Inner ear pathologies impair sodium-regulated ion transport in Meniere's disease.
Inner ear pathologies impair sodium-regulated ion transport in Meniere's disease.
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DOI：
10.1007/s00401-018-1927-7
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发表时间：
2019-03
影响因子：
12.7
通讯作者：
Adams JC
中科院分区：
文献类型：
--
作者：
Eckhard AH;Zhu M;O'Malley JT;Williams GH;Loffing J;Rauch SD;Nadol JB Jr;Liberman MC;Adams JC
梅尼埃病（MD）是一种综合征性内耳疾病，通常与内耳内淋巴液的病理性积聚有关，称为“特发性”内淋巴积水（iEH）。尽管已经提出了许多诱发/加重MD的因素，但其病因仍然难以捉摸。在这里，我们使用免疫组织化学和原位蛋白质-蛋白质相互作用检测试验，证明了小鼠和人类内耳内淋巴囊（eES）骨外部分上皮中盐皮质激素控制的钠转运机制。在一系列广泛的人类颞骨的eES的组织学分析一致显示，在eES的病理变化与iEH和MD的临床病史的情况下，但没有这样的变化被发现的情况下，“继发性”高血压由于其他耳科疾病或健康对照。值得注意的是，两个病因不同的病理变性和发育不全，选择性地影响eES在MD的区别。具有退行性和发育不良eES病理学的MD病例的临床记录显示，临床疾病表现存在明显的组间差异。总体而言，我们首次确定了两种内耳病理，它们始终存在于MD中，并可直接与EH的发病机制相关，并可能影响MD的表型表现。本文的在线版本（10.1007/s 00401 -018-1927-7）包含补充材料，可供授权用户使用。
Meniere’s disease (MD), a syndromal inner ear disease, is commonly associated with a pathological accumulation of endolymphatic fluid in the inner ear, termed “idiopathic” endolymphatic hydrops (iEH). Although numerous precipitating/exacerbating factors have been proposed for MD, its etiology remains elusive. Here, using immunohistochemistry and in situ protein–protein interaction detection assays, we demonstrate mineralocorticoid-controlled sodium transport mechanisms in the epithelium of the extraosseous portion of the endolymphatic sac (eES) in the murine and human inner ears. Histological analysis of the eES in an extensive series of human temporal bones consistently revealed pathological changes in the eES in cases with iEH and a clinical history of MD, but no such changes were found in cases with “secondary” EH due to other otological diseases or in healthy controls. Notably, two etiologically different pathologies—degeneration and developmental hypoplasia—that selectively affect the eES in MD were distinguished. Clinical records from MD cases with degenerative and hypoplastic eES pathology revealed distinct intergroup differences in clinical disease presentation. Overall, we have identified for the first time two inner ear pathologies that are consistently present in MD and can be directly linked to the pathogenesis of EH, and which potentially affect the phenotypical presentation of MD. The online version of this article (10.1007/s00401-018-1927-7) contains supplementary material, which is available to authorized users.
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DOI：
10.1097/mao.0b013e31821553ce
发表时间：
2011-06
期刊：
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
影响因子：
--
作者：
Chung JW;Fayad J;Linthicum F;Ishiyama A;Merchant SN
通讯作者：
Merchant SN
影响因子：
2.1
作者：
Guerkov, Robert
通讯作者：
Guerkov, Robert
影响因子：
2.1
作者：
Frejo, L.;Martin-Sanz, E.;Lopez-Escamez, J. A.
通讯作者：
Lopez-Escamez, J. A.
影响因子：
2.1
作者：
Clemmens, Clarice;Ruckenstein, Michael
通讯作者：
Ruckenstein, Michael
影响因子：
2.8
作者：
Kim, Sung Huhn;Marcus, Daniel C.
通讯作者：
Marcus, Daniel C.