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Cochlear Implant Surgery and Electrically-evoked Auditory Brainstem Response Recordings in C57BL/6 Mice
Published on: January 9, 2019
[Cochlear implant in hypoacusis with alteration of connexin 26]
Angel Ramos1, Carina Rodríguez, Silvia Borkoski
1Servicio de Otorrinolaringología y Patología Cérvico-Facial, Complejo Universitario Hospitalario Materno Insular de Gran Canaria, Las Palmas de Gran Canaria, Las Palmas, España. ramosorl@idecnet.com
Insights
Cochlear implantation benefits children with profound congenital hearing loss, including those with Connexin 26 (DFNB1) gene mutations. While initial results show no significant differences, DFNB1 patients tend to improve faster in speech perception after 12 months.
Area of Science:
- Audiology
- Genetics
- Pediatric Medicine
Background:
- Profound prelingual congenital hearing loss affects young children.
- Connexin 26 (Cx26) gene mutations, specifically the 30-35delG mutation (DFNB1 phenotype), are a common cause of congenital deafness.
- Cochlear implantation is a standard treatment for severe to profound hearing loss.
Purpose of the Study:
- To compare the effectiveness of cochlear implantation in children with and without Cx26 gene mutations.
- To assess audiological and speech perception outcomes 12 months post-implantation.
Main Methods:
- Retrospective study of 36 children under 6 with profound congenital bilateral sensorineural hearing impairment.
- Two groups: 15 with DFNB1 (homozygous 30-35delG Cx26 mutation) and 21 without Cx26 mutation.
- All received Nucleus 24K or ST cochlear implants with non-traumatic electrode insertion; 12-month follow-up.
Main Results:
- No significant differences in pure tone audiometry or standard logoaudiometric tests between the DFNB1 and non-mutation groups.
- Children with DFNB1 mutations showed a trend towards faster improvement in vowel and bisyllabic word recognition at 12 months.
Conclusions:
- Cochlear implantation is an effective therapeutic option for profound prelingual congenital hearing loss.
- The presence of Cx26 mutations (DFNB1) does not contraindicate cochlear implantation and may be associated with quicker early speech perception gains.
Objective:
The objective of this paper is to assess the benefits of cochlear implantation in a population of profound prelingual congenital deaf children with mutation of Connexin 26 (DFNB1 phenotype), compared with a population of profound congenital deaf children without mutation of this gene.
Patients And Method:
This retrospective study was carried out in 36 children with cochlear implants under the age of 6. All had profound congenital bilateral sensorineural hearing impairment, without cochlear malformation. Fifteen children were diagnosed as having DFNB1 and homozygous 30-35delG mutation, and 21 had no mutation of Connexin 26 (Cx26). All of them used Nucleus 24K or ST cochlear implants, with complete non-traumatic insertion of the electrodes, and follow-up was 12 months.
Results:
There is no significant difference in pure tone audiometry and logoaudiometric tests between the 2 groups, children diagnosed as having DFNB1 (homozygous 30-35delG mutation) and children without mutation of Cx26. However, the population with DFNB1 shows a tendency to achieve better results more quickly in vowels and bisyllabic word tests 12 months after implantation.
Conclusions:
The cochlear implant is an effective therapy for children with profound prelingual congenital hearing loss with mutation of Cx26.
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