title: Audiometric characteristics of USH2a patients.
abstract: Temporal processing, frequency discrimination and frequency resolution, three basic mechanisms involved in speech perception, were studied in 11 USH2a patients from 10 Dutch families. Measurements included loudness scaling, gap detection, determination of auditory filter shapes and difference limen for frequency. The results were compared to values obtained from listeners with normal hearing, DFNA8/12 patients, DFNA13 patients and unselected patients with presbyacusis. Overall, the results for USH2a were most similar to those obtained in patients with sensorineural hearing loss caused by hair cell defects. To our knowledge, this is the first study that presents results on audiometric measurements combined with psychophysical measurements in a group of USH2a patients.
authors.affiliation: Department of Otorhinolaryngology/Audiology, Radboud University Medical Centre Nijmegen, Nijmegen, The Netherlands. j.leijendeckers@kno.umcn.nl
authors.forename: Joop M
authors.initials: JM
authors.lastname: Leijendeckers
authors.forename: Ronald J E
authors.initials: RJ
authors.lastname: Pennings
authors.forename: Ad F M
authors.initials: AF
authors.lastname: Snik
authors.forename: Arjan J
authors.initials: AJ
authors.lastname: Bosman
authors.forename: Cor W R J
authors.initials: CW
authors.lastname: Cremers
mesh_terms: D000328:Adult; D001299:Audiometry; D016326:Extracellular Matrix Proteins / Q000235:genetics*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D008144:Loudness Perception; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011304:Presbycusis / Q000175:diagnosis / Q000503:physiopathology; D011571:Psychoacoustics; D013067:Speech Perception; D052245:Usher Syndromes / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology
chemical_list: D016326:Extracellular Matrix Proteins; C112735:USH2A protein, human