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 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 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.