【作者】 马文；

【导师】 李海燕；

【作者基本信息】 山东大学 ， 耳鼻咽喉科学， 2010， 硕士

【摘要】 第一部分：大前庭导水管综合征HRCT影像特点目的探讨高分辨CT (high resolution CT, HRCT)不同扫描方式对婴幼儿大前庭导水管综合症(large vestibular aqueduct syndrome, LVAS)的诊断价值,寻求对大前庭导水管综合症的最佳诊断方法。资料与方法对86例感音神经性聋患者行HRCT横断位扫描,对原始数据行冠状位、矢状位及斜矢状位的多平面重组,所得图像由2位影像学医师及1位耳科医师分别进行诊断,比较其漏诊、误诊、正确率并比较作出诊断所需观察图像序列数,选出最佳诊断影像；诊断结束后,对该86例患者分成非LVAS和LVAS两组,随机抽取非LVAS 20例(40耳)作为对照组,确诊的LVAS作为病变组对所有HRCT原始资料进行测量,测量数据包括：①外半规管前、后脚壶腹连线L1长度(X)；②于直线L1中点作垂直线L2,测量L2介于骨岛和前庭腔内侧壁的长度(Y)；③外半规管前、后脚壶腹连线(L1)与前脚壶腹部至前庭骨岛切线夹角(α)和后脚壶腹部至前庭骨岛切线夹角(β)大小并进行比较。结果三位医师诊断LVA轴位分别为26耳(4耳为误诊)、24耳(2耳为误诊)、24耳；冠状位分别为18耳、18耳、20耳；矢状位分别为21耳(1耳误诊)、24耳(1耳误诊)、19耳；斜矢状位分别为26耳、26耳、26耳。轴位所需图像序列数平均为6个,冠状位平均为9个,矢状位为4个,斜矢状位为1.5个；对照组：X长度为6.22mm(6.22±0.55mm),Y平均长度为3.21mm(3.21±0.3mm),α+β之和平均31(31±5)度；LVAS组X长度为6.28mm(6.28±0.55mm),Y长度为4.24±0.24mm(3.94～5.25mm),α+β之和61±6度(55～87度),,采用U检验,对照组、病变组X长度差异无统计学意义(p＞0.05),对照组、病变组比较Y长度差异具有统计学意义(p＜0.05)；α+β角之和两组比较差异具有统计学意义(p＜0.05)。结论HRCT轴位螺旋扫描,斜矢状位多平面重组为诊断LVAS的最佳图像。误诊、漏诊率低,所需图像序列最少,诊断正确率高,可以提高感音神经性耳聋患者的病因检出率；Y长大于3.92mm可作为诊断LVAS的标准,前庭α+β角之和大于55度合并前庭导水管扩大可作为诊断LVAS的又一标准。第二部分：婴幼儿大前庭导水管综合征ASSR和ABR听力学特点目的利用听觉稳态诱发反应(auditory steady-state response, ASSR)与听性脑干反应(auditory brainstem response, ABR)对大前庭水管综合征(largevestibular aqueduct syndrome, LVAS)患儿进行听力测试,探讨其ASSR和ABR的听力学特点。资料与方法对63例(121耳)确诊为LVAS和同期来我院就诊的20例非LVAS感音神经性聋患儿的听力学资料进行ASSR和ABR测试,分别记录双耳ASSR 0.5kHz、1kHz、2kHz、4kHz的反应阈值,列表并进行统计学分析,观察其变化趋势,同时记录ABR引出V波最小刺激强度,与同侧耳ASSR反应阈值进行比较,观察其相关性。结果LVAS组：ASSR在0.5～4 kHz反应阈值呈上升趋势,直至4 kHz反应阈消失,近成直线关系,随低频听阈值的升高,斜率值减小：给出最大刺激频率中,1kHz反应阈最后消失;其中59例(118耳)ASSR 0.25-4KHZ阈值呈上升趋势,直至4kHZ反应阈消失,呈斜坡行曲线,且随低频阈值的升高,斜率值减小；3例(5耳)0.25-4 KHZ反应阈近呈平坦型曲线,平均听阈均小于60dBHL；8例(16耳)ASSR 0.25-4 KHZ反应阈值呈“八”型,反应阈低值为1或2kHZ,至4kHZ反应阈再次升高；在所有给出最大刺激频率中,1kHZ反应阈最后消失,仅有5例(8耳)8 kHZ在刺激强度小于92 dBHL引出反应,提示所有频率中最先消失的为8 kHZ,其次为4 kHZ。非LVAS组,0.25-4kHZ 36耳听力曲线呈平坦型,2耳为”W”型,1耳为陡降型,1耳为上升型。当ASSR听阈介于50～80 dBHL时,若ABR以dBnHL为单位,其对应的反应阈数值小于ASSR听阈数值当ASSR听阈＞100 dBHL时,ABR 109.6 dBnHL已不能引出V波：84耳ABR最大声刺激未引出v波,其对应ASSR仍可全部或部分频率引出反应阈。非LVAS组：0.5～4 KHz阈值曲线多为平坦型,2～4 kHz均值与ABR V波反应阈相近。结论大前庭水管综合征患者ASSR 0.5～4 kHz各频率反应阈值呈升高趋势直至消失,在所有频率中,最先受累者为4kHz；其数值与ABR反应阈数值大小具有阶段性改变,其改变区间在80～100 dBHL。更多还原

【Abstract】 Part One:CT imaging features in large vestibular aqueduct syndrome in infantsObjectives To evaluate the role of multiplanar reconstruction oblique images,and find a best scan to diagnose the large vestibular aqueduct. Methods High-resolution CT scanning was performed in 86 patients with sensorineural hearing loss. The original data was processed, and the MPR images were respectively observed by two radiology doctors and one otolaryngology doctor, comparing the numbers of scans of designators. Results Three doctors reached the conclusion of LVA were respectively 26、24、24 ears in the axial’s,18、18、20 in the coronal’s scan and 26、26、26 ears in the multiplanar reconstruction oblique images.While 4 ears were misdiagnoses of 26 ears and 2 of 24 ears in the axial.12 and 18 scan of designators average required respectively in the axial and coronal. Only 4 scan of designators were required in the oblique images. Conclusion Multiplanar reconstruction oblique images is the best method in diagnosis of LVA which has a high corresponding ratio and can also elevate the etiologic diagnosis ratio of sensorineural hearing loss in the least scan of designators. Part Two:Audiological features of auditory steady-state response and auditory brain stem response in large vestibular aqueduct syndrome in infantsObjectives To explore characteristics of auditory steady-state response (ASSR) and auditory brainstem response (ABR) in patientswith large vestibular aqueduct syndrome (LVAS). Methods Characteristics of ASSR and ABR of 63 patients (121 ears) with LVAS and 20 patients (40ears) of SHL without LVAS were recorded and analyzed. Main Results In the LVAS group,ASSR raised in a nearly straight-line from 0.5 to 4 kHz thresholds until the threshold of response 4 kHz, with an increase of low-frequency hearing threshold, the value of the slope was reduced. When the biggest boost in frequency was given, response threshold at 1kHz finally disappeared. When stimulated by the maximal sound of ABR,84 ears could not draw forth the V wave, whose corresponding ASSR could draw forth the response threshold in all or part frequencies. In the other group, ASSR from 0.5 to 4kHz thresholds rose mostly in a flat type. The average value of ASSR from 2 to 4 kHz thresholdswas similar to that of wave V of ABR. Conclusions ASSR of LVAS from 0.5 to 4 kHz thresholds rose in a nearly straight-line relationship until the threshold of response disappeared. The correlation was obvious with an increase of frequency. ASSR and ABR thresholds were significantly correlated and the ASSR thresholds had a stage change with the change interval between 80 and 100 dBHL.The prompt and proper treatments are strongly suggested in the patients with fluctuant hearing loss for their hearing benefit.更多还原