Inner Ear Gene Therapy: An Overview from Bench to Bedside.

Hearing loss represents a highly prevalent and debilitating sensory disorder affecting roughly one in five people worldwide. In a majority of patients with congenital hearing loss, genetic mutations cause the disease. Up until recently, therapeutic options for individuals with hearing loss were limited to hearing aids and different types of auditory implants.

Silicone-based AC102-loaded cochlear implant coatings protect residual hearing in an animal model of cochlear implantation.

Cochlear implant users with residual hearing benefit synergistically from combined electrical stimulation via the cochlear implant and preserved residual hearing after surgery. However, direct mechanical trauma and subsequent inflammation may deteriorate hearing function. AC102, a novel otoprotective pyridoindole with anti-apoptotic and anti-oxidative properties significantly improved hearing recovery following cochlear implantation when administered intratympanically prior to surgery.

Long-Term Follow-Up After Translabyrinthine IAC Tumor Removal With Simultaneous Cochlear Implantation.

Objective: To evaluate the long-term hearing outcome after translabyrinthine resection of tumors within the internal auditory canal (IAC) with simultaneous cochlear implantation (CI).

Study Design: Prospective study.

Setting: Tertiary referral center.

A preoperative dose of the pyridoindole AC102 improves the recovery of residual hearing in a gerbil animal model of cochlear implantation.

Sensorineural hearing loss (SNHL) is the most common sensory deficit worldwide. Due to the heterogeneity of causes for SNHL, effective treatment options remain scarce, creating an unmet need for novel drugs in the field of otology. Cochlear implantation (CI) currently is the only established method to restore hearing function in profound SNHL and deaf patients.

Prestin in Human Perilymph, Cerebrospinal Fluid, and Blood as a Biomarker for Hearing Loss.

Objective: Determining the concentration of prestin in human blood, cerebrospinal fluid (CSF), and perilymph (PL), and evaluating its suitability as a clinical biomarker for sensori-neural hearing loss (SNHL).

Study Design: Human blood, CSF, and PL samples were intraoperatively collected from 42 patients with tumors of the internal auditory canal or with intracochlear tumors undergoing translabyrinthine or middle fossa tumor removal. Prestin concentration was measured using enzyme-linked immunosorbent assay and linear regression analyses were performed to investigate its associations with audiological as well as vestibular test results.

Insertion trauma of a novel inner ear catheter for intracochlear drug delivery.

Introduction: Even with recent research advances, effective delivery of a compound to its target cells inside the inner ear remains a challenging endeavor due to anatomical and physiological barriers. Direct intracochlear drug administration with an inner ear catheter (IEC) aims to overcome this obstacle and strives to provide a safe and efficient way for inner ear pharmacotherapy. The goal of this study was to histologically and audiologically evaluate the traumatic properties of a novel IEC for intracochlear drug delivery in a large animal model.

"To implant or not to implant": electrically evoked auditory brainstem response audiometry for decision-making in vestibular schwannoma resection with CI.

Vestibular schwannomas (VS) are often associated with debilitating hearing loss. Therefore, preservation and rehabilitation of hearing have become major therapeutic goals of VS management. Recently, cochlear implantation (CI) has been established as an effective treatment option for VS-associated hearing loss.

Investigation of inner ear drug delivery with a cochlear catheter in piglets as a representative model for human cochlear pharmacokinetics.

Hearing impairment is the most common sensory disorder in humans, and yet hardly any medications are licensed for the treatment of inner ear pathologies. Intricate pharmacokinetic examinations to better understand drug distribution within this complex organ could facilitate the development of novel therapeutics. For such translational research projects, animal models are indispensable, but differences in inner ear dimensions and other anatomical features complicate the transfer of experimental results to the clinic.

Functional Outcome After Simultaneous Vestibular Schwannoma Resection and Cochlear Implantation With Intraoperative Cochlear Nerve Monitoring.

Objective: Electrically evoked auditory brainstem response audiometry has emerged as a suitable option to intraoperatively assess cochlear nerve function during vestibular schwannoma resection. This study aimed to analyze the functional outcome and implant usage of patients with preserved auditory nerve responses after simultaneous translabyrinthine schwannoma resection and cochlear implantation.

Study Design: Prospective study.

Single-incision cochlear implantation and hearing evaluation in piglets and minipigs.

Objectives: Various animal models have been established and applied in hearing research. In the exploration of novel cochlear implant developments, mainly rodents have been used. Despite their important contribution to the understanding of auditory function, translation of experimental observations from rodents to humans is limited due to the size differences and genetic variability.

Combined Open Surgical and Endoscopic Approach for Management of a Meningoencephalocele After Iatrogenic Perforation of the Anterior Skull Base in a Young Infant.

Traumatic iatrogenic perforation of the anterior skull base is a rare complication following endonasal intubation in preterm infants. Subsequent meningoencephaloceles with concomitant cerebrospinal fluid (CSF) fistulas bear the risk of severe complications, therefore early diagnosis and closure of the skull defect are crucial. However, there is no consensus on the management of such cases of meningoencephaloceles.

Intranasal application of adeno-associated viruses: a systematic review.

Adeno-associated viruses (AAVs) represent some of the most commonly employed vectors for targeted gene delivery and their extensive study has resulted in the approval of multiple gene therapies to treat human diseases. The intranasal route of vector application in gene therapy offers several advantages over traditional ways of administration. In addition to targeting local tissue like the olfactory epithelium, it provides minimally invasive access to various organ systems, including the central nervous system and the respiratory tract.

Peaks in online inquiries into pharyngitis-related symptoms correspond with annual incidence rates.

Objective: To assess whether web-based public inquiries into pharyngitis-related search terms follow annual incidence peaks of acute pharyngitis in various countries from both hemispheres.

Methods: Google Trends (GT) was utilized for systematic acquisition of pharyngitis-related search terms (sore throat, cough, fever, cold). Six countries from both hemispheres including four English (United Kingdom, United States, Canada, and Australia) and two non-English speaking countries (Austria and Germany) were selected for further analysis.