Semicircular Canal Dehiscence

  • 文章类型: Journal Article
    背景:上半规管裂开(SSCD)是耳囊的裂开,通常位于上半规管上方。该数据库构成了迄今为止最大的SSCD患者系列。
    目的:为了确定术前因素,如果有的话,在大型SSCD患者队列中有助于术后结局并评估症状缓解。
    方法:单一机构,回顾性图表回顾收集的患者人口统计,术中发现,以及手术前后的症状.对不成对的分类变量进行Fisher精确t检验,显著性水平为p<0.05。
    结果:进行了350例SSCD修复。中位年龄为52岁(范围:17-86岁,±6.4年),中位随访时间为4.6个月(范围:0.03-59.5个月,±6.8个月)。术前听力损失与女性性别显著相关(p=0.0028)。术前报告最多的症状是耳鸣(77.4%),头晕(74.0%),尸检(66.3%),扩增(63.7%),和不平衡(62.6%)。在接受单侧和双侧SSCD修复的患者之间,术后症状缓解率最高的是尸检(74.9%,p<0.001),扩增(77.3%,p=0.00027),高音(77.4%,p=0.023),听力(62.9%,p=0.0063),和头晕(54.6%,对于单侧SSCD修复的患者,p<0.001)。
    结论:经中颅窝入路手术修复可显著解决听觉,前庭,SSCD患者的神经系统症状。尽管这是迄今为止最大的单一机构SSCD研究之一,未来的多机构,前瞻性研究将有助于验证这些结果。
    BACKGROUND: Superior Semicircular Canal Dehiscence (SSCD) is a dehiscence of the otic capsule which normally lies over the superior semicircular canal. This database constitutes the largest series of SSCD patients to date.
    OBJECTIVE: To determine what preoperative factors, if any, contribute to postoperative outcomes and evaluate symptom resolution in a large SSCD patient cohort.
    METHODS: A single-institution, retrospective chart review collected patient demographics, intraoperative findings, and pre-and postoperative symptoms. Fisher\'s exact t-test was performed for unpaired categorical variables, with a significance level of p < 0.05.
    RESULTS: 350 SSCD repairs were performed. The median age was 52 years (range: 17-86 years, ± 6.4 years), and the median follow-up duration was 4.6 months (range: 0.03-59.5 months, ± 6.8 months). Preoperative hearing loss was significantly associated with female sex (p = 0.0028). The most reported preoperative symptoms were tinnitus (77.4%), dizziness (74.0%), autophony (66.3%), amplification (63.7%), and disequilibrium (62.6%). Between patients who received unilateral versus bilateral SSCD repair, the greatest postoperative symptomatic resolution was seen in autophony (74.9%, p < 0.001), amplification (77.3%, p = 0.00027), hyperacusis (77.4%, p = 0.023), hearing (62.9%, p = 0.0063), and dizziness (54.6%, p < 0.001) for patients with unilateral SSCD repair.
    CONCLUSIONS: Surgical repair via the middle cranial fossa approach can significantly resolve auditory, vestibular, and neurological symptoms of patients with SSCD. Although this is one of the largest single-institution SSCD studies to date, future multi-institutional, prospective studies would be beneficial to validate these results.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
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  • 文章类型: Review
    我们报告了一名男子患有上半规管裂开并伴有严重的听前庭症状。患者有创伤后应激障碍和创伤性脑损伤的病史,他被推测为精神分裂症,有自杀念头.患者接受了手术治疗,症状完全缓解,精神分裂症的诊断得到了恢复。这个案例突出了耳鸣和幻听之间的重叠,因为两者都导致没有外部刺激的听觉感知。确定患者听觉症状的适当原因对于提供适当的医疗和可能的手术护理是不可或缺的。
    We report the case of a man suffering from superior semicircular canal dehiscence with severe audiovestibular symptoms. The patient had a history of posttraumatic stress disorder and traumatic brain injury, and he had a presumed diagnosis of schizophrenia, with suicidal ideation. The patient was treated surgically with complete resolution of his symptoms and a retraction of his schizophrenia diagnosis. This case highlights the overlap between tinnitus and auditory hallucinations, as both result in auditory perception without an external stimulus. Ascertaining the appropriate cause of a patient\'s auditory symptoms is integral to providing appropriate medical and possibly surgical care.
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  • 文章类型: Case Reports
    一名73岁的女性因持续的左前良性阵发性位置性眩晕而无法接受多种重新定位程序和训练物理治疗师练习而被转诊到我们部门。前庭听觉评估和岩骨的高共振计算机断层扫描证实存在5.4毫米大的无症状左前半规管裂开。同一患者中两个明显不同的神经实体之间的联系通过其他图像进一步维持。T2加权和3维迷宫序列证实存在部分“自动堵塞”的上半规管开裂,该开裂逐渐捕获了靠近上半规管杯的更大耳石颗粒。
    A 73-year-old female was referred to our department for persistent left anterior benign paroxysmal positional vertigo refractory to multiple repositioning procedures and training physiotherapist exercises. The audiovestibular assessment and high resonance computed tomography of the petrosal bone confirmed the presence of a 5.4 mm large paucisymptomatic left anterior semicircular canal dehiscence. The connection between the 2 apparently distinct neurotological entities in the same patient was further sustained by additional imagery. T2-weighted and 3-dimensional labyrinthine sequences confirmed the presence of a partially \"auto-plugged\" superior semicircular canal dehiscence which progressively entrapped greater otolith particles proximal to the cupula of the superior semicircular canal.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    颞骨炎性假瘤是一种良性特发性炎症过程,在临床实践中很少遇到。我们描述了一个新的病例,该患者在颞骨活动性炎性假瘤的情况下发展为上半规管裂开综合征。
    一名女性患者发现颞骨炎性假瘤。乳突切除术和类固醇治疗后,随后,她患上了上半规管裂开综合征。
    患者最初接受鼓膜切开术和压力平衡管放置治疗单侧积液。计算机断层扫描和磁共振成像成像显示溶解乳突肿块。完整的乳突切除术可诊断为炎性假瘤,并对患者进行长期辅助糖皮质激素治疗。在此过程之后,她被发现有症状的上半规管裂开综合征的新发展。她最终对上半规管裂开进行了乳突修复。
    描述了患者的临床过程,重点是颞骨活动性炎性假瘤背景下上半规管裂开综合征的发展。
    患者的影像学和临床病史与颞骨炎性假瘤一致。她接受了长期术后皮质类固醇的治疗性乳突切除术。在她手术后的过程中,她出现了眩晕的症状,听力损失,和尸检。随后,她被诊断出患有上半规管裂开,并接受了裂开的乳突修复术。手术后,她的症状令人满意地得到了缓解。
    颞骨炎性假瘤是一种罕见的良性炎症过程。我们介绍了在炎性假瘤背景下发展的上半规管裂开综合征的第一个描述。
    Inflammatory pseudotumor of the temporal bone is a benign idiopathic inflammatory process that is rarely encountered in clinical practice. We describe a novel case of a patient who developed superior semicircular canal dehiscence syndrome in the setting of active inflammatory pseudotumor of the temporal bone.
    One female patient found to have inflammatory pseudotumor of the temporal bone. After treatment with mastoidectomy and steroids, she subsequently developed superior semicircular canal dehiscence syndrome.
    The patient initially underwent myringotomy and pressure equalization tube placement for a unilateral effusion. Imaging with computed tomography and magnetic resonance imaging revealed a lytic mastoid mass. A complete mastoidectomy was diagnostic for inflammatory pseudotumor and the patient was treated with adjuvant long-term corticosteroids. After this procedure, she was discovered to have new development of symptomatic superior semicircular canal dehiscence syndrome. She eventually underwent a trans-mastoid repair of her superior semicircular canal dehiscence.
    The patient\'s clinical course is described with emphasis on the development of superior semicircular canal dehiscence syndrome in the setting of active inflammatory pseudotumor of the temporal bone.
    The patient\'s radiographic and clinical history were found to be consistent with inflammatory pseudotumor of the temporal bone. She underwent a therapeutic mastoidectomy with long-term post-operative corticosteroids. During her post-operative course, she developed symptoms of vertigo, hearing loss, and autophony. She was subsequently diagnosed with superior semicircular canal dehiscence and treated with a trans-mastoid repair of the dehiscence. Her symptoms satisfactorily resolved after this surgery.
    Inflammatory pseudotumor of the temporal bone is a rare benign inflammatory process. We present what is to our knowledge the first description of superior semicircular canal dehiscence syndrome developing in the setting of inflammatory pseudotumor.
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  • 文章类型: Journal Article
    OBJECTIVE: To explore the usefulness of the responses of video head impulse testing (vHIT) in assessing symptomatic patients with superior semicircular canal dehiscence (SSCD).
    METHODS: This was a prospective case-control study performed in a tertiary skull base referral Centre in the UK. It included all patients ¬diagnosed with SSCD from January 2015 to January 2019 and compared to a control group of age and gender-matched unaffected indi¬viduals. The main outcome of the study was semicircular canal vestibule-ocular reflex (VOR) gains during vHIT assessment and link to patients\' symptoms.
    RESULTS: A total of 28 patients were diagnosed with SSCD during the study period and completed the vHIT assessment. Reduced VOR gains (<0.8) were noted in 57% of patients (n = 16). Half of these (n = 8) were in canals other than the superior semicircular (posterior only: n = 5; lateral and posterior: n = 3). Three patients (10.7%) had abnormal responses in 2 canals. The canals in the contralateral side were affected in 56.5% of the cases. There was no correlation with the patients\' symptoms. Results were directly comparable with the control group vHIT results with no identifiable statistically significant differences on comparison of the ipsilateral SSCD side with a randomly selected side from the control group (all comparisons: P > .05).
    CONCLUSIONS: SSCD can affect the vestibular responses from all 3 semicircular canals; not necessarily the superior one. Similar responses were found in a control group of normal subjects. Although the use of vHIT in the assessment of SSCD is not diagnosis-specific, it can still help with identifying the impact of surgery on all canals prior to any intervention in order to avoid bilateral vestibular failure.
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  • 文章类型: Case Reports
    UNASSIGNED: Report a series of cases in which patients have concomitant superior semicircular canal dehiscence (SSCD) and a dehiscent tegmen tympani with Dural contact to the malleus head (DCMH).
    UNASSIGNED: An analysis of radiologic and audiologic data in 4 patients who presented with SSCD and DCMH at a tertiary care institution. A pertinent literature review was performed.
    UNASSIGNED: Four patients (5 ears) had SSCD and DCMH. In 3 patients with unilateral DCMH, the mean maximum air-bone gap was 15 dB in the ear with DCMH compared to 50 dB in the ear without DCMH. Of the 5 ears with DCMH, the mean air conduction threshold at 250 Hz was 17 dB compared to 42 dB in the 3 ears without DCMH.
    UNASSIGNED: We report the findings of DCMH in a series of 4 patients with bilateral SSCD. This limited series suggests that ears with SSCD and DCMH have less of an air-bone gap than would be expected, as 1 would expect an additive effect of DCMH and SSCD on the air-bone gap.
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  • 文章类型: Case Reports
    In this case report, the air-conducted cervical vestibular evoked myogenic potentials (AC cVEMP) test was only sensitive for the left superior semicircular canal dehiscence (SCD), even though the contralateral SCD was of equal length (2.5 mm). Furthermore, a lysis of the processus lenticularis incudis caused a real conductive hearing loss in the left ear. A diminished left AC cVEMP was thus expected, but the opposite was shown (increased corrected amplitude, lowered detection threshold). The patient only experienced hearing loss, so middle ear surgery was performed to repair the lysis. The postoperative AC cVEMP showed a further \"uncovering\" of the SCD with increased corrected amplitude on the left but no vestibular symptoms. The significance of an SCD should be interpreted with caution, even when the AC cVEMP and the imaging are significant. Furthermore, AC cVEMPs should not be considered as evidence for the absence or presence of conductive hearing loss.
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