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。     

内耳道膜迷路磁共振三维快速液体衰减反转恢复序列检查在突发性聋中的应用

目的　评估内耳道膜迷路磁共振三维快速液体衰减反转恢复序列（three-dimensional fluid-attenated inversion recovery，3D-FLAIR）在突发性聋诊治中的应用。 方法　根据内耳道膜迷路3D-FLAIR结果，将2017年1月～2018年6月我科收治60例突发性聋患者分为3D-FLAIR阳性组和阴性组，对两组听力学特点、是否伴有眩晕等及预后进行分析和比较。结果　3D-FLAIR阳性组较阴性组眩晕发生率更高，差异有显著性（P =0.001）；3D-FLAIR阳性组听力曲线以全聋型频或平坦型为主，且阳性组较阴性组听力损失更严重，预后更差。结论　听力损失较重尤其伴眩晕的突发性聋，若其内耳道膜迷路3D-FLAIR显示内耳有可能存在延迟强化征象，其预后较阴性组欠佳。建议对所有突发性聋患者，常规行内耳道膜迷路3D-FLAIR检查，有助于判断预后。     

内耳膜迷路积水的基础研究和临床诊治

我们对内耳膜迷路积水的认识一般来源于梅尼埃病。1938年Hallpike和Cairns发现梅尼埃病的病理学改变为内耳膜迷路积水，又称内淋巴积水。其实，膜迷路积水是许多耳科疾病内耳损伤后相同的病理改变，梅尼埃病只是其中一种，称为有症状特发性膜迷路积水。内耳膜迷路积水的基础研究，尤其是动物模型的建立，成为为研究眩晕机制且最终根治眩晕的关键。掌握不同种类膜迷路积水疾病的临床特点，有助于眩晕的诊断、鉴别诊断和临床治疗。     

1.5T MRI经静脉钆造影在内耳膜迷路积水诊断中的临床应用[论著]

目的　探讨1.5T MRI经静脉3D-Flair内耳钆造影在内耳膜迷路积水诊断中的临床应用价值。方法　收集2018年5月至2020年11月鄂尔多斯市东胜区人民医院耳鼻咽喉科门诊就诊的17例（17耳）梅尼埃病和16例（16耳）前庭性偏头痛患者。其中梅尼埃病组（实验组）男11例（11耳）,女6例（6耳）,年龄28~69岁,中位年龄56岁,病程1~17年;前庭性偏头痛组（对照组）16例（16耳）, 男3例（3耳）,女13例（13耳）,年龄31~67岁,中位年龄48.5岁,病程6个月~3年。两组病例均先行1.5T MRI内耳水成像和3D-Flair序列扫描（层厚1 mm）,第1次扫描后即行静脉注射钆剂,剂量为0.4 ml/kg,通过肘静脉以3.0 ml/min的速度注射入体内,注射钆剂后4.5 h行第2次3D-Flair序列扫描。由2名有经验的核磁室医师依据内耳膜迷路MRI评分法观察比较内耳半规管、前庭、耳蜗各 转水成像及钆造影前后3D-Flair图像,并进行评分判断是否存在内耳膜迷路积水。结果　33例患者均获得满意的MRI图像,其中17例梅尼埃病患者中16例患耳提示膜迷路积水,积水显示率为94.11%;16例前庭性偏头痛患者中1例症状耳提示膜迷路积水,积水显示率为6.25%。实验组和对照组比较,实验组积水率高于对照组,两者比较差异有统计学意义（χ2=25.48,P <0.05）。结论  1.5T MRI经静脉双倍剂量内耳3D-Flair钆造影在内耳膜迷路积水诊断中具有一定的临床应用价值。     

伴阵发性睡眠性血红蛋白尿症的突聋病例报告

突聋(sudden sensorineural hearing loss,SSHL)是耳鼻咽喉科常见病、多发病,突聋的病因及发病机制目前并未完全阐明,局部因素及全身因素都可能引起突聋,常见的可能病因包括血管性疾病、病毒感染、微循环障碍、血液粘稠度改变、膜迷路积水、自身免疫性疾病、传染性疾病、肿瘤、生活习惯、睡眠障碍、心理因素等。本文报道1例伴阵发性睡眠性血红蛋白尿症(paroxysmal nocturnal hemoglobinuria,PNH)病史的突聋病例,PNH是一种后天获得性造血干细胞基因突变所致的红细胞缺陷性溶血病,并发症之一为血栓形成。推测突聋是由PNH继发血栓形成引起,此病例罕见,经溶栓、纠正贫血及激素治疗,患者听力恢复良好。通过此病例分析讨论,希望可以为临床的诊疗提供参考。     

突发性聋与内淋巴积水

内淋巴积水一直被看作梅尼埃病的病理基础,但目前的有关研究表明某些突发性聋患者中也存在内淋巴积水,且所占比例不容忽视。本文从特发性突聋的病因、病理、耳蜗电图改变、积水的比率和意义以及突聋的治疗等方面来综述突发性聋与内淋巴积水关系的研究进展。     

自身免疫性聋的分子作用机制

本回顾分析了Ⅱ型胶原、Ⅸ型胶原、内耳膜迷路30ku蛋白、层粘连蛋白、68ku蛋白、P0蛋白、Raf-1蛋白、β-微管蛋白等几种在自身免疫性耳聋和梅尼埃病中的自身抗原及其致聋的分子机制。     

迟发性膜迷路积水的临床研究现状

迟发性膜迷路积水（delayed endolymphatic hydrops，DEH）是类似梅尼埃病但又有明确病因的疾病。1975年Nadol等首次报道，1978年Schuknecht对其分类和命名进行了全面阐述，DEH指患者原有一侧耳极重度感音神经性聋，以后又发生同侧或对侧膜迷路积水，从耳聋发生到以后出现膜迷路积水症状的间隔期可长达数十年。     

比较内耳内淋巴显像和耳蜗电图及甘油试验在梅尼埃病诊断中的价值

目的:研究甘油试验、耳蜗电图、3T MRI在梅尼埃病(MD)患者中的关系。方法:评估20例患者,把稀释的钆造影剂通过鼓室注射入患者双侧的鼓室腔,24h后,通过3T MRI评估膜迷路积水的程度,此外为检测耳蜗积水,进行耳蜗电图和甘油试验。结果:经耳蜗电图检测,11例(55%)患者有阳性结果。经甘油试验检测,12例(60%)患者有阳性结果。同时采用甘油试验和耳蜗电图检测同一组患者时,75%的患者有阳性结果。用钆造影剂的3T MRI检查,19例(95%)患者有阳性结果。结论:与耳蜗电图及甘油试验检测相比,鼓室内注射钆造影剂的3T MRI,对内耳膜迷路积水的诊断更有价值。     

突发性聋与内淋巴积水

内淋巴积水一直被看作梅尼埃病的病理基础，但目前的有关研究表明某些突发性聋患者中也存在内淋巴积水，且所占比例不容忽视。本文从特发性突聋的病因、病理、耳蜗电图改变、积水的比率和意义以及突聋的治疗等方面来综述突发性聋与内淋巴积水关系的研究进展。     

以耳部症状首发、合并鼻咽占位的肉芽肿性多血管炎1例并文献复习

目的 探讨1例临床少见的以耳部症状首发、合并鼻咽部占位的肉芽肿性多血管炎(GPA)的临床特征、实验室检查、病理表现及诊疗过程。 方法 回顾性分析患者的病例资料,总结病例特点并回顾国内外GPA相关文献。 结果 患者以中耳炎、迷路炎症状为首发表现,合并鼻咽部占位,病程中逐渐出现面瘫,三叉神经刺激症状加重。多次留取耳及鼻咽部活检示急慢性炎症细胞浸润。升级抗生素,同时为避免中耳炎侵犯岩骨及颅内行乳突开放术。中耳局部炎症改善后其耳痛、面瘫等仍不缓解,但激素治疗有效,遂进一步完善自免病相关检查并再次行鼻咽部活检,最终确诊为GPA,予激素及免疫抑制剂治疗得以控制症状。术后3个月暂无显著肺部及肾脏受累表现。 结论 临床上发现不典型的中耳炎或常规治疗反复不愈,且逐渐进展出现内耳、颅神经侵犯表现如眩晕发作、面神经麻痹等,同时激素治疗有效,且合并鼻咽部占位、鼻窦炎影像学表现,或累及其他器官如肺、肾脏时,均应考虑到GPA的可能。当反复留取病理活检未能取得特异性确诊依据时,动态监测抗中性粒细胞胞浆抗体、红细胞沉降率、尿潜血、胸部CT、血肌酐等也具有重要的提示意义。     

Decreased miRNA-320e correlates with allergy in children with otitis media with effusion

ObjectiveOtitis media with effusion (OME) is a common childhood disease and the main cause of conductive hearing loss in this age group. Many factors predispose to OME but allergy is still widely disputed. The answer may lay in the molecular mechanisms of ear exudate formation and the recent studies showed miRNAs might take part in it. MiRNAs are also potent regulators of allergic response. As miRNAs are present in the middle ear, we hypothesized their expression differs between allergic and non-allergic patients and reflects the difference in pathomechanism of effusion formation between these two groups.Materials and methodsThis study aimed to establish the expression of 5 different miRNAs (miR-223-3p, miR-451a, miR-16-5p, miR-320e, miR-25-3p) in ear exudates in children diagnosed with OME. The allergy group consisted of 18 patients whereas the non-allergic group had 36 patients. MicroRNA was isolated from the middle ear fluid collected during myringotomy and transcribed into cDNA. MiRNA expression was measured with TaqMan™ MicroRNA Assays and analyzed with DataAssist software. The comparative CT method was used for calculating the relative quantification of gene expression based on the endogenous control gene expression (U6 snRNA-001973).ResultsMiR-320e expression was significantly decreased in allergic children with OME. Other studied miRNAs also showed reduced expression in allergic children, but the decrease was not significant.ConclusionsMiRNA expression differs between children with and without allergy in the course of OME, but further studies are needed to explain the exact role of miR-320e and its target genes in OME pathology in allergic patients.     

Experience with vertical partial laryngectomy with special reference to laryngeal reconstruction with cervical fascia

In this paper we report our experience of vertical partial laryngectomy using the superficial cervical fascia; we describe the technique and present the functional and oncological results of this method of treatment. A total of 42 patients with squamous cell carcinoma of the true vocal folds, in stage T(1) (n = 28) or T(2) (n = 14), were treated in our department using vertical partial laryngectomy during the decade 1987-1997. Nine patients had post-operative radiotherapy. The shortest follow-up time was three years. There were six recurrences in all, four in the larynx and two in the neck. All four of the laryngeal recurrences were treated with total laryngectomy and are doing well. Both the patients with neck metastases, who were treated with neck dissection, died. Permanent tracheotomy was necessary in one patient. There were no problems with aspiration. The recurrence rate was 14 per cent, the three-year survival index was 95.2 per cent and the three-year larynx preservation index was 90 per cent. According to our experience, vertical partial laryngectomy, using the method we describe, has a good functional and oncological result for stage T(1) and T(2) tumours.     

Polymyositis with dysphagia treated with endoscopic balloon dilatation

Polymyositis is characterized by non-specific inflammatory disease associated with an autoimmune disorder involving muscles of the limbs and neck. We report a case of an 80-year-old man who was referred to our clinic with a chief complaint of dysphagia and muscle weakness in all four limbs. The patient was diagnosed with polymyositis based on pathological findings, muscle weakness, electromyogram findings, and an elevated creatine phosphokinase level. The patient was also positive for HLA-DR3. Intravenous predonine administration was initiated, but dysphagia was not improved. We considered a cricopharyngeal myotomy, but this could not be performed because of heart failure. Endoscopic balloon dilation was performed and dysphagia improved on the same day. Therefore, we suggest that this method is a safe and effective approach for polymyositis with dysphagia.     

Cytokines in children with otitis media with effusion

We assayed 38 middle ear effusions from 23 children aged 4–13 years (mean 7) undergoing tympanostomy tube placements. All fluid was assayed for tumor necrosis factor (TNF) α, interleukin (IL) 1β, IL-8, and IL-10. Cytokine concentrations were measured by means of an enzyme-linked immunosorbent assay. Detectable levels of IL-1β, IL-8, and IL-10 were found in all of the effusions. TNF-α was detected in 18 of the middle ear effusions (47.4%). The mean concentration of TNF-α, IL-1β, IL-8, and IL-10 was, respectively, 0.423 ± 1.39, 30.58 ± 68.7, 7001.9 ± 6743, and 56 ± 58.7 pg/ml. There was a strong, statistically significant correlation between the concentrations of TNF-α and IL-1β (r = 0.87, P = 0.001) and between IL-1β and IL-8 (r = 0.53, P = 0.001). There was no correlation between the concentrations of IL-10 and other cytokines examined or between tympanic membrane pathology and the concentrations of TNF-α, IL-1β, IL-8, or IL-10. The presence of IL-10 in middle ear effusions may be one of the causes of a lack of clinical features of acute inflammation and may lead to a chronic inflammatory state. Received: 25 August 1999 / Accepted: 5 January 2000     

Speech improvement with velopharyngoplasty in patients with lip-jaw-palate clefts

BACKGROUND: Despite successful closure of the hard and soft palate and intensive speech therapy a velopharyngeal insufficiency is not completely avoidable in each case of cleft palate. An improvement by velopharyngoplasty should be possible. PATIENTS AND METHODS: Two hundred and ninety patients suffering from cleft palate were examined before and one year after velopharyngoplasty according to Sanvenero-Rosselli. Four main symptoms of the affected speech were assessed: changes of the resonance, inappropriate nasal air emission, articulary disorders (deviations from articulation areas), and non-physiological facial expression during speaking, each divided into three grades. Afterwards, the complete speech quality was classified. RESULTS: Assessing the symptoms separately and summarised the therapy including velopharyngoplasty was suitable to improve the speech quality in 83.1% of the cases, resulting in a widely unaffected speech. The improvement was greater in younger patients undergoing operation (Gamma-test, p < 0.001). But the final results were independent from age due to poorer initial situation in younger patients (Gamma-test, p < 0.001). CONCLUSIONS: The velopharyngoplasty is an important method for repair of velopharyngeal insufficiency in patients with cleft palate. In each individual case it is necessary to consider carefully if and when this operation should be performed. An intensive interdisciplinary co-operation of all specialists involved in the treatment is indispensable.     

Hearing acuity of children with otitis media with effusion

Hearing levels are reported for a cohort of 222 infants (aged 7 to 24 months) and 540 older children (aged 2 to 12 years) with otitis media with effusion (OME). The infants had an average speech awareness threshold of 24.6 dB hearing level (HL). The older group had mean bone conduction thresholds less than 10 dB HL, and air conduction thresholds averaged 27 dB HL; however, acuity was 7 dB less impaired at 2,000 Hz. The mean three-frequency pure tone average and speech reception threshold were 24.5 and 22.7 dB, respectively. Hearing acuity was not significantly related to age or previous duration of OME. The otoscopic observation of an air-fluid level or bubbles was associated with less hearing impairment; however, a predictive relationship between hearing levels and tympanogram characteristics could not be demonstrated.