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Published on: January 9, 2019
Cochlear implantation effect on deaf children with gap junction protein beta 2 gene mutation
Ying Kong1, Sha Liu, Su-Ju Wang
1Beijing Tongren Hospital, Capital Medical University, Beijing Institute of Otolaryngology, Key Laboratory of Otolaryngology Head and Neck Surgery (Capital Medical University), Ministry of Education, Beijing 100005, China.
Chinese Medical Journal
|April 6, 2013
Summary
Cochlear implantation effectively restores hearing in children with GJB2 gene mutations causing deafness. This study shows significant improvements in speech recognition after surgery, offering hope for auditory rehabilitation.
Area of Science:
- Genetics
- Otolaryngology
- Audiology
Background:
- Gene diagnosis technology allows detection of deafness genes in children with congenital hearing loss.
- Gap junction protein beta 2 (GJB2) gene mutations are found in 26.5% of cochlear implant patients.
- This study evaluates auditory rehabilitation outcomes in deaf children with GJB2 mutations post-cochlear implantation.
Purpose of the Study:
- To assess the effectiveness of cochlear implantation for auditory rehabilitation in children with GJB2 gene mutations.
- To provide reference data for managing similar cases.
Main Methods:
- Genetic deafness gene chip detection and complete gene sequence analysis were used to identify GJB2 mutations.
- Auditory rehabilitation effects were evaluated at 3, 6, and 12 months post-surgery.
- Repeated measures ANOVA analyzed speech recognition (consonant, vowel, two-syllable) at different time points.
Main Results:
- The 235delC mutation was the most common site in GJB2 genetic deafness patients (90.3% detection rate).
- Significant improvements (P < 0.01) were observed in initial consonant, vowel, and two-syllable recognition rates at 3, 6, and 12 months post-operation.
Conclusions:
- Cochlear implantation is a safe and effective auditory reconstruction method.
- Patients with GJB2 hereditary severe sensorineural deafness can achieve effective auditory speech recognition post-implantation.