Natural History in Children up to 10 Years With Moderate to Profound Hearing Loss Due to Mutations...
Sensorineural Hearing LossBilateral6 moreThe purpose of this study is to follow the natural history of non-syndromic hearing loss caused by mutations in two genes (GJB2 or OTOF) in children up to 10 years of age.
Otoferlin Patient Registry and Natural History Study
Otoferlin-related Auditory SynaptopathyHearing ImpairmentThis registry is designed to collect comprehensive information about the molecular genetic diagnoses of individuals with otoferlin-associated hearing impairment and clinical information to support a natural history study.
Correlation of Radiological Lesions With Vestibular Function in Patients With Bilateral Vestibulopathy...
Hearing LossSensorineural3 moreIn 2014 radiologic lesions were detected at one or more semicircular canals using CT and MR imaging of temporal bone in subjects carrying the p.P51S mutation in COCH. These lesions are believed to present at more advanced stages of the hearing and vestibular deterioration. Since then, other authors have described similar lesions in advanced non-genetic hearing and vestibular impairment as well. The purpose of this study is therefore to assess the radiologic investigation using CT and MR imaging of temporal bone to all subjects presenting with bilateral vestibulopathy, using the Barany criteria, compared to the p.P51S population.
Study of Viral Transduction of Human Auditory Sensory Cells for the Development of Gene Therapy...
Hearing LossSensorineuralGene therapy is a promising strategy to treat hearing loss and vestibular disorders, and Associated adenovirus (AAV) is shown as a good viral vector for inner ear therapy in animal models. This study aims to study in vitro viral transduction of AAV in human inner ear cells, collected during non-conservative surgeries for vestibular schwannoma.
Evaluation of the BHX Implant in Children With a BAHS
Hearing LossConductiveThe purpose of this study is to in a proactive manner collect high quality data from pediatric patients who have undergone a bone anchored hearing surgery, using the Ponto BHX implant, in clinical practice. This to further increase the knowledge of bone anchored hearing systems in the pediatric population.
Inventory of Radiological and Vestibular Function in Cochlear Implant Candidates
Hearing LossSensorineural3 moreIn 2014 radiological lesions at one or more semicircular canals (SCC) were described using CT & MR imaging in subjects presenting advanced hearing and vestibular deterioration caused by the p.P51S mutation in COCH. Similar lesions were also described in other non-genetic advanced hearing and vestibular deterioration as well. With this prospective observational study it is the purpose to inventory imaging results of candidates for cochlear implantation which are routinely performed during the preoperative work up, since these patient present severe hearing impairment at both ears. A considerable part of them might present vestibular deterioration as well. It is the purpose to detect possible presence of these SCC lesions on CT and MR in this population and the prevalence of these lesions compared to DFNA9 patients.
Sound Ear Check (SEC) in Hearing Diagnostics in Young Children
Hearing LossOtitis Media With Effusion1 moreStudy Sound Ear Check (SEC) hearing test among 3-10 years old otherwise healthy children referred to tympanostomy tube placement. SEC test prior surgery (with middle ear fluid) and at 1 month control visit (dry middle ear with ventilation tube). Otitis media 6 questionnaire prior surgery and at the 1 month control visit.
Cross-sectional Evaluation of the Subjective Performance and Satisfaction With Ponto Sound Processors...
Hearing LossHearing Loss3 moreThe study is a non-interventional, observational, cross-sectional, study on users of Ponto sound processors. The purpose of this study is to evaluate subjectively assessed hearing performance and satisfaction with Oticon Medical's sound processors fitted with the Genie Medical fitting software.
Investigation of Anatomical Correlates of Speech Discrimination
Sensorineural Hearing LossUnderstanding 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.
K-HEARS MCI: Hearing Health Care for Korean American Older Adults With Mild Cognitive Impairment...
Age-related Hearing ImpairmentPersonal Communication1 moreThe objective of this study is to develop and assess the feasibility, acceptability, and preliminary efficacy of a community-delivered, affordable, and accessible hearing care intervention tailored to the needs of community-dwelling Korean American (KA) older adults with mild cognitive impairment (MCI) and their care partners that integrates a low-cost over-the-counter amplification device and hearing rehabilitation through a randomized controlled pilot study.