大前庭水管综合征患者的听力学特点

目的探讨大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患者的听力学特点。方法对19例(38耳)LVAS患者进行了纯音听阈测试、盖莱氏试验、声导抗测试及颞骨高分辨CT检查。结果19例(38耳)患者CT检查均为单纯前庭水管扩大。纯音测听34耳为混合性聋,低频骨气导差较大,中高频骨气导差较小。28耳鼓室导抗图均为A型,20耳可引出声反射,声反射阈与听阈之差平均为28 dB HL。28耳进行了共振频率测试,中耳共振频率为652±175 Hz,较正常耳低(P<0.01)。18耳进行了盖莱氏试验,均为阳性。结论LVAS患者可表现为感音神经性聋,也可表现为混合性聋。混合性聋患者在听力较好时可引出声反射,是该病的特征性表现。另外,该病呈混合性聋时,还可能意味着病变的可逆性。     

大前庭水管综合征临床分析

目的 (1)了解大前庭水管综合征(LVAS)病史、误诊和遗传情况;(2)进行听功能调查,并对听力损失进行分级;(3)单独前庭水管扩大颞骨CT表现及外口宽度测定;(4)听力损失程度与外口径大小之关系.材料和方法回溯过去10年间,100例(男65,女35)LVAS和60例对照组之颞骨CT扫描,确定LVAS之CT诊断标准,并在轴位CT扫描上测前庭水管外口宽度.对病人进行系统听功能检查,按WHO国际听力伤残标准进行分度.分析听力损失与水管口径大小之关系.结果 100例中99例为双侧,1例为单侧前庭水管扩大,听力下降发生于童年早期,平均发病年龄为3.9岁.有直系和旁系家庭史者7例,因轻微头部外伤听力下降22例,因感冒致聋13例,诊断为突聋9例,误诊为耳毒性聋5例,因过度用力致聋1例.LVAS前庭水管外口宽度平均为7.53 1.2mm,回归统计分析,听力损失程度与外口大小无相关性.对照组30%外口不显影,显影者口径均小于2mm.结论具有波动和进行性感音神经聋的LVAS是一独立的临床疾病,为常染色体隐性遗传,确诊依靠CT检查,目前以预防外伤等保守处理及配助听器是主要治疗方法,因内淋巴囊减压和阻塞术术后听力恶化已被放弃.若听力明显下降,影响入学学习,可考虑耳蜗移植术.     

大前庭水管综合征患者听力随访观察

目的 动态观察大前庭水管综合征(large vestibular aqueduct syndrome, LVAS)患者的听力变化,为更好地保护LVAS 患者的听力提供参考.方法 选取2007年7月至2016年5月经确诊且资料完整、能够准确完成纯音听阈检测的双耳大前庭水管综合征患者21例为研究对象,年龄5~29岁,中位年龄11岁,其中男12例,女9例;12例患者行单侧人工耳蜗植入手术,另一耳佩戴助听器,其余患者均双耳佩戴助听器.动态观察非耳蜗植入耳听力2~7年,中位数3年4个月;在随访期间发现患者听力下降就诊,及时给予扩血管改善循环、营养神经等药物保守治疗1~2周,比较用药前后的听力变化;病情稳定者一年复查一次纯音听阈,动态观察听力变化.结果 21例(30耳)中随访期间有15例发生过听力波动(下降),治疗前500、1 000、2 000、4 000 Hz平均纯音听阈分别为85.3±4.4、91.5±4.3、95.9±5.7、99.1±6.6 dB HL,治疗后分别为66.5±4.1、74.4±4.1、76.8±5.0、80±5.9 dB HL,各频率治疗前后比较差异均有统计学意义(均为P<0.05).结论 大前庭水管综合征患者可表现为波动性或进行性感音神经性听力损失;早期发现、及时治疗,可使LVAS患者的听力维持在一定的水平或使之听力下降的速度明显减缓.     

95例大前庭水管综合征的临床分析

目的　调查大前庭水管综合征 (largevestibularaqueductsyndrome,LVAS)的发病、遗传和听觉损失情况 ,探讨LVAS之前庭水管外口宽度与听力损失的关系。方法　回溯性搜集 1 992～ 2 0 0 2年 ,95例 (1 90耳 )双侧LVAS的病史 ,听力和前庭功能检查 ,用每例LVAS颞骨CT片的标尺 ,测量前庭水管外口的宽度。结果　 95例 (1 90耳 )LVAS中 ,男 65例 ,女 30例 ,男女比为 2 2∶1。有家族史者 1 2例(1 2 6 % )。平均就诊年龄 7 6岁 ,年龄范围从 1 2～ 39岁。本组绝大多数误诊 ,因轻度外伤而致重度感音神经性聋 2 1例 (2 2 % ) ;上呼吸道感染致聋 1 3例 (1 3 6 % ) ,误诊为突发性聋 9例 (9 5 % ) ;拟诊氨基糖甙类中毒 5例 (5 3 % )。重度感音神经性聋 1 72耳 ,占 92 4%。 77例 (1 54耳 )前庭水管外口宽度( x±s)为 (7 5± 1 2 )mm ,听力损失与外口宽度不成比例。听力损害可能与富含蛋白的内淋巴从内淋巴囊倒流于耳蜗和前庭有关。结论　LVAS是独立的疾病 ,具有波动性及进行性感音神经性聋。诊断依靠颞骨CT扫描或磁共振成像 (magneticresonanceimaging ,MRI)。若听力波动后能改善可保守治疗 ,若明显听力下降 ,影响学习 ,可考虑采用耳蜗植入术     

大前庭水管综合征与内耳其他畸形的听性脑干反应特性分析

目的:通过对大前庭水管综合征(LVAS)与内耳其他畸形的听性脑干反应(ABR)特性进行分析,了解在进行ABR检查过程中出现声诱发短潜伏期负相波(ASNR)对诊断LVAS的特异性.方法:对2008-01-2009-09患感音神经性聋(SNHL)的患儿进行颞骨薄层CT扫描,将其中70例内耳其他畸形患儿的影像学结果进行分析,并将研究对象分组,LVAS组(包括大前庭伴其他内耳畸形)38例(76耳),内耳其他畸形组32例(64耳),在进行行为测听或纯音测听、声导抗、耳声发射听力学检查项目的基础上,均进行ABR检测.结果:ABR检查发现24例(41耳)大前庭水管,在2～3 ms处记录到特征性的负相波,检出率为54%,在这41耳中,轻度听力下降2耳、中度听力下降1耳,重度听力下降5 耳,极重度听力下降33 耳. 内耳其他畸形组未检测到负相波.2组差异有统计学意义(P<0.05).结论:在 LVAS与内耳畸形的ABR特性分析中,LVAS无论是否伴其他内耳畸形,无论听力损失的程度如何,均有可能检测出负相波,认为ASNR是大前庭水管综合征临床听力学特征性表现之一.     

大前庭导水管综合征9例临床分析

目的: 探讨大前庭导水管综合征（LVAS）的诊断、听力学表现及治疗。方法:回顾分析我科2000至2005年9例LVAS的病史、听力学及前庭功能检查、影像学检查及治疗结果。结果:9例均为双耳发病，并呈进行性听力下降，8例纯音测听虽以感音神经性聋为主，但低频段存在一定的气骨导差，保守治疗远期效果不理想。结论:对于儿童不明原因的听力下降，首先应排除先天性疾病，合并其他因素造成的听力下降会增加LVAS诊断的难度。对LVAS进行性听力下降者应尽早选配助听器，并考虑行人工耳蜗植入术。     

前庭诱发肌源性电位结合纯音测听在大前庭水管综合征患者的临床应用

目的 通过记录、分析大前庭水管综合征（LVAS）患者的前庭诱发的肌源性电位(VEMP) 和纯音测听（PTA）状况,了解其球囊及前庭下神经功能和纯音听力特征,探讨其在LVAS患者的临床应用及意义。方法 对22例（42耳）LVAS患者行内耳高分辨率CT扫描、纯音测听和VEMP检查,对检查结果结合其临床症状进行总结、分析。结果 双侧短声刺激42患耳中25耳（占59.5%）VEMP表现为高振幅和（或）低阈值;42患耳纯音测听有30耳（占71.4%）表现为低中频（2KHz以下）存在明显气骨导差的混合性聋。VEMP低阈值组,前庭水管内径平均值为（4.30±0.53）mm,纯音听力低中频平均气骨导差值为（36±17）dB HL, 19耳主观听力易受外界原因或发热致颅内压改变而波动;VEMP非低阈值组前庭水管内径平均值为（2.80±0.67）mm,纯音听力低中频平均气骨导差值为（32±15）dB HL,4耳主观听力易受外界原因或发热致颅内压改变而波动。结论 VEMP阈值较低者,其前庭水管平均内径值较大,主观听力也易受外界原因或发热致颅内压增高的改变而波动。而纯音听力损失程度与前庭水管内径平均值无相关性,与VEMP振幅的大小及阈值的高低亦无相关性。     

人工耳蜗在前庭水管扩大患者中的应用

目的　研究前庭水管扩大患者人工耳蜗植入手术的经验。方法　对 15例 (15耳 )双侧前庭水管扩大伴重度感音神经性聋的患者行多导人工耳蜗植入术 ,并对术后结果进行随访。经面隐窝径路行人工耳蜗植入术 ,在圆窗龛前下方行鼓岬钻孔 ,植入多导人工耳蜗电极。结果　①术中电极完全插入耳蜗 ,其中 6耳出现轻微井喷 ;②术后无面瘫、脑脊液耳漏等并发症 ;③所有患者人工耳蜗植入术后都有听觉 ;④术后听阈与耳蜗结构正常植入患者无明显区别 (自由声场平均听阈 30dBHL) ;⑤术后语训在北京同仁医院或中国聋儿康复中心进行 ,听力及言语能力均有不同程度提高 ,其中 2例患者已经进入正常学校就读。结论　前庭水管扩大伴重度感音神经性聋的人工耳蜗植入与耳蜗结构正常患者植入术后效果基本一致 ,人工耳蜗植入可以作为前庭水管扩大伴有重度感音神经性聋的治疗和康复手段     

大前庭水管综合征临床特征分析(附10例报告)

目的分析大前庭水管综合征(1arge vestibular aquduct syndrome,LVAS)临床特征.方法分析10例(20耳)临床资料,主要分析其诱因、临床表现、听力学检查、CT及MRI资料.结果10例均双侧发病.病前有感冒史1例、头部外伤史1例;重度感音神经性聋9例,有眩晕发作2例;突发性加重聋4例、经治疗后听力恢复到发病前状况2例;1例患者(发病时有眩晕发作,平衡功能检查在治疗后第11天进行)行平衡功能检查,结果为重心动摇图睁眼、闭眼呈中间型,双侧视动反应正常,双侧冰水试验无反应;所有患者DPOAE均未引出;所有患者HRCT显示大前庭水管3.0-6.5mm(4.3±1.1mm);行颞骨MRI检查1例,T2相前庭水管及内淋巴囊扩大.结论目前LVAS诊断依赖颞骨CT或MRI,保守治疗疗效不明确,早期诊断早期预防是关键.     

人工耳蜗在前庭水管扩大患者中的应用

目的 研究前庭水管扩大患者人工耳蜗植入手术的经验。方法 对15例(15耳)双侧前庭水管扩大伴重度感音神经性聋的患者行多导人工耳蜗植入术，并对术后结果进行随访。经面隐窝径路行人工耳蜗植入术，在圆窗龛前下方行鼓岬钻孔，植入多导人工耳蜗电极。结果 ①术中电极完全插入耳蜗，其中6耳出现轻微井喷；②术后无面瘫、脑脊液耳漏等并发症；③所有患者人工耳蜗植入术后都有听觉；④术后听阈与耳蜗结构正常植入患者无明显区别(自由声场平均听阈30dBHL)；⑤术后语训在北京同仁医院或中国聋儿康复中心进行，听力及言语能力均有不同程度提高，其中2例患者已经进人正常学校就读。结论 前庭水管扩大伴重度感音神经性聋的人工耳蜗植入与耳蜗结构正常患者植入术后效果基本一致，人工耳蜗植入可以作为前庭水管扩大伴有重度感音神经性聋的治疗和康复手段。     

Clinical analysis of 95 patients with large vestibular aqueduct syndrome]

OBJECTIVE: To investigate the occurrence, genetic inheritance, and hearing loss conditions of large vestibular aqueduct syndrome (LVAS), and to measure the width of external aperture of vestibular aqueduct (VA) of LVAS, and to analyze the relationship between the hearing loss and the width of external aperture. METHODS: A retrospective review was conducted by the history, auditory, and vestibular function examinations in 95 patients (190 ears) of LVAS. The width of VA was measured by CT scanning criterion of each LVAS patients in the past 10 years (from 1992 to 2002). RESULTS: In 95 patients, there were male 65 cases and female 30 cases. The male cases was more than the females by a ratio of 2.2:1. Twelve cases (12.6%) would have familial genetic history. The average of the consult age was 7.6 years (range from 1.2 to 39 years). The most patients were misdiagnosed, the hearing deterioration originated from mild head trauma in 21 cases (22.1%), the hearing loss after common cold in 13 cases (13.6%). Nine cases (9.5%) misdiagnosed as sudden hearing loss, 5 cases (5.3%) as ototoxic hearing loss of Aminoglycoside antibiotic. The profound hearing loss was found in 172 ears (92.4%). The average width of external aperture of LVAS was 7.5 mm +/- 1.2 mm (mean +/- s, 77 cases, 154 ears). The level of hearing loss did not proportional to the width of VA. The hearing impairment could be derived from protein-rich endolymph refluxed into the cochlear and vestibule. CONCLUSION: The LVAS is distinct clinical entity characterized by fluctuate and progressive SNHL. The diagnosis depends upon the CT scan and MR image. If the hearing fluctuated and subsequently improved, the children were kept under conservation treatment. If the significantly decreased of hearing effected school performance, the cochlear implant might be considered.     

大前庭水管综合征与波动性听力损失

目的 分析波动性听力损失在大前庭水管综合征（large vestibular aqueduct syndrome,LVAS）中的特征。方法 总结LVAS13例（23耳）的临床资料，对其波动性听力损失的机理、LVAS诊断及治疗作一讨论。结果 LVAS13例（23耳）中双侧10例，单侧3例，平均年龄7.9岁。多幼年起病，呈缓慢进行性听力减退，波动性突然加重12耳，并伴发作性眩晕3例。6例有提重物、运动、乘飞机、感冒史。3例有遗传性家庭史。纯音听力多呈高频下降型感音神经性聋。眼震电图12耳6耳一侧半规管麻痹。高分辨率CT显示大前庭水管扩大2.5-8mm，有的深达前庭总脚。结论 LAVS多于幼年时出现缓慢进行性听力减退，可伴波动性突发性听力下降、眩晕，常与增加颅内压的活动有关，故应注意预防和避免。     

大前庭导水管综合征的临床特点

目的 分析大前庭导水管综合征的临床特点,为指导医师和家长采取适宜的防范措施提供依据。方法 应用听力学检查(包括纯音测听／儿童游戏测听、声阻抗、听觉脑干诱发电位、耳声发射等)、眼震电图检查和影像学检查(CT和／或MRI检查),对130例(男72例,女58例)进行分析。结果 ①母孕期感冒和出生时有异常者占患病总人数的79％：②有外伤史的占63％：③有3个家族7例发病,占5％;④有耳毒性药物使用史占23．4％：⑤6岁以前首次发病的占83％：⑥占总人数80％的儿童有波动性听力变化,8．5％出现混合性听力下降,69．1％以重度耳聋为首次发病的特点：⑦67％呈现单纯的前庭导水管扩大,且多为双侧畸形：⑧首次发病时间与畸形类型无关：⑨26例可配合眼震电图检查的患者中,稳定期患者半规管功能均对称正常,但在听力下降期进行检查的8例患者中,4例出现了前庭功能异常。结论 尽量避免孕期感染可能有助于减少疾病的出现;预防幼童头部外伤和感冒是减少发病的措施之一：听力波动性变化与前庭功能下降有关联但首次发病时间与畸形类型无关。     

The remediation of hearing deterioration in children with large vestibular aqueduct syndrome

Based on imaging findings, large vestibular aqueduct syndrome (LVAS) in early childhood is the most common cause of sensorineural hearing loss. Children with LVAS are at a high risk of suffering sudden deteriorations in hearing. This study was to review treatment of sudden hearing deterioration in children with LVAS who underwent corticosteroid therapy. We conducted a retrospective study of patients presenting to an academic tertiary medical center. Sixteen children of LVAS were evaluated. Corticosteroid therapy (prednisolone 1-2 mg/(kg day) or equal titer's dexamethasone) was administered as soon as sudden hearing loss developed. The pure tone audiometric result improved more than 10dB at two or more consecutive frequencies and was regarded as a significant response to corticosteroid therapy. Sixteen cases comprising 12 boys and 4 girls were retrospectively analyzed in this study. The mean age at which LVAS was diagnosed was 2.3 years. Mean follow-up for the 16 cases from the first clinic visit to November 2003 was 4.2 years. The initial audiograms varied from down-sloping, valve or rising patterns. In addition, bilateral enlargement of the vestibular aqueduct was found to all children and the mean diameter of right and left ears were 7.23 and 6.83 mm, respectively. Seven children had totally experienced 13 episodes of sudden hearing deterioration. After receiving corticosteroid therapy in time, 11 of 13 episodes had indicated significant responses to treatment, a response rate of 85%. Early detection of LVAS and the timing of treatment are crucial for preventing the residual hearing from deteriorating. As soon as the hearing deterioration of a child with LVAS is recognized, aggressive intervention such as corticosteroid therapy should be performed in no time.     

Chronological changes of hearing in pediatric patients with large vestibular aqueduct syndrome

OBJECTIVES: To describe the chronologic changes of hearing in patients with large vestibular aqueduct syndrome (LVAS) and identify the prognostic factors. STUDY DESIGN: A retrospective chart-review study. METHODS: Twelve consecutive patients with LVAS were recruited at Taipei Veterans General Hospital between July 1986 and July 2000. The records of serial pure-tone audiogram and high-resolution computer tomography were collected. The chronologic figures of pure-tone average (PTA) were sketched. The investigated variables included sex, laterality and type of hearing loss (HL), size of vestibular aqueduct, and the chronologic changes and configurations of hearing. Data were analyzed statistically. RESULTS: The degree of HL in early childhood was from moderate to profound. The worst hearing could be estimated by the hearing level in early childhood. Sixteen of 24 ears were stable. Bilateral, chronologically stepwise-downhill hearing occurred in only one patient (1/12) during the follow-up period. Chronologically, high-tone hearings were worse than those of low-tone, but fluctuations of high-tone hearing were smaller than those of low-tone. The sizes of vestibular aqueduct were predictive of the density of major depression and its depth. CONCLUSIONS: LVAS, a congenital disease, is characterized by fluctuating sensorineural HL. Most hearing at PTA remained stable at least in one ear chronologically. The standard deviation of hearing at 500 Hz was the only prognostic factor for the progression of PTA. An enlarged vestibular aqueduct affects fluctuations of hearing, but the pathogenesis of HL still remains unclear and deserves further investigations.     

Vestibular disturbance in patients with large vestibular aqueduct syndrome (LVAS)

Large vestibular aqueduct syndrome (LVAS) is a common inner ear anomaly responsible for some unusual vestibular and audiological symptoms. The gross appearance of CT scan of the inner ear is generally normal. However, precise measurement of the inner ear components reveals abnormal dimensions, which may account for accompanying auditory or vestibular dysfunction. It has been reported that sudden increase in cerebrospinal fluid pressure can cause further deterioration of hearing due to transmission of pressure to the inner ear through the enlarged vestibular aqueduct. However, vestibular function is not often studied. In this report, audiovestibular function of 10 patients with large vestibular aqueducts was analysed and compared with the severity of the radiological deformity. The literature was reviewed and typical findings were discussed to emphasize varying aspects of audiovestibular function. It was found that some patients with LVAS have some spontaneous or provoked vestibular disturbance such as vertigo after watching revolving objects. The mean value of electronystagmographic abnormality in patients with hearing loss is greater than in patients with normal hearing. However, there is no statistical correlation between the level of hearing loss, electronystagmographic abnormality and severity of radiological deformity.     

Vestibular Disturbance in Patients with Large Vestibular Aqueduct Syndrome (LVAS)

Large vestibular aqueduct syndrome (LVAS) is a common inner ear anomaly responsible for some unusual vestibular and audiological symptoms. The gross appearance of CT scan of the inner ear is generally normal. However, precise measurement of the inner ear components reveals abnormal dimensions, which may account for accompanying auditory or vestibular dysfunction. It has been reported that sudden increase in cerebrospinal fluid pressure can cause further deterioration of hearing due to transmission of pressure to the inner ear through the enlarged vestibular aqueduct. However, vestibular function is not often studied. In this report, audiovestibular function of 10 patients with large vestibular aqueducts was analysed and compared with the severity of the radiological deformity. The literature was reviewed and typical findings were discussed to emphasize varying aspects of audiovestibular function. It was found that some patients with LVAS have some spontaneous or provoked vestibular disturbance such as vertigo after watching revolving objects. The mean value of electronystagmographic abnormality in patients with hearing loss is greater than in patients with normal hearing. However, there is no statistical correlation between the level of hearing loss, electronystagmographic abnormality and severity of radiological deformity.