Hearing LossNCT06237790
Auditory Restoration and Outcomes After Gene Therapy and Cochlear Implantation: a PRospective Cohort Analysis (AURORA)
An observational study of Hearing Loss and Genetic Disease, run by Eye & ENT Hospital of Fudan University at 6 sites in China.
Trial summary
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… Read more about this study
- Who is it for?
- Ages 6 months and olderHealthy volunteers can take part
- Conditions the study is for
- Hearing Loss, Genetic Disease, Speech Perception
- How many people
- 180 participants
- Where
- 6 sitesChina
- Study dates
- 6 Apr 2024to 22 Dec 2029
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 months and older
- All sexes
- Healthy volunteers accepted
- Child, Adult, Older adult
Study population: 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.
Who may be able to join
- 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.
Who cannot join
- 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.
Trial locations and contacts
6 sites in 1 country. Contact the study team or a site near you to ask about taking part.
6 sites in 1 country
China6 sites, 6 recruiting
- Not yet recruiting
Peking Union Medical College Hospital
Beijing, Beijing Municipality, 100730
Guodong Feng, +86 21 5515 9599, fengguodong2013@163.com
- Not yet recruiting
The Second Xiangya Hospital of Central South University
Changsha, Hunan, 410011
Qin Wang, +86 731 8529 5888, 455616210@qq.com
- Not yet recruiting
West China Hospital, Sichuan University
Chengdu, Sichuan, 610041
Yu Zhao, 028-85422114, yuzhao@wchscu.cn
- Not yet recruiting
The First Affiliated Hospital of Nanchang University
Nanchang, Jiangxi, 330006
Yuanping Xiong, 0791-88692748, yuanping_xiong@163.com
- Recruiting
Eye & ENT Hospital of Fudan University
Shanghai, Shanghai Municipality, 200031
Yilai Shu, M.D. & Ph.D., +86 21 6437 7134, yilai_shu@fudan.edu.cn
- Not yet recruiting
The First Affiliated Hospital of Zhengzhou University
Zhengzhou, Henan, 450052
Rongqun Zhai, +86 371 6691 3345, rongqunzhai2009@126.com
About this study
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.
Read the detailed description
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.
- Conditions
- Hearing Loss, Genetic Disease, Speech Perception
- Keywords
- Congenital Hearing Loss, DFNB9, Gene Therapy, Cochlear Implant, Speech Perception
- Medical subject headings
- Hearing Loss, Genetic Diseases, Inborn
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Gene Therapy Group | The study will recruit gene therapy patients who are definitely diagnosed with autosomal recessive deafness 9 (DFNB9). | |
| Cochlear Implant Group | The study will recruit cochlear implant patients with congenital deafness. |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Secondary outcomes (4)
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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: Preoperatively and at 13, 26, and 52 weeks postoperatively, with subsequent assessments every 6 or 12 months up to 5 years after treatment.
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted1 Feb 2024
- Study start6 Apr 2024
- Primary completion10 Oct 2029 (estimated)
- Study completion22 Dec 2029 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Hearing LossStudies of the same condition, open and under way.
- More trials run by Eye & ENT Hospital of Fudan UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 180 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Eye & ENT Hospital of Fudan University
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Yilai Shu, M.D. & Ph.D., Study chair, Eye & ENT Hospital of Fudan University
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06237790
- Sponsor's protocol ID
- 2023185-1
- Status last verified
- Aug 2026
- First submitted
- 26 Dec 2023
- Last updated
- 31 Aug 2026
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 31 Aug 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Auditory Restoration and Outcomes After Gene Therapy and Cochlear Implantation: a PRospective Cohort Analysis (AURORA)
- ClinicalTrials.gov ID
- NCT06237790
- Acronym / sponsor ID
- 2023185-1
- Conditions
- Hearing Loss, Genetic Disease, Speech Perception
- Keywords
- Congenital Hearing Loss, DFNB9, Gene Therapy, Cochlear Implant, Speech Perception
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 180 participants (estimated)
- Lead sponsor
- Eye & ENT Hospital of Fudan UniversityUniversity, hospital or other
- Study start
- 6 Apr 2024 (actual)
- Primary completion
- 10 Oct 2029 (estimated)
- Study completion
- 22 Dec 2029 (estimated)
- First posted
- 1 Feb 2024 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| Gene Therapy Group | – | –The study will recruit gene therapy patients who are definitely diagnosed with autosomal recessive deafness 9 (DFNB9). |
| Cochlear Implant Group | – | –The study will recruit cochlear implant patients with congenital deafness. |
Who may qualify
- Ages
- 6 months and older
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult, Older adult
- Study population
- 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.
Key inclusion criteria (4 listed)
- 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.
Key exclusion criteria (7 listed)
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
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.
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
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.
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
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.
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
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.
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
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.
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
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.
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
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.
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.
4 secondary outcomes
Growth and development assessment
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
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
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
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.
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 31 Aug 2026.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Yilai Shu, M.D. & Ph.D. study chair
Eye & ENT Hospital of Fudan University
6 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Hearing Loss and Genetic Disease, run by Eye & ENT Hospital of Fudan University at 6 sites in China.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 4 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 31 Aug 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 6 sites in China. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Yilai Shu, M.D. & Ph.D. (+86 21 6437 7134, yilai_shu@fudan.edu.cn), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
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.). The study lists 7 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 31 Aug 2026.