Litovsky, Ruth Y PHD

The proposed supplement is relevant to the INCLUDE project, in which the parent grant focuses on adults with Down Syndrome (DS), specifically, associations between hearing status, auditory function, cognition, language and brain imaging through functional and structural approaches. This supplement aims to collect pilot data in children with DS, as there is a paucity of auditory studies in this population, with known incidence of hearing loss in infants and children with being much higher than in the general population. This supplement will provide pilot data in children with DS, establishing feasibility for a new parent R01 on auditory function and its association with cognition, language and brain structure and function in children with DS.

Down syndrome (DS) is a leading known cause of intellectual disability and a highly recognized genetic syndrome that involves multiple medical co-morbidities; hearing deficits in DS are estimated to occur at a rate of 80-90% and are thought to be caused by a combination of structural and functional abnormalities in the external and/or inner ear. This project aims to tackle a timely and significant question regarding the role of hearing loss in DS on auditory function, cognition, language, and structural integrity of brain regions that are important for hearing, meeting the programmatic objectives of INvestigation of Co-occurring conditions across the Lifespan to Understand Down syndrome. Results will provided information regarding clinical diagnosis and intervention to facilitate treatment of hearing disorders in individuals with DS, and more long-term impact will be identification of measures that may be used to evaluate effectiveness of pharmaceutical and therapeutic trials.

This research is aimed at advancing knowledge in auditory science, focusing on binaural hearing in deaf individuals who use bilateral cochlear implants. We aim to better understand what conditions are likely to maximize the success under conditions that emulate noisy environments. The novelty of our approach is the new focus on both speech intelligibility measures and pupil dilation as a measure of listening effort. Findings will have a direct translational component, with regard to development of novel sound coding strategies, rehabilitation and counseling.