Auditory outcome after cochlear implantation in patients with congenital nonsyndromic hearing loss: influence of the GJB2 status.

Abstract

OBJECTIVE:

To compare the audiologic outcome after cochlear implantation between 2 groups of patients with congenital nonsyndromic sensorineural hearing loss.

STUDY DESIGN:

Retrospective cohort study.

SETTING:

Department of Otorhinolaryngology, University hospital (tertiary referral center).

PATIENTS:

From a bigger pool of implanted patients, 2 groups, each numbering 30 were enrolled. The patients from the first group were diagnosed with a Connexin 26 mutation (GJB2), whereas all of the patients from the second cohort were with a wild type genotype. Both groups were age matched, 1 to 7 years old at the age of implantation, with diagnosed congenital nonsyndromic sensorineural hearing loss.

MAIN OUTCOME MEASURES:

Both groups were evaluated with the help evaluation of auditory responses to speech/EARS/test battery - LiP test (Listening Progress Profile), MTP tests 3,6,12 (Monosyllabic-Trochee-Polysyllabic Test), GASP test (Glendonald Auditory Screening Procedure), and others. Follow-up period was at least 36 months.

RESULTS:

Mean test scores were compared at the 1st, 6th, 12th, 24th, and 36th month. LiP outcome was significantly better (p < 0.05) for the GJB2-related cohort for the whole follow-up period except at the first month. MTP3, 6, and 12 tests displayed the same statistically significant outcome in favor of the first group. In the open-set test GASP, the difference was apparent: 1.22, 2.40, 5.59, and 7.40 mean scores at the 6th, 12th, 24, and 36th months for the first cohort versus 0.00, 0.07, 0.81, and 1.74 for the GJB2-unrelated patients.

CONCLUSION:

The results from our study suggest that children with GJB2-related deafness show better auditory performance after cochlear implantation than age-matched children with GJB2-nonrelated sensorineural hearing loss.

• Authors: Todor Miroslavov Popov, Iglika Stancheva, Darina L Kachakova, Julian Rangachev, Dimitar Konov, Sonya Varbanova, Vanio Ivanov Mitev, Radka P Kaneva, Diana Petrova Popova
• Journal: Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology (Otol Neurotol) Vol. 35 Issue 8 Pg. 1361-5 (Sep 2014) ISSN: 1537-4505 [Electronic] United States
• PMID: 24691507 (Publication Type: Journal Article)

Chemical References

• Connexins
• GJB2 protein, human
• Connexin 26

Topics

• Child
• Child, Preschool
• Cochlear Implantation
• Cohort Studies
• Connexin 26
• Connexins (genetics)
• Deafness (genetics, surgery)
• Female
• Genotype
• Hearing Loss, Sensorineural (genetics, surgery)
• Humans
• Infant
• Male
• Mutation
• Retrospective Studies
• Treatment Outcome