Erratum: Preclinical evaluation of the efficacy and safety of AAV1-hOTOF in mice and nonhuman primates.

[This corrects the article DOI: 10.1016/j.omtm.

Preliminary evidence for enhanced auditory cortex activation and mental development after gene therapy in children with autosomal recessive deafness 9.

Individuals with congenital deafness that have received gene therapy represent a unique group who experience hearing recovery and speech development. However, it is unclear how hearing-related cortex changes because of gene therapy. Here we study neural processing in ten patients using functional near-infrared spectroscopy and electroencephalography during a six-month follow-up period.

Programmable NIR Responsive Nanocomposite Enables Noninvasive Intratympanic Delivery of Dexamethasone to Reverse Cisplatin Induced Hearing Loss.

Local intratympanic drug delivery to the inner ear possesses significant otological clinical promise as cisplatin-induced hearing loss (CIHL) therapy, inducing significantly less side effects than systemic drug delivery. However, the multiple detoured barriers, round window membrane (RWM) and poorly controlled drug release hinder successful non-invasive drug delivery through intratympanic administration (IT). Here, a novel near-infrared (NIR) responsive nanocomposite functionalized with saponin, denoted gold nanorod@dexamethasone-mesoporous silica-saponin (AuNR@DEX-MS-saponin, NPs/DEX), is developed to enhance RWM permeation and to control the drug release spatiotemporally.

Hair cell-specific Myo15 promoter-mediated gene therapy rescues hearing in DFNB9 mouse model.

Adeno-associated viral (AAV) vectors are increasingly used as vehicles for gene delivery to treat hearing loss. However, lack of specificity of the transgene expression may lead to overexpression of the transgene in nontarget tissues. In this study, we evaluated the expression efficiency and specificity of transgene delivered by AAV-PHP.

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.

The diagnostic utility of diffusion-weighted magnetic resonance imaging and high-resolution computed tomography for cholesteatoma: A meta-analysis.

Objective: The purpose of this meta-analysis was to compare the efficiency of high-resolution computed tomography (HRCT) and diffusion-weighted magnetic resonance imaging (DWI) in guiding the diagnosis of middle ear cholesteatoma in clinical practice.

Materials And Methods: Cochrane Library, Medline, Embase, PubMed, and Web of Science were searched for studies that evaluated the sensitivity and specificity of HRCT or DWI in detecting middle ear cholesteatoma. A random-effects model was used to calculate and summarize the pooled estimates of sensitivity, specificity, and diagnostic odds ratios.

Hearing of Otof-deficient mice restored by trans-splicing of N- and C-terminal otoferlin.

Mutations to the OTOF gene are among the most common reasons for auditory neuropathy. Although cochlear implants are often effective in restoring sound transduction, there are currently no biological treatments for individuals with variants of OTOF. Previous studies have reported the rescue of hearing in DFNB9 mice using OTOF gene replacement although the efficacy needs improvement.