Bilateral gene therapy in children with autosomal recessive deafness 9: single-arm trial results.

Gene therapy is a promising approach for hereditary deafness. We recently showed that unilateral AAV1-hOTOF gene therapy with dual adeno-associated virus (AAV) serotype 1 carrying human OTOF transgene is safe and associated with functional improvements in patients with autosomal recessive deafness 9 (DFNB9). The protocol was subsequently amended and approved to allow bilateral gene therapy administration.

AAV1-hOTOF gene therapy for autosomal recessive deafness 9: a single-arm trial.

Background: Autosomal recessive deafness 9, caused by mutations of the OTOF gene, is characterised by congenital or prelingual, severe-to-complete, bilateral hearing loss. However, no pharmacological treatment is currently available for congenital deafness. In this Article, we report the safety and efficacy of gene therapy with an adeno-associated virus (AAV) serotype 1 carrying a human OTOF transgene (AAV1-hOTOF) as a treatment for children with autosomal recessive deafness 9.

Unexpected Branch Malformation of the Facial Nerve during Stapes Surgery: Comparison between Intraoperative and Radiographic Findings.

The aim of this study was to determine the prevalence of facial nerve (FN) bifurcation in patients who undergo stapes surgery, and to ascertain the correlation between the intraoperative and radiographic findings in cases where an unexpected branch malformation for patients undergoing stapes surgery. Patients who underwent stapes surgery were retroactively examined for confirmed FN bifurcation. Among the 887 patients, 10 had a bifurcated FN confirmed during surgery and had a preoperative high-resolution computed tomography (HRCT) scan.

Atypical manifestations of acute coronary syndrome - throat discomfort: a multi-center observational study.

To present the clinical characteristics and the misdiagnosis rate of acute coronary syndrome manifested primarily as throat discomfort, we conducted a multicentric and retrospective study in the cardiology and otorhinolaryngology departments. Records of patients with primary complaint of throat discomfort, absence of chest pain at onset, and an ultimate diagnosis of acute coronary syndrome, as well as patients with pharyngitis (as controls) were collected from May 2015 to April 2016. The patients' main manifestations were compared.

Genome-wide miRNA analysis and integrated network for flavonoid biosynthesis in Osmanthus fragrans.

Background: Osmanthus fragrans is an important economical plant containing multiple secondary metabolites including flavonoids and anthocyanins. During the past years, the roles of miRNAs in regulating the biosynthesis of secondary metabolites in plants have been widely investigated. However, few studies on miRNA expression profiles and the potential roles in regulating flavonoid biosynthesis have been reported in O.

Direct Functional Protein Delivery with a Peptide into Neonatal and Adult Mammalian Inner Ear .

The aim of this study was to study an antimicrobial peptide (AMP), aurein 1.2, which substantially increased protein delivery directly into multiple mammalian inner-ear cell types . Different concentrations of aurein 1.

Otolaryngology Providers Must Be Alert for Patients with Mild and Asymptomatic COVID-19.

More than half of COVID-19 patients are afebrile early in the disease course, yet mildly ill or asymptomatic patients can still spread SARS-CoV-2 with high efficiency. Atypically presenting patients may be seen in noninfectious disease settings such as otolaryngology, which is a specialty prone to occupational exposure. Otolaryngologists have been infected with COVID-19 at higher rates than other specialties in China and other countries.

Frequency-specific hearing results after stapes surgery for Chinese population otosclerosis with different degrees of hearing loss.

Frequency-specific hearing studies are important for predicting hearing results and the prognosis after stapes surgery, to prepare for the rehabilitation of frequency-specific hearing in patients with otosclerosis. To evaluate outcomes of stapes surgery of Chinese otosclerosis patients with different degrees of hearing loss. We conducted a retrospective analysis of 213 otosclerosis patients who underwent stapes surgery in our hospital.

Factors Affecting Bimodal Benefit in Pediatric Mandarin-Speaking Chinese Cochlear Implant Users.

Objectives: While fundamental frequency (F0) cues are important to both lexical tone perception and multitalker segregation, F0 cues are poorly perceived by cochlear implant (CI) users. Adding low-frequency acoustic hearing via a hearing aid in the contralateral ear may improve CI users' F0 perception. For English-speaking CI users, contralateral acoustic hearing has been shown to improve perception of target speech in noise and in competing talkers.

Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases.

Objective: To describe the characteristics of the clinical presentation, diagnosis, surgical methods, and outcomes of patients with otogenic cerebrospinal fluid (CSF) leakage secondary to congenital inner ear dysplasia.

Methods: A retrospective review was performed of 18 patients with otogenic CSF leakage secondary to inner ear dysplasia who underwent surgery in our group from 2007 to 2017 and had a follow-up of at least 4 months. The average length of follow-up was three years.

Auditory characteristics of noise-exposed memberscrossing age-related groups.

Objective: To report audiological characteristics in a group of noise-exposed crew members on board ships.

Methods And Materials: Clinical and audiological measurements including pure-tone thresholds, acoustic immittance results and tinnitus questionnaires were collected from both the ship crew members (study subjects) and their land based colleagues (controls).

Results: 1) Noise exposed crew members showed not only high frequency, but also low frequency hearing loss; 2) Hearing impairment increased with age, with 65.

Effect of Tinnitus and Duration of Deafness on Sound Localization and Speech Recognition in Noise in Patients With Single-Sided Deafness.

Patients with single-sided deafness (SSD) often experience poor sound localization, reduced speech understanding in noise, reduced quality of life, and tinnitus. The present study aims to evaluate effects of tinnitus and duration of deafness on sound localization and speech recognition in noise by SSD subjects. Sound localization and speech recognition in noise were measured in 26 SSD and 10 normal-hearing (NH) subjects.

Comparable Electrode Impedance and Speech Perception at 12 Months after Cochlear Implantation Using Round Window versus Cochleostomy: An Analysis of 40 Patients.

Objective: The objective of this study was to examine whether cochlear implantation using the round window (RW) route versus cochleostomy achieves comparable electrode impedance and hearing results.

Methods: This retrospective analysis included 40 patients receiving a cochlear implant (REZ-1): 20 using the RW approach and the remaining 20 using cochleostomy. Electrode impedance and tone, vowel, consonant, disyllable and sentence perception were measured during and after the implantation.

Modulation of Glucose Takeup by Glucose Transport on the Isolated OHCs.

Glucose is a fundamental source of energy for mammalian cells; however, whether glucose is taken up through the lateral walls of cochlear outer hair cells (OHCs) is unknown. The OHC lateral wall is complex, composed of a plasma membrane, cortical lattice, and subsurface cisternae. This study assessed the uptake of glucose by OHCs using live-cell microscopy and examined the distribution of glucose transporter isoforms by immunohistochemistry.

The Benefits of Residual Hair Cell Function for Speech and Music Perception in Pediatric Bimodal Cochlear Implant Listeners.

Objective: The aim of this study was to investigate the benefits of residual hair cell function for speech and music perception in bimodal pediatric Mandarin-speaking cochlear implant (CI) listeners.

Design: Speech and music performance was measured in 35 Mandarin-speaking pediatric CI users for unilateral (CI-only) and bimodal listening. Mandarin speech perception was measured for vowels, consonants, lexical tones, and sentences in quiet.

Music Training Can Improve Music and Speech Perception in Pediatric Mandarin-Speaking Cochlear Implant Users.

Due to limited spectral resolution, cochlear implants (CIs) do not convey pitch information very well. Pitch cues are important for perception of music and tonal language; it is possible that music training may improve performance in both listening tasks. In this study, we investigated music training outcomes in terms of perception of music, lexical tones, and sentences in 22 young (4.

Effect of esterified hyaluronic acid as middle ear packing in tympanoplasty for adhesive otitis media.

Objectives: The goal of this study was to evaluate the effects of middle ear packing agents (MEPA) on post-operative hearing improvement and complications after tympanoplasty in patients with adhesive otitis media (OM).

Materials And Methods: Patients with adhesive OM who underwent tympanoplasty surgery were enrolled in the study between January 2012 and January 2015. A total of 205 patients who received canal wall-down tympanoplasty with ossicular chain reconstruction were randomized into one of the three groups with different MEPA.

Clinical observation on hearing conditions of centenarians in northern district of China.

Conclusion: The hearing conditions of the centenarians were quite poor as regards hearing thresholds and speech detection ability.

Objective: To investigate hearing conditions of centenarians.

Methods: A total of 54 centenarians in Rizhao and Linyi Districts in Shandong Province were investigated to assess hearing conditions of centenerians comprehensively by questionnaire investigation, pure-tone audiometry, acoustic immitance, intelligence evaluation, and speech detection scores.