Ramsay-Hunt综合征2例

Ramsay-Hunt综合征是由水痘一带状疱疹病毒和单纯疱疹病毒引起,以侵犯面神经为主的疾病。水痘一带状疱疹病毒常侵犯单侧周围神经,导致特征性簇集小水泡,病愈后可获得较持久的免疫,一般不会再发生,极少导致脑膜炎。本文回顾分析我院2例Ramsay—Hunt综合征病例,以提高对本病的认识。     

Hunt综合征伴眩晕患者的半规管损伤频率特征

目的 探讨Hunt综合征伴眩晕患者的前庭半规管损伤频率特征以及视频头脉冲试验(video head impulse test,vHIT)在评估其前庭功能中的应用价值.方法 Hunt综合征伴眩晕患者26例(病例组)分别进行高频vHIT、中频摇头试验(head shaking test)和低频冷热试验(caloric test);30例健康人为对照组进行vHIT.以冷热试验单侧半规管反应非对称值(unilateral weakness,UW)、摇头试验诱发摇头眼震(head shaking nystagmus,HSN)、vHIT的增益值(video head impulse test gain,vHIT-G)为观察指标,应用SPSS 16.0统计软件分析对照组vHIT-G、病例组的三项试验结果特点及其相关性.结果 对照组6个半规管的vHIT-G均符合正态分布,方差分析,相互间差异无统计学意义(F=0.005,P＜0.01);双侧前、水平和后半规管平均vHIT-G((x)±s,下同)分别为16.80％±9.80％、16.57％±10.30％、16.52％±11.12％;病例组的患耳前、水平和后半规管vHIT-G分别为46.96％±34.54％、75.35％±35.29％、41.65％±32.87％,均符合正态分布,与对照组对应半规管比较差异有统计学意义(P值均＜0.01);vHIT、摇头试验和冷热试验阳性者分别为23例(88.46％)、22例(84.61％)和24例(92.31％),双侧确切概率法x2检验,P值均＞0.05,差异无统计学意义;UW与水平半规管vHIT-G之间存在正相关(r=0.692,P＜0.01).结论 Hunt综合征伴眩晕患者的半规管功能损伤呈多频或近乎全频性损伤,具有传导阻滞性神经损伤特点;vHIT可以作为Hunt综合征伴眩晕患者前庭功能评定的有效检查方法.     

Hunt综合征的临床诊断与治疗进展

Hunt综合征（Ramsay-Hunt syndrome）是外周性面部麻痹的第二大常见原因,本文系统地回顾了国内外在Hunt综合征领域的相关文献,并总结分析Hunt综合征患者的临床表现、并发症,探讨Hunt综合征的诊断和治疗新进展,以期提高临床对Hunt综合征的认识和治疗。同时,在临床中应注意表现为多发性颅神经炎的Hunt综合征,减少误诊,尽早给予合理治疗,提高治愈率。     

首发表现为咽喉部症状的Hunt综合征诊治分析

我科2006年7月～2011年7月共收治7例首发表现为咽喉部症状的Hunt综合征患者,现报道如下.1 资料与方法1.1 临床资料.71例患者中男5例,女2例,年龄38～65岁,中位年龄50岁;均为单侧,其中左侧3例,右侧4例.     

中西医结合治疗耳带状疱疹30例疗效分析

目的 观察中西医结合治疗耳带状疱疹的疗效.方法 将耳带状疱疹患者60例随机分为两组,治疗组30例口服龙胆泻肝汤、外敷青黛散,配合口服阿昔洛韦片治疗.对照组30例单纯口服阿昔洛韦片治疗.两组均10日为1个疗程,2个疗程后统计疗效.结果 治疗组痊愈15例,显效9例,有效4例,无效2例,总有效率93.33%.对照组痊愈7例,显效4例,有效5例,无效14例,总有效率53.33%.两组总有效率比较差异有统计学意义（P＜0.01）.结论 中西医结合治疗耳带状疱疹疗效明显优于单纯西药治疗,且无明显副作用.     

无环鸟苷治疗Ramsay Hunt综合征

ＲａｍｓａｙＨｕｎｔ氏综合征系由带状疱疹病毒感染所致，面瘫为其主要临床表现之一，恢复常不满意。本文报告１９９０年７月以来，我院应用无环岛苷静脉滴注。经８－２４月随诊。治疗效果较好。治疗组共９例，无环鸟苷２５０ｍｇ静脉点滴，一日两次，连两周，同时口服强的松４０ｍｇ／日及神经营养药。对照组５例仅用同剂量强的松及神经营养药。经随且在瘫完全恢复率为７８．８％。对照组１／５。结果表明无环鸟苷治疗Ｈｕｎｔ氏综     

Hunt综合征合并喉返神经损伤1例报告

Ramsay Hunt综合征是水痘-带状疱疹病毒(Varicella-zoster virus,VZV)引起的以侵犯面神经为主的疾病,临床主要表现为耳部疱疹、疼痛和/或面瘫,还可以出现耳鸣、听力减退、眩晕、恶心、呕吐等症状.病毒可以侵犯Ⅴ～Ⅷ颅神经而引起多发性神经炎,因此少数患者可出现其它颅神经症状[1].现将一例合并声音嘶哑的Hunt综合征报告如下.     

针药联合治疗耳带状疱疹引起面瘫的临床疗效观察简

目的观察药物联合针灸治疗耳带状疱疹引起面瘫的临床疗效。方法选取2011年2月～2013年1月于我科就治的此类患者57例,随机分为观察组29例,对照组28例。观察组采用药物治疗配合针灸疗法,对照组单纯采用药物治疗。10d一个疗程,治疗4个疗程后进行疗效评定。结果观察组临床痊愈19例,显效7例,无效3例,治愈和显效率89．6％;对照组临床痊愈13例,显效6例,无效9例,治愈和显效率67．8％,组间差异有显著性（P〈0．01）。结论药物治疗联合针灸治疗耳带状疱疹引起面瘫临床效果确切,与单纯采用药物治疗相比优势明显。     

Ramsay-Hunt综合征5例

Ramsay-Hunt综合征过去为一种少见的皮肤病,近几年有增多的趋势,现将我院近2年接诊的5例病例报道如下.1资料与方法1.1临床资料.例1,男,35岁,主诉右口眼歪斜5d,口腔皮疹伴耳、咽痛2d.患者5d前无明显诱因出现口角倾斜,右眼不能完全闭合,2d前口腔内右硬腭起水疱伴疼痛.     

Hunt综合征并Ⅶ—Ⅹ颅神经麻痹1例

Hunt综合征并多发颅神经损害者少见，我科近期收治1例。现报告如下。患者，男，69岁。因左耳剧痛，口角歪斜2周入院。自诉2周前饮酒后出现左耳剧痛，耳廓出现疱疹，左眼不能闭合，面部麻木，口角歪斜，眩晕伴呕吐，左耳听力减退，耳鸣，曾在当地用中药治疗无效而来院就诊。入院后次日出现声嘶，饮水呛咳，吞咽困难。检查：左耳廊、外耳道、鼓膜均有疱疹，部分破溃，左侧额纹消失，左眼不能闭合，左鼻唇沟消失，示齿及鼓气不能，口角向右歪斜，左侧舌部味觉消失，左咽反射消失，悬雍垂向右偏，左声带麻痹，呈外展位，发音时右声带高于左侧。…     

颞骨先天性胆脂瘤11例分析

目的 探讨颞骨先天性胆脂瘤的病变部位、范围、临床症状、诊断及手术方式.方法 回顾性分析1998年1月～2009年1月诊治的11例颞骨先天性胆脂瘤患者的临床资料.结果 11例先天性中耳胆脂瘤患者的年龄为9～38岁,中位数为15岁,男6例,女5例,术前平均听阈为39 dB HL,气-骨导差为20～55 dB.病变仅位于鼓室...     

A Clinicopathologic Series of 22 Cases of Tonsillar Granulomas

Objectives Tonsils are uncommonly affected by granulomatous inflammation, often with an obscure cause. This study attempts to elucidate the nature of tonsillar granulomatous inflammation. Design Retrospective clinicopathologic review. Methods Twenty‐two cases of tonsillar granulomas diagnosed between 1940 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. H&E slides and a series of histochemical stains were reviewed, and patient follow‐up was obtained. Results There were 11 males and 11 females, aged 7 to 64 years (mean, 29.9 y). Most of the cases presented bilaterally (n = 19) with sore throat, dysphagia, and/or nasal obstruction. The clinical differential included chronic tonsillitis, tuberculosis, nonspecific infection, sarcoidosis, and a neoplasm. Histologically, the granulomas were focal and scattered, or diffuse, identified in the interfollicular zones (n = 16) and/or the germinal centers (n = 13), and occasionally associated with necrosis (n = 6). Based on histochemical and clinical follow‐up information, the etiology of the granulomas included sarcoidosis (n = 8), tuberculosis (n = 3), Hodgkin's lymphoma (n = 2), toxoplasmosis (n = 1), squamous cell carcinoma (n = 1), and no specific known cause (n = 7). Twelve patients were either alive at last follow‐up or had died with no evidence of disease (mean, 12.4 y), and 9 were either alive at last follow‐up or had died with disease (mean, 24.9 y). One patient was alive with unknown disease status (lost to follow‐up after 13.3 y). Conclusions Although a cause for tonsillar granulomas is frequently identified, a number may not develop an identifiable etiology, with the granulomas probably representing an exaggerated immune response to chronic tonsillitis. However, a careful work‐up must be conducted to exclude specific causes and avoid clinical mismanagement.     

广东省59例大前庭水管综合征患者SLC26 A4基因突变及表型分析

目的：探讨广东省大前庭水管综合征（enlargement of vestibular aqueduct syndrome,EVAS）患者SLC26 A4基因位点突变及相关听力表型,为研究 EVAS 发病机制提供参考。方法采用基因芯片法对59例EVAS患儿进行 SLC26 A4基因 IVS7－2 A＞G：2168 A＞G 位点检测,并行颞骨 CT 影像学检查。结果59例EVAS患者中21例（35．59％）为SLC26 A4双等位基因（纯合或复合杂合）突变,其中16例为IVS7－2 A＞G纯合突变,2例为2168A＞G纯合突变,3例为IVS7－2A＞G、2168A＞G复合杂合突变,这21例CT均显示为双侧前庭水管扩大或其他内耳畸形;38例为 SLC26 A4单等位基因突变,其中31例为 IVS7－2 A＞G 杂合突变,7例为2168A＞G杂合突变,这38例中4例为前庭水管扩大伴Mondini畸形,2例表型正常,其余均为双侧前庭水管扩大。59例患儿均表现为重度－极重度聋。结论本组EVAS患者中SLC26 A4基因IVS7－2 A＞G位点的突变发生率最高,其次为2168A＞G;均表现为双耳重度或极重度感音神经性听力损失。     

Musical Ear Syndrome: Prevalence and characteristics in cochlear implant bearers

IntroductionMusical Ear Syndrome (MES) is an uncommon phenomenon described as the perception of auditory musical sensations not corresponding to any external stimulus. It seems to be more frequent in case of profound hearing loss. Our objective was to evaluate prevalence, characteristics and risk factors in a population of cochlear implant patients.MethodsA retrospective study was conducted in cochlear implant patients, who were adult (> 18 years) in 2020 and underwent cochlear implantation between 1993 and 2019. We analyzed the presence and characteristics of MES.Results118 of the 358 patients (33%) perceived or had perceived auditory musical sensations: 71 (19.8%) before, 100 (28%) after, and 53 (14.8%) both before and after implantation. The musical auditory sensations were usually short and well-tolerated, resembling instrumental music, and occurring several times a day. Thirteen patients (11%) considered them intolerable. Fatigue was a triggering factor in 40 patients (33.9%). Personal and medical characteristics, type of implantation, make of implant, etiology and tinnitus did not emerge as risk factors. On the other hand, MES+ patients were significatively younger (56 ± 17.4 years versus 61.9 ± 17.9 years; P = 0.0009). Despite the phenomenon, patients were satisfied with implant functioning and subjective auditory performance was not affected.ConclusionPrevalence of Musical Ear Syndrome was high in cochlear implant patients, and especially in younger subjects. It is essential to improve knowledge of this phenomenon.     

耳廓蔓状血管瘤治疗20年之体会

目的对解放军总医院22年来的耳廓蔓状血管瘤病例进行总结分析,以求选择更好的治疗方法,提高耳廓蔓状血管瘤的疗效,防止复发。方法1986年以来共收治21例耳廓蔓状血管瘤患者,对其进行临床分析,比较不同治疗方法的效果。结果21例中13人接受过冷冻、硬化剂或平阳霉素局部注射、单纯血管栓塞或局部血管结扎等方法治疗,疗效不佳;对13例患者实施了耳廓血管瘤剥脱术治疗,5例术前采取了血管造影及栓塞治疗,8例未采用血管栓塞治疗。术后病理检查报告均符合蔓状血管瘤。经2年以上随访,2例患者有局部小范围复发,再次进行手术治疗后治愈,再经2年以上随访无复发：其余9例一次手术即获得治愈。结论血管造影及栓塞治疗48～72小时内进行耳廓血管瘤剥脱术是对耳廓蔓状血管瘤的最佳治疗方案。     

A novel controlled local drug delivery system for inner ear disease

PURPOSE: Our goal is to develop a novel drug delivery system that can potentially improve clinical outcomes compared to current methods of dosing drugs such as dexamethasone or gentamicin. This system focuses on a single local application to the inner ear via the round window membrane. HYPOTHESIS: A chitosan-glycerophosphate (CGP)-hydrogel based drug delivery system can be engineered to provide local and sustained drug release to the inner ear. STUDY DESIGN: In vitro: drug release and (CGP)-hydrogel matrix degradation were characterized using dexamethasone as a model drug. In vivo: dexamethasone laden CGP-hydrogel was placed in the round window niche of mice. Perilymph samples were obtained from the oval window and analyzed for dexamethasone. The impact of CGP-hydrogel on auditory function was evaluated. RESULTS: In vitro: A CGP-hydrogel was designed to release 92% of the dexamethasone load over 4 consecutive days with concurrent degradation of the hydrogel matrix. In vivo: After surgical placement of CGP-hydrogel to the round window niche, we detected elevated levels of dexamethasone in perilymph for 5 days. Auditory function testing revealed a temporary hearing loss in the immediate postoperative period, which resolved by the 10th postoperative day. CONCLUSIONS: We report the development of CGP-hydrogel, a biodegradable matrix that achieves local, sustained delivery of dexamethasone to the inner ear. There were no significant complications resulting from the surgical procedure or the administration of CGP-hydrogel to our murine model.     

良性阵发性位置性眩晕诊治分析

目的回顾性分析我科诊治的良性阵发性位置性眩晕(BPPV)患者,分析发病因素及治疗方式,旨在协助临床医生提高精准诊治。方法以眩晕问卷形式收集2010年6月-2016年11月接诊1200例BPPV患者,分别采用Epley复位法、Barbecue复位法、Semont复位法及Brandt-Daroff习服法,药物与心理辅助治疗。分析发病原因、临床特征、治疗方式。结果 1200例BPPV患者中,女性多见,男女比例为1:2.2;60-70岁年龄段发病多;症状仅表现为体位发作性眩晕,无基础病和合并症者多;诊断分型后半规管BPPV1012例(84.33%)、水平半规管BPPV162(13.50%)、上半规管BPPV0例(0.00%)、多个半规管BPPV26例(2.17%);耳石复位是首选治疗方法,首次复位有效率为88%。结论详细收集病史及临床特征,综合评估做出正确诊断,给予个性化治疗是有效治疗BPPV的关键。     

耳蜱虫患者31例临床分析

目的探讨耳蜱虫诊治与预防。方法采用回顾性研究的方法,对我院耳鼻咽喉科门诊就诊的31例外耳蜱虫临床资料并结合文献进行分析。结果本组并发耳部瘙痒、剧痛20例,耳后、下颌角淋巴结肿大7例,水肿性红斑13例,贫血5例。取出蜱虫及予相应对症处理,随访1个月临床治愈。结论本病应着重询问病史、特征性的临床症状及电耳镜检查可以明确诊断,及时正确的治疗,以防止严重并发症的发生。