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Ossicular fusion and cholesteatoma in auriculo-condylar syndrome: In vivo evidence of arrest of embryogenesis

机译:耳con突综合征中的听骨融合和胆脂瘤:胚胎发生停止的体内证据

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摘要

Auriculo-condylar syndrome (ACS) is a rare condition affecting first branchial arch structures. The types of hearing loss and temporal bone findings in ACS have not been reported. We describe a 14-year-old male with constricted pinnae, mandibular dysostosis, glossoptosis, a high-arched palate, hearing loss, and cholesteatoma. Computed tomography imaging demonstrated malleoincudal joint ankylosis. The fused malleoincudal complex was removed during mastoidectomy for cholesteatoma. Electron microscopy and histopathology of the joint suggested the fusion was congenital. This is the first report of ossicular fusion and cholesteatoma in ACS and the most detailed in vivo evidence of disruption of embryogenesis during malleoincudal joint formation. Laryngoscope, 2012
机译:耳con突综合征(ACS)是一种罕见的疾病,会影响第一分支弓结构。尚未报道ACS的听力损失类型和颞骨发现。我们描述了一个14岁的男性,其具有狭窄的耳廓,下颌骨发育不良,舌突,上颚弓,听力下降和胆脂瘤。计算机体层摄影术显示踝关节强直。对于胆脂瘤,在乳突切除术中去除了融合的踝突复合物。关节的电子显微镜检查和组织病理学提示融合是先天性的。这是ACS中听骨融合和胆脂瘤的首次报道,也是在踝关节形成过程中胚胎发生中断的最详细的体内证据。喉镜,2012年

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