Hyperacusis and Phonophobia in Williams Syndrome

Abstract

Event Abstract Back to Event Hyperacusis and Phonophobia in Williams Syndrome O. Zarchi1*, J. Attias1 and D. Gothelf1, 2 1 Schneider Children's Medical Center of Israel, The Behavioral Neurogenetics Center, Israel 2 Tel Aviv University, Sackler Faculty of Medicine, Israel Hyperacusis and phonophobia are among the most salient features of Williams syndrome (WS) and affect the life of most children carrying the syndrome. We have investigated the clinical characteristics and neuroaudiological pathways mediating the hyperacusis in 49 children and adolescents with WS. Compared to controls, subjects with WS showed 20dB lower discomfort level thresholds, high-frequency cochlear hearing, distortion products otoacoustic emission, higher prevalence of ipsilateral acoustic reflex (AR) absence, and prolonged wave I latency of the BAER. Subjects with WS also showed increased suppression effect of the MOC reflex. The findings of the study suggest that hyperacusis in WS is associated with a highfrequency hearing loss resembling the configuration of noise induced hearing loss. The hyperacusis and hearing loss in WS may stem from a deficiency in AR, resulting from auditory nerve dysfunction. Hyperexcitability of the medial olivocochlear efferent system coupled with AR absence further enhance hyperacusis in subjects with WS. Additionally, we present preliminary results suggesting abnormal central auditory processing in WS as evident by mismatch negativity ERP findings. Conference: 12th International Professional Conference on Williams Syndrome, Garden Grove,CA, United States, 13 Jul - 14 Jul, 2008. Presentation Type: Oral Presentation Topic: SESSION 5: Anxiety and Other Psychiatric Problems Citation: Zarchi O, Attias J and Gothelf D (2009). Hyperacusis and Phonophobia in Williams Syndrome. Conference Abstract: 12th International Professional Conference on Williams Syndrome. doi: 10.3389/conf.neuro.09.2009.07.017 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 30 Apr 2009; Published Online: 30 Apr 2009. * Correspondence: O. Zarchi, Schneider Children's Medical Center of Israel, The Behavioral Neurogenetics Center, Petach Tikvah, Israel, omer_zar@yahoo.com Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers O. Zarchi J. Attias D. Gothelf Google O. Zarchi J. Attias D. Gothelf Google Scholar O. Zarchi J. Attias D. Gothelf PubMed O. Zarchi J. Attias D. Gothelf Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.