小耳畸形中外耳严重程度与颞骨畸形的关系：一项计算机断层扫描研究。

文献信息

中文摘要

背景/目的： 小耳畸形常与颞骨畸形相关，但外耳表型在多大程度上反映潜在的CT定义解剖结构仍不确定。本研究旨在比较Marx严重程度分级和Nagata形态学分类与小耳畸形患者颞骨解剖结构的关系。方法：这项回顾性研究纳入26例患者、27只患耳，均具有术前外耳照片和颞骨计算机断层扫描（CT）检查。每只患耳根据Marx和Nagata系统进行分类，并使用改良Jahrsdoerfer CT评分系统评估颞骨解剖结构。采用Spearman秩相关和基于置换的Jonckheere-Terpstra分析评估关联，并额外进行患者水平的敏感性分析。结果：在外耳表型类别内观察到Jahrsdoerfer评分存在相当大的变异，Marx II级和III级之间大量重叠，较低评分集中在IV级。Marx分级与Jahrsdoerfer评分之间的负相关未达到传统统计学显著性（ρ = -0.380，p = 0.051），而Jonckheere-Terpstra检验发现总体下降趋势（置换p = 0.025）。该趋势在患者水平敏感性分析中不再达到统计学显著性。Nagata分类未能显著区分基于CT的放射学严重程度。结论：较大的外耳严重程度可能与较不利的颞骨解剖结构相关，但这种关系在连续的Marx分级中并不一致。Marx和Nagata分类提供了互补的表型信息，但两者均不能一致地反映潜在颞骨畸形的程度。这些初步发现强调表型-CT变异性，并支持在临床有指征时进行个体化的基于CT的解剖评估。

英文摘要

Background/Objectives: Microtia is frequently associated with temporal bone malformations, but the extent to which external ear phenotype reflects underlying CT-defined anatomy remains uncertain. This study aimed to compare the relationships of Marx severity grading and Nagata morphological classification with temporal bone anatomy in patients with microtia. Methods: This retrospective study included 26 patients with 27 affected ears who had both preoperative external ear photographs and temporal bone computed tomography (CT) examinations. Each affected ear was classified according to the Marx and Nagata systems, and temporal bone anatomy was assessed using the modified Jahrsdoerfer CT scoring system. Associations were evaluated using Spearman rank correlation and permutation-based Jonckheere-Terpstra analysis, with an additional patient-level sensitivity analysis. Results: Considerable variation in Jahrsdoerfer scores was observed within external ear phenotypic categories, with substantial overlap between Marx Grades II and III and lower scores concentrated in Grade IV. The inverse association between Marx grade and Jahrsdoerfer score did not reach conventional statistical significance (ρ = -0.380, p = 0.051), whereas the Jonckheere-Terpstra test identified an overall decreasing trend (permutation p = 0.025). This trend no longer reached statistical significance in the patient-level sensitivity analysis. Nagata classification did not significantly discriminate CT-based radiological severity. Conclusions: Greater external auricular severity may be associated with less favorable temporal bone anatomy, but the relationship was not uniform across successive Marx grades. Marx and Nagata classifications provide complementary phenotypic information, but neither consistently reflected the extent of underlying temporal bone malformation. These preliminary findings emphasize phenotype-CT variability and support individualized CT-based anatomical assessment when imaging is clinically indicated.