Hearing Impairment

Researchers are exploring and comparing how patients perceive sounds after receiving either cochlear implants or gene therapy for congenital deafness. This study aims to understand differences in speech perception in various environments, music appreciation, and directional hearing between these two treatments. By assessing cognitive and psychological factors alongside auditory development, the study hopes to inform better rehabilitation plans for gene therapy patients. The study includes two groups of congenital deafness patients: those who have cochlear implants and those who have undergone gene therapy for autosomal recessive deafness 9 (DFNB9). Patients receive standard postoperative care and follow-up. The evaluation covers multiple aspects such as speech perception in quiet and noisy settings, cognitive function, psychological status, and auditory cortex growth over time. Participants will be assessed before treatment and at weeks 13, 26, and 52 after intervention. Evaluations include tests of auditory speech perception, cognitive abilities, psychological health, and brain development related to hearing. The study requires participants and their guardians to provide informed consent and cooperate with follow-up visits. Healthy individuals with normal hearing are also included as controls. The goal is to compare outcomes between the two treatments over one year.

Researchers are evaluating the safety, tolerability, pharmacokinetics (PK), pharmacodynamics (PD), and efficacy of NS101, an anti-FAM19A5 antibody, in both healthy volunteers and patients with sudden sensorineural hearing loss (SSNHL). This exploratory Phase 1b/2a study aims to determine if multiple doses of NS101 are safe and tolerable and whether the treatment can help restore hearing in SSNHL patients who have not sufficiently improved after standard steroid therapy. The study includes a placebo control and uses a double-blind design to ensure unbiased results. Participants receive NS101 or placebo via intravenous infusion at doses of 15 mg/kg or 30 mg/kg administered every two weeks. Healthy volunteers receive treatment for 6 weeks, whereas SSNHL patients receive treatment for 12 weeks. The study has four groups: two dose levels for healthy volunteers with active and placebo arms, and two groups for SSNHL patients receiving either NS101 or placebo. Treatments are given systematically, and participants are randomly assigned to the groups. Throughout the study, participants undergo frequent safety assessments to monitor changes from baseline at multiple time points, including days and weeks after treatment begins. Pharmacokinetic and immunological tests are also performed to track how the drug behaves in the body and its effects. Researchers measure hearing improvement and monitor tolerability closely. The study is expected to continue until January 2027, with participants involved in regular visits and evaluations during and after treatment.

Researchers are evaluating the safety, tolerability, and effectiveness of the gene therapy SENS-501 in children aged 6 to 31 months who have severe to profound hearing loss caused by mutations in the Otoferlin gene. This open-label Phase I/II study focuses on children with pre-lingual hearing loss due to these genetic mutations, aiming to better understand how this treatment works in this young population. The study involves administering SENS-501 directly into one ear using a special injection system. It includes a dose-escalation phase where children receive either a low or high dose, followed by a dose expansion phase where the dose recommended from earlier phases is given. The treatment is given as a single unilateral intracochlear injection, and the administration system's safety, performance, and usability are also evaluated. Participants will be followed for up to 5 years to monitor safety and tolerability and to assess hearing improvement using auditory brainstem response (ABR) and pure tone audiometry (PTA). Additional evaluations include clinical checks of the administration system shortly after treatment. Throughout the study, children will undergo hearing tests and medical assessments to measure outcomes and track any side effects or complications from the treatment and procedure.

Researchers are evaluating the clinical benefit, performance, and safety of a new totally implantable cochlear implant (TICI) system in adults with bilateral sensorineural hearing loss. This type of hearing loss results from damage to the inner ear or auditory nerve, affecting the ability to hear sounds and speech. The study is pivotal and prospective, focusing on adults with this condition to better understand how the implant may improve hearing without visible external parts. Participants will receive the TI1132 implant, which includes a microphone placed under the skin to detect environmental sounds and speech. The implant is fully contained beneath the skin, offering an option to hear without any external devices. The study involves implantation of the device and follows participants through a series of timepoints after activation to assess performance and safety. During the study, participants will undergo hearing tests, including speech recognition in quiet and noise, and complete questionnaires about their hearing experience and quality of life. Researchers will monitor adverse events and device issues from implantation up to 12 months post-activation. The study collects patient satisfaction data and health-related quality of life measures, with follow-ups extending to one year after activation to evaluate long-term outcomes.

Researchers are investigating how hearing aids might affect cognitive function in older adults with age-related hearing loss, also known as presbycusis. This study focuses on individuals aged 60 to 85 and aims to understand whether improving hearing ability can slow down the decline in memory and cognition associated with hearing loss. The research is being conducted at a single center and plans to follow participants for several years to assess changes over time. Participants are divided into two groups: one group receives hearing aids and the other does not receive any intervention. Those in the hearing aid group will use the device continuously for five years. Follow-up visits occur every six months, during which various tests are performed, including hearing assessments, cognitive tests, and brain imaging. The study uses a double-blind, randomized design to compare outcomes between the groups. Throughout the study, participants will have their cognitive functions measured using tools like the Montreal Cognitive Assessment (MoCA) and the Mini-Mental Status Exam (MMSE) every six months. Hearing ability will also be evaluated regularly through audiometry tests. Researchers will monitor the effects of hearing aids on cognitive decline and hearing improvement over the five-year period. Safety and adherence to the hearing aid intervention will be tracked as well.

Researchers are evaluating the safety and effectiveness of a new drug delivery method using dexamethasone-loaded exosomes compared to standard intratympanic dexamethasone and exosome vehicle alone in adults with sudden sensorineural hearing loss (SSNHL). This condition involves rapid hearing loss of 30 decibels or more over three frequencies within 72 hours, with causes often unknown but possibly related to infections, immune responses, or blood flow issues in the ear. The study aims to improve drug delivery to the inner ear and reduce systemic side effects. The trial includes three treatment groups: one receiving standard dexamethasone injections into the middle ear, another receiving exosomes alone, and the third receiving exosomes loaded with dexamethasone. These treatments are given as a single course through intratympanic administration. The researchers will assess how well the treatments improve hearing and how the drugs distribute within the ear, using advanced imaging and hearing tests over several weeks. Participants will undergo hearing assessments including pure-tone average tests, auditory brainstem response, and otoacoustic emissions at baseline and follow-up visits at 1, 4, and 12 weeks after treatment. Safety and tolerability will be closely monitored. The main measurement is the change in hearing levels four weeks after treatment. The study will last up to six months for each participant, with detailed evaluations to understand the drug's effects and safety profile throughout this period.

Researchers are evaluating the safety, tolerability, and early effectiveness of EH002 gene therapy for treating congenital deafness caused by mutations in the OTOF gene, known as DFNB9. This study focuses on participants with severe or profound hearing loss confirmed by genetic testing. The research aims to understand how EH002 may help restore hearing by addressing the genetic cause of this type of deafness. Participants may receive one or two doses of EH002 delivered directly into one or both ears through a surgical injection into the cochlea. The study includes dose escalation groups with varying amounts of EH002 administered. Eligibility requires passing imaging tests like CT or MRI to confirm suitable ear anatomy for surgery, with follow-up visits scheduled to monitor participants closely. During the 26-week study period, researchers will track any serious side effects or dose-related toxicities and assess changes in hearing levels compared to baseline. Participants will undergo genetic testing, audiological assessments, imaging scans, and safety monitoring throughout. The study will also evaluate hearing improvements and any adverse events to better understand the therapy's impact.

Researchers are investigating the use of Clemastine Fumarate combined with engineered sound to treat age-related central auditory processing disorder (CAPD), a condition affecting many people over 40 and causing difficulty hearing in noisy environments. This trial aims to test if the therapy, called Localized Oligodendrocyte Optimization Therapy (LOOT), which showed promise in animal studies, can improve hearing in noise for humans. The study is designed to compare the effectiveness of the drug with or without engineered sound in a controlled, randomized setting. Participants will be randomly assigned to one of four groups: Clemastine Fumarate with engineered sound, Clemastine Fumarate with placebo sound (white or pink noise), placebo drug with engineered sound, or placebo drug with placebo sound. The drug dosage is carefully controlled and given orally over 30 days, while participants listen to the assigned sound stimulation through headphones for one hour daily. The trial includes an adaptive design allowing adjustment of study arms based on interim results. Throughout the study, participants will undergo hearing tests to measure their ability to hear in noisy environments, both before and after treatment. Additional assessments include questionnaires on speech and tinnitus, auditory brainstem response tests, physical exams, blood tests, and EKGs to monitor health and safety. The treatment period lasts one month, with evaluations scheduled around weeks five or six. Safety and hearing improvements are carefully monitored to assess the therapy's impact.

Researchers are studying the accuracy of a new device called the laryngopharyngeal endoscopic esthesiometer and rangefinder (LPEER) to measure throat sensitivity in patients suspected of having obstructive sleep apnea (OSA). This prospective, double-blinded, randomized crossover trial aims to validate this device's measurements by comparing them to standard sleep study results. The study focuses on understanding sensory differences in patients with mild, moderate, and severe OSA and improving knowledge about OSA’s underlying mechanisms. The study involves patients referred for a baseline sleep test who will undergo a sensory test using the LPEER, which delivers air pulses of varying intensity to specific throat areas: the velopharynx, hypopharynx, and aryepiglottic folds. Experienced and less experienced pulmonologists or otolaryngologists will perform these tests to assess measurement reliability. The study design includes randomized crossover assignment of raters to evaluate intra- and inter-rater agreement and diagnostic accuracy through detailed statistical analyses. Participants will attend the sleep laboratory where they will receive the sensory test alongside their routine sleep study. Researchers will measure sensory thresholds at three throat sites and collect data related to sleep apnea severity, oxygen levels, and any adverse events. The study will last up to 15 days for each participant, during which safety and test accuracy will be closely monitored. Results will help determine the usefulness of LPEER for future clinical evaluation and monitoring of OSA patients.

Researchers are evaluating whether brief sounds or tones played during a limited recovery sleep period after 40 hours of sleep deprivation can enhance the restorative effects of sleep and improve performance during the following awake period. This study involves healthy adults aged 18 to 39 and aims to understand how acoustic stimulation during slow-wave sleep affects alertness and cognitive functions. Participants will experience two nights of approximately four-hour recovery sleep after sleep deprivation. During these recovery sleeps, one group will receive acoustic stimulation via the Philips SmartSleep device during slow-wave sleep, while the other group will receive sham treatment without acoustic stimulation. The device is applied prior to bedtime on both recovery nights to deliver or withhold the acoustic signals. Throughout the study, participants' performance and alertness will be assessed using various tests including the Psychomotor Vigilance Task, mathematical processing tests, mood scales, and sleepiness scales over several days. Sleep and wakefulness will also be monitored via polysomnographic recordings and actigraphy. The study includes safety monitoring and adherence checks, with total participation spanning multiple days to capture effects during and after sleep deprivation and recovery.