Sensorineural Hearing Loss, Bilateral

The 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 16 years of age.

The purpose of this study is to characterize and assess the evolution of hearing impairment of patients with adulthood-onset bilateral sensorineural hearing loss carrying mutations on GJB2 gene.

Main objective: Compare speech recognition in noise with tonotopic setting (FS4T) and with tonotopic fitting modified by evolutionary algorithm (EAFS4T) in adult patients implanted for 6 months or more with a MED-EL cochlear implant with FS4T. Secondary objectives: Comparison of FS4T and EAFS4T settings * for speech recognition in quiet * for subjective auditory spatial perception * for subjective auditory and musical perception

Main objective: Compare speech recognition in noise with tonotopic fitting (FS4T) and with conventional non-tonotopic fitting (FS4noT) in adult patients implanted for 6 months with a MED-EL cochlear implant with FS4noT strategy. Secondary objectives: Comparison of FS4noT and FS4T settings * for speech recognition in quiet * for subjective auditory spatial perception

Part A of this trial will evaluate the safety and tolerability of a single surgical administration procedure in one or both ear(s) with one of two dose levels of AAVAnc80-hOTOF and will evaluate the Akouos Delivery Device, together with the Precision Delivery Mechanism, to safely achieve the intended product performance.

This is a retrospective and prospective longitudinal study in participants with Otoferlin Gene-Mediated Hearing Loss.

Main objective: For a bimodal fitting (hearing aid (HA) + cochlear implant (CI)): Comparison of a tonotopy based fitting strategy (TFS4) to a default fitting strategy (FS4) for the speech recognition in noise. Secondary objectives: Comparison of TFS4 to FS4 for speech recognition in quiet. Comparison of TFS4 to FS4 for the auditory skills experienced by the subject.

Main objective: For a bimodal fitting (hearing aid (HA) + cochlear implant (CI)): Comparison of a tonotopy based fitting strategy with synchronization between HA and CI (ABFS) to a tonotopy based fitting strategy without synchronization (ABFnoS) for the accuracy of sound localization. Secondary objectives: Comparison of ABFS to ABFnoS for the bias of sound localization. Comparison of ABFS to ABFnoS for speech perception in noise. Comparison of ABFS to ABFnoS for the auditory skills experienced by the subject.

Main objective: Investigate on new cochlear implanted patients whether the FineHearing strategy of the MED-EL cochlear implant gives better results on musical perception if the depth of stimulation (stimulation or not of the apical areas) is greater. Secondary objectives: Evaluate the effect of stimulation depth on vocal audiometric results, results of differential frequency threshold test and on qualitative sound perception.

On June 23, 2021, the Oticon Medical Neuro Cochlear Implant System (NCIS) was granted premarket approval (PMA) in the US to treat individuals 18 years or older, with bilateral severe-to-profound sensorineural hearing loss, who obtain limited benefit from appropriately fitted hearing aid(s). To help assure the continued safety and effectiveness of an approved device, a post-approval study was required as a condition of approval under 21 CFR 814.82(a)(2). The purpose of this study is to provide longer-term data on the safety and effectiveness of the Neuro Cochlear Implant System under general conditions of use in the postmarket environment.