A strikingly large number (~40%) of children with hearing loss have co-existing developmental disabilities, with the most frequent co-existing diagnosis being cognitive disability. The dual diagnosis of hearing loss and cognitive disability places this special subset of children at enormous risk for life-long disability. Additionally, despite receiving numerous and highly varied interventions for language and communication, children with dual diagnosis have language outcomes disproportionately poorer than anticipated. With a long term objective of improving language and communication outcomes in children with both hearing loss and cognitive disabilities, this proposal will thoroughly characterize the interventions and therapies that this complex population receives so we may begin to evaluate their efficacy. This study proposes a prospective case-control design with a unique cohort of 120 children falling into one of three groups: (a) moderate to profound sensorineural hearing loss with normal cognitive abilities; (b) moderate to profound sensorineural hearing loss with cognitive disabilities (dual diagnosis); and (c) normal hearing with cognitive disabilities. Children will receive standardized neurocognitive, language, and functional auditory assessments. The novel characterization of
intervention strategies for language and communication in this population of children is the first step towards identifying components of language-based intervention that have positive (or negative) impact on overall communication. The current level of evidence (or lack thereof) makes it incredibly challenging to effectively clinically manage this population. This study will fill this critical gap in evidence that impacts the effectiveness of our clinical management. Further, it will create the foundation by which effective evidence-based and targeted interventions can be designed. This project brings together perspectives from multiple disciplines: developmental pediatrics, audiology, otolaryngology, neuropsychology, speechlanguage pathology, and epidemiology- biostatistics. By utilizing the already existing multicollaborative and interdisciplinary infrastructure, findings from this research project will meet urgent clinical and research needs; by providing new knowledge regarding biologic mechanisms and intervention strategies underlying language development of children with hearing loss and cognitive disabilities. This knowledge may be used towards the earlier identification (and earlier intervention) of children at highest risk for the poorest language. It is anticipated that the results from this study will impact current clinical paradigms regarding children with dual diagnosis of hearing loss and cognitive disabilities. Novel therapeutic approaches should consider how to maximize successful language and communication skills that are appropriate for a child's cognitive level. This study is responsive to MCHB strategic research issue #IV in promoting the healthy development of MCH populations particularly children with special health care needs; and strategic research issue #III through the exploration of the quality of intervention care for children with hearing loss and cognitive disabilities. This study also addresses the Healthy People 2010 goal of producing findings to improve health and well-being of people with disabilities.
Listed is descending order by year published.
Meinzen-Derr J, Wiley S, Bishop S, Manning-Courtney P, Choo DI, Murray D. Autism spectrum disorders in 24 children who are deaf or hard of hearing. Int J Pediatr Otorhinolaryngol. 2014;78(1):112-18.
Meinzen-Derr J, Wiley S, Grether S, et al. Functional communication of children who are deaf or hard-of-hearing. J Dev Behav Pediatr. 2014;35(3):197-206.
Phillips J, Wiley S, Barnard H, Meinzen-Derr J. Comparison of two nonverbal intelligence tests among children who are deaf or hard-of-hearing. Res Dev Disabil. 2014 Feb;35(2):463-71.
Meinzen-Derr J, Wiley S, Grether S, Choo DI. Functional performance among children with cochlear implants and additional disabilities. Cochlear Implants Int. 2013 Feb 9. [Epub ahead of print] PubMed PMID: 23407115.
Hart C, Wiley S, Choo D, et al. Developmental disabilities and intracranial abnormalities in children with symptomatic cytomegalovirus and cochlear implants. International Scholarly Research Network Otolaryngology. 2012; Article ID 502746.
Wiley S, Meinzen-Derr J, Grether S, Choo DI, Hughes ML. Longitudinal functional performance among children with cochlear implants and disabilities: a prospective study using the Pediatric Evaluation of Disability Inventory. Int J Pediatr Otorhinolaryngol. 2012 2012 May;76(5):693-7.
Wiley S, Meinzen-Derr J. Use of the ages and stages questionnaire in young children who are deaf/hard of hearing as a screening for additional disabilities. Early Human Development.2012 Nov 6. [Epub ahead of print] PubMed PMID: 23141381.
Special Health Care Needs, Developmental Disabilities, Cognitive & Linguistic Development, Health Care Quality