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Updated: Mar 2, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life
Bas P Hartel1, Josephine W I van Nierop, Wendy J Huinck
1*Department of Otorhinolaryngology †Radboud Institute for Health Sciences, Radboud University Medical Center ‡Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen §Department of Otorhinolaryngology, Leiden University Medical Center, Leiden, the Netherlands.
Objectives:
Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment leads to insufficient speech intelligibility with hearing aids and issues with adequate communication and safety. Cochlear implantation (CI) is the next step in rehabilitation of such patients. This study evaluates the performance and benefit of CI in patients with USH2a.
Design:
Retrospective case-control study to evaluate the performance and benefit of CI in 16 postlingually deaf adults (eight patients with USH2a and eight matched controls). Performance and benefit were evaluated by a speech intelligibility test and three quality-of-life questionnaires.
Results:
Patients with USH2a with a mean age of 59 years at implantation exhibited good performance after CI. The phoneme scores improved significantly from 41 to 87% in patients with USH2a (p = 0.02) and from 30 to 86% in the control group (p = 0.001). The results of the questionnaire survey demonstrated a clear benefit from CI. There were no differences in performance or benefit between patients with USH2a and control patients before and after CI.
Conclusions:
CI increases speech intelligibility and improves quality of life in patients with USH2a.
