Diagnostic Utility of Parent Ratings on the Behavior Assessment System for Children-Third Edition in Children who are Deaf and Hard of Hearing and Diagnosed with Autism Spectrum Disorder.

Between 1 to 2 of every 1,000 children are born deaf or hard of hearing (DHH) and, of those, 30-50% have additional disabilities, including Autism Spectrum Disorder (ASD). Most measures assessing ASD characteristics rely on some degree of behavioral response to sound (e.g., responding to name, listening response), and may not be appropriate for use with children who are DHH. Further, ASD specific measures do not provide information on a child's functional abilities across developmental domains. We conducted a cross-sectional analysis comparing mean T-scores on a standardized multidimensional measure, the Behavior Assessment System for Children, Third Edition, Parent Rating Scale (BASC-3 PRS), across three groups matched for age and sex: children who are DHH and diagnosed with ASD (DHH + ASD; n = 16); children who are DHH without ASD (DHH-ASD; n = 16); and children who are typically hearing with ASD (H + ASD; n = 16). Analyses revealed statistically significant differences across scales of Attention Problems, Atypicality, Withdrawal, Behavioral Symptoms Index, Social Skills, Leadership, Functional Communication, Activities of Daily Living, Adaptive Skills, Autism Probability Indices, and Developmental Social Disorders. Pairwise comparisons showed DHH + ASD and H + ASD mean T-scores were statistically similar and distinct from DHH-ASD mean T-scores on all these scales except for Withdrawal, Leadership, Functional Communication, and Activities of Daily Living, where pairwise comparisons varied. The findings add to the literature on ASD and DHH children and call for further exploration of the BASC-3 as a tool for both evaluation of ASD and the development of individualized treatment plans in this unique population.

Cross-Sectional Analysis of Caregiver-Reported Expressive Language Profiles and Associated Covariates in Individuals with Down Syndrome.

OBJECTIVE: To describe the distribution of expressive language abilities of individuals with Down syndrome (DS) in a clinical sample and characterize demographic, environmental, and medical factors associated with varying expressive language profiles. METHODS: Cross-sectional analysis was completed on a sample of 345 individuals with DS between the ages of 4 and 22 years who were enrolled into a longitudinal clinical database between March 2018 and August 2021. Expressive language-related items on a standardized caregiver-reported questionnaire assessing domains of functioning in neurodevelopmental disorders were used to conduct latent variable modeling and determine caregiver-reported expressive language (CREL) classes across the sample. Linear regression was used to explore associations between CREL classes and predictor variables. RESULTS: Latent variable modeling revealed 3 distinct classes of CREL abilities representing higher, middle, and lower CREL. Individuals in the lower CREL class were more likely to be female, to use sign language or visual communication systems, have reduced pronunciation, attend private or residential school, and to be in a substantially separate classroom. Membership was not predicted by complex medical histories or co-occurring neurodevelopmental diagnoses. CONCLUSION: Caregiver-reported expressive language abilities in a cohort of individuals with DS were variable, with most of the individuals belonging to higher or middle CREL classes, relative to one another. Additional studies are indicated to understand factors that predict higher expressive language ability and explore how to direct services to individuals who are at risk of more profound language delays.

Development and Implementation of a Curriculum for Pediatric Trainees on Caring for Children Who Are Deaf and Hard of Hearing: A Pilot Study.

INTRODUCTION: Children who are deaf or hard of hearing (DHH) are at risk for poor developmental outcomes related to incomplete language access. Evidence based interventions are available to improve early access to language. With a better understanding of caring for DHH children, pediatricians will be more prepared to work with families in improving outcomes for this population. To date, there are no formal curricula on educating pediatric trainees on childhood hearing differences. METHODS: The authors designed a novel pilot curriculum to educate pediatric trainees on caring for DHH children, including screening, diagnosis, signed languages, and hearing technologies. The curriculum was delivered to pediatric interns in a 1-hour seminar. Pre-lecture, immediate post-lecture, and 6-month post-lecture surveys were developed and conducted to evaluate the effectiveness of the curriculum. Descriptive statistics were used to determine differences in understanding concepts before and after the curricular intervention. RESULTS: A total of 55 residents participated in the curriculum over a 14-month study period from 2018 to 2019. There were significant differences in responses between the pre- and post- surveys related to residents' understanding of childhood deafness and their confidence in their ability to care for DHH children. CONCLUSION: Pediatric trainees gained an understanding of the challenges faced by DHH children and of the interventions that aim to provide them with access to language during the critical period of development. As a result, trainees will be in a better position to care for patients and their families after a new diagnosis of a hearing difference and guide them through early language-based interventions.

The Importance of Accessible Language for Development in Deaf and Hard of Hearing Children.

CASE: Brady is a 5-year-old boy who was seen in a multidisciplinary clinic for evaluation of deaf and hard of hearing children. Brady was born full-term after an uncomplicated pregnancy. He was referred for audiological evaluation after his newborn hearing screen and was diagnosed with a severe-to-profound bilateral sensorineural hearing difference at age 6 months. He has no other medical history.Brady was referred for developmental evaluation after completing his medical workup and cochlear implantation at an outside institution. No etiologic cause of his hearing difference was identified, and his diagnosis was presumed to be genetic and nonsyndromic. He had previously undergone right cochlear implantation at age 14 months and left cochlear implantation at age 23 months. Brady received speech and language therapy, with an emphasis on spoken language through early intervention, and met all motor and social milestones at appropriate times. Despite therapy, he continued to show delays in meeting language and communication milestones. Given concerns over persistent language delays after cochlear implantation, he underwent an interdisciplinary speech, language, and psychological evaluation at 3 years 4 months old. At the time of his evaluation, he was noted to have robust social skills but significantly delayed expressive and receptive language skills with language use limited to single words.After the initial evaluation, he was enrolled at a school for the deaf with instruction provided in both spoken English and American Sign Language. In follow-up evaluation at age 4 years 8 months, Brady was described as happy, cooperative, and eager to connect socially. It was noted that he had age-appropriate visual spatial cognitive and motor skills and had made some gains compared with prior assessments in both spoken and sign language. Notably, however, his language abilities and most areas of adaptive living skills remained below what would be expected by his developmental age and in some domains plateaued compared with prior assessments. He was able to produce some words and signs and responded to all prompts using only single words or signs and gestures. Brady's parents present today to your multidisciplinary clinic asking to understand why his language has not progressed further and to learn how they can help him reach his full potential.

Adverse Childhood Communication Experiences Associated With an Increased Risk of Chronic Diseases in Adults Who Are Deaf.

INTRODUCTION: This study explores adverse childhood communication experiences and its RRs for acquiring specific chronic diseases and mental health disorders in adults who are deaf and hard of hearing. METHODS: A cross-sectional design with snowball sampling was used to recruit adults who were deaf and hard of hearing and were born or became deaf in both ears before age 13 years. Patient-reported outcomes surveys in American Sign Language and English were disseminated to collect data about early life communication experiences with caregivers. Modified Poisson regression with robust SEs was used to calculate RR estimates and 95% CIs for all medical conditions with early life communication experiences as main predictors. RESULTS: Data collection occurred from May 2016 to July 2016, October 2016 to April 2018, and October 2018 to May 2019. The U.S. sample consisted of 1,524 adults who were born or became deaf early. After adjusting for parental hearing status and known correlates of medical conditions, poorer direct child-caregiver communication was significantly associated with an increased risk of being diagnosed with diabetes (RRR=1.12, 95% CI=1.01, 1.24), hypertension (RRR=1.10, 95% CI=1.03, 1.17), and heart disease (RRR=1.61, 95% CI=1.39, 1.87). Poor indirect family communication/inclusion increased risks for lung diseases (RRR=1.19, 95% CI=1.07, 1.33) and depression/anxiety disorders (RRR=1.34, 95% CI=1.24, 1.44). The absolute risk increase and number needed to harm are also reported. CONCLUSIONS: Outcomes data reported by patients who were deaf and hard of hearing demonstrated that poorer direct child-caregiver communication and ongoing exclusion from incidental family communication were associated with increased risks for multiple chronic health outcomes. Practices should consider developing and utilizing an adverse childhood communication screening measure to prevent or remediate language deprivation and communication neglect in pediatric patients who were deaf and hard of hearing.

Young Deaf Adults' Knowledge of Human Papillomavirus and Human Papillomavirus Vaccine's Effectiveness in Preventing Cervical, Anal, Penile, and Oral Cancer.

STUDY OBJECTIVE: To describe knowledge and risk perception of human papillomavirus (HPV) among deaf adults who use American sign language (ASL) comparison with hearing adults in the United States. DESIGN: Secondary HPV knowledge data for the deaf subset sample were drawn from the Health Information National Trends survey in ASL that was administered between 2015 and 2018. HPV knowledge data for the hearing subset sample were drawn from cycle 5 of the Health Information National Trends survey in English that was administered in 2017. SETTING: Surveys are a nationally based survey of deaf ASL users in the United States and a nationally based survey of hearing non-ASL users in the United States. PARTICIPANTS: The age of the deaf and hearing subset samples was determined on the basis of catchup vaccine eligibility criteria as outlined by the Centers for Disease Control and Prevention that recommends catchup vaccination in women, men who have sex with men, immunocompromised individuals, and those who identify as transgender. INTERVENTIONS AND MAIN OUTCOME MEASURES: We examined HPV, HPV vaccine, and HPV-related cancer knowledge in deaf and hearing subsets. RESULTS: Our sample consisted of 235 deaf and 115 hearing adults aged 18-26 years. Of the deaf participants 58% (136/235) reported knowledge of HPV compared with 84% (97/115) of hearing participants (P < .001). Hearing participants showed higher accuracy in risk perception of HPV relation to cervical cancer compared with deaf participants (P < .001). Hearing participants were more likely to have heard of the HPV vaccine as well as believe it is successful in preventing cervical cancer compared with deaf participants (P < .001). CONCLUSION: Deaf ASL users are less likely to have knowledge of HPV, virus-related cancer risk, and preventative vaccination compared with hearing peers.