Spiral Ganglion Degeneration Patterns in Endolymphatic Hydrops
Spiral Ganglion Degeneration Patterns in Endolymphatic Hydrops
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DOI：
10.1097/mlg.0b013e31816ba9cd
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发表时间：
2008-07-01
期刊：
影响因子：
2.6
通讯作者：
Megerian, Cliff A.
中科院分区：
文献类型：
--
作者：
Bixenstine, Paul J.;Maniglia, Mauricio P.;Megerian, Cliff A.
目的：内淋巴积水（ELH）和听力损失（HL）之间的机制联系尚不清楚。虽然ELH的严重程度在一些研究中已被证明与HL相关，但重要结构（包括毛细胞和耳蜗神经）的损伤未能证明与ELH严重程度相关。本研究的目的是评估螺旋神经节细胞变性是ELH相关耳蜗损伤的主要病理部位的假设，与ELH的严重程度相关，并且在顶端区域最为深刻。研究设计：在豚鼠模型中进行ELH的手术诱导，随后进行ELH的组织学确认，随后与节段螺旋神经节细胞密度相关。对豚鼠（N = 14）进行单侧ELH诱导，并在4至6个月后处死。使用计算机辅助形态测量分析获得ELH严重程度和螺旋神经节密度。通过计算螺旋神经节变性指数（DI）为每个animal.Results：顶端螺旋神经节表现出显着更大的退化比在基底螺旋神经节（DI：1.93与1.13; P = 0.004）。心尖部螺旋神经节变性程度与总水肿指数（P = .006）和心尖部水肿指数（P = .003）相关性良好。然而，基底螺旋神经节变性与水肿的严重程度没有很好的相关性（总水肿指数：P > 0.05;基底水肿指数：P > 0.05）。结论：ELH相关的病理似乎最初集中在顶端螺旋神经节，恶化的程度与ELH的严重程度相关。这些发现反映了人类状况的一些报告，并暗示ELH和继发性功能障碍的耳蜗损伤机制似乎是从耳蜗顶端开始的神经毒性。
Objectives: The mechanistic association between endolymphatic hydrops (ELH) and hearing loss (HL) is unclear. Although ELH severity has been shown to correlate in some studies with HL, injury of vital structures, including hair cells and the cochlear nerve, have failed to demonstrate correlation with ELH severity. The goal of this study is to evaluate the hypothesis that spiral ganglion cell degeneration is the principle pathologic site of ELH-related cochlear injury, correlates with ELH severity, and is most profound in the apical region.Study Design: Surgical induction of ELH in the guinea pig model was followed by histologic confirmation of ELH and subsequent correlation with segmental spiral ganglion cell densities.Methods: Guinea pigs (N = 14) were subjected to unilateral ELH induction and killed after 4 to 6 months. ELH severity and spiral ganglion densities were obtained using computer-aided morphometric analysis. Densities were normalized by calculating a spiral ganglion degeneration index (DI) for each animal.Results: The apical spiral ganglion demonstrated significantly greater degeneration than that noted in the basal spiral ganglion (DI: 1.93 vs. 1.13; P = .004). The degree of spiral ganglion degeneration in the apex correlates well with a total hydrops index (P = .006) and an apical hydrops index (P = .003). Basal spiral ganglion degeneration however, does not correlate well with hydrops severity (total hydrops index: P > .05; basilar hydrops index: P > .05).Conclusions: ELH-related pathology appears to focus initially on the apical spiral ganglion and the degree of deterioration correlates well with the severity of ELH. These findings mirror some reports in the human condition, and imply that the mechanism of cochlear injury in ELH and secondary dysfunction appears to be a neural toxicity that begins in the apex of the cochlea.