Related Experiment Video
Updated: May 13, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Usher syndrome: characteristics and outcomes of pediatric cochlear implant recipients
Kris R Jatana1, Denise Thomas, Lisa Weber
1Department of Otolaryngology-Head and Neck Surgery, Nationwide Children's Hospital and The Ohio State University, Columbus, Ohio, USA.
Insights
Cochlear implantation in children with Usher syndrome (US) shows promising results. Most children achieve significant speech perception and oral communication skills, despite potential delays in ambulation.
Area of Science:
- Otolaryngology
- Genetics
- Ophthalmology
Background:
- Usher syndrome (US) is a genetic disorder causing hearing loss and progressive vision loss.
- Cochlear implantation is a treatment option for hearing loss in individuals with US.
Purpose of the Study:
- To evaluate the characteristics and outcomes of pediatric cochlear implant recipients with Usher syndrome.
Main Methods:
- Retrospective study of 712 pediatric cochlear implant recipients from 1991-2010.
- Included children diagnosed with US before or after implantation.
- Assessed cochlear anatomy, ambulation, implantation age, speech perception, and communication methods.
Main Results:
- 26 (3.7%) recipients had US; none had cochlear malformations.
- Average age of independent ambulation was 21.9 months; average implantation age was 3.3 years.
- 92% achieved open-set speech perception, with 69.2% using primarily oral communication.
Conclusions:
- Cochlear implantation in children with US is safe and effective.
- Most children develop useful hearing and oral communication skills.
- Vestibular dysfunction in US Type I may delay ambulation but does not preclude successful implantation.
Objective:
To evaluate the characteristics and outcomes of pediatric cochlear implant recipients diagnosed with Usher syndrome (US).
Study Design:
Retrospective study of consecutive pediatric cochlear implant recipients (1991-2010).
Setting:
Tertiary care children's hospital.
Patients:
Children who received a cochlear implant who were diagnosed with US either before or after implantation.
Main Outcome Measures:
Electroretinography and ophthalmologic findings, cochlear anatomy based on preoperative imaging, age of independent ambulation, age at implantation, speech perception level, and communication method.
Results:
Approximately 26 (3.7%) of 712 cochlear implant recipients were diagnosed with US based on the results of electroretinography and/or genetic testing. Preoperative imaging revealed no evidence of cochlear malformations. Average age of independent ambulation was 21.9 months (range, 12-30). Average age at implantation was 3.3 years (range, 6 mo to 11.6 yr). Mean follow-up after implantation was 7.8 years (range, 10 mo to 15.6 yr). Open-set speech perception was present in 92% of children, with use of a primarily oral communication mode in 69.2%.
Conclusion:
In this large series of patients with the diagnosis of US who have undergone cochlear implantation, CT and MRI imaging were normal. Significant delay in independent ambulation was present in this population secondary to abnormal vestibular function associated with US Type I. A majority of children developed significant open-set speech perception and oral communication skills. Implantation of US children provides them with the opportunity to develop useful hearing and oral communication.