Journal article
Auditory Dysfunction Among Individuals With Neurofibromatosis Type 1
G Rance, J Zanin, A Maier, D Chisari, KM Haebich, KN North, G Dabscheck, ML Seal, MB Delatycki, JM Payne
JAMA Network Open | Published : 2021
Abstract
IMPORTANCE Neurofibromatosis type 1 (NF1) affects hearing through disruption of central auditory processing. The mechanisms, functional severity, and management implications are unclear. OBJECTIVE To investigate auditory neural dysfunction and its perceptual consequences in individuals with NF1. DESIGN, SETTING, AND PARTICIPANTS This case-control study included children and adults with NF1 and control participants matched on age, sex, and hearing level. Patients were recruited through specialist neurofibromatosis and neurogenetic outpatient clinics between April and September 2019. An evaluation of auditory neural activity, monaural/binaural processing, and functional hearing was conducted. ..
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Awarded by U.S. Department of Defense
Funding Acknowledgements
The work was supported by Barney Fellowship from the Children's Tumour Foundation, awarded to Ms Maier; a Murdoch Children's Research Institute Clinician Scientist Fellowship awarded to Dr Payne; and HEARing Cooperative Research Center (2014-19) project support (XR1.2.2) awarded to Dr Rance. Part of this work was also supported by award Investigator Initiated Research Award W81XWH-15-1-0619 from Neurofibromatosis Research Program of the US Department of Defense and the Joyce Melrose Crickmer Fund.