11 Clinical Trials for Auditory Loss and Deafness
This is a retrospective and prospective longitudinal study in participants with Otoferlin Gene-Mediated Hearing Loss.
Part A of this trial will evaluate the safety and tolerability of a single unilateral administration of one of two dose levels of AAVAnc80-hOTOF and will evaluate the Akouos delivery device to safely achieve the intended product performance.
The proposed study will investigate whether an auditory brain training program can improve cochlear implant (CI) outcomes in older post-lingually deafened CI users. The study will evaluate the potential benefit of training on speech recognition performance, psychosocial and cognitive function.
This study is a feasibility study of the Nucleus 24 ABI in children without NF2. It will be conducted as a repeated-measures, single subject experiment in order to accommodate the known variability in cochlear implant clinical results. Data obtained in the course of this study will be compared with the existing published outcome data from children with normal hearing and with cochlear implants (CIs). These comparisons may provide additional useful information about the progress of ABI subjects' performance over time. Also, this may allow the results of the current study to be utilized to design future pivotal studies of the device in the pediatric population.
The purpose of this study is to compare the effectiveness of a free computer-based auditory training program, Sound Success (Advanced Bionics, Valencia, CA), with the current standard of care of patient-directed auditory training in improving common measures of speech recognition and cochlear implant (CI) specific quality-of-life in new adult CI recipients in their first year post-activation.
This goal of this study is to learn if a new audiology treatment process called the self-efficacy-based auditory rehabilitation (SEBAR) can improve confidence and success with over-the-counter (OTC) hearing aids. The main questions it aims to answer are: * Can the SEBAR improve participants' confidence related to managing their over-the-counter hearing aids? * Can the SEBAR improve participants' willingness to adopt over-the-counter hearing aids, their satisfaction, quality of life, and their emotional state? Researchers will compare these outcomes of the SEBAR with and without wearing OTC hearing aids. Participants will: * Visit for one appointment to complete a few questionnaires without OTC hearing aids * Wear a pair of ITC hearing aids for a week and use an app to answer questions about their experiences * Visit for a second appointment to complete the same questionnaires with OTC hearing aids.
Regeneron is conducting a study of an investigational new drug called DB-OTO. DB-OTO is a gene therapy that is being developed to treat children who have hearing loss due to changes in the otoferlin gene. The purpose of this study is to: * Learn about the safety of DB-OTO * Determine how well DB-OTO is tolerated (does not cause ongoing discomfort) * Evaluate the efficacy of DB-OTO (how well DB-OTO works)
People with hearing loss experience extra effort when listening, which can lead to severe psychological barriers to communication and social participation. Listening effort can lead to fatigue, mental strain, burnout, medical sick leave, and the need for increased time to recover from regular daily activities. This proposal aims to understand effort changes on a moment-to-moment basis during listening, how long the effort lasts, and how the planning and execution of effort is impacted by the experience of using a cochlear implant.
Since 2000, at least 250,000 U.S. Service members have experienced a blast-related mild traumatic brain injury. A retrospective analysis of over 100,000 post-9/11 Veterans shows that blast injury more than doubles the risk of a diagnosed auditory problem. Many blast-exposed Veterans experience "functional hearing difficulties" (FHDs): problems in challenging listening environments despite clinically normal hearing as measured by the pure-tone audiogram. VA audiologists have begun using low-gain hearing aids to treat FHDs, but there are no concrete guidelines for this application given standard procedures rely on the pure-tone audiogram. This study proposes a data-driven approach called speech-based audiometry (SBA), which optimizes hearing aid gains from a patient's responses to speech stimuli in aided conditions. This trial will assess the behavioral (speech recognition in noise, subjective listening difficulty) and neurophysiological (functional neuroimaging during a speech recognition task) benefits of low-gain hearing aids programmed conventionally or with SBA among blast-exposed Veterans with FHDs.
Understanding speech is essential for good communication. Individuals with hearing loss and poor speech discrimination often have little success with hearing aids because amplifying sound improves audibility, but not clarity of the speech signal. The purpose of this study is to determine the relative importance of the sensory cells of the inner ear and auditory neurons on speech discrimination performance in quiet and in noise. This information may be used as a predictor of hearing aid benefit. The investigators expect to find decreased speech understanding ability resulting from both loss of sensory cells and the loss of auditory neurons.
Patients with biochemically confirmed SLOS are being treated with cholesterol supplementation and antioxidant medication. They are carefully monitored with visits to clinic, laboratory testing including cholesterol and 7-dehydrocholesterol levels, vitamin levels, blood counts and liver and kidney function. On a serial basis, no more often than once a year, the patients undergo a series of tests under anesthesia, including electroretinogram (ERG), brainstem audiometry (ABR), and ophthalmologic exam under anesthesia to follow pigmentary retinopathy.