title: Hearing aid fitting for visual and hearing impaired patients with Usher syndrome type IIa.
abstract: OBJECTIVE
  Usher syndrome is the leading cause of hereditary deaf-blindness. Most patients with Usher syndrome type IIa start using hearing aids from a young age. A serious complaint refers to interference between sound localisation abilities and adaptive sound processing (compression), as present in today's hearing aids. The aim of this study was to investigate the effect of advanced signal processing on binaural hearing, including sound localisation.
  
  
  METHODS
  In this prospective study, patients were fitted with hearing aids with a nonlinear (compression) and linear amplification programs. Data logging was used to objectively evaluate the use of either program. Performance was evaluated with a speech-in-noise test, a sound localisation test and two questionnaires focussing on self-reported benefit.
  
  
  RESULTS
  Data logging confirmed that the reported use of hearing aids was high. The linear program was used significantly more often (average use: 77%) than the nonlinear program (average use: 17%). The results for speech intelligibility in noise and sound localisation did not show a significant difference between type of amplification. However, the self-reported outcomes showed higher scores on 'ease of communication' and overall benefit, and significant lower scores on disability for the new hearing aids when compared to their previous hearing aids with compression amplification.
  
  
  CONCLUSIONS
  Patients with Usher syndrome type IIa prefer a linear amplification over nonlinear amplification when fitted with novel hearing aids. Apart from a significantly higher logged use, no difference in speech in noise and sound localisation was observed between linear and nonlinear amplification with the currently used tests. Further research is needed to evaluate the reasons behind the preference for the linear settings.
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands.|Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: B P
authors.initials: BP
authors.lastname: Hartel
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands.|Department of Biophysics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen, The Netherlands.
authors.forename: M J H
authors.initials: MJH
authors.lastname: Agterberg
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands.|Department of Biophysics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen, The Netherlands.
authors.forename: A F
authors.initials: AF
authors.lastname: Snik
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands.|Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: H P M
authors.initials: HPM
authors.lastname: Kunst
authors.affiliation: Department of Biophysics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen, The Netherlands.
authors.forename: A J
authors.initials: AJ
authors.lastname: van Opstal
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands.|Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: A J
authors.initials: AJ
authors.lastname: Bosman
authors.affiliation: Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands.|Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen, The Netherlands.
authors.forename: R J E
authors.initials: RJE
authors.lastname: Pennings
mesh_terms: D000328:Adult; D001299:Audiometry; D005260:Female; D006310:Hearing Aids; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013017:Sound Localization; D013065:Speech Intelligibility; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D052245:Usher Syndromes / Q000503:physiopathology / Q000628:therapy*; D055815:Young Adult