This cohort study aims to explore the trends and differences in multidimensional perceptual levels of patients after cochlear implants or gene therapy, as well as to comprehensively assess the efficacy of gene therapy for congenital deafness, thus providing a reference for making a well-rounded postoperative rehabilitation protocol for gene therapy patients.
Eligibility
Sex
ALL
Min age
6 Months
Max age
—
Healthy volunteers
Accepted
Inclusion Criteria:
Patients with congenital hearing loss with hearing thresholds ≥65 dB receive either gene therapy (previously received gene therapy and standardized postoperative rehabilitation and follow-up or plan to receive gene therapy), or cochlear implant surgery. Healthy participants with bilateral hearing thresholds within the normal range (≤20 dB), generally matched to the gene therapy group and the cochlear implant group by age and sex.
Age ≥ 6 months, regardless of gender. Mandarin Chinese as the native language. Participants and their guardians must provide informed consent before the trial, voluntarily sign a written consent form, and commit to follow-up at specified time points.
Capable of effective communication with researchers with the assistance of their guardians and willing to cooperate and comply with the researchers' requirements.
The participant's guardians should have a correct understanding of the trial and appropriate expectations regarding potential benefits.
Exclusion Criteria:
Presence of other otological disorders that may interfere with the surgical outcome or interpretation of study endpoints, such as middle/inner ear dysplasia or malformations revealed by CT/MRI scans performed within the past 3 months that may affect the therapeutic effect, vestibular-cochlear nerve abnormalities, acute/chronic otitis media, Meniere's disease, etc.
Presence of other severe congenital diseases. Presence of severe systemic diseases or diseases in the acute phase, such as pulmonary tuberculosis, active hepatitis B or C infection, active herpes zoster infection, pancreatitis, renal insufficiency, etc.
Individuals with low immunity, a history of immune deficiency or organ transplantation.
Individuals with a history of neurological or mental disorders, such as epilepsy or dementia.
Patients with contraindications to surgery or anesthesia, as assessed by a surgeon, anesthetist, or designated personnel, such as cardiovascular or cerebrovascular events in the past 6 months, allergies to the planned medications, etc.
Gene therapy group: gene therapy did not restore hearing; Cochlear implant group: presence of hereditary syndromic deafness or other conditions that seriously affect the efficacy evaluation.
Any other conditions for which the investigators consider the subject unsuitable for participation in this clinical study.
Primary outcome measure(s)
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. Meaningful Auditory Integration Scale (MAIS)/Infant-toddler Meaningful Auditory Integration Scale (IT-MAIS): the MAIS (≥3 years old)/IT-MAIS (0\~3 years old) is an auditory perception test with a total score of 40 points. The higher the score the better the hearing ability.
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. Categories of Auditory Performance (CAP): the CAP is an auditory perception test with a total levels of 8. The higher the level the better the hearing ability.
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. Speech Intelligibility Rating (SIR): the SIR is a speech perception test with a total levels of 5. The higher the level the better the hearing ability.
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. Meaningful Use of Speech Scale (MUSS): the MUSS is a speech perception test with a total score of 50 points. The higher the score the better the hearing ability.
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. Speech, Spatial, and Qualities of Hearing Scale for Parents (SSQ-P): the SSQ-P is an auditory speech and sound location perception test with a total score of 10 points. The higher the score the better the auditory speech and sound location ability.
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. Mandarin Speech Perception (MSP): the MSP is a speech test software included the perception tests of monosyllable, disyllable and sentence recognition in quiet and noise environment. The higher the score the better the auditory speech ability.
Auditory speech perception — Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment. The Angel Test: the Angel Test is a speech test software included the perception tests of environmental sound test, final recognition test, initial recognition test, lexical tone test, music tests, and auditory resolution tests. The higher the score or the lower the thresholds, the better the auditory speech perception.
Trial sites (6)
Facility
City
Region
Status
Peking Union Medical College Hospital
Beijing
Beijing Municipality
Not Yet Recruiting
The First Affiliated Hospital of Zhengzhou University
Zhengzhou
Henan
Not Yet Recruiting
The Second Xiangya Hospital of Central South University
Changsha
Hunan
Not Yet Recruiting
The First Affiliated Hospital of Nanchang University
Nanchang
Jiangxi
Not Yet Recruiting
Eye & ENT Hospital of Fudan University
Shanghai
Shanghai Municipality
Recruiting
West China Hospital, Sichuan University
Chengdu
Sichuan
Not Yet Recruiting
More Eye & ENT Hospital of Fudan University trials in China
This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.
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