[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-study-detail:100642992":3},{"organization":4,"armGroups":7,"interventions":18,"overallOfficials":24,"centralContacts":24,"locations":25,"responsibleParty":64,"collaborators":24,"id":66,"slug":67,"hasResults":68,"nctId":69,"briefTitle":70,"officialTitle":71,"acronym":72,"eligibilityCriteria":73,"healthyVolunteers":68,"sex":74,"minAge":75,"maxAge":76,"enrollmentInfo":77,"targetDuration":24,"studyType":80,"phases":81,"briefSummary":84,"conditions":85,"keywords":89,"overallStatus":28,"whyStopped":24,"lastUpdateSubmitDate":96,"lastUpdatePostDateStruct":97,"startDateStruct":100,"completionDateStruct":102,"leadSponsor":104,"locationsCount":105},{"fullName":5,"class":6},"Skylark Bio Inc.","INDUSTRY",[8,14],{"label":9,"type":10,"description":11,"interventionNames":12},"Older","EXPERIMENTAL","Four subjects aged 2 to 7 years old (inclusive) to receive an intracochlear infusion of SKY-GJB2 in the study ear using a sterile, one-time use investigational medical device.",[13],"Combination Product: SKY-GJB2",{"label":15,"type":10,"description":16,"interventionNames":17},"Younger","Six subjects aged 9 months to less than 2 years old to receive an intracochlear infusion of SKY-GJB2 in the study ear using a sterile, one-time use investigational medical device.",[13],[19],{"type":20,"name":21,"description":22,"armGroupLabels":23,"otherNames":24},"COMBINATION_PRODUCT","SKY-GJB2","SKY-GJB2 is intended to be administered via a one-time unilateral intracochlear infusion using the SKY-CAT delivery device.",[9,15],null,[26,46],{"facility":27,"status":28,"city":29,"state":30,"zip":31,"country":32,"countryCode":33,"cosmosGeoPoint":34,"geoPoint":39,"contacts":40},"Massachusetts Eye and Ear Infirmary (MEEI)","RECRUITING","Boston","Massachusetts","02114","United States","US",{"type":35,"coordinates":36},"Point",[37,38],-71.05977,42.35843,{"lat":38,"lon":37},[41],{"name":42,"role":43,"phone":44,"phoneExt":24,"email":45},"Michael Cheung, MSc, CCRP, Clinical Research Project Manager","CONTACT","617-573-6060","centerforclinicalresearchoperations@meei.harvard.edu",{"facility":47,"status":28,"city":48,"state":49,"zip":50,"country":32,"countryCode":33,"cosmosGeoPoint":51,"geoPoint":55,"contacts":56},"Lehigh Valley Hospital-Cedar Crest","Allentown","Pennsylvania","181103",{"type":35,"coordinates":52},[53,54],-75.49018,40.60843,{"lat":54,"lon":53},[57,61],{"name":58,"role":43,"phone":59,"phoneExt":24,"email":60},"Ravi N. Samy, MD, FACS","610-402-9543","Ravi.Samy@Jefferson.edu",{"name":62,"role":43,"phone":24,"phoneExt":24,"email":63},"Danielle Koehler, RN (Study Coordinator)","Danielle.Koehler@Jefferson.edu",{"type":65,"investigatorFullName":24,"investigatorTitle":24,"investigatorAffiliation":24,"oldNameTitle":24,"oldOrganization":24},"SPONSOR","100642992","phase-1-open-label-study-of-sky-gjb2-in-pediatric-subjects-with-gjb2-mediated-hearing-loss-100642992",false,"NCT07627971","Open-label Study of SKY-GJB2 in Pediatric Subjects With GJB2-mediated Hearing Loss","A Phase 1\u002F2, Open-label, Single Dose Study of SKY-GJB2 Given as a Single Unilateral Intracochlear Administration in Pediatric Subjects With GJB2-mediated Hearing Loss","SONIX","Inclusion Criteria:\n\n* Male or female aged 9 months to 7 years at the time of gene therapy administration\n* Subjects must have at least two (biallelic) pathogenic or likely pathogenic variants in the GJB2 gene\n* Bilateral sensorineural hearing loss as assessed by ABR. Hearing loss at ≥85 dB HL for at least one of the frequencies (500-4000 Hz) in the study treatment ear.\n* Subject's parent(s)\u002Fguardian(s) provide informed consent before the initiation of study-related procedures.\n* Subject is able and willing to comply (or provide assent if old enough) will all study requirements.\n\nExclusion Criteria:\n\n* Subject has non-GJB2 mediated hearing loss including genetic, syndromic, or non-syndromic hearing loss that is not associated with GJB2 mutations.\n* Subject has autosomal dominant nonsyndromic hearing loss due to GJB2 mutation.\n* No response on ABR testing.\n* Bilateral Cochlear Implants.","ALL","9 Months","7 Years",{"count":78,"type":79},10,"ESTIMATED","INTERVENTIONAL",[82,83],"PHASE1","PHASE2","Skylark Bio is conducting a study of an investigational new drug called SKY-GJB2 and its delivery device, the SKY-CAT. SKY-GJB2 is a gene therapy that is being developed to treat children who have hearing loss due to changes in the GJB2 gene (also referred to as Connexin 26). The SKY-CAT is a device that delivers the gene therapy.\n\nThe purpose of this study is to:\n\nLearn about the safety and tolerability (does not cause ongoing discomfort) of SKY-GJB2 Evaluate the efficacy of SKY-GJB2 (how well is works)",[86,87,88],"GJB2-mediated Hearing Loss","GJB2 Gene Mutation","DFNB1A",[90,91,92,93,94,95],"GJB2","Hearing Loss, Bilateral","Hearing Loss","Hearing Loss, Sensorineural","Deafness, Autosomal Recessive 1A (DFNB1A)","Connexin 26","2026-06-25",{"date":98,"type":99},"2026-06-30","ACTUAL",{"date":101,"type":99},"2026-05-28",{"date":103,"type":79},"2028-06",{"name":5,"class":6},2]