[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"leadSponsorName\":\"Akouos, Inc.\",\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:":96},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,3,0,[8,46,74],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":18,"targetDuration":4,"studyType":21,"phases":22,"briefSummary":25,"conditions":26,"keywords":28,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":34,"lastUpdatePostDateStruct":35,"startDateStruct":38,"completionDateStruct":40,"leadSponsor":42,"locationsCount":45},"100555884","phase-1-anti-vegf-gene-therapy-trial-for-vestibular-schwannoma-100555884",false,"NCT06517888","Anti-VEGF Gene Therapy Trial for Vestibular Schwannoma","A Phase 1\u002F2 Trial of AAVAnc80-antiVEGF Gene Therapy in Individuals With Unilateral Vestibular Schwannoma","Criteria for Inclusion:\n\n1. Presence of unilateral, progressive vestibular schwannoma.\n2. Vestibular schwannoma larger than 2 mm.\n3. Profound hearing loss, defined by pure-tone audiometry thresholds or word recognition score, in the affected ear.\n4. Able and willing to comply with all trial requirements, including willingness to participate in a separate long term follow-up study after completion of this trial.\n\nCriteria for Exclusion:\n\n1. Prior diagnosis of NF2 and\u002For bilateral vestibular schwannoma.\n2. Prior surgery or radiation therapy for vestibular schwannoma.\n3. Clinical history consistent with endolymphatic hydrops (documented fluctuating sensorineural hearing loss and\u002For episodic vertigo) in the affected ear.\n4. Profound hearing loss, defined by pure-tone audiometry thresholds or word recognition score, in the unaffected ear.\n5. Prior participation in a clinical trial with an investigational drug within six months prior to administration (Day 0), or any prior participation in a gene therapy clinical trial.","ALL","18 Years",{"count":19,"type":20},27,"ESTIMATED","INTERVENTIONAL",[23,24],"PHASE1","PHASE2","This trial will evaluate the safety and tolerability of a single unilateral administration of one of three dose levels of AAVAnc80-antiVEGF and will evaluate the Akouos delivery device to safely achieve the intended product performance.",[27],"Vestibular Schwannoma",[29,30,31,32],"Vestibular schwannoma","Hearing loss","Acoustic neuroma","anti-VEGF","RECRUITING","2026-04-01",{"date":36,"type":37},"2026-04-06","ACTUAL",{"date":39,"type":37},"2025-01-17",{"date":41,"type":20},"2029-08",{"name":43,"class":44},"Akouos, Inc.","INDUSTRY",4,{"id":47,"slug":48,"hasResults":11,"nctId":49,"briefTitle":50,"officialTitle":51,"acronym":4,"eligibilityCriteria":52,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":4,"enrollmentInfo":53,"targetDuration":4,"studyType":21,"phases":55,"briefSummary":56,"conditions":57,"keywords":59,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":65,"lastUpdatePostDateStruct":66,"startDateStruct":68,"completionDateStruct":70,"leadSponsor":72,"locationsCount":73},"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":54,"type":20},22,[23,24],"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.",[58],"Sensorineural Hearing Loss, Bilateral",[60,61,62,30,63,64],"Otoferlin","Auditory neuropathy (AN)","Deafness, autosomal recessive 9 (DFNB9)","Hearing loss, bilateral","Hearing loss, sensorineural","2026-03-03",{"date":67,"type":37},"2026-03-04",{"date":69,"type":37},"2023-09-15",{"date":71,"type":20},"2028-10",{"name":43,"class":44},9,{"id":75,"slug":76,"hasResults":11,"nctId":77,"briefTitle":78,"officialTitle":79,"acronym":4,"eligibilityCriteria":80,"healthyVolunteers":11,"sex":16,"minAge":4,"maxAge":81,"enrollmentInfo":82,"targetDuration":4,"studyType":84,"phases":4,"briefSummary":85,"conditions":86,"keywords":87,"overallStatus":33,"whyStopped":4,"lastUpdateSubmitDate":65,"lastUpdatePostDateStruct":89,"startDateStruct":90,"completionDateStruct":92,"leadSponsor":94,"locationsCount":95},"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":83,"type":20},150,"OBSERVATIONAL","This is a retrospective and prospective longitudinal study in participants with Otoferlin Gene-Mediated Hearing Loss.",[58],[88],"Otoferlin Gene, sensorineural hearing loss (SNHL), auditory neuropathy, auditory neuropathy spectrum disorder",{"date":67,"type":37},{"date":91,"type":37},"2022-07-14",{"date":93,"type":20},"2029-02",{"name":43,"class":44},10,""]