Idiopathic sudden sensorineural hearing loss in children

OBJECTIVE: Although idiopathic sudden sensorineural hearing loss (ISSHL) is a frequent disease in adults, less is known about incidence and treatment of ISSHL in children. METHOD: A retrospective chart analysis was performed to evaluate the frequency of ISSHL in children aged under 18 years between 2000 and 2003, who were treated in our department. Children received prednisolone intravenously at an initial dose of 3mg/kg bodyweight. Prednisolone dose was reduced to half every second day. The medication was given for a maximum of 14 days or finished 2 days after the hearing normalized in pure-tone audiometry. The follow-up was continued between 3 and 14 months. RESULTS: The complete recovery rate was 57%, and the partial recovery was 36%. Initial hearing loss of 50dB and more was predictive for poor outcome in children (p=0.028). Presence of tinnitus was without relevance for the outcome. The incidence of ISSHL in the local area of about 250,000 inhabitants was 1/10,000 in children. CONCLUSION: ISSHL seems to be a less frequent disease in children than in adults. Severe initial hearing loss is coupled with poor outcome. Under treatment with prednisolone hearing improvement was found in 13 of 14 patients.     

Prognostic factors of sudden hearing loss in children

OBJECTIVE: Sudden hearing loss in children under 15 years old is rare and its pathophysiology remains unclear. The aim of this retrospective study was to define prognostic factors of sudden hearing loss in children under 15 years old. METHODS: Among the patients referred to our institution during the period 1990-1999 for sudden hearing loss, 12 children were considered eligible for this study (mean age=9.5 years old). Patients were divided into three groups according to hearing recovery and seven putative prognostic factors were analyzed. RESULTS: Total hearing recovery (group I: 90-100%) occurred in 28.5% of cases, partial hearing recovery (group II: 11-89%) occurred in 28.5% of cases and absence of recovery (group III: 0-10%) was reported in 43% of cases. Factors such as age, sex, and bilaterality of hearing loss were not correlated with hearing recovery. CONCLUSIONS: Initial severe hearing loss, associated vertigo and 'downward' audiometric curve were three negative prognostic factors of hearing recovery. Tinnitus has only been reported in children with partial or total hearing recovery.     

突发性聋与前庭神经炎病因学比较研究

目的 通过比较研究突发性聋和前庭神经炎,进一步探讨两类疾病在病因学上和前庭神经损伤的差异.方法 观察眩晕诊疗中心2004年1月至2007年7月间37例突发性聋、30例突发性聋伴发眩晕和40例前庭神经炎的前庭功能(前庭双温试验、前庭诱发的肌源性电位和主观垂直视觉检查)和高刺激率ABR的特点及差异.结果 (1)突发性聋与前庭神经炎两组患者年龄分布差别显著不(P>0.05).(2)高刺激率ABR:前庭神经炎组,患侧高刺激ABR异常率为33.3%;突发性聋组,患侧异常18例,患侧异常率占69.3%(18/26).突发性耳聋组高刺激率ABR异常率明显高于前庭神经炎组(P<0.01).(3)突发性聋与前庭神经炎两组患者前庭损伤的差异性比较:前庭神经炎组,前庭上神经损伤占50%,前庭下神经损伤占10%,前庭上下神经损伤占40%;在突发性耳聋组,前庭上神经损伤占53.3%,前庭下神经损伤占6.7%.前庭上下神经损伤占40%.两组在前庭神经损伤的模式上差别不显著(P>0.05).结论 突发性聋(伴眩晕)的主要病因可能是内耳微循环障碍,而前庭神经炎的主要机制可能是病毒感染.突发性聋伴眩晕的和前庭神经炎前庭神经损伤模式相似,均主要累及前庭上神经.     

Prevalence and etiology of hearing loss in rural Nicaraguan children

OBJECTIVE/HYPOTHESIS: The prevalence and causes of pediatric hearing loss (HL) in the developing world are largely unknown. Infectious sequelae, ototoxic medications, and genetic causes may play a larger role in developing countries. In addition, the significance of GJB2 mutation gene in poorly developed areas remains unclear. The intent of this study is to investigate the prevalence and etiology of HL in children living in a remote, impoverished region of northern Nicaragua. STUDY DESIGN: Cross-sectional study. METHODS: Clinical data from two sources were analyzed: data from screening examinations performed in rural schools in the Department of Jinotega, Nicaragua (group A) and pediatric HL patients seen at the Otolaryngology and Audiology Clinic in Jinotega, Nicaragua (group B). Patients with congenital HL were offered a genetic test for GJB2 mutations. Comparisons were made using parametric (analysis of variance) and nonparametric (Kruskal-Wallis) tests. RESULTS: School-based screening examinations (group A) revealed a high prevalence of significant HL (>30 dB) of 18%. The majority of these children had normal otoscopic examinations (58%). A family history of HL was seen in 24% of children who failed screening exams. Positive family history was more common in patients with HL (P < .01) and in specific schools (P < .05). Clinic-based evaluations (group B) reveal a population with predominantly severe-profound HL. Physical dysmorphism was common, yet identifiable syndromic HL was rare. Although familial HL was common (33%), there were no pathologic GJB2 mutations. Other common risk factors in this population were maternal infection during pregnancy, neonatal distress, low birth weight or prematurity, and gentamicin exposure. CONCLUSIONS: HL in this rural, third world environment is more prevalent, and the etiologies responsible in this study group are different from those encountered in industrialized nations. Poor perinatal health care, infectious causes, gentamicin exposure, and hereditary HL are potentially preventable causes that play a major role in this population.     

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.     

Systematic review of the etiology of bilateral sensorineural hearing loss in children

OBJECTIVE: Identification of the etiology of sensorineural hearing loss (SNHL) in children facilitates management and provides important prognostic information. In recent years, the etiology of bilateral SNHL in children has changed due to advances in genetic testing and treatment of perinatal infections. The objective of this study was to determine the frequency of etiologies of moderate-profound bilateral sensorineural hearing loss (SNHL) in children. METHODS: The English literature was searched in Medline for articles published between 1966 and 2002. The inclusion criteria were studies involving bilateral SNHL >/=40dB in children less than 18 years of age. The studies were required to account for all patients, and provide a breakdown of etiologic factors. Etiologies investigated included genetic and non-genetic (prenatal, perinatal, postnatal). To compare differences between the frequencies of etiologies a two-sample t-test was performed assuming unequal variance. Studies were stratified according to perceived confounders: start date of study, study design, and degree of hearing loss. RESULTS: Seven hundred and eighty abstracts were screened for relevancy. Forty-three studies satisfied the inclusion criteria. The common etiologies of bilateral SNHL were unknown (41.5%), genetic non-syndromic (27.2%), prenatal (11.5%), perinatal (9.7%), postnatal (6.6%), and genetic syndromic (3.5%). Unknown and Rubella were significantly less frequent etiologies in the more recent studies, while genetic non-syndromic, asphyxia and prematurity were more common. Genetic non-syndromic hearing loss was more frequent in the prospective studies compared to the population and retrospective studies, but this difference was not significant. Genetic non-syndromic hearing loss was more common among patients with profound hearing loss. CONCLUSION: Accounting for the recent decline in infectious etiologies, the most common causes of bilateral SNHL are unknown (37.7%), genetic non-syndromic (29.2%), prenatal (12%), perinatal (9.6%), postnatal (8.2%), and genetic syndromic (3.2%).     

儿童及成人突发性聋预后的对比分析

目的对比分析儿童及成人突发性聋预后的相关因素,为临床实践及患者预后评估提供依据。方法回顾性分析2008年1月~2016年12月住院治疗的237例（258耳）突发性聋患者的临床资料,其中儿童突发性聋患者（儿童组）26例（29耳）,成人突发性聋患者（成人组）211例（229耳）,采用单因素及多因素分析的方法对比分析两组突发性聋患者的年龄、性别、耳侧、初诊时间、就诊听阈、听力曲线、有否伴有耳鸣、眩晕及耳闷等因素对预后的影响。结果儿童突发性聋患者人数占总人数的11.0%;儿童组就诊听阈平均为（88.7±15.8）dB,高于成人组［（71.8±23.6）dB,(P＜0.05)］;儿童组听力曲线为全聋型的占69.0%,高于成人组（44.5%,P＜0.05）;经治疗后,儿童组的总体有效率为51.7%,成人组总体有效率为45.4%,两者差异无统计学意义（P＞0.05）。对儿童组预后的相关因素分析显示就诊听阈及伴发眩晕对疗效的影响具有统计学意义(P＜0.05);成人组中,初诊时间、就诊听阈及伴发眩晕对疗效的影响具有统计学意义（P<0.05）。结论儿童突发性聋患者就诊时听力损失较成人重,但治疗后两者总体有效率无明显差异（P>0.05）;儿童及成人突发性聋患者中听力损失程度越轻且不伴发眩晕者预后较好;就诊时间越早越有利于成人突发性聋患者的预后;而年龄、性别、耳侧以及是否伴发耳鸣、耳闷对儿童及成人突发性聋的预后无明显影响。     

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.     

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.     

Doppler ultrasonography can be useful to determine the etiology of idiopathic sudden sensorineural hearing loss

Objective

This study aims to evaluate clinical features of both carotid and vertebral arteries in the idiopathic sudden sensorineural hearing loss (ISSNHL) patients and healthy individuals to contribute additional knowledge on vascular compromise theory.

儿童突发感音神经性聋:病因、治疗方法及疗效

尽管已有大量关于成人突发性感音神经性聋(SSNHL)研究的文章发表,但关于儿童SSNHL的数据有限。研究表明,每年SSNHL的发病率可在10.7/10万至27/10万之间。研究表明,SSNHL的发病率随着年龄的增长而增加,18岁时每10万人中有8人,65岁时每10万人中有70人患病。SSNHL是一种耳科急症,必须尽早治疗,以避免永久性听力损失。这对儿童特别重要,因为早年听力损失可能会影响言语和语言发展,影响学业和社会表现;另一个问题是年龄很小的患者无法报告他们的听力损失,这可能导致在治疗最有效的急性期内常常不能被发现。     

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.     

Tube function in sudden hearing loss

The function of the Eustachian tube was assessed in 50 patients suffering from sudden hearing loss, using the pressure chamber impedance method. We investigated the existence of a relationship between sudden hearing loss and the occurrence of a patulous tube. We could not observe more patulous tubes in patients with sudden hearing loss compared to a control group with healthy ears. We registered the parameters "tissue resistance pressure" and "tubal opening pressure", both characterising the passive qualities of the Eustachian tube. There was no indication of a facilitated passive tubal opening nor a tendency to a patulous tube among our patients. Statistical evaluation of these parameters showed no significantly different tubal function on comparing the right and the left ear, as well as the ear affected by sudden hearing loss and the healthy ear. We conclude that there is no relationship between sudden hearing loss and patulous tube.