1. Optimizing Auditory Development in Infants with Hearing Loss and Cognitive Disability Kathryn Arehart, Ph.D. 1, Christine Yoshinaga-Itano, Ph.D. 1 and Sandra Gabbard, Ph.D. 2 1 Speech, Language and Hearing Sciences, UCB 2 Department of Otolaryngology, UCHSC Hearing Loss and Cognitive Disability Hearing loss is common in children with cognitive delays. Of the 12,000 children born each year with hearing loss, 40% will have multiple disabilities and 26% will have developmental delays or significant learning disabilities. More than 75% of children with Down syndrome have hearing loss (Pueschel, 1987). Traditionally: Late Identification, Intervention; Delayed Language Development Age of identification of hearing loss has traditionally been 18 months of age. Start of intervention was even later. In children with cognitive disabilities, hearing loss has often been considered a secondary disability. For these children, fitting of hearing aids and intervention services for habilitation of speech, language and auditory skills have been delayed. Children with hearing loss, especially those with low cognition, have had poor outcomes in terms of language development. Earlier Identification and Intervention Recent advances in technology allow for effective screening for hearing loss in newborns. Since 1999, Colorado has had systems in place to screen hearing in all newborns. Currently, 99% of all infants born in Colorado are screened for hearing loss prior to hospital discharge. Children are now identified with hearing loss and enrolled in intervention before six months of age. Early identification and early intervention of hearing loss in newborns (by 6 months) leads to significantly better language development in hard-of-hearing children with and without cognitive disabilities. The figure below (Yoshinaga-Itano, Sedey, Coulter, & Mehl, 1998) illustrates these improved outcomes. (Language quotients below 80 are delayed). Intervention Improved outcomes depend on appropriate and timely early intervention. This intervention usually begins with the fitting of appropriate amplification (hearing aids) and assistive technologies. Current Needs Laboratory-based studies (e.g., Nozza et al, 1991) have shown infants with normal hearing and normal cognitive development can discriminate phonetic speech differences (e.g., /s/ vs. /sh/; /u/ vs /i/) in both quiet and noisy environments. These types of fine speech discrimination are essential for the development of intelligible speech in hard-of-hearing children. Therefore, a primary goal of hearing aids in young infants is to give them the audibility necessary to perceive these fine speech discriminations. No tools exist for assessing speech perception in young infants with hearing loss. Our Current Work Our goal is to develop tools and technologies for the assessment of speech perception among hard-of-hearing infants with and without cognitive disabilities. These tools and technologies will, in turn, lead to better fitting of amplification technology and to more optimal auditory development in hard- of-hearing infants with and without cognitive disabilities. Mean Language Quotient at 31-36 Mo.