豚鼠环磷酰胺预处理后再免疫对其ABR阈值的影响

目的建制一高阳性率、可供圆窗给药治疗内耳病研究用的自身免疫性内耳病动物模型。方法采用270—370g重的白色红目豚鼠97只作为实验对象，其中32只用于制备粗制内耳抗原，47只经环磷酰胺腹腔注射预处理2d后，再用粗制内耳抗原行皮内多点接种。动物于接种后4、6、8、10、12、14、20d(分别为6、7、7、6、9,6、6只)接受听性脑干反应(ABR)检测。正常对照18只，组1(12只动物)不行任何处理，组2(6只动物)仅行环磷酰胺预处理，不行粗制内耳抗原接种。结果实验组动物接种后4d时ABR阈值升高10dB以上者为67％，8d时为86％．14d时仍为58％，接种后20d时所有动物ABR阈值恢复正常。结论将豚鼠用环磷酰胺预处理后，用粗制同种异体内耳抗原只需单次接种即可建立听力损害发生率高的自身免疫性内耳病动物模型。     

自身免疫性感音神经性聋

自身免疫性感音神经性聋(autoimmune sensorineural hearing loss,ASNHL)是侵犯耳蜗[1、2]及蜗后[3]的自身免疫性疾病,这一概念由美国学者McCabe在1979年     

自身免疫性感音神经性聋

1979年McCabe报告18例自身免疫性感音神经性聋(autoimmune sensorineural hearing loss，ASNHL)，首次论述ASNHL是一种临床疾病实体，是少数几种感音神经性聋(SNHL)中唯一能够有效治愈的。本文就有关ASNHL的内耳免疫学、临床表现、诊断及治疗方面作一概述，重点在临床，以提高对ASNHL的基本认识。     

同种异体内耳组织免疫后豚鼠听功能与内耳形态学变化的关系

目的 观察豚鼠接受环磷酰胺处理及免疫后其听功能与内耳形态学变化的关系。方法 采用86只白色红目豚鼠作为实验对象，其中32只动物提供粗制内耳抗原。实验组（42只）动物于腹腔注射环磷酰胺2d后用粗制内耳抗原接种，接种后4．6、8、10、12、14、20d时接受ABR检测及内耳形态光镜观察。对照组1（6只）不行任何处理，对照组2（6只）注射环磷酰胺，但不接种抗原。结果 接种后4d时67％动物ABR阈值明显提高，8d时为83％，14d时降为58％，20d时所有动物阈值恢复正常。光镜观察发现：接种后4d时，实验动物听功能受损耳出现炎性细胞浸润；8—12d时，所有实验动物内耳均有类似改变；6—14d时，内耳尚有血管周围炎、螺旋神经节细胞变性等改变，相关形态变化均以听功能受损耳明显为重；接种14d时，内耳炎性细胞浸润明显减轻，20d时，绝大多数动物内耳形态基本恢复正常。结论 豚鼠接受环磷酰胺预处理及同种异体内耳抗原接种后，其听功能与内耳形态学的变化基本相一致。     

自身免疫性感音神经性聋的实验研究

以豚鼠内耳组织作为抗原免疫豚鼠，观察免疫前及免疫后不同时期豚鼠听阈几项免疫学参数和内耳病理组织结构的变化。结果发现：免疫后豚鼠内耳组织病理学光镜及扫描电镜观察出现了一些改变，听阈显著提高，Ｔ淋巴细胞转化率显著低于正常，循环免疫复合物显著升高。并对实验结果进行了讨论。     

自身免疫性感音神经性聋的实验研究

以豚鼠内耳组织作为抗原免疫豚鼠、观察免疫前及免疫后不同时期豚鼠听阈几项免疫学参数和内耳病理组织结构的变化，结果发现：免疫后豚鼠内耳组织病理学光镜及扫描电镜观察出现了一些改变，听阈显著提高，Ｔ淋巴细胞转化率显著低于正常，循环免疫复合物显著升高。并对实验结果进行了讨论。     

实验性自身免疫性感音神经性聋的自然演变

应用粗制内耳抗原（CIEAg)与等量福氏佐剂（CFA；IFA）皮下注射免疫豚鼠4次，复制动物模型，模型动物经过9周自然病程演变后。结果：（1）实验动物内耳病变基本消失，平均反应阈（ABR）与对照组平均反应阈差异无显著性。（2）实验性内耳鼻身免疫性损害多导致可逆性听力障碍也可引起难以恢复的听力损害，并对可能机制进行了探讨。     

自身免疫性感音神经性聋研究的回顾分析

目的复习我国近十年来有关自身免疫性感音神经性聋的实验和临床文章。方法检索中国生物医学文献数据库和中国期刊网医学专题全文数据库,以“自身免疫病”、“感音神经性聋”、“抗体”为关键词检索1993~2004年发表的文章。对所检索出的文章从以下几个方面进行分析研究对象的选择、样本含量、对照设立、实验方法和结果、听力学检测结果、治疗方法及疗效等。结果共检出文章27篇,其中实验研究11篇,临床研究7篇,综述7篇,病例报告2篇。样本临床研究文章中,病例数最少14例,最多71例。有2篇文章无对照设立。听力图可分为低频型、高频型、平坦型和钟型。ABR有异常,耳蜗电图有-SP。热休克蛋白阳性率为51%,68KD蛋白阳性率为72%,抗内耳病抗体(IgG)阳性率为30%,抗肺炎克雷白杆菌膜蛋白抗体阳性率为70%。所有患者均采用激素(强地松或地塞米松)治疗,有效率无明确统计。在11篇实验文章中,采用动物造模法、听生理监测法和病理形态学法(生物化学和免疫组织化学),其结果各不相同。结论目前在我国动物造模是否反映自身免疫性感音神经性聋的真实的临床病变尚需更深入研究;诊断标准也要进一步规范化;内耳免疫学检查方法要进一步改进;治疗方法要统一和标准化,且要进一步加强和深入临床研究。     

自身免疫性感音神经性聋

自身免疫性感音神经性聋顾之燕，顾瑞１９９４年９月在河北省承德市召开了全国自身免疫性内耳病专题学术研讨会，会议达到普及与提高相结合的目的，开得很成功。北京、武汉、广州、上海、河南等地的耳鼻咽喉科工作者作了学术报告。但由于信息交流情况了解不够，可能还有一...     

迷路内积血致感音神经性聋的实验研究

目的观察迷路内积血病变特征,为其导致感音神经性聋的病因学理论提供实验基础。方法采用豚鼠自体抗凝微量血液行鼓阶内注射,分别于术前和术后不同时间测试听神经复合动作电位、耳蜗微音电位、颞骨MRI扫描、耳蜗火棉胶切片光镜观察、内耳超薄切片和透射电镜观察。结果鼓阶内灌注不同量血液的2个实验组术后不同时间均出现感音神经性聋,以术后第7天较为严重;听力损失特点表现为低频(0.5kHz)最为显著,中频(2kHz)次之,高频(8kHz)较轻;术后1个月时听功能无明显好转。两实验组间听力损伤程度和类型于术后各时间均无显著性差异。MRI T1加权可显示迷路内积血的高信号,且迷路内的积血在术后不同时间段有不同的形态学特征:由最初的积血(术后第1天)演变成积血与纤维网状样结构共存(术后第7天),到最后(术后30天)仅存纤维网状样结构。透射电镜显示部分动物的外毛细胞内线粒体呈水肿或空泡样变,胞浆稀疏,纤毛倒伏。结论迷路内积血可引起豚鼠感音神经性聋,听力损失以低频区更为严重。不同积血量引起的听力损失程度和类型无显著差异。MRI可良好显示内耳积血不同病程阶段在迷路内的分布与变化。迷路内积血最终可被吸收。     

自身免疫性感音神经性听力减退的测听结果

观察分析７１例（１３０耳）临床拟诊为自身免疫性感音神经性听力减退耳的听力学检查结果。听力图为低频减退者５５耳、平坦型者２６耳、钟型者１７耳、高频减退者３２耳。综合瞬态诱发耳声发射（ＴＥＯＡＥ）、听性脑干反应（ＡＢＲ）和耳蜗电图的－ＳＰ／ＡＰ振幅比的结果分析，这组患耳中低频减退型多为蜗后病变所致，且以３０岁以下者为多见；其余３型多为耳蜗病变所致，且多为３１岁以上者。鉴于约１／３患者以蜗后病变为主，称本病为自身免疫性内耳病似不恰当，值得商榷。     

自身免疫性感音神经性聋体液与细胞转移免疫研究

为探讨体液和细胞转移免疫是否能造成自身免疫性感音神经性聋(autoimmune sensorineuralhearing loss.ASHL),采取ASHL模型动物的血清和T淋巴细胞进行转移免疫,观察被动免疫动物听觉功能和内耳病理形态学变化,发现体液和细胞转移免疫后,部分被动免疫动物出现不同程度的听觉损伤和内耳病理改变,两组听力损失的发生率分别为16.67%和29.17%,依据Witskey法则,进一步证实ASHL是一种客观存在的自身免疫性疾病,体液性和细胞毒性变态反应在ASHL是一种客观存在的自身免疫性疾病.体液性和细胞毒性变态反应在ASHL发病中可能均起着重要作用.     

Autoimmune Sensorineural Hearing Loss in Young Patients: An Exploratory Study

Objectives P0 protein is expressed exclusively in myelinating Schwann cells of the peripheral nervous system. In a previous study from our laboratory, 27% of patients with sensorineural hearing loss (SNHL) had antibodies to P0 protein in their serum. The purpose of the present exploratory study was to examine the relationship between the clinical presentation of SNHL among children and young adults (age range, 5–30 y) and the presence of serum anti‐P0 antibodies. Study Design The data were collected by retrospective questionnaires from Belgian otolaryngologists. Methods Patients were divided for comparison into two groups according to the presence or absence of anti‐P0 antibodies. Results Analyses of clinical data and audiometric results indicated that a progressive hearing loss was more frequently recorded in the patients in the anti‐P0 antibody–positive group (82% [14 of 17]) than in those in the anti‐P0 antibody–negative group (35% [6 of 17]) (P <.005). Conclusions Thus, in the age group in the present study, autoimmune SNHL (as measured in the present study by the presence of anti‐P0 antibodies) is more frequently associated with progressive than with sudden hearing loss. The implications of this finding for preventive screening of hearing loss in children and young adults are discussed.     

葛根素对噪声性聋的防治作用

目的探讨葛根素对噪声性聋的预防及治疗作用。方法 18只白化雄性豚鼠随机分为三组,每组6只,单纯噪声组每日腹腔注射生理盐水2 ml,噪声+葛根素组每日腹腔注射葛根素150 mg/kg,空白对照组每日腹腔注射生理盐水2 ml。三组动物均持续给药10日。单纯噪声组和葛根素组于给药第3日给予噪声刺激,噪声模式采用4 kHz纯音,强度128 dB SPL,时间6小时。并于实验第1、4、7、10日分别对各组豚鼠进行ABR检测。10日后随机抽取各组豚鼠进行耳蜗基底膜铺片、免疫组化染色、硝基自由基含量测定。结果三组豚鼠噪声刺激前ABR阈值无明显差异(P>0.05);实验第4、7、10日时,噪声+葛根素组ABR反应阈较单纯噪声组显著降低(P<0.01),但比空白对照组明显升高(P<0.01)。耳蜗基底膜铺片结果显示,单纯噪声组外毛细胞缺失率约72.65%,噪声+葛根素组外毛细胞缺失率约21.48%,空白对照组外毛细胞无缺失。免疫组化染色的结果显示,噪声+葛根素组的硝基自由基表达较单纯噪声组弱,但比空白对照组强。结论葛根素对噪声性聋有一定的防治作用,可以减少听力下降的幅度,降低毛细胞损伤的比例,但不能完全逆转噪声所致的听力下降。     

Epidemiology of Sensorineural Hearing Loss

The paper is based on the author's epidemiological studies of sensorineural hearing loss in Ghana, Jamaica, Nigeria and the United Kingdom. Basically, the method has been one of direct examination of random or total samples. Such a study is more difficult than a study of conductive hearing loss because there is no qualitative difference between sensorineural hearing loss or of normal hearing. These epidemiological, studies therefore, resolve themselves into measuring the hearing levels of ears which have been otologically screened to exclude conductive hearing losses, calculating values for 'average' hearing levels and their dispersion and determining factors which appear to influence these levels.

Whereas in European and North American populations, noise and 'ageing' appear to be the only factors which influence these average values, in other populations, other factors emerge. In particular, there are dietary factors and the influence of systemic disorders becomes apparent.

The evidence from epidemiology is that sensorineural hearing loss can be considered to be not a collection of distinct disorders but one disorder with a multifactorial aetiology where causative factors may or may not interact with one another.     

Informational Masking in Listeners with Sensorineural Hearing Loss

Measures of energetic and informational masking were obtained from 46 listeners with sensorineural hearing loss. The task was to detect the presence of a sequence of eight contiguous 60-ms bursts of a pure tone embedded in masker bursts that were played synchronously with the signal. The masker was either a sequence of Gaussian noise bursts (energetic masker) or a sequence of random-frequency 2-tone bursts (informational masker). The 2-tone maskers were of two types: one type that normally tends to produce large amounts of informational masking and a second type that normally tends to produce very little informational masking. The two informational maskers are called "multiple-bursts same" (MBS), because the same frequency components are present in each burst of a sequence, and "multiple-bursts different" (MBD), because different frequency components are presented in each burst of a sequence. The difference in masking observed for these two maskers is thought to occur because the signal perceptually segregates from the masker in the MBD condition but fuses with the masker in MBS. In the present study, the effectiveness of the MBD masker, measured as the signal-to-masker ratio at masked threshold, increased with increasing hearing loss. In contrast, the signal-to-masker ratio at masked threshold for the MBS masker changed much less as a function of hearing loss. These results suggest that sensorineural hearing loss interferes with the ability of the listener to perceptually segregate individual components of complex sounds. The results from the energetic masking condition, which included critical ratio estimates for all listeners and auditory filter characteristics for a subset of the listeners, indicated that increasing hearing loss also reduced frequency selectivity at the signal frequency. Overall, these results suggest that the increased susceptibility to masking observed in listeners with sensorineural hearing loss is a consequence of both peripheral and central processes.     

Sudden Bilateral Sensorineural Hearing Loss

Most cases of sudden sensorineural hearing loss (SHL) remain idiopathic, and the majority are unilateral. From 1989 to 1993, 823 patients with sudden SHL were evaluated. Of these, 14 (1.7%) had sudden bilateral SHL. We reviewed the charts of these 14 patients to compare sudden bilateral SHL with sudden unilateral SHL. Usually, bilateral SHL was asymmetric. Most bilateral cases received combined steroid and vasodilator treatment, while unilateral cases were more likely to receive only one of these treatments. By audiological criteria, 67% of bilateral SHL cases improved, while the improvement rate in unilateral SHL was 52%; however, this difference was not statistically significant. In bilateral SHL patients showing improvement, both ears responded. Bilateral SHL patients were older at the onset of hearing loss, had a higher incidence of vascular disease, and were more likely to have positive antinuclear antibody titer. Recognition of similarities and differences between sudden unilateral and bilateral SHL can help in counseling patients.     

隐性遗传性感音神经性聋

报告两个家系隐性遗传性感音神经性聋。一家系三代４人发病，均为男性，系为Ｘ连锁隐性遗传性感音性耳聋；另一家系三代３人发病，为常染色体隐性遗传。此两种类型隐性遗传性聋的家系，临床上较为罕见，为此对其发病特点及其诊断进行了讨论。