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.
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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.
Cohort
Prospective
Not listed
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.
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.
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.
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.
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.
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.
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.
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.
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