人工耳蜗植入术前的影像学检查

目的 :探讨CT和MRI检查对人工耳蜗植入术前评估的价值。方法 :对 2 6例感音性耳聋患者均采用颞骨轴位高分辨率CT螺旋扫描、MR水成像及内耳三维重建。结果 :1 8例先天性感音性耳聋患儿中检出Mondini畸形Ⅰ型 1例 (2耳 ) ,Ⅱ型 1例 (2耳 ) ,内耳纤维化 1例 (2耳 ) ;5例语后聋患者中检出慢性化脓性中耳炎 1例 (2耳 ) ,内耳骨化 1例 (2耳 )。结论 :对于人工耳蜗植入术的术前评估 ,CT检查具有重要价值 ,必不可少 ,MRI检查是必要的补充。对内耳的三维重建 ,MRI优于CT。     

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.     

Autoimmune-mediated sympathetic hearing loss: a case report.

BACKGROUND: Damage to one inner ear is occasionally followed by contralateral sensorineural hearing loss. This has been defined as sympathetic hearing loss. HYPOTHESIS: It is hypothesized that autoimmunity can play a role in the pathogenesis of sympathetic hearing loss. METHODS: A male patient who developed right-sided sympathetic hearing loss at 20 years of age, 11 years after deafness of the left ear caused by a temporal bone fracture, is described. The patient's serum was analyzed for the presence of autoantibodies against inner ear tissues by immunocytochemistry and Western blotting using rat inner ear tissues. The patient's serum was tested specifically for antibodies against heat shock protein 70 by immunodot blot. The presence of autoantibodies known to play a role in systemic autoimmune disease was also examined. RESULTS: Immunocytochemistry on rat temporal bone sections demonstrated autoantibodies in the patient's serum specifically targeted against cochlear outer hair cells. No reactivity of the patient's serum was observed with control tissues including kidney, brain, and liver. Western blotting using homogenized rat cochlear tissues showed that the patient's serum reacted with a 25- and 27-kDa protein. No reactivity was observed with heat shock protein 70 in the immunodot blot analysis. The patient's serum did not contain autoantibodies against antinuclear antibodies, double-stranded DNA, antineutrophil cytoplasmic antibodies, basement membrane, reticulin, intestinal mucosa, muscle, collagen, or mitochondria. CONCLUSION: Observations indicate that this patient suffered sympathetic hearing loss caused by organospecific autoimmunity directed to cochlear outer hair cells.     

Clinical review of inner ear malformation

We had 126 patients with inner ear malformation diagnosed with temporal bone computed tomography (CT) scans at Azabu Triology Hospital between 1996 and 2002. We classified cases of inner ear malformation according to Jackler et al. The incidence of inner ear malformation in our series was as follows; 1. Labyrynthine anomalies 61% (isolated lateral semicircular canal dysplasia 56%, compound semicircular canal dysplasia 4%, semicircular canal aplasia 1%), 2. Cochlear anomalies 24%, 3. Enlargement of the vestibular aqueduct 12%, 4. Narrow internal auditory canal 2%, 5. Complete labyrinthine aplasia 1%, 6. Enlargement of the cochlear aqueduct 0%. The most frequent anomaly was isolated lateral semicircular canal dysplasia. We did not detect any significant clinical features in this anomaly. There were 2 patients with cochlear anomalies who had past histories of meningitis. Some patients with enlargement of the vestibular aqueduct had frequent attacks of fluctuating hearing. Clinically it is important to detect patients with inner ear malformation such as cochlear anomalies and enlargement of the vestibular aqueduct usually accompanied by congenital sensorineural hearing loss. For patients with congenital sensorineural hearing loss, we recommend temporal bone CT scan.     

Image analysis of the inner ear with CT and MR imaging: pre-operative assessment for cochlear implant surgery]

Recent progress in magnetic resonance imaging (MRI) has made it possible to obtain detailed images of the inner ear by delineating the lymphatic fluid within the labyrinth. We analyzed CT scans and MR images in 70 ears manifesting profound deafness owing to inner ear lesions and compared their detective ability for inner ear lesions. The following results were obtained. 1) CT scan examination showed slight to extensive ossification of the labyrinth in six ears (9%), whereas MRI examination revealed low to absent signal intensity of the inner ear in nine ears (13%). Therefore, it was concluded that MRI is more sensitive in detecting abnormalities of the inner ear than CT scan. 2) MRI provided useful information as to whether the cochlear turn is filled with lymphatic fluid or obstructed. This point was one of the greatest advantages of MRI over CT scan. 3) Abnormal findings in either or both the CT scan and the MRI were detected in suppurative labyrinthitis occurring secondary to chronic otitis media, bacterial meningitis and in inner ear trauma. However, such abnormal findings were not detected in patients with idiopathic progressive sensorineural hearing loss, ototoxicity or sudden deafness. These findings should be taken into consideration in pre-operative assessment of cochlear implant candidates.     

The lymphocyte transformation test with type II collagen as a diagnostic tool of autoimmune sensorineural hearing loss

Immunological disorders of the cellular type can be diagnosed by the lymphocyte transformation test (LTT). An autoimmune mechanism of certain cases of sensorineural hearing loss (SNHL) can be evaluated by using human inner ear tissue as an antigen. Recent studies have shown that type II collagen plays an important role, not only in some autoimmune mediated rheumatoid diseases, but also as an antigenic substrate of inner ear tissue in autoimmune sensorineural hearing loss. This paper deals with results of the lymphocyte transformation test using type II collagen as antigen in 68 patients with progressive sensorineural hearing loss (PSNHL) and 68 healthy volunteers. Thirty-four patients showed a strong stimulation in the lymphocyte transformation test, in contrast to only four volunteers in the control group, two of whom had a history of rheumatoid arthritis.     

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.     

Characteristics of sensorineural hearing loss in children with inner ear anomalies

PURPOSE: To determine whether hearing loss in children with inner ear anomalies has some distinctive characteristics when compared to children with hearing loss but without inner ear anomalies. METHODS: Temporal bone computed tomography scans of 69 patients with sensorineural hearing loss were examined for inner ear abnormalities of which 17 were identified. The medical histories of these patients were reviewed for the characteristics of their hearing loss, including initial presentation, natural history, and nature of loss, as well as the family history of hearing loss and risk factors for hearing loss. These were compared to age-matched controls with hearing loss but without inner ear anomalies. RESULTS: Seventeen patients had inner ear anomalies. Records of 14 of these patients were compared to patients without inner ear anomalies. Regarding age of onset, 71.4% of patients with anomalies had onset of their hearing loss at less than 2 years old vs 78.6% without anomalies. Regarding unilateral vs bilateral, 42.9% of patients with anomalies were unilateral vs 28.6% of patients without anomalies. For patients with anomalies, 85.7% were stable and 14.3% were progressive; without anomalies, 71.4% were stable, 21.4% were progressive, and 7.1% were fluctuating. Regarding family history, only 14.3% of patients without anomalies had a positive family history vs 56% of patients with anomalies. CONCLUSIONS: Children with inner ear anomalies and sensorineural hearing loss have an increased incidence of unilateral hearing loss and stable hearing loss as compared to controls with sensorineural hearing loss without inner ear anomalies. In addition, children with inner ear anomalies and sensorineural hearing loss are less likely to have a family history of hearing loss.     

Autoimmune mouse antibodies recognize multiple antigens proposed in human immune-mediated hearing loss.

HYPOTHESIS: Autoimmune diseased mice with hearing loss will have autoantibodies against the various cochlear antigens proposed in clinical autoimmune inner ear disease. BACKGROUND: Serum antibodies of patients with hearing loss recognize several proteins that are proposed as possible antigenic targets in the ear. This often leads to a clinical diagnosis of autoimmune inner ear disease, although it is not clear how these antibodies cause inner ear disease. Therefore, to better understand the relationship of autoantibodies and ear disease, an examination was made of serum autoantibodies in the MRL/MpJ-Fas(lpr) autoimmune mouse with hearing loss. Similar antibody patterns in the mouse would provide an animal model in which to investigate potential autoimmune mechanisms of this clinical ear disorder. METHODS: Sera from MRL/MpJ-Fas(lpr) autoimmune mice and normal C3H mice were tested by the enzyme-linked immunosorbent assay technique for reactivity against various reported cochlear antigens: heat shock protein 70 (bovine, human, bacterial), laminin, heparan sulfate proteoglycan, cardiolipin, and collagen types II and IV. RESULTS: The autoimmune mouse sera showed significantly greater antibody reactivity against all of the antigens when compared with normal mouse sera. CONCLUSIONS: Serum antibodies from autoimmune mice recognized several putative autoantigens reported for patients with hearing loss, suggesting that comparable antigen-antibody mechanisms might be operating. However, the recognition of multiple antigens did not identify any one as being the specific target in autoimmune hearing loss. The correlation of antibodies in the MRL/MpJ-Fas(lpr) autoimmune mouse and human studies indicates this animal model should aid further investigations into potential cochlear antigens in autoimmune hearing loss.     

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.     

Assessment of Serum Antibodies in Patients With Rapidly Progressive SensorineuralHearing Loss and Meniere's Disease

The immunoreactivity of sera from patients with rapidly progressive sensorineural hearing loss (SNHL) or Meniere's disease with bovine inner ear material was determined using the Western blot technique. Patients with other otologic conditions, autoimmune disorders, or arthritic disorders and age-matched randomly chosen patients with no hearing complaints served as controls. Twenty-two percent of the patients with bilateral rapidly progressive SNHL and 30% of the patients with Meniere's disease had antibodies that reacted with a 68 kd antigen in the inner ear material. In the control groups, the incidence of reactivity was 5.0% (P<.001). When the results of this study were compiled with data collected previously, it was found that of 279 patients with bilateral rapidly progressive SNHL, 90 (32%) were positive with the 68 kd protein. Thus, the anti-68 kd antibody may provide a good marker for an immune etiology of these patients' hearing loss.     

Experimental autoimmune sensorineural hearing loss

Alterations in host immunity result in a number of disorders affecting multiple organ systems, including the inner ear. The mechanism of injury is poorly understood; as such, this study investigated whether an experimental model of autoimmune sensorineural hearing loss (ASNHL) could be established. Animals were immunized with heterologous inner ear antigen and then evaluated for evidence of evolving inner ear autoimmunity. These animals uniformly developed antibodies to inner ear antigen in their sera and perilymph and 12 of 38 ears showed significant increases in action potential thresholds. Histopathological lesions consisted of loss of cochlear neurons, perivascular plasma cell infiltrates, edema, and extravasation of erythrocytes. These findings are characteristic of autoimmune injury and suggest the establishment of an experimental model of ASNHL in which to further investigate this clinical disorder.     

同种内耳抗原免疫致聋豚鼠的子代内耳功能和形态学观察

目的观察自身免疫性感音神经性聋(autoimmune sensorineural hearing loss，ASHL)母鼠所产子代内耳听觉和前庭生理功能、病理形态学的变化，初步探讨母鼠体内针对内耳组织抗原免疫反应是否可以造成子代的内耳损伤。方法同种内耳抗原(homogeneous inner ear antigens，HIEAg)持续免疫孕豚鼠，采用耳蜗电图(包括听神经复合动作电位)、总和直流电位、耳蜗微音器电位和眼震电图仪(记录自发性和冷热空气试验)测试母鼠和子鼠的听觉和前庭功能，并检测针对HIEAg的血清特异性体液和细胞免疫反应，采用火棉胶切片和HE染色，光镜观察内耳病理组织学改变。结果ASHL母鼠所产子鼠中，部分(3／7)出现听觉损伤，并发现其血清中特异性抗体水平升高，内耳出现免疫炎性病理损伤。非ASHL母鼠和对照组母鼠所产子代未见明显异常。结论ASHL雌鼠所产子代可出现感音神经性聋，其内耳损伤和功能障碍极可能与针对内耳组织的自身免疫反应(尤其是体液免疫)有关。     

共同腔畸形人工耳蜗手术适应证及手术方法的探讨

目的：探讨共同腔畸形人工耳蜗手术适应证以及人工耳蜗电极植入人路的选择。方法：在对重度或全聋患者进行人工耳蜗植入术前影像掌检查中,发现了6例耳蜗、前庭、外半规管呈共同腔畸形,其中5例有残留听力,1例未查到残留听力。结果：6例影像学检查呈共同腔畸形患者中,对5例有残留听力患者进行了人工耳蜗植入,其中3例选择了常规入路植入电极,2例选择了经乳突侧入路植入电极,术后均建立了人工耳蜗的听觉反应。1例因未查到残留听力,放弃了人工耳蜗手术治疗。结论：有残留听力的共同腔畸形患者,如果能够接受术后听觉言语识别效果差的事实,可以进行人工耳蜗手术。无残留听力或无法了解到有听觉反应的共同腔畸形患者,在现有技术条件下应放弃人工耳蜗植入手术。     

The use of magnetic resonance in the study of sensorineural hearing loss in children

Although newborn screening of congenital hearing loss through otoacustic emissions allow prompt recognition, imaging techniques, such as CT and MRI are needed to get a morphological diagnosis. Furthermore they can be very useful in unilateral cases, whose clinical presentation is belated and more insidious. Our aim is to show the utility of MRI in the study of inner ear congenital anomalies, whose presentation is belated. Thus from a series of 88 consecutive patients in which a MRI was performed as screening of assymetric sensorineural hearing loss, we selected 6 cases aged between 6 and 20. Four of them showed an inner ear anomaly on MRI. We present these anomalies commenting the findings on CT and MRI. Imaging techniques are required to start hearing rehabilitation programs early on patients with bilateral inner ear anomalies. But also they are very useful in the evaluation of unilateral assymetric sensorineural hearing loss, in young patients, even if only some frequencies are damned, to determine the nature of hearing loss.     

先天性自身免疫性感音神经性聋防治的实验研究

目的 探讨免疫抑制剂环磷酰胺是否可以有效地预防和治疗先天性自身免疫性感音神经性聋.方法 同种粗制内耳抗原免疫雌性豚鼠,在妊娠期间给予免疫抑制剂环磷酰胺预防,对于未给予免疫抑制的母鼠所产子鼠给予环磷酰胺治疗,观察实验动物内耳听觉功能和病理形态学变化.结果 同时给予免疫抑制的母鼠及其各自所产子鼠均未出现明显的听觉损伤和内耳病理改变;出现听觉功能障碍和内耳病理变化的子鼠经环磷酰胺治疗后,其听觉功能有所提高(主要为低频区).结论 环磷酰胺可有效地预防母体内针对内耳组织特异性自身免疫反应所造成的子鼠先天性感音神经性聋,但疗效有限.     

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.     

Cochlear implantation for children with GJB2-related deafness

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.     

不完全分隔内耳畸形患者人工耳蜗植入研究进展

不完全分隔内耳畸形作为内耳畸形的一种,是导致重度、极重度感音神经性聋的病因之一.其曾被认为是人工耳蜗植入手术的禁忌症.但是随着相关研究的进展,人工耳蜗植入已成为其主要治疗手段.本文就不完全分隔内耳畸形的概念、分类、及其所致的重度、极重度感音神经性聋患者人工耳蜗植入手术的相关研究进展作一综述.     

Inner ear-specific autoantibodies

The sera of patients with idiopathic sensorineural hearing loss (SNHL) were examined, using qualitative immunoblotting (Western blotting), for the presence of inner ear (IE)-specific autoantibodies. The water-soluble extracts and the sodium dodecyl sulfate-soluble extracts were prepared as the antigens from bovine IE tissues and several other organs in order to determine the specificity of autoantibodies in their sera to the IE. Some patients (n = 46) tested had autoantibodies that reacted with 68-, 62-, 55-, 50-, or 47-kd antigenic determinants found in all tissues, while others (n = 13) showed IE-specific autoantibodies which reacted with 220-, 60-, 58-, 33–35-, or 32-kd determinants. The presence of these autoantibodies from patients with progressive SNHL may have important implications with regard to their etiology and possibly their sensitivity to therapeutic intervention. It is now necessary to purify these IE-specific antigens and determine their clinical usefulness.