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Cochlear implantation in patients with Pendred syndrome
Taylor E Patterson1, Victoria B Gonzalez2, Jeffrey D Carron2
1University of Mississippi School of Medicine, 2500 North State Street, Jackson, MS 39216, United States of America.
American Journal of Otolaryngology
|May 24, 2021
Summary
Cochlear implantation effectively treats severe hearing loss in children with Pendred syndrome. Early intervention with cochlear implants supports speech and cognitive development for mainstream education.
Area of Science:
- Otolaryngology
- Genetics
- Audiology
Background:
- Pendred syndrome is a common genetic cause of sensorineural hearing loss.
- Inner ear malformations are characteristic of Pendred syndrome.
- Traditional hearing aids offer limited benefit for severe hearing loss in these patients.
Purpose of the Study:
- To evaluate the outcomes of cochlear implantation in pediatric patients diagnosed with Pendred syndrome.
- To assess the efficacy of cochlear implants in improving audiological results and development.
Main Methods:
- Retrospective case series of nine pediatric patients with Pendred syndrome.
- Patients underwent cochlear implantation between 2003 and 2017.
- Preoperative imaging and audiological data were analyzed.
Main Results:
- All patients had bilateral enlarged vestibular aqueducts and cochlear dysplasia.
- Successful electrode implantation with minimal complications was achieved.
- Favorable post-operative audiological outcomes were observed.
Conclusions:
- Cochlear implantation is a successful treatment for severe-to-profound hearing loss in children with Pendred syndrome.
- Early cochlear implantation facilitates speech and cognitive development comparable to normal hearing peers.
- This enables mainstream educational integration for affected children.
