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.
Natural History Study for Charcot Marie Tooth Disease
Charcot-Marie-Tooth DiseaseCharcot-Marie-Tooth51 moreThe goal of this Natural History Study for Charcot-Marie-Tooth is to acquire, record, and analyze patient-reported data and associated genetic reports, Electronic Health Records (EHRs) and clinical notes to identify the burden, diagnostic journey, and prevalence of disease that will aid scientists in their work toward finding a cure. Participants will be asked to complete a Natural History Survey.
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.
Immunity After Cochlear Implantation and Perilymph Molecular Profiles in Sensorineural Hearing Loss...
Sensorineural Hearing LossMany patients receiving cochlear implant (CI) have some residual hearing prior to implantation surgery. However, approximately one third of them will lose it in next 6 months after cochlear implantation. Although the mechanisms involved in the residual audition loss remain unknown, animal experiments suggest the role of inflammatory or immune reaction in the cochlea. The goal of this project is to search in the perilymph (a fluid which fills the scala vestibuli and scala tympani of the cochlea) some predictive biomarkers of the residual hearing loss using modern proteomics and immunological techniques. A parallel search for blood biomarkers of post-implantation residual hearing loss and for molecular and cellular evidences of immune response to cochlear implantation will be performed. The study will recruit 50 subjects-candidates for cochlear implantation surgery; 30 normally hearing individuals eligible for other types of otological interventions will form a control group. For all the participants blood samples will be collected and preserved. In addition, the perilymph sampling will be performed during cochlear implantation surgery. This project relies on the calculation that from 50 patients post-cochlear implantation, 15 patients will form a group with maintained residual hearing and 15 will display delayed hearing loss. For these 30 subjects together with the control group the blood biomarkers search will be performed. For the group of implanted patients, the follow-up will last for 12 months with 6 visits in total : the inclusion visit V0 during which the study will be presented and the consent form will be signed the surgery visit V1 with blood sample and perilymph collection the activation visit V2, 1 month ± 1 week after V1, visit V3 3 months ± 7 days after V1, V4 6 months ± 7 days after V1, V5 12 months ± 1 month after V1. During each of this visit blood sampling will be performed. For the control group, the follow up will be 6 months long with 4 visits in total arranged during the routine follow-up appointments: the inclusion visit V0 during which the study will be presented and the consent form will be signed the surgery visit V1, the 1 month visit V2 and the 6 months visit V3 during which the blood sampling will be performed.
Exploiting the Potential of Neural Attentional Control to Overcome Hearing Impairment
Hearing LossAttention Impaired1 moreThis study will improve the understanding of the cerebral mechanisms that underlie the control of auditory selective attention and evaluate the potential of neuromodulation to enhance neural attention control as a possible way to overcome hearing impairment. First, electroencephalography (EEG) will be applied to identify neural marker of auditory attention in individuals with hearing loss (HL), tinnitus (TI) and normal hearing (NH). Afterwards, the importance of the identified markers for attention control will be tested using non-invasive transcranial alternating current stimulation (tACS) and neurofeedback (NF).
EarGenie: Assessment of a Minimum Viable Product
Hearing Impaired ChildrenThe goal of this clinical trial is to evaluate safety and preliminary efficacy of a novel device (EarGenie MVP) to assess hearing function in infants, using a small number of infants with normal hearing. The main questions it aims to answer are: Is our device safe? Does the device provide preliminary results consistent with previous results from a commercial functional Near Infrared Spectroscopy (fNIRS) research device? Participants will attend one test session and have their hearing assessed with the EarGenie MVP device.
Effects of Web-Based Adolescent Health Promotion Program in Hearing Impaired Adolescents
Health PromotionOur research is to evaluate the effect and applicability of the adolescent health promotion program, which includes multiple behavioral interventions, on physical activity, nutrition and mental health of hearing impaired adolescents living in the community.