Rethinking the Accessibility of Hearing Assessments for Children with Developmental Disabilities.

We aim to determine the accessibility of gold-standard hearing assessments - audiogram or auditory brainstem response (ABR) - during the first 3 months of hearing health care for children with and without developmental disabilities. Electronic health records were examined from children (0-18 years) who received hearing health care at three hospitals. Children with developmental disabilities had a diagnosis of autism, cerebral palsy, Down syndrome, or intellectual disability. Assessments from the first 3 months were reviewed to determine if ≥ 1 audiogram or ABR threshold was recorded. To evaluate differences in assessment based on disability status, logistic regression models were built while accounting for age, race, ethnicity, sex, and site. Of the 131,783 children, 9.8% had developmental disabilities. Whereas 9.3% of children in the comparison group did not access a gold-standard assessment, this rate was 24.4% for children with developmental disabilities (relative risk (RR) = 3.79; p < 0.001). All subgroups were at higher risk relative to the comparison group (all p < 0.001): multiple diagnoses (RR = 13.24), intellectual disabilities (RR = 11.52), cerebral palsy (RR = 9.87), Down syndrome (RR = 6.14), and autism (RR = 2.88). Children with developmental disabilities are at high risk for suboptimal hearing evaluations that lack a gold-standard assessment. Failure to access a gold-standard assessment results in children being at risk for late or missed diagnosis for reduced hearing. Results highlight the need for (1) close monitoring of hearing by healthcare providers, and (2) advancements in testing methods and guidelines.

Identifying reduced hearing in children who have developmental disabilities: Insights for inclusive research practices with electronic health records.

Introduction: Recent advancements in big data analytics and the formation of large-scale clinical data repositories provide a unique opportunity to determine the current state of pediatric hearing health care for children who have developmental disabilities. Before answering unresolved questions about diagnostic practice, it is paramount to determine a standard and reliable method for identifying children who have reduced hearing because clinical management is affected by hearing status. The purpose of this study was to compare 5 different methods for identifying cases of reduced hearing from pure-tone thresholds based on developmental disability status. Methods: Using retrospective clinical data from 100,960 children (0-18 years), hearing status was determined for a total of 226,580 encounters from three clinical sites. 9% of the children had a diagnosis of intellectual disability, autism spectrum disorder, Down syndrome, or cerebral palsy. Results: Results revealed that encounters from children who have developmental disabilities were more likely to have insufficient data to allow hearing status to be determined. Moreover, methods with higher data demands (i.e., number of thresholds and ear-specific thresholds) resulted in fewer classifiable encounters. The average child age when hearing status was classified for the first time was older for children who have developmental disabilities than for children in the comparison group. Allowing thresholds to build up over multiple test sessions did result in more children who have developmental disabilities being classified than for single-encounter methods, but a meaningful decrease in child age at the time of classification was not seen for this strategy. Compared to the comparison group, children who have developmental disabilities were more likely to have reduced hearing that was stable over time, yet their hearing status was determined at older ages. Discussion: Results provide key guidance to researchers for how to determine hearing status in children for big data applications using electronic health records. Furthermore, several assessment disparities are spotlighted for children who have developmental disabilities that warrant further investigation.

Technology assisted language intervention (TALI) for children who are deaf/hard of hearing: promising impact on pragmatic skills.

Children who are deaf and hard of hearing (DHH) often demonstrate pragmatic language difficulties which can impact academic and social outcomes. This randomized control trial for DHH children, ages 3-12 years, explored the Technology-Assisted Language Intervention (TALI), incorporating augmentative and alternative communication technology (AAC) into traditional speech/language therapy, compared to treatment-as-usual (TAU) to determine impact on pragmatics. Pragmatic outcome measures included parent reported Pragmatics Profile of the CELF-5 (for children age ≥5 years) and CELF-P Descriptive Pragmatics Profile (for children <5 years) in addition to parent reported Social and Communication domains of the Vineland Adaptive Behavior Scales,Third Edition (VABS). Over 24 weeks, children ≥5 years in the TALI made significantly more progress (increase in raw scores) on the Pragmatics Profile compared to children in TAU (12.7 points vs. -6.0 points; p = 0.04) and also showed significant gains on two of the three subscales. For children ≥5 years, no significant VABS changes were seen in either intervention group. For children <5 years, there were no statistically significant differences in growth on the CELF-P total pragmatics raw score or on any subdomain. However, children in TALI had significant increases in the mean VABS Communication (86.7-99.1) and Social domain standard scores (91.8-97.4;p = 0.01), while gains for children in TAU on the Communication and Social domain standard scores were not statistically significant. These promising results support the need for additional research exploring the effectiveness of AAC supported speech/language therapy to enhance DHH children's pragmatic language skills. Trial registration: ClinicalTrials.gov identifier: NCT02998164.

Visual Reinforcers Designed for Children with Developmental Disabilities.

A library of visual reinforcers has been created to facilitate visual reinforcement audiometry (VRA) testing in children with developmental disabilities. The library includes 45 reinforcer sets-photos or videos grouped by a common theme-that were created based on commonly reported interests of children with developmental disabilities. Each reinforce set contains a minimum of 20 unique photo or video files that can be downloaded in two formats: one for commercially available VRA reinforcement systems and another for a custom setup. The library is freely available for download online under a Creative Commons License (Creative Commons Attribution-NonCommercial 4.0 International License). Use of these materials has the potential to improve behavioral testing outcomes for children with developmental disabilities, including children with restricted interests. Future research is needed to determine the effectiveness of implementing these materials in clinical settings.

Relational Factors in Pragmatic Skill Development: Deaf and Hard of Hearing Infants and Toddlers.

In this article, we review relational factors in early childhood believed to contribute in unique ways to pragmatic skill development in deaf and hard of hearing (DHH) infants and toddlers. These factors include attending to infant interactions with caregivers and others, supporting development of theory of mind through play and use of mental state language (ie, describing one's own or others' thoughts, feelings, and beliefs), and providing accessible opportunities for social interaction. On the basis of a review of the literature and clinical experience, we offer prescriptive strategies for supporting DHH children's development in these areas. To improve outcomes for DHH children, medical care providers and allied health professionals have a responsibility to support the development of young DHH children's pragmatic abilities by understanding these variables, coaching caregivers regarding their importance, and facilitating referrals for support when necessary.

The Assessment of Pragmatic Skills in Young Deaf and Hard of Hearing Children.

The development of pragmatic skills does not often receive attention by professionals who are recommending or undertaking assessment of deaf and hard of hearing (DHH) children, yet social communication is vital for linguistic, social, emotional, and academic development. We acknowledge the challenges that DHH children have with pragmatic skills, advocate for monitoring of pragmatic development for all DHH children by medical professionals, and provide direction for assessment of pragmatic skills in young DHH children, particularly for clinicians and teachers who are tasked with that work. Pragmatic assessment is challenging because it must involve observations of the child in interaction with a communication partner, either directly during a specific interaction or through the reflections of a familiar adult. In this article, we recommend two complementary assessment procedures for young DHH children who use spoken language. Assessment 1 recommends that a parent or caregiver completes The Pragmatic Checklist to provide a picture of the child's functional communication. In assessment 2, the information gained through the checklist is complemented by using direct observations of a child in interaction with an adult or a peer. The Pragmatic Protocol uses a video-recorded conversation sample between the child and familiar person that is analyzed by a DHH professional for 30 different pragmatic behaviors. We conclude this article with a recommendation for pediatricians and health care professionals to monitor pragmatic developmental milestones in DHH children, to refer them for pragmatic assessments, and to collaborate with researchers to develop valid, reliable tools that adequately capture the pragmatic skill strengths and needs of DHH children.

Using Visual Supports to Facilitate Audiological Testing for Children With Autism Spectrum Disorder.

Purpose One in 59 children is diagnosed with autism spectrum disorder (ASD). Due to overlapping symptoms between hearing loss and ASD, children who are suspected of having ASD require an audiological evaluation to determine their hearing status for the purpose of differential diagnosis. The purpose of this article is twofold: (a) to increase audiologists' knowledge of ASD by discussing the challenges associated with testing and interpreting clinical data for children with ASD or suspected ASD and (b) to provide visual supports that can be used to facilitate audiological assessment. Method Eight children (ages 4-12 years) were recruited as video model participants. Videos were filmed using scripts that used concise and concrete language while portraying common clinical procedures. Using the video models, corresponding visual schedules were also created. Conclusion Although obtaining reliable hearing data from children with ASD is challenging, incorporating visual supports may facilitate testing. Video models and visual schedules have been created and made freely available for download online under a Creative Commons License (Creative Commons-Attribution-NonCommercial-ShareAlike 4.0 International License). Incorporating visual supports during clinical testing has the potential to reduce the child's and family's stress, as well as to increase the probability of obtaining a reliable and comprehensive audiological evaluation. Future research is warranted to determine the effectiveness and feasibility of implementing these tools in audiology clinics. Supplemental Material https://doi.org/10.23641/asha.10086434.

A summary of current understanding regarding children with autism spectrum disorder who are deaf or hard of hearing.

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.

Clinical use of the autism diagnostic observation schedule-second edition with children who are deaf.

The Autism Diagnostic Observation Schedule-Second Edition (ADOS-2) was administered to eight children who are deaf and who are native American Sign Language (ASL) users with previous autism spectrum disorder (ASD) diagnosis. Classification on two different module selection criteria was compared based on: (1) standardized administration rules (signs not counted as equivalent to words) and (2) commonly utilized clinical administration (sign language complexity treated equivalently to spoken language complexity). Differential module selection resulted in discrepant classification in five of the eight cases (63%) and suggests that ADOS-2 via standardized test administration may result in a failure to identify autism among children who are deaf with primary communication in ASL. Two of the eight children (25%) did not exceed the cutoff for an ASD classification on either module administered despite previous ASD diagnosis. Overall results suggest that caution should be used when utilizing the ADOS-2 with children who are deaf who primarily communicate using ASL.