Cochleosaccular dysplasia: a morphometric and histopathologic study in a series of temporal bones.
Cochleosaccular dysplasia: a morphometric and histopathologic study in a series of temporal bones.
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耳蜗囊发育不良：一系列颞骨的形态测量和组织病理学研究。
DOI：
10.1097/00129492-200407000-00021
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发表时间：
2004
期刊：
影响因子：
--
通讯作者：
Oliveira,CarlosACP
中科院分区：
文献类型：
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作者：
Sampaio,AndreLL;Cureoglu,Sebahattin;Schachern,PatriciaA;Kusunoki,Takeshi;Paparella,MichaelM;Oliveira,CarlosACP
目的：本研究的目的是对一系列患有耳蜗囊发育不良的颞骨进行形态测量分析，以阐明这种疾病的内耳变化程度。研究设计：对一系列聋哑病例进行的这项人类颞骨组织病理学研究涉及形态测量分析，包括血管纹和螺旋韧带面积测量以及螺旋神经节和毛细胞计数。主题：根据Scheibe描述的组织病理学结果，从35例聋哑症患者中选择了13块颞骨。二十个正常的年龄匹配的对照组被用于comparison.Results：所有颞骨的主要组织病理学结果描述的Scheibe，以及严重受影响的纹血管。七个颞骨有囊性区的条纹和三个有结石。耳蜗囊发育不良的颞骨横截面积在所有耳蜗转弯处均小于正常，但螺旋韧带横截面积无差异。Reissner膜在三个颞骨中是水肿的，Corti器在至少一个耳蜗中缺席。在7个颞骨的黄斑中存在凝块。12个颞骨显示出一定程度的螺旋神经节细胞丢失。在任何颞骨中均未观察到毛细胞。3例有耳聋家族史。结论：病理表现多样，局限于球囊和中阶。这种变化可能反映了耳蜗囊发育不良的不同病因。
Objective:The objective of this study was to perform a morphometric analysis of a series of temporal bones with cochleosaccular dysplasia to clarify the extent of inner ear changes in this disease.Study Design:This human temporal bone histopathologic study of a series of deaf–mute cases involves morphometric analysis, including stria vascularis and spiral ligament area measurements and spiral ganglion and hair cells counts.Subjects:Thirteen temporal bones were selected from 35 with deaf mutism based on the histopathologic findings described by Scheibe. Twenty normal age-matched control subjects were used for comparisons.Results:All temporal bones had the main histopathologic findings described by Scheibe, as well as severe affected stria vascularis. Seven temporal bones had cystic areas in the stria and three had concretions. Cross-sectional strial areas in temporal bones with cochleosaccular dysplasia were smaller than normal in all cochlear turns; however, no difference was found in spiral ligament cross-sectional areas. Reissner’s membrane was hydropic in three temporal bones and the organ of Corti was absent in at least one cochlear turn in five. Concretions were present in the macula of seven temporal bones. Twelve temporal bones showed some level of spiral ganglion cell loss. No hair cells were observed in any temporal bone. A familial history of deafness was found in three cases.Conclusion:Pathologic findings were variable and limited to the saccule and scala media. The variation, perhaps, reflects the different etiologies involved in the origin of cochleosaccular dysplasia.