A Study in Subjects With Otoferlin Mutation-related Hearing Loss Using RNA Base-eDiting Therapy...
Congenital Hearing LossThe purpose of the study is to determine whether HG205 as CRISPR/Cas13 RNA base-editing therapy is safe and effective for the treatment of hearing loss caused by p.Q829X mutation in OTOF gene.
The Study on Effect and Neural Network Mechanism of Transcranial Direct Current Stimulation for...
TinnitusSubjective7 moreThis clinical study is prospective, single-center, randomized, controlled, double-blind clinical trail, which entitled transcranial electrical stimulation for the treatment of acute tinnitus approved by Sun Yat-sen University, and intends to recruit 86 patients with sudden deafness and tinnitus. For acute subjective tinnitus, a common otological disease, the study gave the experimental group patients received tDCS with electrodes positioned over the left temporal cortex for 5 days. To assess the efficacy of conventional medical therapy and tDCS by comparing changes in anterior and posterior tinnitus-related subjective scale scores, such as THI, VAS, BAI, BDI, PSQI, and hearing recovery, in patients who received tDCS, to determine whether tDCS is effective in improving acute tinnitus, and whether it is superior to conventional tinnitus treatment. In addition, the study will continue to follow patients for 1 month,3 months, and 6 months after the end of treatment to observe the long-term sustained efficacy of tDCS. This clinical trail will also evaluate tDCS from the perspective of compliance and safety, and explore the factors affecting the efficacy of this therapy.
Novel Coding Strategies for Children With Cochlear Implants
DeafnessThe purpose of this study is to understand performance with a cochlear implant. The long-term goals of this research are to improve sound perception with cochlear implants and to better understand the functioning of the auditory system. Information from individuals with and without cochlear implants will be compared.
Pilot Study to Evaluate the Long-term Chronic Care of Patients Who Could or do Utilize an Osseointegrated...
Hearing LossConductive4 moreThe primary objective of the study is to evaluate OID hearing intervention on cognition among patients who could vs. do utilize an OID. We will also evaluate the long-term effects of OID use on secondary outcomes measures (hearing performance, quality-of-life, social interaction, communication, physical functioning, etc)
Clinical Investigation of the Safety and Performance of HiResTM Ultra Cl HiFocusTM SlimJ Electrode...
Cochlear Hearing LossThis is a prospective study designed to evaluate the safety and performance of the HiResTM Ultra Cl HiFocusTM SlimJ Electrode and HiResTM Ultra 3D Cl HiFocusTM SlimJ Electrode (Ultra X) under normal conditions of use as it is approved for this device. Participants receive interventions as part of routine medical care.
Hear Again, Work Again
Hearing LossSensorineural10 moreProject synopsis: This prospective cohort study investigates impact of (1) hearing loss and (2) cochlear implantation on cost and health state on one hand, and employment, productivity and social wellbeing on the other hand in a professionally active group using validated questionnaires. Participants: The investigators aim to include 100 professional active adults between 18 and 65 years old. All participants have a bilateral severe-to-profound sensorineural or mixed hearing loss. Due to several reasons such as an optimal hearing aid fitting, the presence of a residual hearing or physical contra-indications, half of these participants will not be implanted with CI. Additionally, 100 participants with a single-sides deafness (SSD) in the acute or chronic setting (SDD present for at least three months) will be included. Study design Study measures: All audiological tests and patient-reported outcome measures will be included in this test protocol and repeated throughout the follow-up visits depending on aided or unaided setting. The cognitive evaluation will be executed during the first and last test moment. The investigators anticipate that the protocol will take 1 hour per follow-up in the hospital (audiological testing and to go over the questionnaires), plus an extra hour at home to fill out the questionnaires. For the cognitive tests, an extra hour in the hospital will be scheduled. Hypothesis As a primary endpoint, the investigators anticipate demonstrating that severe-to-profound hearing loss has a significant impact on sick leave and self-reported productivity. Health state will also be analyzed as a secondary endpoint because the investigators anticipate only marginal improvement (if any) on these instruments due to the lack of sensitivity and responsiveness, even in this population. The investigators will also determine the rate of usage and non-usage at this long-term follow-up to demonstrate the utility of cochlear implants. Statistical analysis IBM SPSS Statistics (IBM; Armonk, NY) will be used for the statistical analyses. The participants' hearing profiles will be summarized using descriptive statistics (median, and range). In view of the sample size, non-parametric tests and linear mixed models (to describe evolution in time and difference between groups) will be used. Quantitative data will be presented as median and range (minimum and maximum). Descriptives will be used to summarize the outcomes of the subjective data logging. For the speech perception in noise results, a Wilcoxon signed-rank test will be used. In addition, to correct for the multiple speech in noise test configurations, Holm's correction will be applied. The level of significance will be set at p.0.05. Data storage REDCap (Research Electronic Data Capture) is a secure, web-based application designed exclusively to support data capture for research studies. REDCap provides an interface for data entry (with data validation) and audit trails for tracking data manipulation and export procedures. Data will be pseudomized before storage in REDCap.
Assessment of The Efficacy of Intratympanic Platelet Rich Plasma for Treatment of Sensorineural...
Hearing LossSudden3 moreAssessment of the efficacy of Intratympanic platelet rich plasma injection for treatment of idiopathic suden sensorineural hearing loss
A Monocentric Study Evaluating Pupillometry as an Objective Measurement for CI Fittings
Hearing LossSensorineural5 moreThis trial is a monocentric, prospective and controlled study of the pupil response to detect hearing threshold and comfortable loudness of normal-hearing (NH) and CI-subjects.
National Registry of Rare Kidney Diseases
Adenine Phosphoribosyltransferase DeficiencyAH Amyloidosis85 moreThe goal of this National Registry is to is to collect information from patients with rare kidney diseases, so that it that can be used for research. The purpose of this research is to: Develop Clinical Guidelines for specific rare kidney diseases. These are written recommendations on how to diagnose and treat a medical condition. Audit treatments and outcomes. An audit makes checks to see if what should be done is being done and asks if it could be done better. Further the development of future treatments. Participants will be invited to participate on clinical trials and other studies. The registry has the capacity to feedback relevant information to patients and in conjunction with Patient Knows Best (Home - Patients Know Best), allows patients to provide information themselves, including their own reported quality of life and outcome measures.
Efficacy of Auditory Training and Combined Auditory-working-memory Training in Improving Communication...
Hearing LossUsing a randomized controlled trial, we are going to examine whether a training program that incorporates both auditory training and working memory training (AT-WMT) would generate significantly better results in communication and cognition than an auditory training program and a mindfulness training program (active-control). Participants are 120 older adults (40 per group) with untreated mild-to-moderate sensorineural hearing impairment .