■本报告是一系列患者的病例,其发现可疑为医学文献中先前未描述的迷宫裂开综合征。我们描述了16例患者的临床和测试结果,这些患者的CT发现怀疑面神经鼓室段水平半规管的壶腹末端开裂。
■观测案例系列。
■神经前庭转诊中心。
■要纳入本研究,患者于2019年在我们中心就诊,并进行了高分辨率CT扫描,准直度为0.6mm。鉴定并进一步分析了被鉴定为怀疑面部神经穿过水平半规管(HSC-FND)的壶腹端的骨骼开裂的患者。
■患者症状的病例系列回顾性记录回顾,物理发现,测听法,前庭测试,并进行CT扫描。注意到其他开裂部位的CT发现。与同期手术治疗的外淋巴瘘(PLF)患者进行了比较。
■对听觉症状进行了病史和体格检查,前庭症状,和加剧的因素。and.对测听法和前庭测试进行了审查,以确定哪些测试最有可能出现异常。CT扫描根据对HSC-FND的怀疑程度独立分级。最后,将HSC-FND作为唯一裂开部位的患者与HSC-FND加其他裂开部位(HSC-FND+O)的患者以及经手术治疗的PLF患者组进行比较.
■在18名患者中,16符合纳入标准。对HSC-FND可疑的人中有9人(56%)在迷宫的其他部分有裂痕。其他开裂部位包括:六个上半规管开裂(SSCD),两个耳蜗面部裂开和一个耳蜗颈动脉裂开。最常见的听觉症状是尸音,其次是耳鸣和听觉饱满。最常见的前庭症状是冲动感(被推到一侧的感觉),然后是眩晕。眩晕最常见的加重因素是紧张,和声音。最常见的异常前庭测试是鼻Valsalva测试,除一名患者外,所有患者均为阳性。两组的回忆和检查观察结果相似,但与HSC-FND+O组相比,HSC-FND组不太可能出现热量减弱或ECOG异常.值得注意的是,与HSC-FND+0组相比,在HSC-FND组中更经常发现cVEMP具有更低的阈值。突出显示了一个示例案例。与PLF患者的比较显示,在出现尸检症状方面存在统计学上的显着差异,丰满和冲动的感觉。比较测试时,HSC-FND患者更可能有异常cVEMP,而PLF患者更可能有不对称听力。在HSC-FND患者中,双侧疾病的发生率也比PLF患者更常见。
■已经描述了一种新的迷宫裂开,其中面神经的鼓段穿过水平半规管的壶腹末端。HSC-FND患者可以以与HSC-FND+0患者相似的方式存在,除了如上所述之外,具有相似的测试结果。一种裂开如SSCD的鉴定并不排除另一种裂开如HSC-FND的存在。HSC-FND可能是SSCD手术后持续症状的来源,如病例所示。与PLF患者相比,HSC-FND患者似乎更有可能出现尸检症状,丰满度,pulsion,异常cVEMP,疾病的双边性,对称的听力
UNASSIGNED: This report is a case series of patients with findings suspicious for a labyrinthine dehiscence syndrome not previously described in the medical literature. We describe the clinical and test findings in 16 patients with CT findings suspicious for dehiscence of the ampullated end of the horizontal semicircular canal at the tympanic segment of the facial nerve.
UNASSIGNED: Observational case series.
UNASSIGNED: Neurotology vestibular referral center.
UNASSIGNED: To be included in this study the patients were seen at our center in 2019 and had a high-resolution CT scan with a collimation of 0.6 mm. Patients who were identified as having findings suspicious for dehiscence of bone where the facial nerve crosses the ampullated end of the horizontal semicircular canal (HSC-FND) were identified and further analyzed.
UNASSIGNED: Case series retrospective record review of patient symptoms, physical findings, audiometry, vestibular testing, and CT scans was performed. CT findings of other dehiscent sites were noted. A comparison to surgically treated perilymph fistula (PLF) patients of the same period was performed.
UNASSIGNED: History and physical exam were reviewed for auditory symptoms, vestibular symptoms, and exacerbating factors. and. Audiometry and vestibular testing were reviewed to determine which tests were most likely to be abnormal. CT scans were independently graded according to degree of suspicion for HSC-FND. Finally, patients with HSC-FND as the sole dehiscence identified were compared to those who had HSC-FND plus other dehiscent sites (HSC-FND+O) and to the group of surgically treated PLF patients.
UNASSIGNED: Of 18 patients, 16 met inclusion criteria. Nine (56%) of those suspicious for HSC-FND had dehiscences in other parts of the labyrinth. Additional dehiscent sites included: six superior semicircular canal dehiscences (SSCD), two cochlear facial dehiscences and one cochlear carotid dehiscence. The most common auditory symptoms were autophony followed by tinnitus and aural fullness. The most common vestibular symptoms were pulsion sensation (feeling of being pushed to one side) followed by vertigo spells. The most common exacerbating factors for vertigo were straining, and sound. The most commonly abnormal vestibular test was nasal Valsalva testing, which was positive in all but one patient. Anamnesis and examination observations were similar in both groups, but the HSC-FND group were less likely to demonstrate a caloric weakness or an abnormal ECOG compared to the HSC-FND+O group. Of note, cVEMP was more often found to have lower thresholds in the HSC-FND group compared to the HSC-FND+O group. An example case is highlighted. Comparison to the PLF patients revealed statistically significant difference in the presenting symptoms of autophony, fullness and pulsion sensation. When comparing testing, HSC-FND patients were more likely to have an abnormal cVEMP and PLF patients were more likely to have asymmetric hearing. The incidence of bilateral disease was also more common among the HSC-FND patients than the PLF patients.
UNASSIGNED: A new labyrinthine dehiscence has been described to occur where the tympanic segment of the facial nerve crosses over the ampullated end of the horizontal semicircular canal. HSC-FND patients can present in a similar manner as HSC-FND+O patients with similar test findings except as mentioned above. The identification of one dehiscence such as SSCD does not preclude the presence of another dehiscence such as HSC-FND. HSC-FND could be the source of persistent symptoms post SSCD surgery as illustrated in the case presented. HSC-FND patients seem to identify themselves compared to PLF patients by a much more likely presenting symptoms of autophony, fullness, pulsion, abnormal cVEMP, bilaterality of disease, and symmetric hearing.