首次在国内报告小鼠前庭迷路的体外发育。采用周祥宁和Van De Water建立的HEMA水凝胶器官培养法培养第12～13天CBA/C57小鼠胚胎的内耳原基7～8天。内耳原基的上部发育成3个半规管、椭圆囊和球囊。半规管的壶腹嵴由一排感觉毛细胞和2～3...首次在国内报告小鼠前庭迷路的体外发育。采用周祥宁和Van De Water建立的HEMA水凝胶器官培养法培养第12～13天CBA/C57小鼠胚胎的内耳原基7～8天。内耳原基的上部发育成3个半规管、椭圆囊和球囊。半规管的壶腹嵴由一排感觉毛细胞和2～3排支持细胞构成。嵴的上方有正在发育的嵴顶。椭圆囊斑和球囊斑的表面都有一层耳石膜。这些囊斑内可见一层感觉毛细胞和1～2层支持细胞。超微结构研究发现壶腹嵴、椭圆囊斑和球囊斑的毛细胞有排列规则的静纤毛和表皮板,毛细胞的上部有丰富的线粒体,还可见内质网和高尔基体等细胞器。内耳体外培养可用以研究前庭迷路的发育机理、耳毒性药物、放射线等致病因素对前庭迷路的损害以及前庭迷路毛细胞的再生,对研究前庭迷路疾病引起的眩晕病的预防和治疗有积极作用。展开更多
Objective: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. Methods: History, examination, and audiometric testing in the proband, brother, and son; quantitative...Objective: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. Methods: History, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. Results: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. Conclusions: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging.展开更多
文摘首次在国内报告小鼠前庭迷路的体外发育。采用周祥宁和Van De Water建立的HEMA水凝胶器官培养法培养第12～13天CBA/C57小鼠胚胎的内耳原基7～8天。内耳原基的上部发育成3个半规管、椭圆囊和球囊。半规管的壶腹嵴由一排感觉毛细胞和2～3排支持细胞构成。嵴的上方有正在发育的嵴顶。椭圆囊斑和球囊斑的表面都有一层耳石膜。这些囊斑内可见一层感觉毛细胞和1～2层支持细胞。超微结构研究发现壶腹嵴、椭圆囊斑和球囊斑的毛细胞有排列规则的静纤毛和表皮板,毛细胞的上部有丰富的线粒体,还可见内质网和高尔基体等细胞器。内耳体外培养可用以研究前庭迷路的发育机理、耳毒性药物、放射线等致病因素对前庭迷路的损害以及前庭迷路毛细胞的再生,对研究前庭迷路疾病引起的眩晕病的预防和治疗有积极作用。
文摘Objective: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. Methods: History, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. Results: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. Conclusions: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging.
