儿童遗传性与先天性巨细胞病毒相关听力损失的听觉与视觉皮层体积MRI分析
本研究对GJB2相关、cCMV相关听力损失儿童及正常对照儿童的3D T1加权序列进行FreeSurfer体积分割，比较听觉与视觉皮层体积及半球不对称性。结果显示GJB2相关听力损失主要伴视觉皮层体积改变，cCMV相关听力损失主要伴听觉皮层体积改变，且两组均存在特定区域的左侧或右侧偏侧化。
要点
- GJB2相关听力损失需关注视觉皮层体积改变，cCMV相关听力损失需关注听觉皮层体积改变。
- 两组听力损失与正常对照均可见特定听觉区左侧偏大、视觉区右侧偏大的不对称模式。
- 不同病因听力损失对皮层发育影响不同，影像评估时应结合病因分析。
核心结论
- GJB2相关听力损失与视觉皮层体积改变相关，cCMV相关听力损失与听觉皮层区域改变相关。
- 特定听觉区在GJB2相关、cCMV相关听力损失及正常对照中均显示显著左侧偏大；视觉区在cCMV相关听力损失及对照中显示显著右侧偏大。
研究设计与样本
- 采用3D T1加权序列进行FreeSurfer体积分割，分析Heschl回、颞极平面、颞平面、颞上回外侧、初级视觉皮层及楔叶的皮层体积。
- 采用线性混合模型分析病例与对照的皮层体积差异，校正年龄、性别和场强；采用侧化指数评估半球不对称性。
关键影像表现/征象
- GJB2相关听力损失与对照组相比，右侧、左侧及总楔叶皮层体积存在显著差异。
- cCMV相关听力损失与对照组相比，右侧Heschl回和颞平面、左侧颞上回外侧及总Heschl回和颞平面存在显著差异。
诊断标准与关键数值
- GJB2相关听力损失组右侧（P=.03）、左侧（P=.03）及总楔叶（P=.02）皮层体积与对照组存在显著差异。
- cCMV相关听力损失组右侧Heschl回（P=.02）、右侧颞平面（P<.001）、左侧颞上回外侧（P=.002）、总Heschl回（P=.02）及总颞平面（P<.001）与对照组存在显著差异。
- GJB2相关SNHL中左侧Heschl回显著更大（P=.01）；cCMV相关听力损失及对照组中左侧Heschl回、颞平面和颞上回外侧显著更大，右侧楔叶显著更大（P<.01）。
- 统计显著性阈值设定为P<.05。
临床应用与阅片要点
- 阅片评估儿童听力损失时，GJB2病因可重点关注视觉皮层体积改变，cCMV病因可重点关注听觉皮层区域改变。
- 注意不同病因听力损失均可见左侧听觉区偏大和右侧视觉区偏大的自然不对称，避免误判为异常。
局限性
- 本研究为回顾性研究。
- GJB2相关听力损失组样本量较小（7例）。
以上解读依据论文摘要（全文未开放获取或许可不允许翻译转载）。
英文原文（摘要）· 点击展开
BACKGROUND AND PURPOSE: Although effects of deafness on the brain have been reported, little is known about the differential impact of genetic versus acquired etiologies. Our purpose was to assess cortical volumetric differences and hemispheric asymmetry in children with gap junction protein beta-2 (GJB2)- and congenital cytomegalovirus (cCMV)-related hearing loss compared with age- and sex-matched controls.
MATERIALS AND METHODS: In this retrospective study, 3D T1-weighted sequences of children with cCMV-related hearing loss, GJB2-related hearing loss, and normal hearing were analyzed using FreeSurfer volumetric segmentation. Cortical volumes of Heschl gyrus (HG), planum polare (PP), planum temporale (PT), lateral aspect of superior temporal gyrus (LSTG), primary visual cortex, and cuneus were assessed. We performed a linear mixed model analysis to investigate cortical volumetric differences between 1:3 case:control-matched groups, adjusting for age, sex, and field strength. The laterality index was calculated to assess hemispheric asymmetry, and a 2-tailed 1-sample t test was used to compare the distribution of values against zero. Statistical significance was determined at a threshold of P < .05.
RESULTS: Twenty-two children with cCMV-related hearing loss, 7 children with GJB2-related hearing loss, and 87 age- and sex-matched children with normal hearing were included. Significant differences were observed between the cortical volumes in the right (P = .03), left (P = .03), and total cuneus (P = .02) between GJB2-related hearing loss and controls; HG (P = .02) and PT (P < .001) on the right; LSTG (P = .002) on the left; and the total HG (P = .02) and PT (P < .001) between cCMV-related hearing loss and controls. HG was significantly larger on the left in GJB2-related SNHL (P = .01), and the HG, PT, and LSTG were significantly larger on the left with a significantly larger right cuneus in both cCMV-related hearing loss and controls (P < .01).
CONCLUSIONS: GJB2-related hearing loss is associated with volumetric alterations in the visual cortices, and alterations in the auditory cortical regions occur in cCMV-related hearing loss. Particular auditory areas showed significant leftward asymmetry in GJB2- and cCMV-related hearing loss and controls. In contrast, the visual areas showed significant rightward asymmetry in both CMV-related hearing loss and controls.
引用
- Hiremath, Wagner, Cushing, et al. Genetic and Congenital Cytomegalovirus-Related Hearing Loss in Children: Volumetric MRI Analysis of Auditory and Visual Cortices. AJNR Am J Neuroradiol. 2026. PMID: 42235969. doi:10.3174/ajnr.A9176
供执业医师工作参考，不替代临床判断；数值与分级以指南原文为准。