced locations and emphasizes the role of culture(s) in shaping FCEI-DHH. This article also cautions against the direct application of the 10 FCEI-DHH Principles presented in this issue across the globe without consideration of cultural implications. Cultural perceptions of decision-making processes and persons who can be decision-makers in FCEI-DHH are explored. Deaf culture(s) and the benefits of exposure to DHH adults with diverse backgrounds are introduced. Structural inequities that impact families' access to FCEI-DHH programs/services and systems, within and among nations and regions, are noted. The need to consider the cultural influences on families is emphasized; this applies to all levels of FCEI, including the development of systems through implementation of supports.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Foundation Principles.

This article is the fifth in a series of eight articles that comprise a special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. The 10 FCEI-DHH Principles are organized conceptually into three sections (a) Foundation Principles, (b) Support Principles, and (c) Structure Principles. Collectively, they describe the essential Principles that guide FCEI for children who are DHH and their families. This article describes the Foundation Principles (Principles 1 and Principle 2). The Foundation Principles emphasize the essential elements of ensuring that families with children who are DHH can access early intervention (EI) and other appropriate supports, as well as highlight the need for provision of EI that is family-centered. Implementation of these FCEI-DHH Principles is intended to improve the lives and the outcomes of children who are DHH and their families around the globe.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Structure Principles.

This article is the seventh in a series of eight articles that comprise a special issue on family-centered early intervention for children who are deaf or hard of hearing and their families, or FCEI-DHH. This article, Structure Principles, is the third of three articles (preceded by Foundation Principles and Support Principles) that describe the 10 FCEI-DHH Principles. The Structure Principles include 4 Principles (Principle 7, Principle 8, Principle 9, and Principle 10) that highlight (a) the importance of trained and effective Early Intervention (EI) Providers, (b) the need for FCEI-DHH teams to work collaboratively to support families, (c) the considerations for tracking children's progress through developmental assessment, and (d) the essential role of progress monitoring to continuously improve systems.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Guiding Values.

This article is the second of eight articles in this special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. Five foundational values that guide FCEI-DHH are described, providing an evidence-informed, conceptual context for the 10 FCEI-DHH Principles and other articles presented in this issue. These values are applicable for Early Intervention (EI) Providers and other professionals on FCEI teams, as well as for FCEI-DHH programs/services and systems. The five key values include (1) being family-centered, (2) responding to diversity, (3) involving invested parties, especially families and individuals who are DHH, (4) supporting holistic child development, and (5) ensuring fundamental human rights. These evidence-informed values are considered essential to the effective provision of FCEI-DHH supports.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Introduction.

This article is the first of eight articles in this special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH), or FCEI-DHH. In 2013, a diverse panel of experts published an international consensus statement on evidence-based Principles guiding FCEI-DHH. Those original Principles have been revised through a coproduction process involving multidisciplinary collaborators and an international consensus panel, utilizing the best available evidence and current understanding of how to optimally support children who are DHH and their families. This revision (referred to as expanded Principles) was motivated by the need to incorporate (a) input from family leaders and DHH leaders, (b) broader international and cultural perspectives, (c) new empirical evidence, and (d) research in human development. This Introduction provides an overview of the rationale, purposes, and main content areas to be addressed throughout the special issue.

A prospective, randomized, single-blind study to compare two methods of treating cystic lesions in the jaw.

OBJECTIVE: The study aimed to assess the usefulness of a bovine bone substitute material in treating cystic lesions in the jaw with a maximum diameter of <4 cm. MATERIAL AND METHODS: In this prospective, randomized, single-blind intervention study of 116 patients, 61 underwent cystectomy with a subsequent filling of the defect using a bovine xenograft, and 55 underwent cystectomy alone. Volumetric measurement of the cysts was performed preoperatively and 6 and 12 months postoperatively using the available digital volume tomography data sets. Follow-up appointments were made 14 days and 1, 3, 6, and 12 months postoperatively. RESULTS: Almost complete regeneration was seen in both treatment groups within 12 months, with no significant difference in absolute volume loss between the 2 groups (P = .521). Examination 14 days after surgery revealed a tendency for more wound healing disorders with the use of a bone substitute (P = .077). It was no longer possible to detect any further differences in later examinations. CONCLUSION: Using bovine bone substitute material has no radiologically measurable advantage over cystectomy alone without defect filling regarding bone regeneration. In addition, there was a tendency for more wound-healing disorders to occur in the bone substitute group.

Learning about neurodiversity from parents - Auditory gestalt perception of prelinguistic vocalisations.

BACKGROUND: Infants with Rett syndrome (RTT) may have subtle anomalies in their prelinguistic vocalisations but the detection of these is difficult, since their conspicuous vocalisations are often interspersed with inconspicuous ones. AIMS AND METHODS: Extending a previous study with predominantly non-parents, the present study sampled parents of children with RTT and aimed to examine their gestalt perception of prelinguistic vocalisations. METHODS AND PROCEDURE: Parents (n = 76) of female children with RTT listened to vocalisation recordings from RTT and typically developing (TD) infants, including an inconspicuous vocalisation from a RTT girl. For each recording, parents indicated if the vocalisation was produced by a RTT or a TD child. RESULTS: Overall correct to incorrect identification rate was 2:1, which was comparable to that of the previous study. Intriguingly, parents of RTT children seemed to be sensitive to features characterising the vocalisations of RTT infants, which has especially influenced their perception of the inconspicuous vocalisation from a RTT girl. CONCLUSIONS AND IMPLICATIONS: These results invite further research on the potential characterising differences between vocalisations from TD infants and infants with divergent neurodevelopment.

Exploring spirituality and quality of life in individuals who are deaf and have intellectual disabilities.

PURPOSE: While positive contributions of religion and spirituality (R/S) to quality of life (QOL) are confirmed by a growing body of evidence, only limited research has involved people with intellectual disabilities and so far, no studies included prelingually deaf individuals with intellectual disabilities. This study explores the role of R/S in people with intellectual disabilities and deafness living in three therapeutic living communities specifically adapted to their needs. METHODS: Forty-one individuals (mean age: 46.93 years, 43.9% female) with prelingual deafness and mild to moderate intellectual disability participated in structured sign language interviews adapted to their cognitive-developmental level, regarding their QOL, individual spirituality and participation in spiritual practices in the community. Participants' QOL was assessed with an established short measure for QOL (EUROHIS-QOL) adapted to easy-to-understand sign language. With 21 participants, qualitative interviews were conducted. In addition, proxy ratings from caregivers were obtained. RESULTS: The participants' ratings of their individual spirituality (r = 0.334; p = 0.03) and spiritual practices-in-community (r = 0.514; p = 0.00) correlated positively with their self-reported QOL. Qualitative findings illustrate the importance of R/S and give insights into R/S concepts and practices. CONCLUSIONS: Personal spirituality and participating in spiritual practices are positively related to self-reported quality of life in deaf individuals with intellectual disability (ID). As a consequence, access to spiritual and religious services should be included in comprehensive programs and society at large.

Preschool Children with Hearing Loss: Social Communication and Parenting Stress.

Studies on parenting stress (PS) in parents of children with hearing loss (HL) have found relationships between child behavior, language skills and parenting stress. The role of early social communication skills has not been researched before. The aim of this cross-sectional study was to investigate the relationship between child behavior, social communication and PS. The study was performed in a subgroup of a total population sample from the AChild (Austrian Children with Hearing Impairment-Longitudinal Databank) study. Preschool children (n = 81) with all degrees of HL and average cognitive functioning and their families were included, and the Parenting Stress Index (PSI) was used. Through factor component analysis, compound scores for externalizing/internalizing problem behavior and hyperactivity were analyzed. Although mean PS was not elevated, the proportion of those with elevated scores was higher compared with the norm population. There was a strong correlation between child behavior problems and PS (strongest correlation: externalizing problem behavior r = 0.643; p < 0.001). All three problem behaviors accounted for 49.7% of the variance in PS. An indirect effect of social communication on PS was almost completely mediated by problem behavior (especially hyperactivity). The importance of social communication development with respect to problem behavior and PS is highlighted.

The link between social communication and mental health from childhood to young adulthood: A systematic review.

This systematic review investigates the current state of the literature on the association between social communication (SC) skills and mental health outcomes in children and young adults. We ran searches using terms describing SC and mental health in the following databases: PubMed, the Psychology and Behavioral Sciences Collection, APA PsychInfo, the Education Resource Information Center (ERIC) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL). We included studies that both measured SC before age 21 years and assessed a mental health component, such as behavior/conduct problems, anxiety, depression, or emotional problems, before age 30. Only peer-reviewed articles published in or after 2000 were retained. In total, 27 studies were included in the narrative synthesis. Overall, the methodological quality was good. A longitudinal design was used in 12 studies, seven of which sourced data from the Avon Longitudinal Study of Parents and Children. Most studies investigated the broad concept of pragmatic language and its influence on internalizing, externalizing, and peer-relationship problems. The analyzed publications included population-based studies and studies investigating special populations with existing mental health problems or at increased risk of developing them. The population-based cross-sectional studies showed significantly higher rates of SC problems in children with mental health problems while those with a longitudinal design found earlier onset and more persistent mental health problems in children who had the most severe SC difficulties. Studies with special populations confirmed more SC problems as compared to typically developing children and worse mental health outcomes in children with additional SC difficulties. The available literature thus shows consistently significant, mostly moderate associations between SC skills and mental health outcomes. SC difficulties earlier in life can predict mental health problems and impact their trajectories. Systematic review registration: [https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=286598], identifier [CRD42022286598].

Validity and feasibility of a predictive language screening tool in 2-year-old children in primary pediatric care.

Objective: To assess the predictive validity and feasibility of the newly developed language screening tool, SPES-2 (Sprachentwicklungsscreening), for 2-year-old children in pediatric primary care. Methods: A prospective cohort study recruited 2,044 non-selected German-speaking children undergoing a regular well-baby check-up at the age of 2 years. Thirty primary care pediatricians spread over urban and rural areas screened the children using a short parent-reported questionnaire and direct assessment of word comprehension. To validate the screening tool, language skills were assessed using a standardized language screening tool in the complete sample 1 year later. Data of a random sample of 621 children were analyzed. Feasibility of the screening tool was evaluated using questionnaires completed by the participating pediatricians. Results: The new screening tool, SPES-2, demonstrated good diagnostic accuracy with AUC (Area under the Roc Curve) of 0.885, a sensitivity of 0.74, and specificity of 0.86, using a parent-reported questionnaire (expressive vocabulary, two-word combinations, parental concerns) as stage 1, followed by a stage 2 direct assessment of word comprehension by the pediatrician. The second stage was restricted to children who failed the parental screening. The screening identified children with high, moderate, and low risk of significant language deficits (SLD) at the age of 3 years, permitting tailored follow-up assessment and parental counseling. Practicality and acceptability of the screening were mostly rated as high. Pediatricians regarded the availability of follow-up diagnostic services and parent guidance as most important for a general implementation of the new instrument. Conclusion: The language screening tool, SPES-2, was valid for the identification of significant language deficits 1 year later, and considered as feasible within primary pediatric care.

Communicative deficits associated with maladaptive behavior in individuals with deafness and special needs.

Background: At least one in three individuals who are prelingually deaf has special needs, most commonly due to intellectual disabilities. The scant literature on challenging behavior in this population, however, suggests high rates of prevalence and an important need to better understand the contributing factors. Aim: We sought to analyze the prevalence of maladaptive behavior and its association with intellectual functioning, adaptive skills, language skills, and social communication in a population of adults with deafness and special needs. Methods: Participants were 61 individuals from three therapeutic living communities established for people with deafness and special needs. The participants had a mean age of 54.7 years, 64% were male. Intellectual functioning was measured with two versions of the Snijders-Oomen Non-verbal Intelligence Scale. The Vineland-II Scales were used to assess adaptive and maladaptive behavior. Language skills were measured with instruments specifically adapted for this population, including the Reynell Developmental Language Comprehension Scale, the comprehension scale of the Child Development Inventory, and the Profile of Multiple Language Proficiencies. Due to high correlations between instruments, a composite language score was used. A specific questionnaire to measure social communication in adults with intellectual disabilities was also utilized. Results: The mean nonverbal developmental reference age was 6.5 years, whereas the equivalent for the language measures was about 3.5 years. The prevalence rate of elevated maladaptive behavior was 41% (v-scale score ≥18) and 18% of the participants had a clinically significant score (v-scale score ≥21). Regression analyses showed that only language and social communication skills were significantly associated with maladaptive behavior, while intellectual functioning and adaptive skills were not. Conclusion: These findings emphasize the importance of the constant promotion of communicative skills, as those people with better language and social communication skills demonstrate lower levels of maladaptive behavior.

Intellectual Disability Profiles, Quality of Life and Maladaptive Behavior in Deaf Adults: An Exploratory Study.

Individuals who are prelingually deaf and have intellectual disabilities experience great challenges in their language, cognitive and social development, leading to heterogeneous profiles of intellectual and adaptive functioning. The present study describes these profiles, paying particular attention to domain discrepancies, and explores their associations with quality of life and maladaptive behavior. Twenty-nine adults with prelingual deafness (31% female) and mild intellectual functioning deficits (mean IQ = 67.3, SD = 6.5) were administered the Vineland Adaptive Behavior Scales-II (VABS-II) and an adapted sign language version of a quality of life scale (EUROHIS-QOL 8). Intellectual disability domain discrepancies were characterized as at least one standard deviation difference between the social domain and IQ and the practical domain and IQ, and a significant difference, according to the VABS-II manual, between the social and practical domains. Domain discrepancies were found between intellectual functioning and both the practical (58.6%) and social domain (65.5%). A discrepancy between intellectual and social functioning was significantly associated with a higher level of internalizing maladaptive behavior (T = 1.89, p < 0.05). The heterogeneous profiles highlight the importance of comprehensive assessments for adequate service provision.

Development and Validation of a Language Screening for Implementation in Pre-School Settings.

Background: To prevent or mitigate long-lasting learning problems and emotional, behavioral, and social-adaption difficulties associated with language disorders, age-appropriate German language competence at school entry level is essential. Therefore, universal screening of children in their penultimate year of pre-school has been established in Upper Austria. So far, the screenings administered by speech and language pathologists to identify risk of language disorder (LD) were not based on standardized materials. Objective: To develop a screening instrument to identify increased risk of LD and to evaluate its validity and feasibility within the constraints of regular universal pre-school language screening. Design: A two-component screening instrument including direct assessment of expressive and receptive grammar was used in a sample of 374 children with German as their dominant language attending a public pre-school in their penultimate year (age 4-5 ½ years) in the state of Upper Austria. Assessment by use of standardized German language tests including a variety of linguistic domains was considered reference standard for diagnosing LD. Feasibility was assessed by a self-developed questionnaire completed by the administrators of the screening. Results: The combination of the expressive and receptive grammar scales demonstrated excellent accuracy (area under the curve score 0.928). A cut-off of 18 resulted in a failing rate of 21.8% and showed good sensitivity (84.2%) and specificity (85.3%). Acceptance by children and testers, time-economy and sustainability of the screening were mostly rated as high.

Predictors of Early Language Outcomes in Children with Connexin 26 Hearing Loss across Three Countries.

GJB2-associated hearing loss (GJB2-HL) is the most common genetic cause of hearing loss in children. However, little is known about the clinical characteristics and early language outcomes in population-oriented samples including children with different degrees of hearing loss. Insight into these characteristics are relevant for the counselling of parents. Our sample consisted of 66 children at approximately 2 years of age (17-32 months) with bilateral hearing loss due to GJB2 from three population-based cohorts in Austria, Australia and the Netherlands. Predictors of early vocabulary, including demographic, audiological, genetic and intervention variables and the role of medical comorbidities and nonverbal cognition were examined. The vocabulary scores of children with GJB2-HL were approximately 0.7 standard deviations (SDs) below the norms of children with typical hearing. Age at access to family-centered early intervention and first-born position among siblings predicted language outcomes, whereas the degree of hearing loss and genetic subtype were not significantly correlated with expressive vocabulary. In children with GJB2-HL, early access to family-centered early intervention significantly affected language outcomes at the age of two.