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先天性重度-极重度感音神经性聋且助听器效果不佳是人工耳蜗植入（cochlear implantation, CI）的适应证，但CI效果与诸多因素有关，其中听觉剥夺时间和植入年龄是关键因素之一。相对来说，植入年龄越小，CI效果越好，这可能与听觉中枢发育关键期及可塑性水平有关。出生后听觉中枢的发育依赖于听觉刺激，先天性聋患者由于关键期内缺乏听觉刺激，听觉中枢的结构和功能发育延迟，听皮层发生跨模态重组，耳蜗慢性电刺激可使听皮层神经元突触重塑，皮层结构重组，皮质反应逐渐成熟，听觉功能改善，这可能与即刻早期基因c-fos和基质金属蛋白酶-9(matrix metallopeptidase-9, MMP-9)、脑源性神经生长因子(brain-derived neurotrophic factor, BDNF)等神经元可塑性相关因子表达有关，并受兴奋性-抑制性神经递质调控。先天性遗传性聋小型猪在基因表型、听觉发育规律、颞骨解剖结构、内耳形态和听觉中枢结构等多方面与人类高度相似性，比传统先天性聋动物模型更具优势，可利用先天性遗传性聋小型猪模型研究先天性听觉剥夺和耳蜗慢性电刺激后听觉中枢发育及可塑性的影响，深入探究听觉中枢可塑性机制。
Abstract：Non-aidable severe to profound congenital hearing loss is an indication for cochlear implantation(CI).The outcome of CI is associated with various factors, among which the time of auditory deprivation and implantation age are crucial. Generally, the younger the implantation age, the better the outcome, likely related to the critical period of auditory center development and plasticity. Development of the auditory center after birth relies on auditory stimulation.Congenitally deaf patients experience delayed structural and functional development of the auditory center due to lack of auditory stimulation during the critical period, with cross-modal reorganization in the auditory cortex. Chronic electrical stimulation of the cochlea leads to synaptic remodeling of auditory cortex neurons, cortical structure reorganization, and gradual maturation of cortical responses, improving auditory functions. This might be associated with expression of neuronal plasticity-related factors such as c-fos, matrix metallopeptidase-9(MMP-9), and brain-derived neurotrophic factor(BDNF), and is regulated by excitatory-inhibitory neurotransmitters. Miniature pigs congenital hereditary deafness bear high similarities to humans including genetic phenotypes, auditory development patterns, temporal bone anatomy, inner ear morphology, and central auditory system structures, offering more advantages than traditional animal models of congenital deafness. They can be employed to study the impacts of auditory deprivation and chronic cochlear electrical stimulation on central auditory development and plasticity and explore the mechanism of plasticity.
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基本信息:
中图分类号:R764.43
引用信息:
[1]唐菲,吕萍,杨崇灵,等.先天性聋听觉中枢发育及可塑性的研究进展[J].中华耳科学杂志,2024,22(03):498-503.
2024-06-15
2024-06-15