An observational study in Hearing Loss, Genetic Disease and Speech Perception, sponsored by Eye & ENT Hospital of Fudan University. Recruiting at 6 sites in China. Open to participants aged 6 Months and older, including healthy volunteers. Per ClinicalTrials.gov, last updated 2026-08-31.
Sponsored by Eye & ENT Hospital of Fudan University · Observational
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.
Cochlear implant patients exhibit improved speech perception in quiet environments after surgery, but their music perception and speech perception under noise are still unsatisfactory. Gene therapy is a novel and promising treatment for congenital hearing loss to recover natural hearing sensations. To date, for patients with congenital deafness, there has been no study on their ability to perceive and understand sounds, such as speech perception in noise, music and directional perception, after hearing recovery owing to gene therapy. In addition, the difference between the two treatments in those perceptual levels postoperatively remains unknown.
Therefore, the investigators designed a single-center cohort study. Based on the intervention method, congenital deafness patients are divided into two groups: the cochlear implant group and the gene therapy group. In the present study, a full-scale evaluation of the two groups will be conducted. The battery encompasses auditory speech perception, cognition, psychological status, and auditory cortex development. A comparative analysis will be conducted to examine multidimensional differences between the two groups, shedding light on the divergent outcomes of gene therapy and cochlear implants for patients with congenital deafness.
796 studies on the registry are indexed under Hearing Loss; 193 are open to participants now.
This study's planned enrollment of 180 is above the median of 100 across 192 observational studies indexed under Hearing Loss.
Browse Hearing Loss studies →Eye & ENT Hospital of Fudan University is the lead sponsor of 126 studies on the registry; 70 are open to participants now.
Counted across the registry records on this site, refreshed daily.
The number of enrollments in the gene therapy group is expected to be 90, and that of the cochlear implant group is expected to be at least 90, according to the pairing ratio of at least 1:1 between the cochlear implant group and gene therapy group. An estimated 30 healthy subjects are expected to be recruited (optional, subject to recruitment feasibility) for electroencephalography and/or functional near-infrared spectroscopy assessments.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
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 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 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 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.
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Hepatitis B is a virus that infects the liver. These are separate tests: HBsAg positive usually means a current infection (a recent vaccination can briefly cause it too); total anti-HBc (HBcAb) positive means an infection now or in the past; HBV DNA measures how much virus is in the blood.
Some studies accept a past infection if HBV DNA is undetectable, sometimes with antiviral medicine during the study. Check what this study allows.
A reason a treatment, test or procedure should not be given to a particular person, such as an allergy, another illness or a medicine they take.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Hepatitis B tests
Hepatitis B is a virus that infects the liver. These are separate tests: HBsAg positive usually means a current infection (a recent vaccination can briefly cause it too); total anti-HBc (HBcAb) positive means an infection now or in the past; HBV DNA measures how much virus is in the blood.
Some studies accept a past infection if HBV DNA is undetectable, sometimes with antiviral medicine during the study. Check what this study allows.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Contraindication
A reason a treatment, test or procedure should not be given to a particular person, such as an allergy, another illness or a medicine they take.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
The study will recruit gene therapy patients who are definitely diagnosed with autosomal recessive deafness 9 (DFNB9).
The study will recruit cochlear implant patients with congenital deafness.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Auditory speech perception
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.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Growth and development assessment
Griffiths development scales(GDS)/Wechsler Intelligence Scale for Children, 4e (WISC-IV): the GDS (0\~6 years old)/ WISC-IV (8-16 years old) is a cognitive function test. For children aged 6-8 years, the assessment instrument was selected according to their developmental status and ability to cooperate with the testing procedures.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Psychological status
Strengths and Difficulties Questionnaire(SDQ): the SDQ is a psychological status test with a total score of 25 points. The lower the score the better the psychological status.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Cortical auditory processing
Electroencephalogram (EEG): the EEG is a non-invasive brain imaging method that uses brain maps to assess changes in auditory and speech-related cortex.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Cortical auditory processing
Functional near-infrared spectroscopy (fNIRS): the fNIRS is a non-invasive brain imaging method that uses brain maps to assess changes in auditory and speech-related cortex.
Time frame: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Started Apr 2024 (actual). Primary completion is estimated for Oct 2029, 66 months after the start.
Of 77 non-industry observational studies under Hearing Loss, half reached primary completion within 50 months of starting (the median), counting those still running; a quarter within 14 and three quarters within 179. This study's planned 66 months is above that.
Counting every sponsor, half of 98 studies reached it within 42 months; a quarter within 15 and three quarters within 83.
How these figures are worked out, and which studies are left out of them.
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