A New Model for Congenital Vestibular Disorders.
A New Model for Congenital Vestibular Disorders.
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先天性前庭疾病的新模型。
DOI：
10.1007/s10162-018-00705-z
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发表时间：
2019
期刊：
影响因子：
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通讯作者：
Peusner,KennaD
中科院分区：
文献类型：
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作者：
Lilian,SigmundJ;Seal,HayleyE;Popratiloff,Anastas;Hirsch,JuneC;Peusner,KennaD
许多内耳发育障碍在临床上表现为运动发育迟缓以及维持姿势和平衡方面的挑战，表明中央前庭回路受累。由于缺乏合适的动物模型，人们对儿科病例中前庭回路如何重新连接知之甚少。基于此，我们实验室设计并验证了鸡胚模型来研究先天性前庭疾病的前庭发育。通过手术将 2 日龄鸡胚 (E2) 右侧正在发育的内耳或“耳囊”沿前后轴旋转 180°，形成“前后轴旋转耳囊雏鸡”或 ARO 雏鸡。 ARO 雏鸡具有可产生的半规管截断或缺失的囊病理学。囊是先天性前庭疾病儿童中最常见的内耳缺陷。在 E13 ARO 雏鸡中，囊包含所有三个嵴和椭圆斑和球囊，但上嵴和椭圆斑的前后范围缩短。此外，切向前庭核（主要的鸟类前庭核）的主细胞数量在旋转侧减少了 66%。孵化后，ARO 雏鸡与正常雏鸡的翻正反射时间没有差异。然而，与正常雏鸟不同的是，ARO 雏鸟的头部持续向右倾斜，在执行翻正反射后，ARO 雏鸟会跌跌撞撞，走路时脚底变宽。鉴定 ARO 雏鸡异常发育的大脑区域的结构和功能可能有助于改善先天性前庭障碍患者的治疗。
Many developmental disorders of the inner ear are manifested clinically as delayed motor development and challenges in maintaining posture and balance, indicating involvement of central vestibular circuits. How the vestibular circuitry is rewired in pediatric cases is poorly understood due to lack of a suitable animal model. Based on this, our lab designed and validated a chick embryo model to study vestibular development in congenital vestibular disorders. The developing inner ear or “otocyst” on the right side of 2-day-old chick embryos (E2) was surgically rotated 180° in the anterior–posterior axis, forming the “anterior–posterior axis rotated otocyst chick” or ARO chick. The ARO chick has areproduciblepathology of a sac with truncated or missing semicircular canals. A sac is the most common inner ear defect found in children with congenital vestibular disorders. In E13 ARO chicks, the sac contained all three cristae and maculae utriculi and sacculi, but the superior crista and macula utriculi were shortened in anterior–posterior extent. Also, the number of principal cells of the tangential vestibular nucleus, a major avian vestibular nucleus, was decreased 66 % on the rotated side. After hatching, no difference was detected between ARO and normal chicks in their righting reflex times. However, unlike normal chicks, ARO hatchlings had a constant, right head tilt, and after performing the righting reflex, ARO chicks stumbled and walked with a widened base. Identifying the structure and function of abnormally developed brain regions in ARO chicks may assist in improving treatments for patients with congenital vestibular disorder.