Deafness (Hearing Loss)

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 1b2a 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 mgkg or 30 mgkg 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.

This research aims to evaluate the clinical benefit, performance, and safety of a totally implantable cochlear implant TICI system in adults with sensorineural hearing loss, which is hearing loss caused by damage to the inner ear or auditory nerve. The study is pivotal and pre-market, focusing on how this system, which includes a microphone placed under the skin to detect speech and sounds without any visible external parts, can affect hearing ability and daily life. Participants will be implanted with the TI1132 device, a totally implantable cochlear implant system. The study involves a single group design where all participants receive this investigational implant. The research team will monitor and assess the implants performance and safety over time from the implantation through post-activation periods. During the study, participants will undergo hearing tests and complete questionnaires before implantation and up to 12 months after activation of the device. The tests will measure speech recognition performance in quiet and noise, and various quality of life and hearing questionnaires will evaluate the impact on daily living. Safety will be monitored by tracking adverse events and device issues. The total participation period includes assessments up to 12 months post-activation, with the study completion expected in August 2028.

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 drugs 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 therapys impact.

Researchers are evaluating OpCT-001, a cell therapy made from photoreceptor precursor cells derived from human induced pluripotent stem cells iPSCs, in adults with primary photoreceptor disease. This Phase 12a multisite study aims to assess the safety, tolerability, and effects on visual function and retinal anatomy in approximately 54 participants. The study includes adults with various inherited retinal diseases such as retinitis pigmentosa and Usher syndrome. The study is divided into two parts Phase 1 involves a dose-escalation design with up to four dose levels of OpCT-001 given by subretinal injection to up to 24 legally blind participants. Phase 2 will enroll up to 15 participants per cohort in two cohorts to evaluate two selected dose levels from Phase 1. In Phase 2, participants will be randomized to one of the two doses, and masking applies to participants, investigators, and site personnel outside the surgical team. Participants will be closely monitored through Week 52 after treatment to assess safety and effects on vision and retinal structure using tests like spectral domain optical coherence tomography SD-OCT. Researchers will measure the incidence and severity of treatment-emergent adverse events and changes in retinal layer thickness. The study includes genetic confirmation of diagnosis, vision tests, retinal structure exams, and ongoing safety follow-up over approximately one year post-treatment.

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 therapys 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 devices 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 OSAs 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.

This research aims to develop a universal method for adapting speech audiometry tests into different languages, focusing on children. Speech audiometry is commonly used to assess hearing in children, but many languages lack suitable test materials. This study introduces the first comprehensive protocol that systematically addresses linguistic, phonological, and audiological factors to adapt and standardize the Mainzer Audiometric Test for Children MATCH for other languages. The adaptation process includes six phases selecting and validating test items for recognizability among children, ensuring linguistic accuracy by comparing phoneme distributions, recording speech materials in a sound-treated environment following ISO standards, equalizing item intelligibility through adult speech recognition tests, standardizing the test on normal-hearing children aged 3 to 6 years, and evaluating diagnostic validity by comparing speech recognition thresholds with pure-tone audiometry in clinical samples. The study also uses ROC analysis to determine sensitivity, specificity, and optimal cutoffs for detecting hearing loss. Participants include children aged 2 to 7 years with either normal hearing or stable sensorineural hearing loss, divided into age groups for testing. The study involves assessments such as speech recognition testing, pure-tone audiometry, and tympanometry. Researchers monitor test-retest reliability and diagnostic performance over about one year. The primary outcome is the correlation between adapted MATCH speech recognition thresholds and pure-tone audiometry thresholds. Secondary outcomes include diagnostic accuracy and test consistency.