[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"sensorineural-hearing-loss-bilateral\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:sensorineural-hearing-loss-bilateral":25},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,10,0,[8,45,76,104,123,153,173,198,219,242],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":15,"eligibilityCriteria":16,"healthyVolunteers":11,"sex":17,"minAge":4,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":23,"conditions":24,"keywords":4,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":33,"lastUpdatePostDateStruct":34,"startDateStruct":37,"completionDateStruct":39,"leadSponsor":41,"locationsCount":44},"100470205","natural-history-in-children-up-to-16-years-with-mild-to-profound-hearing-loss-due-to-mutations-in-gjb2--otof-genes-100470205",false,"NCT05402813","Natural History in Children up to 16 Years With Mild to Profound Hearing Loss Due to Mutations in GJB2 \u002F OTOF Genes","Longitudinal Study of the Natural History of Two Autosomal Recessive Non Syndromic Deafness (DFNB1A and DFNB9) in Children up to 16 Years of Age","Otoconex","Main Inclusion Criteria:\n\nParticipants meeting all the following main inclusion criteria will be eligible to participate in the study:\n\n* Aged ≤ 16 years on the date of signed informed consent for cohort 1 and ≤ 10 years for cohort 2;\n* With a diagnosis of non-syndromic, bilateral, mild to profound, sensorineural hearing loss (according to the American Speech Language-Hearing Association);\n* With documented genotyping results showing mutation(s) in GJB2 or OTOF genes;\n* Written informed consent as required by local regulations.\n* Either without Cochlear Implant, or with unilateral or bilateral Cochlear Implant(s)\n\nExclusion Criteria:\n\nParticipants presenting with any of the following main exclusion criteria will not be included in the study\n\n* Other type of deafness, such as unilateral deafness, persistent conductive deafness, malformation syndrome, syndromic deafness, known familial deafness linked to mutations in other genes than OTOF or GJB2;\n* Documented genotyping results showing pathogenic mutation(s) in other gene(s) than GJB2 or OTOF genes in the tested panel;\n* Unable and\u002For unwilling to comply with all the protocol requirements and\u002For study procedures.","ALL","16 Years",{"count":20,"type":21},180,"ESTIMATED","OBSERVATIONAL","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.",[25,26,27,28,29,30,31],"Sensorineural Hearing Loss, Bilateral","AUNB1","DFNB1A","Congenital Deafness","DFNB9","OTOF Gene Mutation","GJB2 Gene Mutation","RECRUITING","2026-05-28",{"date":35,"type":36},"2026-06-01","ACTUAL",{"date":38,"type":36},"2022-11-18",{"date":40,"type":21},"2031-06",{"name":42,"class":43},"Sensorion","INDUSTRY",1,{"id":46,"slug":47,"hasResults":11,"nctId":48,"briefTitle":49,"officialTitle":49,"acronym":50,"eligibilityCriteria":51,"healthyVolunteers":11,"sex":17,"minAge":52,"maxAge":53,"enrollmentInfo":54,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":56,"conditions":57,"keywords":58,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":67,"lastUpdatePostDateStruct":68,"startDateStruct":70,"completionDateStruct":72,"leadSponsor":74,"locationsCount":75},"100543295","cross-sectional-and-prospective-study-to-characterize-early-onset-presbycusis-100543295","NCT06354010","Cross-sectional and Prospective Study to Characterize Early-onset Presbycusis","SONG","Inclusion Criteria:\n\n1. Female or Male patients ≥30 and ≤55 years old\n2. Bilateral hearing loss first noticed after the age of 16 years old\n3. Documented genotyping results showing mutations in GJB2 gene.\n\nExclusion Criteria:\n\n1. Deafness with a known, non-genetic cause\n2. To the opinion of the investigator, unable and\u002For unwilling to comply with all the protocol requirements and\u002For study procedures","30 Years","55 Years",{"count":55,"type":21},100,"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.",[25],[59,60,61,62,63,64,50,65,66],"Adulthood-onset bilateral sensorineural hearing loss","GJB2","Presbycusis","hearing impairement","Deafness","Connexin 26","Genetic deafness","Adult deafness","2026-05-04",{"date":69,"type":36},"2026-05-08",{"date":71,"type":36},"2024-06-14",{"date":73,"type":21},"2027-07",{"name":42,"class":43},2,{"id":77,"slug":78,"hasResults":11,"nctId":79,"briefTitle":80,"officialTitle":81,"acronym":4,"eligibilityCriteria":82,"healthyVolunteers":11,"sex":17,"minAge":83,"maxAge":4,"enrollmentInfo":84,"targetDuration":4,"studyType":86,"phases":87,"briefSummary":89,"conditions":90,"keywords":91,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":95,"lastUpdatePostDateStruct":96,"startDateStruct":98,"completionDateStruct":100,"leadSponsor":102,"locationsCount":44},"100572739","comparison-of-speech-understanding-between-tonotopy-based-fitting-and-setting-based-on-evolutionary-algorithms-100572739","NCT06737185","Comparison of Speech Understanding Between Tonotopy-based Fitting and Setting Based on Evolutionary Algorithms","Comparison of Speech Understanding Between Tonotopy-based Fitting and Evolutionary Algorithm-based Setting: a Prospective Cross-sectional Study","Inclusion Criteria:\n\n* Adult patient (\\>= 18 years old) speaking French\n* Patient who fulfils the criteria for cochlear implantation\n\nExclusion Criteria:\n\n* retro-cochlear pathology: auditory neuropathy, vestibular schwannoma","18 Years",{"count":85,"type":21},22,"INTERVENTIONAL",[88],"NA","Main objective:\n\nCompare 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.\n\nSecondary objectives:\n\nComparison of FS4T and EAFS4T settings\n\n* for speech recognition in quiet\n* for subjective auditory spatial perception\n* for subjective auditory and musical perception",[25],[92,93,94],"cochlear implant","evolutionary algorithms","tonotopy-based fitting","2026-04-30",{"date":97,"type":36},"2026-05-06",{"date":99,"type":36},"2025-01-15",{"date":101,"type":21},"2028-01-15",{"name":103,"class":43},"MED-EL Elektromedizinische Geräte GesmbH",{"id":105,"slug":106,"hasResults":11,"nctId":107,"briefTitle":108,"officialTitle":109,"acronym":4,"eligibilityCriteria":82,"healthyVolunteers":11,"sex":17,"minAge":83,"maxAge":4,"enrollmentInfo":110,"targetDuration":4,"studyType":86,"phases":112,"briefSummary":113,"conditions":114,"keywords":115,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":95,"lastUpdatePostDateStruct":117,"startDateStruct":118,"completionDateStruct":120,"leadSponsor":122,"locationsCount":44},"100571669","speech-perception-of-a-tonotopy-based-fitting-for-cochlear-implant-recipients-for-6-months-with-conventional-setting-100571669","NCT06723262","Speech Perception of a Tonotopy-based Fitting for Cochlear Implant Recipients for 6 Months With Conventional Setting","Contribution to Speech Perception of a Tonotopy-based Fitting for Patients With Cochlear Implants for 6 Months With a Conventional Setting: Prospective Cross-sectional Study",{"count":111,"type":21},25,[88],"Main objective:\n\nCompare 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.\n\nSecondary objectives:\n\nComparison of FS4noT and FS4T settings\n\n* for speech recognition in quiet\n* for subjective auditory spatial perception",[25],[116],"cochlear implant strategy anatomy-based fitting",{"date":97,"type":36},{"date":119,"type":36},"2025-01-01",{"date":121,"type":21},"2028-06-30",{"name":103,"class":43},{"id":124,"slug":125,"hasResults":11,"nctId":126,"briefTitle":127,"officialTitle":128,"acronym":4,"eligibilityCriteria":129,"healthyVolunteers":11,"sex":17,"minAge":4,"maxAge":4,"enrollmentInfo":130,"targetDuration":4,"studyType":86,"phases":131,"briefSummary":134,"conditions":135,"keywords":136,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":143,"lastUpdatePostDateStruct":144,"startDateStruct":146,"completionDateStruct":148,"leadSponsor":150,"locationsCount":152},"100502415","phase-1-gene-therapy-trial-for-otoferlin-gene-mediated-hearing-loss-100502415","NCT05821959","Gene Therapy Trial for Otoferlin Gene-mediated Hearing Loss","A Trial of AAVAnc80-hOTOF Gene Therapy in Individuals With Sensorineural Hearing Loss Due to Otoferlin Gene Mutations","Criteria for Inclusion:\n\n1. Participants may be of any age, based on Cohort Criteria\n2. At least two mutations in the otoferlin gene\n3. Clinical presentation of Profound bilateral sensorineural hearing loss as assessed by ABR\n4. Preserved distortion product otoacoustic emissions (DPOAEs)\n5. Able and willing to comply with all study requirements, including willingness to participate in a separate long term follow-up study after completion of this trial\n\nCriteria for Exclusion:\n\n1. Persistent ear infections, anatomic or other abnormalities of the ear, and\u002For medical conditions that would contraindicate undergoing surgery, anesthesia, and\u002For administration of investigational gene therapy\n2. Cochlear Implant(s) in the ear(s) to receive AAVAnc80-hOTOF\n3. Prior participation in a clinical trial with an investigational drug, within six months prior to administration, or any prior participation in a gene therapy clinical trial",{"count":85,"type":21},[132,133],"PHASE1","PHASE2","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.",[25],[137,138,139,140,141,142],"Otoferlin","Auditory neuropathy (AN)","Deafness, autosomal recessive 9 (DFNB9)","Hearing loss","Hearing loss, bilateral","Hearing loss, sensorineural","2026-03-03",{"date":145,"type":36},"2026-03-04",{"date":147,"type":36},"2023-09-15",{"date":149,"type":21},"2028-10",{"name":151,"class":43},"Akouos, Inc.",9,{"id":154,"slug":155,"hasResults":11,"nctId":156,"briefTitle":157,"officialTitle":158,"acronym":4,"eligibilityCriteria":159,"healthyVolunteers":11,"sex":17,"minAge":4,"maxAge":160,"enrollmentInfo":161,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":163,"conditions":164,"keywords":165,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":143,"lastUpdatePostDateStruct":167,"startDateStruct":168,"completionDateStruct":170,"leadSponsor":172,"locationsCount":5},"100483210","otoferlin-gene-mediated-hearing-loss-natural-history-study-100483210","NCT05572073","Otoferlin Gene-mediated Hearing Loss Natural History Study","A Natural History Study in Individuals With Otoferlin Gene-mediated Hearing Loss","Inclusion Criteria:\n\n1. Clinical presentation of bilateral sensorineural hearing loss (SNHL), including auditory neuropathy (AN) \u002F auditory neuropathy spectrum disorder (ANSD) phenotype or medical history of AN \u002F ANSD phenotype earlier in life\n2. Mutation(s) in the otoferlin gene\n3. Able and willing to comply with all study requirements, as evidenced by successful completion of the informed consent (and assent, if applicable) process\n\n   Additional Criteria for Inclusion in the Prospective Phase:\n4. Presence of OAE \u002F CM and absent \u002F abnormal ABRs in at least one ear (that does not have a cochlear implant) within 12 months prior to or at the Month 0 visit\n\nExclusion Criteria:\n\n1. Unwillingness or inability of the potential participant and\u002For legally authorized representative to comply with all protocol requirements\n2. Presence of cochlear nerve deficiency and\u002For cochlear nerve dysplasia\n\n   Additional Criteria for Exclusion from the Prospective Phase:\n3. Presence of bilateral cochlear implants at the time of record review or planned within the next 6 months\n4. Presence of middle ear or auditory brainstem implant(s) at the time of record review or planned within the next 6 months\n5. Any condition that would not allow the potential participant to complete follow-up assessments during the course of the study and\u002For, in the opinion of the Investigator, makes the potential participant unsuitable for the study\n\nNote: Potential participants will not be excluded based on their sex, gender, race, or ethnicity","44 Years",{"count":162,"type":21},150,"This is a retrospective and prospective longitudinal study in participants with Otoferlin Gene-Mediated Hearing Loss.",[25],[166],"Otoferlin Gene, sensorineural hearing loss (SNHL), auditory neuropathy, auditory neuropathy spectrum disorder",{"date":145,"type":36},{"date":169,"type":36},"2022-07-14",{"date":171,"type":21},"2029-02",{"name":151,"class":43},{"id":174,"slug":175,"hasResults":11,"nctId":176,"briefTitle":177,"officialTitle":178,"acronym":4,"eligibilityCriteria":179,"healthyVolunteers":11,"sex":17,"minAge":83,"maxAge":4,"enrollmentInfo":180,"targetDuration":4,"studyType":86,"phases":182,"briefSummary":183,"conditions":184,"keywords":186,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":190,"lastUpdatePostDateStruct":191,"startDateStruct":193,"completionDateStruct":195,"leadSponsor":197,"locationsCount":44},"100512670","comparison-in-new-cochlear-implanted-subjects-of-a-tonotopy-based-bimodal-fitting-and-a-conventional-fitting-100512670","NCT05955469","Comparison in New Cochlear Implanted Subjects of a Tonotopy-based Bimodal Fitting and a Conventional Fitting","Comparison of Speech Understanding Between a Tonotopy-based Bimodal Fitting and a Default Bimodal Fitting in Newly Cochlear-implanted Patients: a Double-blind Randomized Crossover Study.","Inclusion Criteria:\n\n* Adult patient (\\>= 18 years old) speaking French\n* Patient who fulfils the criteria for cochlear implantation\n\nExclusion Criteria:\n\n* retro-cochlear pathology: auditory neuropathy, vestibular schwannoma\n* patient with residual hearing \\\u003C 70 dB hearing level (HL) at 500 Hz and 1000 Hz on the contralateral ear",{"count":181,"type":21},20,[88],"Main objective:\n\nFor 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.\n\nSecondary objectives:\n\nComparison of TFS4 to FS4 for speech recognition in quiet. Comparison of TFS4 to FS4 for the auditory skills experienced by the subject.",[25,185],"Bilateral Hearing Loss",[187,188,189],"Cochlear implant","Bimodal fitting","Anatomy-based fitting","2026-01-16",{"date":192,"type":36},"2026-01-20",{"date":194,"type":36},"2024-11-05",{"date":196,"type":21},"2027-01-01",{"name":103,"class":43},{"id":199,"slug":200,"hasResults":11,"nctId":201,"briefTitle":202,"officialTitle":203,"acronym":4,"eligibilityCriteria":204,"healthyVolunteers":11,"sex":17,"minAge":83,"maxAge":4,"enrollmentInfo":205,"targetDuration":4,"studyType":86,"phases":206,"briefSummary":207,"conditions":208,"keywords":209,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":211,"lastUpdatePostDateStruct":212,"startDateStruct":214,"completionDateStruct":216,"leadSponsor":218,"locationsCount":44},"100508303","comparison-in-new-cochlear-implanted-subjects-of-a-tonotopy-based-bimodal-fitting-with-or-without-synchronization-100508303","NCT05898659","Comparison in New Cochlear Implanted Subjects of a Tonotopy-based Bimodal Fitting With or Without Synchronization","Comparison of a Bimodal Fitting With Synchronization and a Conventional Bimodal Fitting in Newly Implanted Cochlear Patient. Prospective Monocentric Randomized Double-blind Crossover Study.","Inclusion Criteria:\n\n* Adult patient (\\>= 18 years old) speaking French\n* Patient who fulfils the criteria for cochlear implantation\n* Total hearing loss for less than 5 years\n\nExclusion Criteria:\n\n* retro-cochlear pathology: auditory neuropathy, vestibular schwannoma\n* patient with residual hearing \\\u003C 70 dB hearing level (HL) at 250 Hz and 500 Hz and \\\u003C 80 dB HL at 1000 Hz on the contralateral ear",{"count":181,"type":21},[88],"Main objective:\n\nFor 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.\n\nSecondary objectives:\n\nComparison 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.",[25],[92,210,94],"bimodal fitting","2026-01-12",{"date":213,"type":36},"2026-01-14",{"date":215,"type":36},"2023-10-17",{"date":217,"type":21},"2026-12-15",{"name":103,"class":43},{"id":220,"slug":221,"hasResults":11,"nctId":222,"briefTitle":223,"officialTitle":224,"acronym":4,"eligibilityCriteria":225,"healthyVolunteers":11,"sex":17,"minAge":83,"maxAge":4,"enrollmentInfo":226,"targetDuration":4,"studyType":86,"phases":228,"briefSummary":229,"conditions":230,"keywords":231,"overallStatus":32,"whyStopped":4,"lastUpdateSubmitDate":234,"lastUpdatePostDateStruct":235,"startDateStruct":237,"completionDateStruct":239,"leadSponsor":241,"locationsCount":44},"100407873","auditory-performances-with-different-stimulation-depths-in-cochlear-implanted-subjects-using-a-fine-structure-strategy-100407873","NCT04591093","Auditory Performances With Different Stimulation Depths in Cochlear Implanted Subjects Using a Fine Structure Strategy","Study of Musical Perception and Post-operative Auditory Performance as a Function of the Depth of Stimulation in Subjects Implanted With a MED-EL Cochlear Implant and Using a FineHearing Strategy Single-center Randomized Cross-over Study","Inclusion Criteria:\n\n* Adult patient (≥ 18 years old) speaking French\n* Patient who fulfils the criteria for cochlear implantation\n* Patient with a postoperative insertion angle of the apical electrode \\> 450°\n* Patient with 12 active electrodes on the day of activation.\n\nExclusion Criteria:\n\n* Retro-cochlear pathology: auditory neuropathy, vestibular schwannoma",{"count":227,"type":21},32,[88],"Main objective:\n\nInvestigate 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.\n\nSecondary objectives:\n\nEvaluate the effect of stimulation depth on vocal audiometric results, results of differential frequency threshold test and on qualitative sound perception.",[25],[92,232,233],"stimulation strategy","sound coding strategy","2025-11-18",{"date":236,"type":36},"2025-11-19",{"date":238,"type":36},"2021-07-10",{"date":240,"type":21},"2026-09-30",{"name":103,"class":43},{"id":243,"slug":244,"hasResults":11,"nctId":245,"briefTitle":246,"officialTitle":246,"acronym":247,"eligibilityCriteria":248,"healthyVolunteers":11,"sex":17,"minAge":83,"maxAge":4,"enrollmentInfo":249,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":251,"conditions":252,"keywords":255,"overallStatus":258,"whyStopped":4,"lastUpdateSubmitDate":259,"lastUpdatePostDateStruct":260,"startDateStruct":262,"completionDateStruct":264,"leadSponsor":265,"locationsCount":4},"100451106","post-approval-study-to-assure-the-continued-safety-and-effectiveness-of-neuro-cochlear-implant-system-in-adult-users-100451106","NCT05154188","Post Approval Study to Assure the ContInued saFety and effectIveness of Neuro Cochlear Implant System in Adult Users","PACIFIC","Inclusion Criteria:\n\n* Individuals 18 years of age or older\n* Obtain limited benefit from appropriately fitted hearing aids\n* Bilateral severe-to-profound sensorineural hearing loss \\_ Severe-to-profound hearing loss is determined by a pure-tone average (PTA) superior or equal (≥) to 70 dB HL at 500, 1000 and 2000 Hz. Limited benefit from amplification is defined by scores of 50% or less on a validated sentence recognition test in quiet (AzBio sentences), in the best-aided listening condition \\_\n\nExclusion Criteria:\n\n* Previous cochlear implantation\n* Ossification or any other cochlear anomaly that might prevent complete insertion of the electrode array\n* Active external or middle ear infections or tympanic membrane perforation in the ear to be implanted\n* Presence of medical contraindications to middle-ear or inner-ear surgery or anesthesia as required\n* Diagnosis of retro-cochlear pathology\n* Diagnosis of auditory neuropathy\n* Unrealistic expectations on the part of the subject regarding the possible benefits, risks, and limitations that are inherent to the surgical procedure and use of the prosthetic device\n* Unwillingness or inability to comply with all investigational requirements\n* Additional cognitive, medical, or social handicaps that would prevent completion of all study requirements",{"count":250,"type":21},60,"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).\n\nTo 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).\n\nThe 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.",[25,253,254],"Sensorineural Hearing Loss, Severe","Sensorineural Hearing Loss, Profound",[256,257],"candidates for a cochlear implantation surgery","patients aged 18 years and older","NOT_YET_RECRUITING","2024-03-27",{"date":261,"type":36},"2024-03-28",{"date":263,"type":21},"2025-09-01",{"date":171,"type":21},{"name":266,"class":43},"Oticon Medical"]