Between 1 to 2 of every 1,000 children are born deaf or hard of hearing (DHH) and, of those, 30–50
CASE: Emily is a 10-year-old girl who is in fifth grade. She has known congenital blindness secondary to septo-optic dysplasia with bilateral optic nerve hypoplasia and precocious puberty. Emily was referred to a Developmental-Behavioral Pediatrics clinic for concerns of academic, social, and language challenges. Emily was born at term gestation after an uncomplicated pregnancy. At 4 months of age, she underwent ophthalmologic evaluation because of nystagmus, reduced visual tracking and response to light, and increased startle response to touch. An magnetic resonance imaging of the brain and orbits demonstrated bilateral hypoplastic optic nerves and the absence of posterior pituitary. Subsequent endocrinological evaluation for pituitary function was reassuring. Emily's early developmental milestones were delayed across all domains. She participated in early intervention programming including speech/language, physical, and occupational therapy with interval improvement in skills. She also received supports for low vision. In the elementary school, she received supports and services for low vision in a general education classroom. It was observed that Emily had reduced interest in her peers, a strong preference for routine, and distinctive play interests. As elementary school progressed, Emily had increasing challenges with academic achievement, despite performing well on formal testing in second grade. At a recent ophthalmology visit, Emily's best-corrected visual acuity was noted to be 20/800 in each eye. Neuropsychological testing was completed with visual accommodation and administration of measures with minimal visual requirements. Cognitive testing revealed variable verbal intellect and language skills. Academic testing revealed strong reading abilities and a relative weakness in math. Adaptive measures were notable for reduced function and highlighted social vulnerabilities. Parent measures regarding mood and behavior were not concerning. Emily's speech was noted to have a very distinctive prosody with notable response latency. Echolalia and scripting were appreciated, and Emily often asked about names and used made-up words. When excited, Emily flapped her arms and hands, jumped up and down, or clapped her hands quickly. Socially, Emily was engaged and seemed eager to please. She was able to participate in back-and-forth conversation. Although she often responded to social bids, she frequently directed the conversation to her own areas of interest. Emily looked in the direction of the examiner when talking to the examiner and when the examiner spoke. Although a diagnosis of autism spectrum disorder is under consideration, what special considerations are necessary in the context of congenital blindness?
Alazami syndrome, caused by biallelic pathogenic variants in LARP7, is a recently-described rare genetic disorder, with 17 patients currently reported in the literature. We present a case of a male infant referred for genetics evaluation at 5 months of age, found at 17 months of age to have Alazami syndrome. He was promptly referred for developmental evaluation, where he was found to be higher functioning than prior reports of individuals with this condition. This demonstrates the neurodevelopmental phenotypic variability seen in rare genetic disorders; it also demonstrates the important role of developmental programs to measure and track outcomes and provide support for infants with genetic disorders that put them at risk of developmental disabilities.
This is the first book of a 2-part manual that is written by leaders in the field of educating young children with developmental disabilities. The authors have crafted this well-written book as a guide to evidence-based treatment, with a focus on applying interventions that are based on the principles of applied behavior analysis (ABA). This book is informative and easy to read, with a target audience that includes both family members and professionals. Book 1 of this 2-part manual is ideal for readers who are beginning to learn about ABA or behaviorally based interventions. Perhaps of greatest importance, this book provides robust evidence for the effectiveness of ABA as an intervention that improves developmental outcomes in children with Down syndrome. Included is a 14-page list of peer-reviewed studies that the authors compiled to illustrate this point. Specifically, the authors have highlighted studies that demonstrate improved outcomes in multiple domains, including motor, social-communication, cognitive development, and self-care skills. Through extensively reviewing the research, the authors have done a great service to families and professionals who can use this information to advocate for such intervention. In chapter 1, the authors describe their journey in creating a behaviorally based model of intervention for young children with Down syndrome that incorporates a strength-based approach. Within chapter 1, the authors also introduce the reader to educational models and the importance of inclusion when using their approach. Chapter 2 provides crucial background information on the behavioral phenotype of individuals with Down syndrome. Strengths characteristic of youth with Down syndrome, including social interest and visual processing skills, are highlighted. The reader is also presented with ideas on how these strengths can be leveraged to promote the continued learning and development of the child. In chapter 3, elements of discrete trial training are broken down and explained in detail, with several examples provided. In chapter 4, the authors make a strong recommendation that a behavior analyst or someone with extensive training and experience in behavior analysis be included among a child's educational team. This chapter further empowers families to advocate for their children by providing an introduction to their rights and special education law. The book concludes with 3 stories written by families who describe their experiences raising children with Down syndrome, including the challenges and triumphs. These stories are sure to provide families and clinicians alike with insight, hope, and wisdom. Families also give first-person accounts of the role that ABA and inclusion has played in their child's learning. Included in Appendix A are sample forms that caregivers can use to collect data during discrete trial teaching or naturalistic instruction. Appendix B includes a vast collection of additional resources that will surely prove useful to families. The authors have taken on the challenge of writing this manual for a diverse audience, including families and professionals. As such, some parts of the book may be too simplified or detailed depending on the reader's level of experience. The authors navigate this by providing 2 separate manuals, book 1 and book 2. The first book is a wonderful primer for families or professionals who have little knowledge of ABA. Book 2 in this series is said to focus on step-by-step procedures for teaching skills to children with Down syndrome and intervening in behaviors that get in the way of learning. Those with a strong background in ABA may only require book 2 to implement these programs. In sum, in the first book of this 2-part series, the authors present a concise manual that highlights the evidence behind using ABA-based strategies to educate young children with Down syndrome. In doing so, they dispel the myth that ABA is an intervention that is only used for youth with autism spectrum disorders. This important work is the culmination of the authors' 20 years of direct work and experience in this field. We recommend this book for all families as a guide on how to implement and advocate for the use of behaviorally based interventions on young children with Down syndrome.
The goal of this study was to compare the effects of before school physical activity (PA) and sedentary classroom-based (SC) interventions on the symptoms, behavior, moodiness, and peer functioning of young children (M age = 6.83) at risk for attention-deficit/hyperactivity disorder (ADHD-risk; n = 94) and typically developing children (TD; n = 108). Children were randomly assigned to either PA or SC and participated in the assigned intervention 31 min per day, each school day, over the course of 12 weeks. Parent and teacher ratings of ADHD symptoms (inattention, hyperactivity/impulsivity), oppositional behavior, moodiness, behavior toward peers, and reputation with peers, were used as dependent variables. Primary analyses indicate that the PA intervention was more effective than the SC intervention at reducing inattention and moodiness in the home context. Less conservative follow-up analyses within ADHD status and intervention groups suggest that a PA intervention may reduce impairment associated with ADHD-risk in both home and school domains; interpretive caution is warranted, however, given the liberal approach to these analyses. Unexpectedly, these findings also indicate the potential utility of a before school SC intervention as a tool for managing ADHD symptoms. Inclusion of a no treatment control group in future studies will enable further understanding of PA as an alternative management strategy for ADHD symptoms.
This study examines whether positively biased self-perceptions relate to social behaviors in children with attention-deficit/hyperactivity disorder (ADHD) as compared to control children. The social behaviors of children with ADHD (n=87) were examined relative to control children (CTL; n=38) during a laboratory-based dyadic social interaction task. Children with ADHD were subgrouped into those with a positive illusory bias (PIB) in their self-perceptions (ADHD + PIB) versus those without such a bias (ADHD - PIB). Using a behavioral coding system adapted for this study, ADHD + PIB, ADHD - PIB, and CTL participants were compared on objectively coded social behaviors occurring within the context of the social interaction task. Whereas both ADHD groups displayed more disruptive behavior than controls, only the ADHD + PIB group displayed less prosocial behavior and less effortful behavior. This study breaks new ground by examining positively biased self-perceptions as they relate to social behavior in children with ADHD and provides promising new insight into the social problems experienced by these children.
This study examined effectiveness of a Group Curriculum (GC) for parents of 3- to 6- year-old children with disruptive behavior. The curriculum is based on the book Parenting the Strong-Willed Child. A total of 39 parents were randomly assigned to the GC condition or a wait-list control condition. Assessments occurred at baseline, postintervention (6 weeks after baseline), and 2-month follow-up. Findings indicated that the GC condition was associated with lower levels of child problem behavior and improved parenting at postintervention relative to the control condition. Parents were also satisfied with the intervention. Uncontrolled 2-month follow-up data suggested that changes were maintained from postintervention to follow-up for all outcome measures.
Objective: Physical activity associates with mental health and neurocognitive function, showing potential for addressing ADHD symptoms. As a preliminary assessment of this potential, the authors piloted a before-school physical activity intervention for young children. Method: Seventeen children (Grades K-3) exhibiting four or more hyperactivity/impulsivity symptoms on the Disruptive Behavior Disorders Rating Scale (Pelham, 2002) completed about 26 min of continuous moderate-to-vigorous physical activity daily over eight school weeks. The authors administered cognitive, motor, social, and behavioral functioning measures at pre- and postprogram, assessed response inhibition weekly, and coded negative behaviors daily. Results: Several measures showed significant or marginally significant change over time (effect size = 0.35-0.96) with additional measures showing meaningful effect size values (≥ 0.20). Response inhibition effects were most consistent. Most participants (64% to 71%) exhibited overall improvement according to postprogram parent, teacher, and program staff ratings. Conclusion: Physical activity shows promise for addressing ADHD symptoms in young children.
Children with Attention-Deficit/Hyperactivity Disorder (ADHD) are thought to have fundamental deficits in the allocation of attention for information processing. Furthermore, it is believed that these children possess a fundamental difficulty in motoric timing, an assertion that has been explored recently in adults and children. In the present study we extend this recent work by fully exploring the classic Wing and Kristofferson (1973) analysis of timing with typically developing children (n=24) and children with ADHD (n=27). We provide clear evidence that not only do children with ADHD have an overall timing deficit, they also time less consistently when using a similar strategy to typically developing children. The use of the Wing and Kristofferson approach to timing, we argue, will result in the discovery of robust ADHD-related timing differences across a variety of situations.
The purpose of this study was to address the following question: Why do parents first seek help with parenting when they report their child’s disruptive behaviors are within normative levels? Two groups were selected for study from a sample of 91 parents of 3- to 6-year-olds who sought help with parenting: Parents reporting disruptive behavior problems in the normative range ( n = 22) or above a clinical cutoff ( n = 19). Logistic regression was used to examine two competing hypotheses about family variables which may account for why parents seek help when reporting normative levels of disruptive behaviors: Alternative family stress (i.e., high levels of family stress other than child disruptive behavior) and parent enhancement and validation (i.e., enhance and validate parenting although child behavior is in normal range). No support emerged for the alternative family stress hypothesis whereas multiple indicators (better use of disciplinary strategies, seeking services for a first born child, and being more highly educated) provided support for the parent enhancement and validation hypothesis.