A Comparative Analysis of Speech Perception Between Cochlear Implant Patients and DFNB9 Patients Receiving Gene Therapy

Recruiting

Brief 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 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.

Recruitment & Eligibility

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.
Age ≤18 years old, 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 under the guardian's assistance 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 that affected the therapeutic effect revealed in CT/MRI scans within 3 months, vestibular-cochlear nerve abnormalities, acute/chronic otitis media, Meniere's disease, etc.
Presence of other severe congenital diseases.
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.
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 for surgery or anesthesia 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 Measures

Name	Time	Method
Auditory speech perception	Preoperation, week 13, week 26 and week 52	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 and lexical tone test in quiet environment. The higher the score the better the auditory speech ability.

Secondary Outcome Measures

Name	Time	Method
Auditory cortex development	Preoperation, week 13, week 26 and week 52	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.
Psychological status	Preoperation, week 26 and week 52	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.
Cognitive function	Preoperation, week 13, week 26 and week 52	Griffiths development scales(GDS)/Wechsler Intelligence Scale for Children, 4e (WISC-IV): the GDS (0\~6 years old)/WISC-IV (7\~16 years old) is a cognitive function test. The higher the score the better the cognitive function.

Locations (1): Eye & ENT Hospital of Fudan University
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Shanghai, Shanghai, China