Unpartitioned versus incompletely partitioned cochleae: Radiologic differentiation
Unpartitioned versus incompletely partitioned cochleae: Radiologic differentiation
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DOI：
10.1097/00129492-200407000-00020
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发表时间：
2004-07-01
影响因子：
2.1
通讯作者：
Saatci, T
中科院分区：
文献类型：
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作者：
Sennaroglu, L;Saatci, T
目的：在评估患者的过程中，我们意识到“Mondini畸形”一词被用来描述两种不同类型的耳蜗不完全分隔。第一个由一个未分隔的、完全空的耳蜗组成，其中缺乏标量间隔膜和整个蜗轴，使耳蜗呈囊性外观;伴随着这种病变的是一个明显扩张的前庭。第二种病理学符合Mondini畸形的经典描述，包括正常的基底转向和囊性顶点（其中中间和顶端转向形成囊性腔），前庭扩张和前庭水管扩大。本研究的目的是探讨两种不同类型的不完全分区的内耳畸形的放射学特征的基础上的差异。研究设计：我们进行了回顾性审查颞骨计算机断层扫描（CT）的结果。方法：受试者为18名患有重度双侧感音神经性听力损失的患者，他们接受了高分辨率CT，通过岩骨在轴向切片中获得mm厚的图像。将CT结果审查为I型（IP-I）和II型（IP-II）不完全分区。不完全分隔I型（未分隔耳蜗，囊状耳蜗前庭畸形）定义为耳蜗缺乏整个蜗轴和标量间隔膜的畸形，导致囊性外观，伴有前庭严重扩张。在不完全分隔II型（不完全分隔耳蜗，Mondini畸形）中，存在由正常基底转向和囊状顶端组成的耳蜗，伴有最小程度扩张的前庭和扩大的前庭导水管（VA）。测量包括耳蜗，前庭，前庭水管，和内听道（IAC），以确定这些pathologics.Results的特征：13耳IP-I和18耳IP-II异常。两种异常的耳蜗大小与正常所见无显著差异。只是内部结构不同。所有IP-I耳均出现前庭明显扩张。虽然这两种异常表现为前庭扩张，但IP-II患者的前庭扩张程度最小。IP-I异常的IAC大于IP-II异常。所有IP-II病例均伴有VA增大，而IP-I患者中只有1例VA可在CT上显示。在所有病例中，VA表现两侧对称：正常或扩大。结论：本研究中放射学表现的内耳测量证实存在两种不同类型的不完全分隔：IP-I（未分隔的耳蜗，囊性耳蜗前庭畸形）和IP-II（不完全分隔的耳蜗，典型的Mondini畸形）。I型畸形的分化程度低于II型畸形。典型的Mondini畸形有三个组成部分（囊性顶端，前庭扩张，前庭水管大），而I型畸形有一个空的，未分区的耳蜗和前庭扩张，没有扩大VA。Mondini畸形代表一种较晚的畸形，因此发育不良程度较I型轻。不仅外观，而且内耳结构的测量将有助于确定畸形的类型。
Objective: In the process of evaluating our patients, we realized that the term "Mondini deformity" was being used to describe two different types of incomplete partition of the cochlea. THE First one consisted of an unpartitioned, completely empty cochlea where the interscalar septum and entire modiolus were absent, giving the cochlea a cystic appearance; a grossly dilated vestibule accompanied this lesion. The second pathology fitted the classic description of Mondini deformity, consisting of a normal basal turn and cystic apex (where the middle and apical turns form a cystic cavity), dilated vestibule, and enlarged vestibular aqueduct. This study was planned to investigate the differences between the two types of incomplete partition for inner ear malformations based on radiologic features.Study Design: We conducted a retrospective review of temporal bone computed tomography (CT) findings.Methods: The subjects were 18 patients with profound bilateral sensorineural hearing loss who had high-resolution CT with contiguous 1-mm thick images obtained through the petrous bone in axial sections. The CT results were reviewed as incomplete partition type I (IP-I) and type II (IP-II). Incomplete partition type I (unpartitioned cochlea, cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and interscalar septa, resulting in a cystic appearance and there is an accompanying grossly dilated vestibule. In incomplete partition type II (incompletely partitioned cochlea, the Mondini deformity), there is a cochlea comprised of a normal basal turn and cystic apex accompanied by a minimally dilated vestibule and enlarged vestibular aqueduct (VA). Measurements involving the cochlea, vestibule, vestibular aqueduct, and internal auditory canal (IAC) were done to determine the characteristic features of these pathologies.Results: Thirteen ears had IP-I and 18 ears had IP-II anomaly. The size of the cochleae in both anomalies showed no significant difference from the normal findings. Only the internal architecture was different. All ears with IP-I had grossly dilated vestibules. Although both anomalies demonstrated vestibular dilatation, the degree of that was minimal in patients with IP-II. In IP-I anomalies, the IAC was larger than that in IP-II anomalies. An enlarged VA accompanied all cases of IP-II, whereas only one of the patients with IP-I had VA that could be demonstrated on CT. In all cases, the VA findings were symmetric on both sides: normal or enlarged.Conclusion: Inner ear measurements on radiologic findings in this study confirmed the presence of two different types of incomplete partition: IP-I (unpartitioned cochlea, cystic cochleovestibular malformation) and IP-II (incompletely partitioned cochlea, the classic Mondini deformity). The type I malformation is less differentiated than the type II malformation. Classic Mondini deformity has three components (a cystic apex, dilated vestibule, and large vestibular aqueduct), whereas type I malformation has an empty, unpartitioned cochlea and dilated vestibule without an enlarged VA. Mondini deformity represents a later malformation, so the degree of dysplasia is milder than that in type I. Not only the appearance, but also the measurements of the inner ear structures would be helpful in determining the type of malformation.