Autoimmune recurrent facial palsy and bilateral sudden sensorineural hearing loss following Ramsay Hunt-like syndrome

Objective

To describe the first published case of recurrent facial nerve palsy associated with bilateral sudden sensorineural hearing loss of autoimmune origin.

Case report

A 33-year-old male presented with acute facial palsy on the left following a vesicular herpetic eruption in the external ear canal on the same side. Serologic measurements demonstrated an elevation of IgM antibodies against herpes simplex virus but not for varicella-zoster virus, confirming a Ramsay Hunt-like syndrome due to herpes simplex virus. The following four months, the patient exhibited other three episodes of facial palsy, well responded to steroid treatment. During the clinical course, a sudden sensorineural hearing loss was also diagnosed, initially on the left side and then on both sides. The autoimmune markers such as the antinuclear antibody and the anti-gangliosides antibodies (anti-GM1, anti-GQ1b) were found positive. Despite steroid treatment, hearing did not show any improvement, remaining moderate on the right and severe on the left.

Conclusion

Recurrent facial nerve palsy and bilateral sudden sensorineural hearing loss could be the expression of autoimmune disturbances. The initial triggered factor could be the herpes simplex virus infection, such as a Ramsay Hunt-like syndrome.     

感音神经性听力损失的MRI特征

感音神经性听力损失(SNHL)是耳科常见疾病,主要表现为听力损失伴或不伴耳鸣、眩晕、耳闷等症状,SNHL病因复杂多样,目前尚未有一个确切的病因。近年来磁共振影像技术(MRI)得到了较好的发展和运用,内听道MRI在显示软组织方面更具优势,可用来研究SNHL患者的内耳疾病,如研究小脑前下动脉来进一步阐述内耳微循环,轧造影显示梅尼埃病患者膜迷路积水,以及功能MRI对脑区活动的研究等等。本文主要以内听道MRI影像学特征来探讨SNHL的潜在病因,为耳科医生诊疗SNHL提供新思路。     

感音神经性聋并迟发性眩晕（附13例报告）

报告感音神经性聋并迟发性眩晕（ＳＨＬ－ＤＶ）１４耳，其中由中耳炎及其有关手术所致者８耳，突聋和原因不明各３耳，１４耳由耳聋至眩晕发作相隔期平均２２．６年，眩晕病程平均６．２年，２耳行内淋巴囊减压术；３耳由中耳炎所致进严重耳聋，行迷路切除术，３耳行庆大霉素鼓室灌注术，６耳行链霉素外规管灌注术，１４耳平均随访２．３年，迷路切除术者眩晕消除，听力丧失，氨基糖甙类药物灌注者眩晕控制，听力有不同程度保存，提     

急性低频感音神经性聋的短期临床疗效分析

目的探讨小剂量、短疗程皮质类固醇激素对急性低频感音神经性聋的疗效。方法选取28例(30耳)急性低频感音神经性聋患者作为研究对象,随机分为强的松治疗组16例(耳)与对照组12例(14耳),两组患者在口服等剂量强的松基础上,对照组加用血管扩张剂和神经营养剂治疗,1周后观察疗效。结果两组患者总有效率(完全恢复和部分恢复)为83.3%(25/30),总治愈率(完全恢复)46.7%(14/30);激素治疗组的有效率87.5%(14/16),治愈率50.0%(8/16),对照组有效率78.6%(11/14),治愈率42.9%(6/14),两组间的疗效比较,差异无统计学意义(P>0.05)。结论急性低频感音神经性聋的治疗有别于突发性耳聋,治疗上无需使用血管扩张剂及神经营养剂等,初始小剂量皮质类固醇激素治疗本病有效。     

Acute sensorineural hearing loss immediately following a local anaesthetic dental procedure

Acute sensorineural hearing loss is an uncommon phenomenon. We describe the first case of a 42-year-old lady who presented with acute sensorineural hearing loss occurring immediately after a dental procedure. Possible mechanisms are discussed. She was treated with high dose oral steroids, low molecular weight dextran and vasodilators with benefit.     

急性低频感音神经性耳聋的临床观察

目的探讨急性低频感音神经性耳聋(acute low-tone sensorineural hearing loss,ALHL)的临床特点和疗效,提高对该疾病的诊断和认识。方法回顾性分析62例ALHL患者的临床表现、听力学检查和治疗情况,总结其临床发展规律。结果发病年龄以青中年为主,女性明显多于男性,多为单耳发病,表现为耳闷或伴耳鸣,听力下降,不伴眩晕,所有患者纯音听阈均表现为轻中度低频感音神经性耳聋,治疗前后分别为(38.71±6.82)dB和(20.56±9.44)dB,两者比较差异具有统计学意义(P<0.05)。鼓室图"A"型,49例(80.9%)镫骨肌反射引出,40例(64.5%)Metz试验阳性,62例ABR均正常。62例患者治疗前DPOAE在0.5～1 kHz的引出率仅为18.7%,反应幅值明显降低,治疗后DPOAE在0.5～1 kHz的引出率提高至43.8%,幅值亦有所提高。结论 ALHL以突发的耳闷和(或)伴耳鸣为主要表现,常单耳发病,青中年女性为主,听力学定位诊断为蜗性聋,仅累及低频区,皮质类固醇激素治疗有较好的疗效。     

Sudden sensorineural hearing loss after heroin injection

Sudden sensorineural hearing loss is a symptom of cochlear injury. Potential aetiologies are vascular diseases, viral infections, allergic reactions, autoimmune disorders, and traumatic rupture of the intralabyrinthe membrane. Unlike in unilateral cases bilateral sensorineural hearing loss is often associated with specific disease entities. We report a case of sudden bilateral deafness after intravenous heroin abuse. The putative pathophysiological mechanisms are discussed.     

突发性耳聋伴眩晕患者听力预后分析

摘要：目的回顾性分析突发性耳聋（sudden deafness,SD）患者的听力损失程度、预后与发病时是否伴有眩晕发作的关系。探索突发性耳聋单发听力损失、突发性耳聋伴眩晕的诊断、治疗及预后评价。 方法按中华医学会耳鼻咽喉头颈外科学分会颁布的突发性耳聋诊断标准,2012年1月~2015年12月收治突发性耳聋患者196例。对所有患者进行扩管溶栓、营养神经、抗病毒及高压氧治疗,按2007年中华耳鼻咽喉学会颁布的突发性耳聋疗效及分级标准,针对听力恢复进行疗效评价。结果伴有眩晕症状患者发病率为30.7%,听力损失在50 dB以上,听力恢复有效率23%;不伴有眩晕症状患者发病率为69.3%,听力恢复有效率73%。结论伴发眩晕的突发性耳聋患者,往往其听力损失严重,且预后不佳。     

影响突发性聋疗效的相关因素研究

目的探讨突发性聋预后的相关因素,指导其预后判断。方法回顾性分析2007年10月-2012年7月710例（748耳）突发性聋患者,应用有序Logistic回归分析,筛选与突发性聋预后相关的因素,对于有统计学意义的因素用非参数检验再次验证,以指导预后分析。结果年龄、病程、伴眩晕症状、治疗前耳聋程度、伴糖尿病与疗效有相关性;性别、耳聋侧别、伴高血压与疗效无相关性,病程长短与疗效有统计学意义（P〉0．05）;不伴眩晕的患者治疗有效率要明显高于伴眩晕的患者（P〈0．05）;糖尿病患者与非糖尿病患者的有效率比较有统计学意义（P〈0．05）;初诊听阈程度与疗效差异有统计学意义（P〈0．05）。结论影响突发性聋预后的因素有年龄、病程、伴发症状、伴糖尿病、治疗前耳聋稃彦．     

儿童流行性腮腺炎并发急性胰腺炎和感音神经性聋1例及文献复习

目的：探讨流行性腮腺炎并发急性胰腺炎和感音神经性聋的临床特征,听力学表现,早期诊断与预后。方法：报告并分析1例流行性腮腺炎并发急性胰腺炎和感音神经性聋患者的临床资料,并复习有关文献。结果：流行性腮腺炎病毒感染可致多器官损害,出现一种或多种并发症,而同时并发急性胰腺炎和感音神经性聋临床少见,多表现为单侧突发的完全性聋,预后差。结论：流行性腮腺炎可同时并发包括感音神经性聋在内的一种或多种并发症,听性脑干反应对流行性腮腺炎并发感音神经性聋的早期诊断有一定的价值。     

Pediatric sudden sensorineural hearing loss after diving

OBJECTIVE: Sensorineural hearing loss after diving from a low height has been rarely reported especially in children. METHODS: We present and discuss a new case of pediatric sudden sensorineural hearing loss after diving. RESULTS AND CONCLUSION: Medical and surgical approaches (indications and timing of explorative tympanotomy) to this occurrence are still controversial.     

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。     

Post operative sensorineural hearing loss after middle ear surgery

Mild Sensorineural hearing loss subsequent to middle ear surgery has till today been an important complication to middle ear surgery inspite of advances in surgical techniques, operative instruments, monitoring devices and better treatment options. Lack of proper knowledge about this problem is because of under reporting of exact magnitude of hearing loss on account of difficulty in measuring hearing threshold of patients in immediate postoperative period as it may lead to post operative infection and discomfort to the patient. In our study of 80 cases carried out at ENT department, Baroda Medical College and S.S.G Hospital, Baroda, we have utilized weber’s lateralisation principle and measured bone conduction thresholds of patients undergoing middle ear surgery for evaluation of postoperative Sensorineural loss as a result of middle ear surgery. Probable causes of post operative hearing loss in a patient undergoing middle ear surgery are, noise due to drills, continuous suction irrigation, vibrations, inner ear injury, manipulation of ossicles and a few unknown reasons.     

老年与中青年突发性聋的疗效比较

目的 了解老年与中青年突发性聋患者的不同临床特征和疗效。方法 回顾 分析123例（129耳）突发性聋资料,分成两组：老年组（60≥岁）72例（76耳）,中青年组（＜60岁）51例（53耳）。所有患者行皮质类固醇激素、能量合剂等治疗,一部分患者加用高压氧和东菱克栓酶辅助治疗,5例上述治疗无效患者行甲强龙鼓室灌注治疗。结果分析用χ2检验和ｔ检验。结果 两组治疗后的总有效率（痊愈、显效、有效之和）分别为59.2％和69.8％,差异无统计学意义（χ2＝3.26,P＞0.05）,总显效率（痊愈、显效之和）为34.2％和56.6％,差异有统计学意义（χ2＝6.37,P＜0.05）。患者发病至就诊时间差异有统计学意义（t=2.14,P＜0.05）。治疗前健患侧听力差分别为(28.5±13.9)dB和(42.1±28.2)dB ,差异有统计学意义（t=3.64,P＜0.01）。老年组患者合并糖尿病、高血脂症（25/76,48/76）的比例较中青年组（9/53,12/53）高。结论 老年突发性聋患者往往有基础的听力损失,发病呈渐进性和隐匿性,虽然总有效率与中青年患者相似,但显效率较低。类固醇激素鼓室灌注经圆窗渗透对一般治疗无效的部分患者仍有效。     

聪耳熄鸣丸治疗感音神经性耳聋、耳鸣的疗效观察

目的 研究聪耳熄鸣丸治疗感音神经耳聋、耳鸣的临床疗效。方法 随机选取来我院就治的感音神经性聋、伴耳鸣病人120例,分治疗组和对照组进行对比。结果 治疗组和对照组对耳鸣的疗效,分别为68％和40％;治疗组和对照组对耳聋的疗效分别为68％和50％,有显著性差异。结论 聪耳熄鸣丸是治疗感音神经性耳聋,耳鸣的有效药物。     

Missing perilymph but leaking blood-endolymph and vestibulocochlear nerve barriers in idiopathic sudden sensorineural hearing loss: A case study

ObjectivesTo evaluate the pathological changes in the blood-perilymph, blood-endolymph, and blood-nerve barriers of a patient with idiopathic sudden sensorineural hearing loss (SSNHL).MethodsPotential ossification or fibrosis in the inner ear was evaluated using temporal bone CT and MRI acquired using the 3-dimensional T2-weighted sampling perfection with application-optimized contrasts using a flip angle evolution sequence. Pathological changes in the barriers were analyzed by MRI obtained 4 h after a single-dose intravenous injection of gadolinium chelate using a medium inversion-time inversion recovery imaging with magnitude reconstruction sequence.ResultsThe perilymph was absent, while significant enhancements of the vestibulocochlear nerve and the endolymphatic compartments were detected.ConclusionSignificant injuries in the blood-endolymph and blood-vestibulocochlear nerve barriers and disabled perilymph production may contribute to the development of SSNHL with poor response to treatments.     

Middle cranial fossa arachnoid cysts causing sensorineural hearing loss

Arachnoid cyst of the middle cranial fossa has never been reported to present with sensorineural hearing impairment. We report the case of a 15-year-old girl who complained of progressive hearing loss on the right side for 4 years. The pure tone audiometry revealed sensorineural hearing loss with an average of 63 dB, and the auditory brain stem response demonstrated absence of all waves on the right side. Magnetic resonance imaging showed giant arachnoid cysts occupying the bilateral middle cranial fossa and extending to the parietal and prepontine areas with the atrophic change of the right facial-acoustic nerve cord and a decrease in signal intensity of the right cochlea and vestibule. The cystoperitoneal shunting procedure was suggested, but the parents hesitated about the surgical treatment. The patient has not received any surgical intervention, and her hearing has been stable for 1 year during the follow-up period. This case represents the first report that sensorineural hearing loss is a possible presenting symptom when arachnoid cysts of the middle cranial fossa are massive.     

Epileptiform electroencephalogram abnormality in children with congenital sensorineural hearing loss

Objectives

This work was designed to study electroencephalogram findings in children with congenital sensorineural hearing loss and correlate these findings with the SNHL parameters as duration, etiology, severity, and type.

Methods

Ninety children with bilateral congenital sensorineural hearing loss served as the study group. They were free from any neurological disorders or symptoms that are commonly associated with abnormal electroencephalogram as convulsions or loss of consciousness. Twenty children having normal hearing with no history of otological or neurological disorders served as the control group. All children participating in the study were subjected to full medical and audiological history, otological examination, neurological examination, audiological evaluation and electroencephalogram recording.

Results

Mean age of the children in the control group was 3.56 ± 2.1 years and mean age of the children in the study group was 3.8 ± 2.2 years. While none of the control children had abnormal electroencephalogram, 38 (42.2%) of children with congenital SNHL had epileptiform electroencephalogram abnormality. The epileptiform abnormality was generalized in 14 children (36.8%), focal temporal in 17 children (44.7%) and focal other than temporal in 7 children (18.4%). According to the hemispheric side affected, the abnormality was right in 14 children (36.8%), left in 10 children (26.3%) and bilateral in 14 children (36.8%). No statistically significant predominance of specific site or side of the epileptiform abnormality was found. Similarly, no statistical significant prevalent of the epileptiform abnormality was found in relation to the age or sex of children, duration of hearing loss or etiology of hearing loss (i.e., genetic vs. neonatal insults). On the other hand, the epileptiform abnormality was statistically prevalent in children with moderate degree of hearing loss, and in children with auditory neuropathy spectrum disorder.

Conclusions

The epileptiform electroencephalogram abnormality is a common finding in children with congenital sensorineural hearing loss especially those with auditory neuropathy spectrum disorder, suggesting the affection of the central nervous system despite the absence of neurological symptoms or signs. These findings raise the question of the requirement of medical treatment for those children and the effect of such treatment in their rehabilitation.