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

Inclusion Criteria:

• Patients with congenital hearing loss with hearing thresholds ≥65 dB receive eithergene therapy (previously received gene therapy and standardized postoperativerehabilitation and follow-up or plan to receive gene therapy), or cochlear implantsurgery. Healthy participants with bilateral hearing thresholds within the normalrange (≤20 dB), generally matched to the gene therapy group and the cochlear implantgroup by age and sex.

• Age ≥ 6 months 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 timepoints.

• Capable of effective communication with researchers under the guardian's assistanceand willing to cooperate and comply with the researchers' requirements.

• The participant's guardians should have a correct understanding of the trial andappropriate expectations regarding potential benefits.

Exclusion Criteria:

• Presence of other otological disorders that may interfere with the surgical outcomeor interpretation of study endpoints, such as middle/inner ear dysplasia ormalformations that affected the therapeutic effect revealed in CT/MRI scans within 3months, 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 aspulmonary tuberculosis, active hepatitis B or C infection, active herpes zosterinfection, pancreatitis, renal insufficiency, etc.

• Individuals with low immunity, a history of immune deficiency or organtransplantation.

• Individuals with a history of neurological or mental disorders, such as epilepsy ordementia.

• Patients with contraindications for surgery or anesthesia assessed by a surgeon,anesthetist, or designated personnel, such as cardiovascular or cerebrovascularevents 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 affectthe efficacy evaluation.

• Any other conditions for which the investigators consider the subject unsuitable forparticipation in this clinical study.