[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"congenital-hearing-loss\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:congenital-hearing-loss":33},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,4,0,[8,55,89,114],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":15,"eligibilityCriteria":16,"healthyVolunteers":17,"sex":18,"minAge":19,"maxAge":20,"enrollmentInfo":21,"targetDuration":4,"studyType":24,"phases":25,"briefSummary":27,"conditions":28,"keywords":35,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":43,"lastUpdatePostDateStruct":44,"startDateStruct":47,"completionDateStruct":49,"leadSponsor":51,"locationsCount":54},"100498831","cochlear-implants-in-young-children-with-ssd-100498831",false,"NCT05775367","Cochlear Implants in Young Children With SSD","Cochlear Implantation in Infants and Toddlers With Single-Sided Deafness","Little SSD","To be eligible to participate in this trial as a study subject, an individual must meet all of the following criteria:\n\n1. Provision of signed and dated parental permission form\n2. Unilateral severe-to-profound sensorineural hearing loss, congenital or acquired prior to the age of 2 years, defined as:\n\n   1. Unaided residual hearing thresholds that yield a 3 frequency pure tone average (PTA; average at 500, 1000, and 2000 Hz) of ≥70 dB hearing loss (HL) in the ear to be implanted\n   2. Hearing thresholds in the contralateral ear of ≤20 dB HL (3 frequency PTA of 500, 1000, and 2000 Hz).\n\n   i. Thresholds should be measured using inserts wherever possible, or in the sound field to record the better-hearing ear alone. Required thresholds will include 250, 500, 1000, 2000, and 4000 Hz for air conduction. All other octave frequencies should be attempted. Bone conduction thresholds should be obtained at 500, 1000, 2000, and 4000 Hz. Masking should be attempted where appropriate. As participants are very young and masking is difficult for this population, suspected shadow audiograms will be sufficient if objective testing has confirmed a severe-to-profound unilateral hearing loss. Testing should confirm a severe-to-profound hearing loss, defined as a 3-frequency PTA (500, 1000, and 2000) \\>70 dB HL in one ear and ≤20 dB HL in the contralateral ear. The Primary Investigator and Lead Diagnostic Audiologist must agree on this diagnosis for enrollment.\n3. Insufficient functional access to sound with appropriately fit amplification and aural habilitation (based on best practices and standard of care) defined as:\n\n   1. PEACH + parent questionnaire scores below the expected value for children who lack the requisite language to complete open-set word recognition testing or\n   2. Recorded Multisyllabic Lexical Neighborhood Test (MLNT) scores ≤ 30% in the ear to be implanted for children with the requisite language to complete open-set word recognition testing.\n4. Between 7 months and 2 years, 11 months of age at implantation\n5. Normal cochlear nerve as evaluated by imaging, required imaging by MRI brain\u002FInternal auditory canal (IAC) without contrast with heavily weighted T2 images. CT optional at the physician's discretion.\n6. No malformation of the cochlea\n7. No evidence of progressive hearing loss of the contralateral ear\n8. Willing to\u002Fhas undergone hearing aid trial as warranted based on achieving an aided speech intelligibility index (SII) of \\> .65 when fitted via Desired Sensation Level (DSL) methods.\n9. Realistic parental expectations: a verbal acknowledgment of the potential benefits and risks, and postoperative variation in performance. For instance, cochlear implantation will not restore normal hearing\n10. Willing to obtain recommended meningitis vaccinations per Centers for Disease Control (CDC) recommendations\n11. Development and cognition within the normal range as measured by the Developmental Assessment of Young Children (DAYC-2).\n12. Parental commitment to study parameters including being able and willing to participate in the evaluation schedule, involvement in prescribed therapy, and travel to the investigational site and study-related activities. Parents must be willing to encourage wearing implant during waking hours.\n\nTo participate as an SSD control subject, an individual must meet all the following criteria:\n\n1. Provision of signed and dated parental permission form\n2. Unilateral severe-to-profound hearing loss - congenital or acquired prior to 2 years of age.\n\n   1. Unaided residual hearing thresholds that yield a 3 frequency PTA (500, 1000, and 2000 Hz) of ≥70 dB HL in the affected ear.\n   2. Hearing thresholds in the contralateral ear of ≤20 dB HL (3 frequency PTA of 500, 1000, and 2000 Hz)\n   3. Unable to use or benefit from traditional amplification\n3. Five years of age at the time of testing\n4. Development and cognition within the normal range as measured by the Early Stanford Binet 2 (SB2).\n\nTo participate as a TH control subject, an individual must meet all the following criteria:\n\n1. Provision of signed and dated parental permission form\n2. Bilateral hearing thresholds that yield a PTA of ≤20 dB HL (3 frequency PTA of 500, 1000, and 2000 Hz).\n3. Five years of age at the time of testing\n4. Development and cognition within the normal range as measured by the Early SB2.\n\nAn individual who meets any of the following criteria will be excluded from participation in this study as a study subject:\n\n1. English is not primary language of the home\n\n   1. Speech perception materials are presented in English\n   2. Parental questionnaires are administered in English\n2. Conductive hearing loss in either ear\n3. Cochlear nerve deficiency\n4. Ossification\u002FFibrosis of the cochlea precluding implantation\n5. Inability to participate in follow-up procedures (i.e., unwillingness, geographic location)\n6. History of refractory chronic otitis media or condition that contraindicates anesthesia\n\nAn individual who meets any of the following criteria will be excluded from participation in this study as a control subject:\n\n1. English is not primary language of the home\n\n   1. Speech perception materials are presented in English\n   2. Parental questionnaires are administered in English\n2. Inability to participate in testing (i.e., unwillingness)",true,"ALL","7 Months","71 Months",{"count":22,"type":23},60,"ESTIMATED","INTERVENTIONAL",[26],"NA","The goal of this clinical trial is to investigate the safety and effectiveness of cochlear implantation in infants and toddlers with single-sided deafness.\n\nThe main questions it aims to answer are:\n\n* Are cochlear implants an effective treatment of single-sided deafness in infants and toddlers?\n* Are cochlear implants a safe treatment for single-sided deafness in infants and toddlers?\n\nParticipants will receive a cochlear implant and be followed until they are five years old. During those five years, the investigators will program the device and monitor auditory development.\n\nChildren will be asked to:\n\n* Undergo cochlear implantation\n* Wear their cochlear implant processor whenever they are awake.\n* Participate in traditional hearing tests\n* Participate in traditional hearing testing\n* Participate in localization testing\n* Participate in hearing in noise testing\n* Participate in word recognition testing\n* Participate in speech, language, and educational evaluations\n\nThe researchers will compare results to children with typical hearing in both ears and children with single-sided deafness who have not received an implant to observe any differences between the groups.",[29,30,31,32,33,34],"Single Sided Deafness","Unilateral Deafness","Hearing Loss, Unilateral","Hearing Loss","Congenital Hearing Loss","Deafness One Ear",[36,37,38,39,40,41],"cochlear implant","uhl","ssd","children","hearing loss","deafness","RECRUITING","2026-06-23",{"date":45,"type":46},"2026-06-26","ACTUAL",{"date":48,"type":46},"2023-07-07",{"date":50,"type":23},"2030-05-30",{"name":52,"class":53},"University of North Carolina, Chapel Hill","OTHER",1,{"id":56,"slug":57,"hasResults":11,"nctId":58,"briefTitle":59,"officialTitle":60,"acronym":4,"eligibilityCriteria":61,"healthyVolunteers":17,"sex":18,"minAge":62,"maxAge":63,"enrollmentInfo":64,"targetDuration":4,"studyType":24,"phases":66,"briefSummary":67,"conditions":68,"keywords":73,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":80,"lastUpdatePostDateStruct":81,"startDateStruct":83,"completionDateStruct":85,"leadSponsor":87,"locationsCount":54},"100594828","perceptual-evaluation-and-rehabilitation-system-development-for-congenital-hearing-loss-100594828","NCT07024524","Perceptual Evaluation and Rehabilitation System Development for Congenital Hearing Loss","Development and Application of A Multimodal Perceptual Evaluation and Precision Rehabilitation System in Congenital Deafness","Inclusion Criteria:\n\nFor Congenital Deafness Group: Patients with congenital hearing loss with hearing thresholds ≥65 dB who have received hearing aids, cochlear implantation, or gene therapy; Age between 1 and 35 years old, regardless of gender.\n\nFor Healthy Controls Group: Participants with normal hearing thresholds (≤20 dB) whose age and gender matched to the patient group.\n\nMandarin Chinese as the native language. Participants and\u002For their guardians must provide informed consent before the trial, voluntarily sign a written consent form, and commit to receive evaluation at specified time points.\n\nCapable of effective communication with researchers under the guardian's assistance and willing to cooperate and comply with the researchers' requirements.\n\nParticipants and\u002For their guardians should have a correct understanding of the trial and appropriate expectations regarding potential benefits.\n\nExclusion Criteria:\n\nPresence of other otological disorders that may interfere with the surgical outcome or interpretation of study endpoints, such as otitis media, Meniere's disease, etc.\n\nPresence of other severe congenital diseases, such as congenital heart disease. Presence of severe systemic diseases or in the acute onset of diseases, such as pulmonary tuberculosis, active hepatitis B or C infection, active herpes zoster infection, pancreatitis, renal insufficiency, etc.\n\nIndividuals with low immunity, a history of immune deficiency or organ transplantation.\n\nIndividuals with a history of neurological, mental disorders, or moderate-to-severe cognitive dysfunction, such as epilepsy, dementia, autism spectrum disorders, etc.\n\nAny other conditions for which the investigators consider the subject unsuitable for participation in this clinical study.","1 Year","35 Years",{"count":65,"type":23},210,[26],"This study aims to establish a novel multimodal evaluation and precision rehabilitation system for patients with congenital hearing loss who have received hearing aids, cochlear implantation, or emerging gene therapy. The proposed system will integrate age-stratified difficulty levels, combined with a gamified interaction platform or software designed to assess and train three core perceptual domains: auditory speech perception, music perception, and spatial hearing capabilities.",[33,69,70,71,72],"Gene Therapy","Speech Perception","Rehabilitation","Reliability and Validity",[74,75,76,77,78,79,71],"Congenital hearing loss","Gene therapy","Multimodal assessment","Auditory speech perception","Music perception","Spatial hearing","2025-07-24",{"date":82,"type":46},"2025-07-28",{"date":84,"type":46},"2025-07-04",{"date":86,"type":23},"2028-05-05",{"name":88,"class":53},"Eye & ENT Hospital of Fudan University",{"id":90,"slug":91,"hasResults":11,"nctId":92,"briefTitle":93,"officialTitle":94,"acronym":4,"eligibilityCriteria":95,"healthyVolunteers":11,"sex":18,"minAge":96,"maxAge":4,"enrollmentInfo":97,"targetDuration":4,"studyType":24,"phases":99,"briefSummary":100,"conditions":101,"keywords":4,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":104,"lastUpdatePostDateStruct":105,"startDateStruct":107,"completionDateStruct":109,"leadSponsor":111,"locationsCount":113},"100571585","a-study-of-eh002-gene-therapy-for-otoferlin-gene-mutation-mediated-hearing-loss-100571585","NCT06722170","A Study of EH002 Gene Therapy for Otoferlin Gene Mutation-mediated Hearing Loss","A Study on the Safety, Tolerability, and Preliminary Efficacy of EH002 in the Treatment of DFNB9 Congenital Deafness","Inclusion Criteria:\n\n1. The participant and\u002For their legal guardian must provide informed consent before the study, voluntarily sign the written informed consent form, and be willing to attend follow-up visits as scheduled by the study.\n2. The participant must communicate effectively with the investigator and comply with the investigator's requirements, with the assistance of a legal guardian if necessary. Young children without mature language skills must be able to cooperate and comply with the investigator's requirements with the help of a legal guardian.\n3. The participant and\u002For legal guardian must understand the study correctly and have appropriate expectations regarding the potential benefits.\n4. The participant must be at least 6 months old, with no restrictions on gender.\n5. The participant must be diagnosed with DFNB9 congenital deafness, confirmed by genetic testing showing homozygous or compound heterozygous mutations in the OTOF gene.\n6. Audiological inclusion criteria: Severe or profound hearing loss (≥65 dB).\n7. The participant must meet the surgical requirements, including the absence of middle- or inner-ear malformations, vestibulocochlear nerve developmental abnormalities, and ear inflammation, as confirmed by CT or MRI scans within 3 months or at the time of screening. Additionally, the participant must be deemed eligible for surgery.\n\nExclusion Criteria:\n\n1. The genetic diagnosis does not indicate an OTOF mutation.\n2. Other types of deafness that are unsuitable for otological surgery, such as middle- or inner-ear developmental abnormalities or malformations, vestibulocochlear nerve abnormalities, conductive hearing loss, mixed hearing loss, or malformation syndromes, as detected by CT or MRI scans within 3 months.\n3. Pre-existing otological conditions that would interfere with the planned surgery or the interpretation of study endpoints, such as acute or chronic otitis media, Meniere's disease, acoustic neuroma, or unrecovered sudden sensorineural hearing loss.\n4. A history of substance abuse, or treatment with any known ototoxic drugs (e.g., aminoglycosides, cisplatin, loop diuretics) within the last 6 months, or antiviral medications or immunotherapy within the last 3 months, or vaccination within the last month.\n5. Individuals with compromised immunity or a history of immunodeficiency, including HIV-positive status, other acquired or congenital immunodeficiency disorders, or a history of organ transplantation.\n6. Patients with severe systemic diseases or severe acute illnesses, such as tuberculosis, active hepatitis B or C infection, active herpes zoster infection, pancreatitis, renal insufficiency, or gastrointestinal ulcers.\n7. Patients with surgical or anesthetic contraindications as determined by the surgeon, anesthesiologist, or designated personnel. This includes those who have had a cardiovascular or cerebrovascular event within the last 6 months, such as myocardial infarction, heart failure, angina, stroke, transient ischemic attack, or any other cardiac condition deemed unsuitable by the investigator or an allergy to any planned medications.\n8. Currently participating in or planning to participate in any interventional clinical trial involving drugs or devices within the next year or having received the last dose of another clinical trial within 5 half-lives.\n9. Presence of an implant in the ear (on the side intended for surgery) at the time of screening, such as a cochlear implant.\n10. Neutralizing antibody titer against AAV1 \\>1:2000.\n11. Presence of other severe congenital disorders.\n12. A history of definite neurological or psychiatric disorders, including epilepsy or dementia.\n13. Patients requiring long-term anticoagulant therapy that cannot be temporarily discontinued.\n14. A history of chemotherapy or radiation therapy.\n15. Any other condition that, in the opinion of the investigator, makes the participant unsuitable for this clinical study.","6 Months",{"count":98,"type":23},24,[26],"The study is designed to evaluate the safety, tolerability, and preliminary efficacy of EH002 for the treatment of congenital deafness caused by mutations in the OTOF gene. Participants may receive one or two injections of the EH002 gene therapy in one or both ears.",[102,33,103],"DFNB9","Hearing Loss, Sensorineural","2025-07-22",{"date":106,"type":46},"2025-07-25",{"date":108,"type":46},"2024-11-22",{"date":110,"type":23},"2029-11",{"name":112,"class":53},"Yilai Shu",2,{"id":115,"slug":116,"hasResults":11,"nctId":117,"briefTitle":118,"officialTitle":119,"acronym":4,"eligibilityCriteria":120,"healthyVolunteers":11,"sex":18,"minAge":4,"maxAge":96,"enrollmentInfo":121,"targetDuration":4,"studyType":123,"phases":4,"briefSummary":124,"conditions":125,"keywords":4,"overallStatus":127,"whyStopped":4,"lastUpdateSubmitDate":128,"lastUpdatePostDateStruct":129,"startDateStruct":131,"completionDateStruct":133,"leadSponsor":135,"locationsCount":4},"100544197","genetic-feature-of-congenital-hearing-loss-in-chinese-population-100544197","NCT06365749","Genetic Feature of Congenital Hearing Loss in Chinese Population","Exome Sequencing Explored the Genetic Characteristics Congenital Hearing Loss in Chinese Population","Inclusion Criteria:\n\n* Age up to 6 months\n* Fail in the neonatal hearing screening programs, including oto-acoustic emissions and automated auditory brain stem response\n* Promise to complete the tests required at baseline and follow-ups by the legal representative\n* Informed consent by the legal representative\n\nExclusion Criteria:\n\n* Congenital infections related to acquired or congenital hearing loss, including rubella virus, cytomegalovirus, herpes simplex virus, rubella virus, toxoplasma gondii and treponema pallidum infections\n* Other explicit otologic conditions which could induce hearing loss, including cerumen, otitis media, congenital middle ear abnormalities, microtia and external ear abnormalities\n* A drug with ototoxicity usage during pregnancy\n* Other severe congenital anomalies",{"count":122,"type":23},50,"OBSERVATIONAL","Congenital hearing loss, as well as hearing loss present at birth, is one of the most common chronic conditions in children, with a prevalence of permanent bilateral hearing loss of 2.83 per 1000 children of primary school age, which is mainly caused by genetic factors. The goal of this observational study is to learn about novel causative genes in infants with hearing loss in the Chinese population. The main problem it aims to deal with are:\n\n* to present the genetic characteristics of the infant with hearing loss in the Chinese population\n* to build up a prognostic model base on diverse data.\n\nParticipants will be asked to receive audiological tests and collection of the peripheral blood sample.",[33,126],"Congenital Deafness","NOT_YET_RECRUITING","2024-04-09",{"date":130,"type":46},"2024-04-15",{"date":132,"type":23},"2024-04-23",{"date":134,"type":23},"2026-11-23",{"name":136,"class":53},"Dan Bing"]