Study on gene therapy for DPOAE and ABR threshold changes in adult Otof-/- mice / 临床耳鼻咽喉头颈外科杂志

Objective:This study aims to analyze the threshold changes in distortion product otoacoustic emissions（DPOAE） and auditory brainstem response（ABR） in adult Otof-/- mice before and after gene therapy, evaluating its effectiveness and exploring methods for assessing hearing recovery post-treatment. Methods:At the age of 4 weeks, adult Otof-/- mice received an inner ear injection of a therapeutic agent containing intein-mediated recombination of the OTOF gene, delivered via dual AAV vectors through the round window membrane（RWM）. Immunofluorescence staining assessed the proportion of inner ear hair cells with restored otoferlin expression and the number of synapses.Statistical analysis was performed to compare the DPOAE and ABR thresholds before and after the treatment. Results:AAV-PHP. eB demonstrates high transduction efficiency in inner ear hair cells. The therapeutic regimen corrected hearing loss in adult Otof-/- mice without impacting auditory function in wild-type mice. The changes in DPOAE and ABR thresholds after gene therapy are significantly correlated at 16 kHz. Post-treatment,a slight increase in DPOAE was observeds,followed by a recovery trend at 2 months post-treatment. Conclusion:Gene therapy significantly restored hearing in adult Otof-/- mice, though the surgical delivery may cause transient hearing damage. Precise and gentle surgical techniques are essential to maximize gene therapy's efficacy.

Evaluation of the electrode position by CBCT following cochlear implantation / 中华耳鼻咽喉头颈外科杂志

Objective@#To observe the position of the electrode in the cochlea following cochlear implantation by cone beam computed tomography (CBCT).@*Methods@#Twenty-five children who received cochlear implantation and CBCT examinations in the Department of Otorhinolaryngology Head and Neck Surgery from the Second Hospital of Shandong University between January 2016 and December 2017 were selected. There were 15 males (17 ears) and 10 females (10 ears). The age ranged from 0.5 to 7.0 years old, with a median age of 1.6 years. 23 patients with unilateral implantation and two patients with bilateral implantation. The implants were all Med-El standard 12-electrode contact arrays. The CBCT was used to determine the position of the electrode in the cochlea, the distance between the electrode contacts and modiolus, and intracochlear insertion length of the electrode arrays. SPSS Statistics, version 22.0 was used for data processing and statistical analysis.@*Results@#CBCT images could clearly demonstrate the structure of round window, oval window, modiolus, osseous cochlear duct, osseous spiral lamina, and electrodes. The electrode arrays of all the children were fully implanted into the cochlea. 26 of the electrode arrays were located in the scala tympani and one of them was inserted into the scala vestibular. The mean intracochlear insertion length of the electrode arrays was 30.23 (95%CI, 30.04-30.71) mm. The distance between the electrode contacts and modiolus gradually increased at the 1-4th electrode contacts and reached the maximum at the 4th electrode contacts with an average of 1.10 mm (95%CI, 1.02 to 1.18 mm), and then gradually decreased from the 5th electrode contacts.@*Conclusions@#CBCT can clearly show cochlear fine structures and therefore accurately assess the electrode position following cochlear implantation.