三种原因不明内耳病抗内耳组织自身抗体的检测

提取、制备豚鼠内耳膜迷路抗原，成功地建立了免疫转印法（ｉｍｍｕｎｏｂｌｏｔｔｉｎｇ），对３３例不明原因内耳病（不明原因进行性感音性聋１０例、突发性聋１５例、Ｍｅｎｉｅｒ病８例）进行分析，发现１０例（３０．３％）患者血清中存在抗内耳组织自身抗体，提示自身免疫反应可能参与部分原因不明内耳病的发病过程。本研究表明：应用免疫转印技术检测抗内耳组织自身抗体，对进一步认识和探讨自身免疫因素在原因不明内耳病的发病机制中的作用有重要价值。     

三种原因不明内耳病抗内耳组织自身抗体的检测

提取，制备豚鼠内耳膜迷路抗原，成功地建立了免疫转印法，对３３例不明原因内耳病（不明原因进行性感音性聋１０例、突发性聋１５例、Ｍｅｎｉｅｒ病８例）进行分析，发现１０例（３０．３％）患者血清中存在抗内耳组织自身抗体，提示自身免疫反应可能参与部分原因不明内耳病的发病过程，本研究表明，应用免疫转印技术检测抗内耳组织自身抗体，对进一步认识和探讨自身免疫因素在原因不明内耳病的发病机制中的作用有重要价值。     

感音神经性聋病人的抗内耳血管抗体测定的研究

目的 :研究某些感音神经性聋患者血清中是否存在抗内耳血管抗体。方法 :通过临床详细询问病史、纯音测听、声导抗测试及耳声发射筛查 ,选择耳蜗性聋患者 4 0 7例为研究对象 ,其中突发性耳聋 35例 ,Ménière′s病 4 1例 ,不明原因耳蜗性耳聋 331例。以豚鼠内耳石蜡切片作为抗原 ,用间接免疫荧光法检测患者血清中的抗内耳血管抗体。结果 :4 0 7例患者中有 6 9例血清中抗内耳血管抗体阳性 ,阳性率为 16 .95 %。其抗体类型有抗毛细血管内皮抗体 ,抗血管壁弹力纤维抗体 ,抗血管壁内膜和外膜抗体等。 6 9例患者中 ,单纯抗血管壁抗体阳性者 31例 ,占 4 4 .93% ,其余 38例患者还同时存在其它抗内耳抗体。在 6 9例患者中临床诊断突发聋者 12例 ,Ménière′s病者 13例 ,不明原因耳蜗性聋者 4 4例 ,占6 3.77%。 6 9例患者纯音测听结果显示低频听力下降者 4 2例 ,平坦型听力下降者 2 4例 ,高频听力下降者 3例。结论 :某些感音神经性聋患者血清中存在各种类型的抗内耳血管抗体。     

突发性聋与血脂及血液流变学变化的关系

突发性聋（突聋）为耳科常见病,其病因和发病机制未明,多数学者认为耳蜗微循环障碍是导致突聋的重要原因之一。本研究对55例突聋患者的病史、血液流变学和血脂进行检测分析,以探讨其与突聋发生的关系。     

突聋患者内耳MRI钆造影与cVEMP的相关性研究

目的通过对单侧突发性耳聋患者进行纯音测听、内耳钆造影MRI检查、cVEMP检查以探讨突聋患者的可能发病机制及在突聋患者中膜迷路积水与cVEMP异常的相关性。方法根据纯音测听结果对65例单侧突发性耳聋患者分为低频组、高频组、平坦组、全聋组,进行内耳钆造影MRI及cVEMP检查,比较各个组间钆造影阳性率和cVEMP阳性率;按照钆造影结果分组,分别计算钆造影阳性组、阴性组的cVEMP阳性率。结果 65例患者中,低频组14人,钆造影阳性率35.7%(5/14),cVEMP阳性率50%(7/14);高频组11人,钆造影阳性率0(0/11),cVEMP阳性率9.1%(1/11);平坦组17人,钆造影阳性率17.6%(3/17),cVEMP阳性率35.3%(6/17);全聋组23人,钆造影阳性率13.0%(3/23),cVEMP阳性率43.5%(10/23);低频组、平坦组、全聋组两两之间的钆造影阳性率无明显差异性;各个频率两两之间cVEMP阳性率未存在完全性明显差异;钆造影阳性共11例,cVEMP阳性共24例。其中钆造影阳性组中cVEMP阳性率63.64%(7/11),钆造影阴性组中cVEMP阳性率31.48%(17/54),二者差值具有统计学意义(P‐0.05)。结论内耳钆造影MRI提示突发性耳聋目前仍是病因不明的疾病,膜迷路积水是低频型突发性聋的一种可能发病机制,而平坦型、全聋型突聋的膜迷路积水可能为内耳损伤后出现的一种病理结果;cVEMP可能在不同频率的突聋类型检出率无明显独特性,在一定程度上可能可以提示突发性聋患者存在膜迷路积水情况。     

力源精纯溶栓酶治疗突发性聋的疗效观察

突发性感音神经性聋（突聋），为较常见的内耳病，其病因和病理迄今尚未阐明，然而导致突聋的任何原因最终不可避免地影响内耳微循环。因此，通过改善内耳微循环来治疗突聋已为众学者所采用。我科于１９９６年６月开始应用力源精纯溶栓酶（降纤酶组）治疗突聋４２例，与４５例应用扩血管药物治疗者（对照组）进行对比，取得更好的疗效。报告如下。１　资料与方法１．ｌ　临床资料 两组均为有完整资料可查的住院患者，经纯音测听、声导抗测试及ＡＢＲ检查证实为感音神经性聋，符合中华医学会上海会议（１９９６年）制定突发性聋诊断标准［１…     

人体抗内耳组织自身抗体的检测方法

相关研究表明某些感音神经性聋患者血清中存在各种类型的抗内耳组织自身抗体。为了对临床上此类疾病的诊断提供客观依据,我们参考了临床上其它学科诊断自身免疫性疾病自身抗体的检测方法,用豚鼠内耳石蜡切片,采用免疫荧光法检测此类耳聋患者血清中的抗内耳组织自身抗体,具体方法如下。     

人体抗内耳组织自身抗体的检测方法

相关研究^[1]表明某些感音神经性聋患者血清中存在各种类型的抗内耳组织自身抗体。为了对临床上此类疾病的诊断提供客观依据，我们参考了临床上其它学科诊断自身免疫性疾病自身抗体的检测方法，用豚鼠内耳石蜡切片，采用免疫荧光法检测此类耳聋患者血清中的抗内耳组织自身抗体，具体方法如下。     

突发性聋与颈静脉球憩室关系初探

为探讨颈静脉憩室（ＪＢＤ）与突发性聋（突聋）的相关性，报告收治突聋１９例中，ＣＴ及磁共振血管造影显示右侧ＪＢＤ３例，共颈静脉孔和乙状窦均明显扩大。其产生内耳功能障碍的机理可能为：①ＪＢＤ压迫与阻塞耳蜗导水管和（或）前庭导水管，甚至内淋巴囊；②ＪＢＤ内的迂回血流对内耳的流体机械力学影响。提示：原因不明的内耳功能障碍病人，应作影像学检查，考虑有无ＪＢＤ的相关影响。     

突发性聋患者血清脂质、脂蛋白及载脂蛋白水平的分析

目的：为了探讨血清脂质代谢与突发性聋（突聋）的关系。方法：检测了２２例突聋患者及２７例对照者的血清甘油三酯、胆固醇、高密度脂蛋白胆固醇、低密度脂蛋白胆固醇、载脂蛋白ＡＩ及载脂蛋白Ｂ１００的含量。结果：经统计分析发现：突聋患者的甘油三酯水平升高（Ｐ＜０．０５），载脂蛋白Ｂ１００降低（Ｐ＜００５），而胆固醇、高密度脂蛋白胆固醇、低密度脂蛋白胆固醇、载脂蛋白ＡＩ与对照组比较差异无显著性（Ｐ＞００５）。结论：本组突聋患者体内存在脂类代谢异常，脂类代谢，尤其甘油三酯代谢异常可能与突聋的发生有关     

Sudden sensorineural hearing loss associated with inner ear anomaly.

OBJECTIVE: This study was conducted to evaluate the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss and in control subjects. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS AND INTERVENTION: We evaluated 366 patients (165 men and 201 women; age range, 3-91 yr) with sudden sensorineural hearing loss and 228 control subjects without sensorineural hearing loss using magnetic resonance imaging. Three hundred fifty-six patients had unilateral and 10 patients had bilateral sudden sensorineural hearing loss. RESULTS: Eleven (2.9%) of 376 ears with sudden sensorineural hearing loss had inner ear anomaly. Nine patients (2.5%) had inner ear anomaly associated with sudden sensorineural hearing loss, but none of the 228 control subjects had the anomaly. The current study demonstrated that the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss was significantly higher than in control subjects. CONCLUSION: Our study reveals that inner ear anomaly may be associated with sudden sensorineural hearing loss in 2.5% of patients.     

Detection of humoral immune response to inner ear proteins in patients with sensorineural hearing loss]

BACKGROUND: The precise mechanism of inner ear disease is still unknown. An autoimmune reaction could be one of several possible pathogenic factors involved in progressive sensorineural hearing loss. Heat shock protein 70 is suggested to play an important role in the development of autoimmune diseases. The aim of this study is the investigation of humoral immune reactivity to inner ear components in patients with sensorineural hearing loss. METHODS: The presence of antibodies to inner ear components was determined by immuno-blotting extracted bovine or human inner ear proteins. Study groups consisted of patients with idiopathic progressive sensorineural hearing loss (group A), patients with Menière's disease (group B), patients with sudden hearing loss (group C), patients with otosclerosis (group D), patients with Cogan's disease (group E), and individuals without hearing problems (group F). RESULTS: 40% of the patients with progressive sensorineural hearing loss showed reactivity against a 68-kDa protein extracted from bovine inner ear. In contrast to this, only 5% of healthy individuals and 10% with Menière's disease showed reactivity against the 68-kDa protein from bovine inner ear or against bovine heat shock protein 70. Some of the patients who showed reactivity against bovine inner ear proteins were tested with human inner ear and human heat shock protein 70; all of these showed reactivity. Approximately 6% of the patients with sudden hearing loss (group C), otosclerosis (group D), and Cogan's disease (group E) showed reactivity to inner ear proteins. A non-specific humoral immune reaction against inner ear proteins with molecular weights of 30, 40, 50, 60, and 220 kDa was observed in all patients. DISCUSSION: These results indicate a humoral immune reactivity against heat shock protein 70, which might be responsible for the pathogenesis of progressive sensorineural hearing loss.     

Clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss

OBJECTIVES/HYPOTHESIS: The role of antiendothelial cell antibodies in systemic vasculitis has been reported. The aim of the study was to define the clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss. STUDY DESIGN: A prospective study in patients with sudden sensorineural hearing loss. METHODS: Serum samples were taken from 59 consecutive patients with sudden sensorineural hearing loss at time of presentation and from 28 normal control subjects. Indirect immunofluorescence assay was used to detect antiendothelial cell antibodies. RESULTS: The prevalence of antiendothelial cell antibody detection was 54% (32 of 59 patients), with a statistically significant difference between patients and control subjects (P =.0004). Antiendothelial cell antibody positivity was significantly associated with absent recovery of hearing loss (P =.0020). CONCLUSIONS: The cytotoxicity to endothelial cells of the inner ear by antiendothelial cell antibody-positive sera might play a role in causing the stria vascularis damage in immune-mediated sudden sensorineural deafness. The appearance of antiendothelial cell antibody is related to the poor outcome of hearing loss, and its detection could be helpful in the selection of particular patients with sensorineural hearing loss for specific immunosuppressive treatments.     

Validity of the Western blot immunoassay for heat shock protein-70 in associated and isolated immunorelated inner ear disease

OBJECTIVE: To assess the validity of the Western blot immunoassay for heat shock protein-70 (hsp-70) for diagnosis of autoimmune inner ear disease. STUDY DESIGN: Retrospective study of 53 patients affected by sudden deafness (n = 19), idiopathic progressive sensorineural hearing loss (n = 24), and Meniere's disease (n = 10) who were treated from 1995 to 1999. The clinical course and response to corticosteroid were evaluated. METHODS: A purified hsp-70 antigen from bovine kidney cell line was used for the Western blot immunoassay. RESULTS: Only five patients (9.4%) showed anti--hsp-70 antibodies: Two presented a sudden sensorineural hearing loss (sudden deafness group), two showed an idiopathic progressive sensorineural hearing loss (idiopathic progressive sensorineural hearing loss group), and one was affected by fluctuating hearing loss (Meniere's disease group). A systemic autoimmune condition was observed in 29.1% of patients with idiopathic progressive sensorineural hearing loss. CONCLUSIONS: The low sensitivity of Western blot immunoassay for patients affected by idiopathic progressive sensorineural hearing loss and Meniere's disease may result from either the long time elapsed from the hearing loss and vertigo to the initial examination or from the increased percentage of cases of systemic autoimmune disease present in patients with idiopathic progressive sensorineural hearing loss. More studies to detect the immune-mediated inner ear disease in Western blot immunoassay-negative patients are required.     

Acute hearing loss in patients with hematological disorders

Objective: This study adopted an inner ear test battery to investigate the causes of acute sensorineural hearing loss in patients with hematological disorders. Methods: During the past 20 years, the authors have experienced 14 patients with hematological disorders, i.e. leukemia or aplastic anemia, having acute sensorineural hearing loss. An inner ear test battery comprising audiometry and cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP (oVEMP), and caloric tests was performed. Results: Diagnoses comprised of sudden sensorineural hearing loss in 12 patients and endolymphatic hydrops in four patients (two patients had one ear with sudden sensorineural hearing loss while the other ear had endolymphatic hydrops). Percentages of recruitment phenomenon showed a significant difference between endolymphatic hydrops and sudden sensorineural hearing loss. Abnormal percentages for mean hearing level (86%), cVEMP test (71%), oVEMP test (25%), and caloric test (14%) exhibited a significant sequential decline in these patients. Conclusion: Acute sensorineural hearing loss in a patient with leukemia or aplastic anemia may be related to either sudden sensorineural hearing loss or endolymphatic hydrops. A significant sequential decline in the function of the cochlea, saccule, utricle, and semicircular canals indicates that the pars inferior is more vulnerable to blood insult than the pars superior.     

Binding of serum immunoglobulins to human inner ear tissue in inner ear hearing loss: methodologic limits

The immunopathological processes possibly involved in cryptogenic sensorineural hearing loss were investigated. In a pilot study the sera from 66 patients were tested by the indirect immunofluorescence technique for immunoglobulins which bind to normal human inner ear tissue. The reaction was positive in two thirds of all patients with sudden hearing loss, especially bilateral. This was mostly for IgG, and to the organ of Corti. Others have found that Corti's organ showed marked degenerative changes in cases of sudden hearing loss, examined by histology. The reaction was positive in half of the patients with progressive sensorineural hearing loss. It was noticeable that positive reactions for IgA were twice as frequent in sera of patients with progressive hearing loss than in those with sudden deafness. In a control study, the sera of ten patients were tested in parallel by the same method on histological sections of inner ears taken from four individuals. Similar results were found for the sera of two patients only. The sera of the remaining eight patients revealed very inconsistent reaction patterns. These preliminary results may indicate that temporary or permanent humoral (auto) immune mechanisms could have occurred in certain inner ear diseases. So far however reliable diagnostic methods have not been established, and not enough patients have been followed up.     

Ear involvement in Wegener's granulomatosis

In 16 of 19 patients with biopsy-proved Wegener's granulomatosis the early manifestations were limited to the ear and nose. The audiological data of 13 patients revealed middle ear involvement in 16 of 26 ears. Twenty-one of 26 ears presented with a low to moderate sensorineural hearing loss. One ear remained deaf after a sudden hearing loss in the early stage of the disease. Serologically, 4 of 6 tested patients with sensorineural hearing loss demonstrated antibodies against sarcolemma. One patient showed antinuclear antibodies. It is remarkable that these antibodies can often be detected in classic inner ear disorders. The course of inner ear function, serum findings and the success of immunosuppressive therapy in Wegener's granulomatosis are comparable with immunologically mediated vasculitis in the inner ear.     

Magnetic resonance imaging-detected inner ear hemorrhage as a potential cause of sudden sensorineural hearing loss

Purpose

The aim of this study is to assess the value of magnetic resonance imaging in identifying the etiology of sudden sensorineural hearing loss, and to correlate the high signals in the labyrinth with clinical features to identify if inner ear hemorrhage could be implicated.

Materials and methods

In this retrospective study, inner ear magnetic resonance imaging was given to 112 patients with sudden sensorineural hearing loss in the First Affiliated Hospital of Sun Yat-sen University from 2011 to 2012. The clinical features of patients with high signals in the labyrinth on magnetic resonance imaging were analyzed.

Results

Abnormal magnetic resonance images were identified in 13 (11.6%) patients. Retrocochlear pathology was found in six patients, including two cases of lacunar infarction, one case of multiple ischemias in the brainstem and bilateral centrum semiovale, two cases of acoustic neuroma, and one case of inner ear hemangioma. There were seven cases showing high signals in the labyrinth on unenhanced T1-weighted and fluid-attenuated inversion recovery images. Clinical features of these seven patients were characterized by irreversible profound hearing impairment and vestibular dysfunction. These findings were consistent with the hypothesis that their symptoms were caused by an inner ear hemorrhage.

Conclusion

The results indicate the importance of magnetic resonance imaging in sudden sensorineural hearing loss in patients. Moreover, patients with vestibular dysfunction and sudden profound hearing loss may have an inner ear hemorrhage evident by interpreting clinical and magnetic resonance imaging results.     

Otosyphilis mimics immune disorders of the inner ear

Syphilis is a well established cause of hearing loss. Sensorineural hearing loss may develop in the congenital or acquired form. The clinical course of the early acquired and late congenital forms are similar: sudden or rapidly progressive bilateral sensorineural hearing loss with mild vestibular symptoms. Cochleovestibular involvement in early acquired syphilis has been related to a basilar meningitis with lymphocytic infiltration of the labyrinth and VIIIth nerve. However, neurosyphilis and inner ear syphilis are not the same disease. Prompt diagnosis and treatment with corticosteroids and penicillin are mandatory to reduce the immune response and fibrosis of the labyrinth and the endolymphatic sac. Unfortunately, early acquired syphilis is frequently overlooked in the differential diagnosis of other forms of sensorineural hearing loss, particularly autoimmune inner ear disease. Given the increasing number of luetic infection cases, especially in immunocompromised patients, this condition should be considered in any sexually active patients affected by sudden hearing loss. Cases of inner ear syphilis are presented. Immunopathology of luetic inner ear infection is discussed and compared with immune disorders of the inner ear.     

Otosyphilis mimics immune disorders of the inner ear

Abstract

Syphilis is a well established cause of hearing loss. Sensorineural hearing loss may develop in the congenital or acquired form. The clinical course of the early acquired and late congenital forms are similar: sudden or rapidly progressive bilateral sensorineural hearing loss with mild vestibular symptoms. Cochleovestibular involvement in early acquired syphilis has been related to a basilar meningitis with lymphocytic infiltration of the labyrinth and VIIIth nerve. However, neurosyphilis and inner ear syphilis are not the same disease. Prompt diagnosis and treatment with corticosteroids and penicillin are mandatory to reduce the immune response and fibrosis of the labyrinth and the endolymphatic sac. Unfortunately, early acquired syphilis is frequently overlooked in the differential diagnosis of other forms of sensorineural hearing loss, particularly autoimmune inner ear disease. Given the increasing number of luetic infection cases, especially in immunocompromised patients, this condition should be considered in any sexually active patients affected by sudden hearing loss. Cases of inner ear syphilis are presented. Immunopathology of luetic inner ear infection is discussed and compared with immune disorders of the inner ear.