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