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.

Enlarged Vestibular Aqueduct and Cochlear Implants: The Effect of Early Counseling on the Length of Time Between Candidacy and Implantation.

OBJECTIVE: To determine if discussing cochlear implantation (CI) with patients with enlarged vestibular aqueducts (EVA) and their families before reaching audiological criteria for CI candidacy effects the length of time between reaching audiological candidacy and CI surgery, and to describe the universal newborn hearing screening (UNHS) results and communication modality in this sample. PATIENTS: Forty-two patients (25 females) with confirmed EVA and cochlear implants. INTERVENTION(S): Diagnostic CI visit. MAIN OUTCOME MEASURES: The primary outcome measure is the difference in length of time between reaching audiological candidacy for CI and surgical implantation between those who had preliminary discussions regarding CI with their medical and healthcare providers before reaching audiological candidacy versus who had discussions after reaching candidacy. The secondary outcome measure is the result of the UNHS and primary mode of communication used by each patient. RESULTS: Discussing CI before reaching audiological candidacy was associated with a significantly shorter duration between reaching audiological candidacy and receiving CI (median = 3.1 mo; interquartile range [IQR] = 1.7-5.4) as compared with discussing CI after reaching candidacy (median = 5.8 mo; IQR = 3.2-11.2; p = 0.012). Participants born after the implementation of the UNHS, 16 of 24 patients referred on one or both ears. Communication modalities were evenly divided between utilizing sign-support English and oral/aural communicators only. CONCLUSIONS: Discussion of CI in patients with EVA before reaching audiological candidacy reduces the amount of time the child is without adequate auditory access and contributes to a constructive and interactive preparatory experience.

Dually diagnosed: a retrospective study of the process of diagnosing autism spectrum disorders in children who are deaf and hard of hearing.

Utilizing a retrospective chart review of 30 children who have been dually diagnosed with hearing loss and autism spectrum disorders (ASDs), this study explores the process of arriving at the diagnosis of ASD in this population. Factors of interest include the age of ASD diagnosis in children who are deaf and hard of hearing, the types of professionals involved in making the diagnosis, and the measures used for assessment. Complications in the diagnostic process are highlighted.

Prognosis tool based on a modified children's implant profile for use in pediatric cochlear implant candidacy evaluation.

OBJECTIVES: We developed a prediction tool to assist in evaluation of pediatric candidates for cochlear implantation (CI) and to help plan for preoperative and postoperative support. METHODS: Between 1995 and 2005, 277 patients underwent CI at Children's Hospital Boston. Of these 277 patients, 250 had at least 2 years of post-CI follow-up and adequate pre-CI information for rating by our prediction tool. Of the 250, 106 were randomly selected for inclusion. The patients were divided into group A (auditory/oral communicator); group B (auditory/oral communicator with visual assistance), group C (visual/manual communicator with auditory/oral skills assistance), and group D (will not derive communicative benefit from implant). Predictions were performed with clinical assessment and two statistical techniques: regression modeling and classification and regression tree (CART) analysis. RESULTS: Among patients who became auditory/oral communicators (group A), clinical assessment predicted that outcome accurately 65% of the time, CART analysis had intermediate sensitivity (79%), and regression modeling was the most sensitive (95%). Groups B through D were predicted 45% of the time by regression modeling, 90% of the time by clinical assessment, and 100% of the time by CART analysis. CONCLUSIONS: A combination of speech-language, medical, and educational constructs can provide a reliable prediction of the communication outcome. Our goal for the prognosis tool is to make it part of the overall candidacy process in supporting decision-making about CI and planning for post-CI therapy.

Not a "sound" decision: is cochlear implantation always the best choice?

OBJECTIVE: To review the candidacy criteria used to counsel parents of profoundly deaf children, to determine if these criteria have changed over time, and to evaluate eventual communication outcomes for these patients. DESIGN: Retrospective review of 483 pediatric cochlear implant candidates from September 1995 to December 2006 seen at a tertiary care pediatric hospital. RESULTS: Out of 483 implant candidates, 191 patients were initially felt not to be favorable candidates based on CI team evaluation. Of this group, 3 had insufficient records to review and were excluded. The remaining 188 patients underwent a detailed analysis of specific possible contraindications to implantation. This included audiologic, medical and psychosocial parameters. The data was divided into two time periods: Group 1 included 44 patients from 1995 to 2000, and Group 2 included 144 patients from 2001 to 2006. In Group 1, there was a higher percentage of children with language deprivation and developmental concerns and patients not ready, compared to Group 2 which had a higher percentage of families not ready and inadequate support systems. Group 1 had a higher percentage of patients who ultimately underwent cochlear implant, but otherwise the two groups were largely similar. CONCLUSION: Analysis of our data showed that the degree of concern that the cochlear implant team has in relationship to specific candidacy criteria has changed over time. Recommendations against a cochlear implant were often revisited after initial concerns were addressed. The use of a team approach, in conjunction with a validation tool, is important for establishing criteria for successful cochlear implantation in children to support appropriate counseling of patients and families and to plan post-implant management.