[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-study-detail:100470205":3},{"organization":4,"armGroups":7,"interventions":23,"overallOfficials":33,"centralContacts":38,"locations":44,"responsibleParty":56,"collaborators":10,"id":58,"slug":59,"hasResults":60,"nctId":61,"briefTitle":62,"officialTitle":63,"acronym":64,"eligibilityCriteria":65,"healthyVolunteers":60,"sex":66,"minAge":10,"maxAge":67,"enrollmentInfo":68,"targetDuration":10,"studyType":71,"phases":10,"briefSummary":72,"conditions":73,"keywords":10,"overallStatus":46,"whyStopped":10,"lastUpdateSubmitDate":81,"lastUpdatePostDateStruct":82,"startDateStruct":85,"completionDateStruct":87,"leadSponsor":89,"locationsCount":90},{"fullName":5,"class":6},"Sensorion","INDUSTRY",[8,15,19],{"label":9,"type":10,"description":11,"interventionNames":12},"Cohort 1a",null,"Patients without Cochlear Implant, with or without Hearing Aid(s) at study entry",[13,14],"Other: Pure Tone Audiometry Assessment","Other: Quality of Life Questionnaires",{"label":16,"type":10,"description":17,"interventionNames":18},"Cohort 1b","Patients receiving unilateral or bilateral Cochlear Implant(s) during the study period, after study entry",[13,14],{"label":20,"type":10,"description":21,"interventionNames":22},"Cohort 2","Patients with Cochlear Implant(s) (unilateral or bilateral) at study entry",[13,14],[24,29],{"type":25,"name":26,"description":27,"armGroupLabels":28,"otherNames":10},"OTHER","Pure Tone Audiometry Assessment","Collection of Pure Tone Audiometry data performed in routine practice during study period",[9,16,20],{"type":25,"name":30,"description":31,"armGroupLabels":32,"otherNames":10},"Quality of Life Questionnaires","Collection of Quality of Life questionnaire's answers during study period",[9,16,20],[34],{"name":35,"affiliation":36,"role":37},"Natalie LOUNDON, MD","Necker Hospital","PRINCIPAL_INVESTIGATOR",[39],{"name":40,"role":41,"phone":42,"phoneExt":10,"email":43},"Lionel HOVSEPIAN, MD","CONTACT","+33 (0)7 86 31 13 76","lionel.hovsepian@sensorion-pharma.com",[45],{"facility":36,"status":46,"city":47,"state":10,"zip":10,"country":48,"countryCode":49,"cosmosGeoPoint":50,"geoPoint":55,"contacts":10},"RECRUITING","Paris","France","FR",{"type":51,"coordinates":52},"Point",[53,54],2.3488,48.85341,{"lat":54,"lon":53},{"type":57,"investigatorFullName":10,"investigatorTitle":10,"investigatorAffiliation":10,"oldNameTitle":10,"oldOrganization":10},"SPONSOR","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":69,"type":70},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.",[74,75,76,77,78,79,80],"Sensorineural Hearing Loss, Bilateral","AUNB1","DFNB1A","Congenital Deafness","DFNB9","OTOF Gene Mutation","GJB2 Gene Mutation","2026-05-28",{"date":83,"type":84},"2026-06-01","ACTUAL",{"date":86,"type":84},"2022-11-18",{"date":88,"type":70},"2031-06",{"name":5,"class":6},1]