Detailed MR imaging assessment of endolymphatic hydrops in patients with SLC26A4 mutations.
Detailed MR imaging assessment of endolymphatic hydrops in patients with SLC26A4 mutations.
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DOI：
10.1016/j.anl.2020.05.018
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发表时间：
2020-06
期刊：
影响因子：
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通讯作者：
Keita Tsukada;S. Usami
中科院分区：
文献类型：
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作者：
Keita Tsukada;S. Usami
SLC 26 A4突变是耳聋患者中第二常见的突变。大多数SLC 26 A4突变患者的前庭导水管（LVA）较大。近年来，有报道在高分辨率增强3 T-MRI的基础上发现LVA患者存在内淋巴积水（ELH）。然而，尚未进行详细评估。我们提供了第一份关于双等位基因SLC 26 A4突变LVA患者ELH的报告。在本研究中，我们重点关注1）双等位基因SLC 26 A4突变的LVA患者中ELH的发现，（2）内淋巴管（艾德）和内淋巴囊（ES）的两种不同钆增强方法研究对象和方法5例SLC 26 A4突变患者行3 T-MRI增强扫描，采用鼓室内（IT）或静脉注射（IV）法。结果所有患者均至少有一耳有ELH。在前庭（8/10耳）和耳蜗（7/10耳）中确定了ELH。关于艾德和ES，所有的耳朵进行MRI与IT注射Gd在ES或VA或两者的黑色区域;然而，所有的耳朵接受IV注射没有黑色区域，并得到了很好的enhanced.ConclusionsA大多数的患者有严重的ELH在耳蜗前庭内淋巴，与两种不同的模式中观察到的MRI结果的艾德和ES。
ObjectiveMutations inSLC26A4represent the second most common mutations in deafness patients. The majority of patients withSLC26A4mutations have a large vestibular aqueduct (LVA). Recently, some reports showed the presence of endolymphatic hydrops (ELH) in patients with LVA on the basis of high-resolution enhanced 3T-MRI. However, detailed evaluation has not been performed. We provide the first report on ELH in LVA patients with biallelicSLC26A4mutations. In this study, we focused on 1) the findings of ELH in LVA patients with biallelicSLC26A4mutations, and 2) the findings of the endolymphatic duct (ED) and endolymphatic sac (ES) by using two different gadodimide (Gd) enhancement methods.Subjects and methodsFive patients withSLC26A4mutations underwent enhanced 3T-MRI using the intratympanic (IT) or intravenous (IV) injection of Gd for the diagnosis ELH.ResultsAll of the patients had ELH in at least one ear. ELH was identified in the vestibule (8/10 ears) as well as in the cochlea (7/10 ears). With regard to the ED and ES, all ears for which MRI was performed with an IT injection of Gd had black areas in the ES or VA or both; however, all of the ears receiving an IV injection had no black areas and were well enhanced.ConclusionsA majority of the patients had severe ELH in the cochleo-vestibular endolymph, with two different patterns observed in the MRI findings of the ED and ES.