The shortened cochlea: its classification and histopathologic features
The shortened cochlea: its classification and histopathologic features
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DOI：
10.1016/s0165-5876(01)00642-5
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发表时间：
2002-03-15
影响因子：
1.5
通讯作者：
Paparella, MM
中科院分区：
文献类型：
--
作者：
Zheng, YQ;Schachern, PA;Paparella, MM
简介：Mondini发育不良一词被用来描述几乎任何先天性的骨迷路畸形，导致对这类畸形的困惑和似乎相互矛盾的观察和结论。本研究的目的是对耳蜗数小于2.5圈的颞骨进行组织病理学分类和描述。方法：在我们收集的1800块颞骨标本中，发现有耳蜗者12例，21例，耳蜗圈数小于2.5圈。年龄从死产到50岁不等。尸检时采集了颞骨，对其进行处理，并将其包埋在火棉胶中。切片厚度20微米，每隔10个切片行苏木精-伊红染色，光镜下观察。记录转数、耳蜗长、耳蜗底完整性、蜗体长度、半规管和前庭畸形、前庭导水管和中耳扩大。21例年龄匹配的无耳蜗性畸形患者的颞骨作为对照进行耳轮长度测量。结果：耳蜗短畸形分为三组：(1)共腔畸形。耳蜗性发育不良(1耳)--严重的耳蜗性发育不良，无完整的基底弯；(2)Mondini发育不良(11耳)--1.5圈。(3)Mondini样发育不良A型(5耳)--2个转向耳蜗区，包括一个完整的基底部转向和一个完整的蜗底骨；B型(四耳)-1.5-2个转向耳蜗底、蜗底骨质发育不良或缺失(有或不存在内耳道和耳蜗骨之间的交通)和一个完全性的基底部转向。结论：短耳蜗型先天畸形的范围具有很大的变异性。为了准确评估迷路畸形，内耳畸形应该根据迷路中的发现进行分类。我们建议使用共腔耳蜗术。Mondini发育不良和Mondini样发育不良来描述这些可变的异常。(C)2002爱思唯尔科学爱尔兰有限公司。保留所有权利。
Introduction: The term 'Mondini dysplasia' has been used to describe virtually any congenital abnormality of the osseous labyrinth resulting in confusion and seemingly contradictory observations and conclusions about this type of deformity. The purpose of this study is to histopathologically classify and describe temporal bones whose cochleas have less than 2.5 turns. Methods: Of the 1800 temporal bones in our collection, 21 from 12 cases were found to have cochleas with less than 2.5 cochlear turns. Ages ranged from stillborn to 50 years. Temporal bones were harvested at autopsy, processed and embedded in celloidin. Sections were cut at a thickness of 20 mum and every 10th section stained with hematoxylin-eosin and examined using light microscopy. The number of turns, length of cochlea, integrity of cochlear base, length of modiolus, abnormalities of the semicircular canals and vestibule, enlargement of the vestibular aqueduct and middle ears were documented. Twenty-one temporal bones from age-matched patients without cochlear deformities were used as controls for modiolar length measurements. Results: Malformation of the shortened cochlea was histopathologically classified into three groups as follows: (1) Common cavity. cochlear dysplasia (one ear)-severe dysplasia of the cochlea without a complete basal turn; (2) Mondini dysplasia (11 ears)-1.5 cochlear turns. a complete basal turn, an incomplete or absent interscalar septum and a complete bone at the base of the modiolus; and (3) Mondini-like dysplasia type A (five ears)-2 turns to the cochlea including a complete basal turn and complete bone at the base of the modiolus; and type B (four ears)-1.5-2 turns to the cochlea, hypoplasia of or a missing bone at the base of the modiolus (either with or without a communication between the internal auditory canal and the cochlea) and a complete basal turn. Conclusion: The range of congenital malformations in short cochlea is highly variable. Fundamental to the accurate evaluation of a labyrinthine anomaly, malformations of the inner ear should be classified according to the findings in the labyrinth. We suggest the use of common cavity cochlear dysplasia. Mondini dysplasia and Mondini-like dysplasia to describe these variable anomalies. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.