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

Launched by EYE & ENT HOSPITAL OF FUDAN UNIVERSITY · Jan 25, 2024

Keywords

ClinConnect Summary

This clinical trial is studying how well patients with hearing loss understand speech after receiving either cochlear implants or gene therapy. The goal is to see how these two treatments compare and to better understand the effectiveness of gene therapy for congenital deafness, which is hearing loss present at birth. By doing this, researchers hope to create better rehabilitation plans for patients who receive gene therapy.

To be eligible for this trial, participants must be children or young adults (up to 18 years old) with congenital hearing loss who can communicate in Mandarin Chinese. They should either have already received gene therapy and have a follow-up plan or are planning to receive it, or they will be getting cochlear implants. Participants and their guardians will need to give informed consent, meaning they understand what the trial involves. Throughout the study, participants will have regular check-ins to assess their progress. It’s important to note that certain medical conditions or other ear disorders could prevent someone from participating, as these issues might affect the results of the study.

Gender

ALL

Eligibility criteria

• 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 ≥ 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.