Cochlear implantation under local anesthesia: a possible alternative for elderly patients.

INTRODUCTION: As average life-expectancy increases, a sufficient hearing rehabilitation for elderly patients with severe-to-profound sensorineural hearing loss becomes more important. Cochlear implantation is a relatively safe surgical procedure also for elderly patients, the higher risk is caused by general anesthesia. We report on four patients who underwent cochlear implantation under local anesthesia. METHODS: After detailed preoperative examinations (audiological tests, imaging, genetic tests, evaluation of motivation and compliance of the patient), four patient with severe-to-profound hearing loss were selected for cochlear implantation under local anesthesia. For the electrode insertion, we used the posterior suprameatal approach technique. Pre- and postoperative pure tone audiometry and speech-perception tests were conducted to prove the success of the procedure. RESULTS: The mentioned technique was applied; the average length of the operation was 52 min. The intraoperative measurements showed normal impedance and normal neuronal response telemetry, all the patients had sound experience during the intraoperative examination of the engineer. No complications were observed. The postoperative audiological tests showed a significant increase in the hearing perception. CONCLUSION: Cochlear implantation under local anesthesia is a safe and fast procedure for elderly patients. The intraoperative sound experience can give an extra motivation in the postoperative rehabilitation. Our results prove that by carefully selected elderly patients cochlear implantation can assure a significant increase in speech perception. We can establish that the new posterior suprameatal approach technique combined with local anesthesia presents a viable future option for those patients who were inoperable beforehand because of high risks of general anesthesia.

Analysis of GJB2 mutations and the clinical manifestation in a large Hungarian cohort.

PURPOSE: Pathogenic variants of the gap junction beta 2 (GJB2) gene are responsible for about 50% of hereditary non-syndromic sensorineural hearing loss (NSHL). In this study, we report mutation frequency and phenotype comparison of different GJB2 gene alterations in Hungarian NSHL patients. METHODS: The total coding region of the GJB2 gene was analyzed with Sanger or NGS sequencing for 239 patients with NSHL and 160 controls. RESULTS: Homozygous and compound heterozygous GJB2 mutations were associated with early onset serious clinical phenotype in 28 patients. In 24 patients, two deletion or nonsense mutations were detected in individuals with mainly prelingual NSHL. In compound heterozygous cases, a combination of deletion and missense mutations associated with milder postlingual NSHL. A further 25 cases harbored single heterozygous GJB2 mutations mainly associated with later onset, milder clinical phenotype. The most common mutation was the c.35delG deletion, with 12.6% allele frequency. The hearing loss was more severe in the prelingual groups. CONCLUSION: The mutation frequency of GJB2 in the investigated cohort is lower than in other European cohorts. The most serious cases were associated with homozygous and compound heterozygous mutations. In our cohort the hearing impairment and age of onset was not altered between in cases with only one heterozygous GJB2 mutation and wild type genotype, which may exclude the possibility of autosomal dominant inheritance. In early onset, severe to profound hearing loss cases, if the GJB2 analysis results in only one heterozygous alteration further next generation sequencing is highly recommended.