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.     

A sudden bilateral hearing loss caused by inner ear hemorrhage

IntroductionLabyrinthine hemorrhage is a rare cause of sudden deafness and generally concerns only on one side.Case summaryAn 84-year-old man with a past medical history of myelomonocytic chronic leukemia (CMML) suffered from sudden bilateral hearing loss associated with vertigo. The audiogram revealed a left cophosis and a right profound deafness. Videonystagmography showed a left vestibular deficit. The MRI showed a spontaneous strong T1 weighted signal in the left and right labyrinths, corresponding to a bilateral inner ear hemorrhage (IEH). Dizziness resolved rapidly following vestibular physiotherapy, in contrast to hearing which did not improve at all and let the patient isolated in his environment. The patient successfully underwent cochlear implantation so that he could communicate.DiscussionMost IEHs are unilateral and due to anticoagulants treatments and hematological diseases. Only rare cases have described bilateral labyrinth hemorrhage. This is the first case reported of bilateral labyrinth hemorrhage due to CMML.     

双侧突发性耳聋患者临床特征与预后分析

目的 分析双侧突发性耳聋患者的临床特征及预后效果,了解与该病发生及影响预后的相关因素.方法 对12例双侧突发性耳聋患者的临床资料进行系统性分析,包括临床表现、听力学检查、血液学、影像学检查、治疗方法和疗效评估等,分析致病和影响预后的危险因素,总结该病发生发展及转归的特点.结果 12例患者痊愈2耳,有效1耳,总有效率为12.5%.患者双耳同时发病7例,相继发病5例.可明确突聋诱因的6例,首发症状为听力下降21耳、耳鸣17耳,眩晕8耳.12耳既往有听力损失和耳鸣病史,发病后≤7天治疗者20耳,>7天4耳.12例患者听力损失呈下降型听力曲线15耳,治疗有效1例;全聋型5耳,切迹型2耳,治疗无效;山型1耳,平坦型1耳,痊愈.白细胞计数异常8例,在听力损失程度、耳鸣评价方面,外周血白细胞计数异常与正常患者间差异有统计学意义(P<0.05).免疫抗体检查异常3例;IgE异常增高3例;血清促甲状腺激素测定异常增高1例;葡萄糖增高3例、血清脂质和脂蛋白异常7例,脂类代谢功能异常4例.结论 双侧突发性聋的病情复杂,预后较差.详细分析病史、临床表现和特异性的血液及免疫学系统检查,有助于制定个性化诊疗方案.     

The double-membrane break syndrome in sudden hearing loss.

Some patients with sudden hearing loss actually have two membrane breaks, one at the oval or round window and one further inside the cochlea. One may heal spontaneously and the other not, or both, or neither. It is very likely impossible to detect this early from the threshold audiogram, because the intracochlear breaks allows endolymph and perilymph mixing which spreads over much of the anatomically normal clchlea causing diffuse loss of function. This theory is illustrated by three case histories.     

Iloprost-induced sudden hearing loss

We report a patient who developed sudden, bilateral, sensorineural hearing loss during therapeutic use of iloprost for Raynaud's phenomenon. The sudden hearing loss was attributed to iloprost use and completely reversed in eight days with conservative therapy. Iloprost may be a potentially ototoxic drug, causing sudden hearing loss.     

Functional hearing loss presenting as sudden hearing loss: a case report

A case of functional hearing loss presenting as sudden sensorineural hearing loss in the only hearing ear of a musician is presented. Pure-tone audiometric evaluation showed good intratest and intertest consistency. The pitfalls of diagnosis, ultimately made by brain stem evoked response audiometry, are discussed in light of the literature on sudden and functional hearing loss. Psychiatric evaluation revealed features consistent with hysterical conversion. It is argued that it is important to establish the exact etiologic agent of functional hearing loss despite the difficulty of diagnosis so that the patient may receive appropriate treatment.     

Hemophagocytic syndrome in the course of sudden sensorineural hearing loss

Objective: This paper aims to comprehensively document a rare case of sensorineural hearing loss accompanied by hemophagocytic syndrome which could be one of the causes of cochlear dysfunction. Methods: A 63-year-old man presented with right-sided sudden hearing loss and dizziness and pure-tone audiometry showed a right-sided sensorineural hearing loss. The patient was treated with steroid pulse infusion therapy, ATP and vitamin B(12). Results: Bone marrow specimen revealed hemophagocytic cells and the diagnosis of hemophagocytic syndrome was made. A blood culture grew Capnocytophaga. The prognosis for hearing recovery was poor. Conclusion: Although viral infection and cochlear circulatory dysfunction have been suggested to be associated with sudden sensorineural hearing loss, hemophagocytic syndrome due to bacterial infection which produces high cytokine levels might cause dysfunction of the inner ear.     

突发性极重度聋与全聋的预后特点及差异

Objective To clarify the different prognostic characteristics between profound sudden sensorineural hearing loss (SSNHL) and total SSNHL. Methods The patients with SSNHL who visited Eye Ear Nose and Throat Hospital from June 2007 to September 2008 were reviewed retrospectively. All the 204 patients, with pure tone average (PTA) threshold more than 90 dB, were enrolled and divided into two groups, including total SSNHL and profound SSNHL groups. The relationship between recovery rate and prognostic factors including the age, complications, time period between onset and therapy was analyzed.Results There were 57 cases of total SSNHL and 147 cases of profound SSNHL in this series. Tinnitus was complained in more than 90% of the patients in both groups, which was higher than that of dizziness and ear fullness. Dizziness was present in 64. 9% (37/57) patient with total SSNHL group and 45.6% (67/147)patients with profound SSNHL, which had significant difference between the two groups (x2 = 5.72,P =0. 017). The PTA threshold improvement in total SSNHL group and profound SSNHL group was (36. 4 ±19.3) dB and (40. 2 ±21.3) dB respectively, which was no significant difference between the two groups (t = 1. 165 ,P =0. 245). The cured patients were all those received therapy within 1 week following the onset of SSNHL, which was of 2. 6% (1/38) patients in the total SSNHL group and 14. 3% (14/98) patients in the profound SSNHL group(P =0. 045). Furthermore, 3.5% (2/57) patients in total SSNHL group as well as 29. 9% (44/147) patients in profound SSNHL group obtained a good result with PTA threshold ≤50 dB after therapy(x2 = 15.92,P = 0. 001 ). In addition, the favorable prognosis was related with the onsettherapy time point( P = 0. 001 ), but not related to the patients' age. Conclusion Profound SSNHL and total SSNHL though both with PTA threshold > 90 dB had significant differences recovery rate and need to be studied separately.     

突发性极重度聋与全聋的预后特点及差异

目的 研究突发性极重度聋和全聋的预后特点及差异.方法 回顾性研究复旦大学附属眼耳鼻喉科医院2007年6月至2008年9月收治的初始平均纯音听阈(pure tone average,PTA)>90 dB、随访完整的204例患者,分为全聋和极重度聋两组,对两组的预后进行比较,并对预后与年龄、并发症、发病-就诊时间进行相关性分析.结果 全聋组57例,极重度聋组147例,耳鸣、眩晕、耳闷三大伴随症状中,耳鸣的伴随率最高,两组均达90%以上,眩晕的伴随率全聋组为64.9%(37/57),极重度聋组为45.6%(67/147),两组间差异有统计学意义(x2=5.72,P=0.017).治疗后全聋组PTA下降(36.4±19.3)dB,极重度聋组下降(40.2±21. 3)dB,差异无统计学意义(t=1.165,P=0.245).两组痊愈者均为1周内接受治疗者,全聋组痊愈率为2.6%(1/38),极重度聋组为14.3%(14/98),两组间差异有统计学意义(Fisher确切概率法,P=0.045).全聋组治疗后PTA≤50 dB的患者占3.5%(2/57),极重度聋组达29.9%(44/147),两组间差异有统计学意义(x2=15.92,P=0.001).两组的预后与发病-就诊的时间有关(P值均为0.01),与年龄无关.结论 初始PTA>90 dB的突发性聋可分为极重度聋与全聋,两者预后有差异,需分开进行研究.     

突发性极重度聋与全聋的预后特点及差异

Objective To clarify the different prognostic characteristics between profound sudden sensorineural hearing loss (SSNHL) and total SSNHL. Methods The patients with SSNHL who visited Eye Ear Nose and Throat Hospital from June 2007 to September 2008 were reviewed retrospectively. All the 204 patients, with pure tone average (PTA) threshold more than 90 dB, were enrolled and divided into two groups, including total SSNHL and profound SSNHL groups. The relationship between recovery rate and prognostic factors including the age, complications, time period between onset and therapy was analyzed.Results There were 57 cases of total SSNHL and 147 cases of profound SSNHL in this series. Tinnitus was complained in more than 90% of the patients in both groups, which was higher than that of dizziness and ear fullness. Dizziness was present in 64. 9% (37/57) patient with total SSNHL group and 45.6% (67/147)patients with profound SSNHL, which had significant difference between the two groups (x2 = 5.72,P =0. 017). The PTA threshold improvement in total SSNHL group and profound SSNHL group was (36. 4 ±19.3) dB and (40. 2 ±21.3) dB respectively, which was no significant difference between the two groups (t = 1. 165 ,P =0. 245). The cured patients were all those received therapy within 1 week following the onset of SSNHL, which was of 2. 6% (1/38) patients in the total SSNHL group and 14. 3% (14/98) patients in the profound SSNHL group(P =0. 045). Furthermore, 3.5% (2/57) patients in total SSNHL group as well as 29. 9% (44/147) patients in profound SSNHL group obtained a good result with PTA threshold ≤50 dB after therapy(x2 = 15.92,P = 0. 001 ). In addition, the favorable prognosis was related with the onsettherapy time point( P = 0. 001 ), but not related to the patients' age. Conclusion Profound SSNHL and total SSNHL though both with PTA threshold > 90 dB had significant differences recovery rate and need to be studied separately.     

Histopathology of sudden hearing loss

Eleven temporal bones from eight patients who had clinical histories of sudden hearing loss (SHL) were studied to assess the possible etiopathogenesis. The origin of SHL in seven ears from five patients was obscure, but appeared to be due to multiple causes. Common histopathologic changes in the cochlea, although complex, included atrophy of the organ of Corti and loss of cochlear neurons. Loss of cochlear neurons was the main finding in ears of viral infection. Labyrinthine fibrosis and formation of new bone were seen in two ears associated with vascular insult and in two ears of autoimmune disease. Different histopathologic findings causing SHL were observed even in cases with the same etiology. A case of SHL showing endolymphatic hydrops as the main histopathologic finding is described.     

Idiopathic sudden sensorineural hearing loss

Idiopathic sudden sensorineural hearing loss remains one of the major unsolved otologic emergencies. In this paper the most important recent clinical literature is reviewed, a new method of clinical staging is presented, and unexplored potential treatments are presented. The method of clinical staging presented here is based on four elements represented by the acronym HEAR. The individual elements of the staging are hearing threshold (H), elapsed time from onset (E), audiogram shape (A), and related vestibular symptoms (R). Insufficiently explored potential treatments of sudden hearing loss include antiviral drugs, rheologic agents, and free radical scavengers.     

Non-inherited manifestation of bilateral branchial fistulae, bilateral pre-auricular sinuses and bilateral hearing loss: A variant of branchio-oto-renal syndrome

A rare manifestation of branchio-oto-renal like syndrome as an isolated finding with normal chromosomal analysis in a 6 year old child with bilateral branchial simises, bilateral pre-auricular simuses, bilateral auricular malformation, and bilateral hearing loss is presented here.     

Sudden-onset bilateral hearing loss: case report

An ischaemic infarction of the anterior inferior cerebellar artery (AICA) is usually associated with vertigo, hearing loss, facial palsy, nystagmus, or truncal ataxia; it is often accompanied by other brainstem or cerebellar signs. Sudden- onset bilateral hearing loss without associated neurological symptoms is infrequent in the literature. We report a case of sudden bilateral hearing loss, later diagnosed as AICA infarction without other symptoms.     

Vogt-小柳-原田综合征被误诊为突发性聋1例报告

患者,男,39岁,因右耳听力下降1周伴眩晕于2013年12月20日入院。患者1年前无明显诱因出现双耳鸣,持续性,低调;1周前患者突发眩晕,伴视物旋转,恶心呕吐,双耳鸣加重,右耳听力下降。头颅MRI检查无明显异常。纯音听力检查示：右耳感音神经性聋,高频下降,平均听阈为42dBHL,左耳正常。声导抗检查示：双耳A型曲线。发病后多次在神经内科就诊,行改善血液循环、营养神经等治疗,无好转。     

Magnesium treatment for sudden hearing loss

Magnesium treatment has been repeatedly shown to reduce the incidence of both temporary and permanent noise-induced hearing loss. We hypothesized that it might also improve the permanent threshold shift in patients with acute-onset hearing loss. In a prospective, randomized, double-blind, placebo-controlled trial, 28 patients with idiopathic sudden sensorineural hearing loss were treated with either steroids and oral magnesium (study group) or steroids and a placebo (control group). Compared to the controls, the magnesium-treated group had a significantly higher proportion of patients with improved hearing (>10 dB hearing level) across all frequencies tested, and a significantly greater mean improvement in all frequencies. Analysis of the individual data confirmed that more patients treated with magnesium experienced hearing improvement, and at a larger magnitude, than control subjects. Magnesium is a relatively safe and convenient adjunct to steroid treatment for enhancing the improvement in hearing, especially in the low-tone range, in patients with sudden sensorineural hearing loss.