More Than Hearing Loss

More than 40% of children with hearing loss (HL) also display additional disabilities, placing them at serious risk for life-long communication impairment. Our previous work indicates that children with the dual diagnosis of hearing loss and a cognitive disability have language outcomes dramatically poorer than expected. Reasons behind the negative synergy between HL and cognitive disabilities are unclear and result in suboptimal management. Effective intervention is required to ensure children with HL reach their full potential. However, rigorous evaluation of language-based intervention strategies for children with dual diagnosis has not occurred.

Study objective

Our long term goal is to improve clinical language and communication outcomes in children with both hearing loss and cognitive or intellectual disabilities. The objective of this study is to determine the effect of cognitive abilities and hearing loss on language and thoroughly characterize the language-based intervention strategies.

Study Participants

We are recruiting three groups of children (age 3-6 years) who have:

1. Hearing loss with normal cognitive abilities.
2. Hearing loss with low cognitive abilities or with intellectual disabilities (dual diagnosis).
3. Normal hearing with low cognitive abilities.

All degrees of permanent bilateral hearing loss are included in this study.

Improving functional outcomes early in life will help improve transition to adolescence and adulthood with less reliance on services and supports. However, research to improve functioning has been focused on how hearing loss affects auditory, speech, and language outcomes. It seems logical to assume that improvement in these domains will translate to improvement in functional performance; however our preliminary data suggest that the functional outcomes in children who are deaf or hard-of-hearing are below what is predicted. Unfortunately, there is a lack of studies researching factors influencing functional performance and day-to-day activities of children who are deaf or hard of hearing.

Study Objective

This project seeks to understand the specific nature of language and nonverbal cognitive abilities in children who are deaf or hard-of-hearing. Project participants will include individuals with and without cognitive impairment. We will observe their day-to-day functional skills and participation in daily life activities at home and at school.

The novel characterization of functional skill acquisition in this population of children is the first step towards identifying critical periods of development for targeted intervention strategies.

The current level of evidence (or lack thereof) regarding functional skills in children with hearing loss is stifling progress towards effective interventions.

This study will fill a critical gap in evidence that impacts the effectiveness of our management, interventions, and counseling of families. Further, it will create the foundation by which effective evidence-based and interventions targeting functional independence can be designed.

This project brings together perspectives from multiple disciplines: special education, deaf education, developmental pediatrics, audiology, otolaryngology, neuropsychology, speech-language pathology, and epidemiology- biostatistics.

Knowledge from this study may be used towards the earlier identification (and earlier intervention) of children at high risk for poor outcomes. Novel therapeutic approaches should consider how to maximize successful language, communication, and functional skills that are appropriate for a child’s cognitive abilities.

Study Participants

This study will enroll children (n=60) with bilateral permanent hearing loss, with and without cognitive impairment. The evaluation of children with normal hearing (n=30) with similar cognitive abilities provides a comparison group allowing researchers to understand the effects of hearing loss on functional skills.

Children are followed for 2 years and receive standardized neurocognitive, language, and functional-adaptive skill assessments, including parent and teacher-report measures at yearly intervals.

Autism spectrum disorders (ASDs) impact as many as 10% of children with hearing loss, yet no validated screening or diagnostic ASD-specific tools exist for this population. Despite improved screening and identification of ASD in the general population, children with permanent hearing loss (PHL) continue to experience delayed or missed identification of a co-existing ASD. The scarcity of valid or adapted tools is a barrier to receiving appropriate ASD-specific intervention services for children with PHL.

If we hope to provide early identification and intervention opportunities for all children with ASD, we must prioritize accurate screening and diagnostic tools for all children. Valid and reliable diagnostic instruments with high sensitivity and specificity will dramatically reduce the time and expense currently required to determine diagnostic status of children with PHL.

Study Objective

The overall objective of this project is to assess the validity of specific ASD assessments in a population of children with PHL. This project will have significance in the field by documenting the accuracy of current screening and evaluation measures for children with PHL and subsequently facilitating more rapid diagnosis of ASD among children with PHL.

A major strength of this proposal is our ability to recruit children with dual diagnosis of PHL and ASD. We will work with the ASD experts in the Autism Treatment Network, which include developmental pediatricians, speech language pathologists and psychologists. We’ll also work with expert clinicians in the Ear and Hearing Center who understand the development of children with PHL.

This line of research is innovative because it focuses on a novel population of children for whom no consistent strategy exists for diagnosing ASD. These findings will advance science by providing researchers methods for identifying and hence studying children with the dual diagnosis of hearing loss and ASD at younger ages, which could impact genetic, biomedical, and intervention research.

Study Participants

We will recruit children ages 3-12 years who have a permanent hearing loss and have been diagnosed with ASD. We will also recruit children with permanent hearing loss who have no additional disabilities. Children will receive age-appropriate screening tools and diagnostic assessments.