改良Contour试验测试正常听力与感音神经性听力损失儿童响度增长

目的 应用改良Contour试验测试听力正常与感音神经性听力损失儿童的响度增长,探讨改良Contour试验的临床应用价值.方法 对26例(26耳)听力正常儿童、25例(25耳)感音神经性听力损失儿童采用临床对照试验用改良Contour试验进行响度测试.在初测2～3周后进行复测.采用多因素方差分析进行统计学分析.结果 在听力正常组、不同程度听力损失组间(F=1386.32,P值＜0.001)及不同响度分级间(F=682.21,P＜0.001)响度的差异均有统计学意义.听力正常组与听力损失组动态范围间差异有统计学意义(F=214.26,P＜0.001).感音神经性听力损失儿童响度增长特征:听力损失越重,响度增长越快,动态范围越小.听力正常组与听力损失组初复测结果高度相关(r值分别为0.97和0.91),可信度较高.结论 儿童助听器验配应尽可能获取个体的响度测试结果以帮助助听器调试到最适合该患儿使用.改良Contour试验易为儿童接受、可靠性好,值得进一步研究用于临床,以帮助提高助听器验配质量.     

Attitude and knowledge of hearing loss among medical doctors selected to initiate a residency in Mexico

Early diagnosis and intervention of hearing loss are directly influenced by the knowledge and attitude towards this condition among medical personnel, particularly in countries where screening is not performed routinely. The objective of this paper was to evaluate the attitude and knowledge of hearing loss in a group of physicians. A questionnaire with five Likert-type items and five multiple choice and fill-in-the-blank items was completed by 2727 physicians selected to start a medical residency. Results suggested that physicians’ knowledge level on the matter is deficient and their attitude is far from the ideal; however, physicians selected for a residency in audiology showed slightly better results.     

全植入式助听器治疗中重度耳聋

目的 探讨全植入式助听器(Carina)治疗中重度和重度耳聋的安全性和效果.方法 分析随访12个月以上的三例接受全植入式助听器手术的中重度至重度耳聋患者术后康复的效果.比较术前术后纯音测听和言语测听的差异,观察植入体日常使用情况及与传统助听器效果的比较.结果 手术顺利,无并发症.术后手术侧无助听听阈(听力级,下同)平均升高8.3 dB,四个频率(0.5、1、2、4 kHz)纯音听阈功能性增益平均为35.4 dB,言语听阈平均降低22.5 dB,患者主观满意度评分明显高于传统助听器.结论 全植入式助听器是治疗中重度和重度耳聋患者的有效方法 ,其远期疗效需要进一步观察.     

Tinnitus with or without hearing loss: are its characteristics different?

The present study was carried out in order to analyze the clinical characteristics of tinnitus both in normal hearing subjects and in patients with hearing loss. The study considered 520 consecutive tinnitus sufferers. The following parameters were considered: age, sex, subjective disturbance caused by tinnitus, subjective judgment of tinnitus intensity, tinnitus laterality, tinnitus duration, tinnitus measurements, normal hearing or associated hearing loss. Among the patients considered, 223 have normal hearing while 297 have a hearing deficit. The hearing impairment was found to be in most cases of sensorineural type. The subjective discomfort is higher in presence of hearing loss. Subjects with hearing loss needed significantly higher masking levels. No evident differences in the residual inhibition (RI) result between the two groups were found. The present study confirms that tinnitus is most frequently associated with hearing loss. The characteristics of tinnitus in normal hearing subjects, except for the subjective judgment of tinnitus intensity, the pitch and the RI, are significantly different for those observed in subjects with hearing loss. The association of tinnitus and hearing deficit seems to increase the perceived severity of the symptom.     

A case of unilateral sensorineural hearing loss caused by a venous malformation of the internal auditory canal

Intracranial vascular malformations can be classified as telangiectasis, varices, cavernous malformations, venous malformations (VMs), or arteriovenous malformations. VMs are congenital vascular malformations of the brain thought to be anomalies of the normal venous drainage. VMs are the most common intracranial vascular malformations documented by brain imaging and by autopsy series. However, vascular lesions of the internal auditory canal (IAC) are extremely rare. We report here a case of a VM arising within the IAC and expanding to the brainstem, causing a unilateral profound sensorineural hearing loss (SNHL). To the best of our knowledge, this is the first documented case of a VM as a rare cause of a unilateral SNHL in a child.     

Advantages of a new miniature hearing aid for mild to moderate hearing loss

OBJECTIVES/HYPOTHESIS: To evaluate the performance of a new, miniature, behind-the-ear hearing aid designed for individuals with mild to moderate high-frequency hearing loss who need an aid but are reluctant to try one. The aid is essentially invisible, leaves the ear canal open, and can be fit in less than 30 minutes without an ear impression. The cost is less than $500. STUDY DESIGN: A 4-week trial of the aid in 63 ears (62 subjects) with mild to moderate bilateral hearing loss. METHODS: A questionnaire was completed at the end of the study by each subject asking them to evaluate several features of the aid (cosmesis, comfort, understanding speech, amplification, and so forth) and to compare their unaided performance in quiet and in noise with the test hearing aid. A rating scale of 1 to 10 was used, with 10 being excellent and 1 poor. RESULTS: Subjective improvement in understanding speech in both quiet (5.8-->7.3) and noise (4.6-->5.9) occurred with the aid. Cosmesis, comfort, and appearance were highly rated (mean scores, > 8). CONCLUSIONS: This aid appears to have several features (comfort, cost, performance, and cosmesis) that make it ideal as a first aid for patients with mild to moderate losses.     

Characteristics of tinnitus with or without hearing loss: Clinical observations in Sicilian tinnitus patients

Objective

To analyze the clinical characteristics of tinnitus both in normal hearing subjects and in patients with hearing loss.

Methods

The study considered 312 tinnitus sufferers, 176 males and 136 females, ranging from 21 to 83 years of age, who were referred to the Audiology Section of the Department of Bio-technology of Palermo University. The following parameters were considered: age, sex, hearing threshold, tinnitus laterality, tinnitus duration, tinnitus measurements and subjective disturbance caused by tinnitus. The sample was divided into two groups: Group 1 (G1) subjects with normal hearing; Group 2 (G2) subjects with hearing loss.

Results

Among the patients considered, 115 have normal hearing while 197 have a hearing deficit. There is a slight predominance of males respect to females that is more evidenced in G2 (61.42% of males vs. 38.58% of females). The highest percentage of tinnitus results in the decades 41–50 for G1 and >70 for G2 with a statistically significant difference between the two groups (P < 0.0001).The hearing impairment results in most cases of sensorineural type (74.62%) and limited to the high frequencies (58.50%), moreover the 72.10% of the patients with SNHL had a high-pitched tinnitus while the 88.37% of the patients high-frequency sensorineural hearing loss had a high-pitched tinnitus (P < 0.0001). As for the subjective discomfort, the catastrophic category resulted most representative among subjects with normal hearing with a statistically significant difference between the two groups but no significant correlation was found between the level of tinnitus intensity and the tinnitus annoyance confirming the possibility that tinnitus discomfort is elicited by a certain degree of psychological distress as anxiety, depression, irritability and phobias that do not allow the phenomenon of the ‘habituation’.

Conclusion

This work, according to literature data, suggests that the hearing status and the elderly represent the principal tinnitus related factors; moreover tinnitus characteristics differ in the two groups for tinnitus pitch. There is, in fact, a statistically significant association between high-pitched tinnitus and high-frequency SNHL suggesting that the auditory pathway reorganization induced by hearing loss could be one of the main source of the tinnitus sensation.     

Quantification of hearing loss in patients with posterior semicircular canal dehiscence

Conclusion: Patients with posterior semicircular canal dehiscence (PSCD) have low frequency conductive hearing loss similar to patients with superior semicircular canal dehiscence (SSCD) secondary to a pathologic third window. Objectives: PSCD can result in conductive hearing loss, but the magnitude of this hearing loss remains to be quantified. Patients with SSCD have been shown to have low frequency conductive hearing loss. The underlying pathophysiology of hearing loss from PSCD and SSCD is similar and related to a pathologic third window. Method: A PubMed search was completed for a meta-analysis of patients with PSCD. Articles with quality audiograms were obtained. Air conduction thresholds for ears with posterior semicircular canal dehiscence were compared to the opposite ear as well as normal control data. Results: Eight articles with 21 patients with PSCD and quality audiograms were included. Two patients had bilateral PSCD and one of those was excluded because hearing thresholds were at the limit of the audiometer. Patients with posterior semicircular canal dehiscence have statistically significant lower air conduction thresholds in frequencies at and below 2000 Hz.     

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

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.     

遗传性聋一个家系五例的分子病因学分析

目的 分析遗传性聋一个家系五例耳聋患者的分子病因学.方法 对一个遗传性聋家系的5例耳聋患者进行GJB2,线粒体DNA 12S rRNA A1555G,SLC26A4和MITF 4种基因进行突变检测.结果 此家系的5例耳聋患者中有3例检测到携带致病性突变.其中两人携带Mt DNA A1555G点突变.一人携带SLC26A4 279T>A/2168A>G复合杂合突变;另外两人虽表现为Waardenburg综合征但未查找到突变位点.结论 耳聋患者间的同证婚配可导致家族内遗传性聋分子病因的复杂化.     

130例婴幼儿听力损失的听力学和基因学分析

目的 探讨0～3岁听障婴幼儿的基因学致聋因素,分析基因与环境致聋因素的相关性.方法 对130例听障患儿进行了客观听力学评估,并进行线粒体DNA(mtDNA)12SrRNA、GJB2基因、SLC26A4基因检测.84例患儿完成了影像学评估.结果 130例患儿中,前庭水管扩大综合征54例,听神经病7例.有明确高危因素患儿85例,占65.4%(85/130);23例有耳毒性药物用药史,其中以庆大霉素居多;13例有家族史,2例家族中存在近亲结婚.基因检测结果 显示130例患儿中,42例检测到SLC26A4基因致病突变,占32.3%(42/130);6例患儿检测到GJB2基因致病突变,占4.6%(6/130);130例患儿中未检测到mtDNA 12S rRNA C1494T以及A1555G突变.结论 在0～3岁的婴幼儿听力筛查诊断中,36.9%的听障患儿为遗传因素致聋.早期进行听力诊断联合分子诊断可提早发现病因,有利于随后的干预和康复治疗,同时也有利于进行有效的遗传咨询.     

新生儿听力损失高危因素与听力筛查研究进展

目的随着听力筛查工作不断的推广以及听力筛查技术不断的革新,新生儿听力损失的高危因素也逐渐明确,本文就以下听力损失高危因素进行相关阐述：①低胎龄出生儿;②新生儿感染性疾病;③新生儿缺氧性疾病;④高胆红素血症;⑤耳毒性药物的影响;⑥遗传因素;⑦环境因素,并对相关高危因素损害听力机制进行阐明;探讨了目前各种听力筛查方法在听力筛查工作中的优势、不足之处以及改进方法。进而得出结论：针对有高危因素的新生儿,应严格按照听力筛查工作流程对其进行严格的筛查,同时联合耳聋基因筛查,长期对其听力进行检测和随访。     

An office procedure to detect vestibular loss in children with hearing impairment

As coexisting vestibular and cochlear lesions are of etiological importance, evaluation of children with congenital or early acquired hearing impairment (HI) should include vestibular assessment. A rotation test requires specific equipment and allows only detection of bilateral vestibular impairment. An impulse or head thrust test allows assessment of one ear at a time, detects more pronounced caloric side differences and can be performed without any equipment. We report a consecutive series of children with profound sensorineural HI investigated at a tertiary hospital unit. Age at taking first steps without help, the results of temporal bone images (CT/MRT) and vestibular tests were collected retrospectively from patient files. The children were 12 to 90 months old at the time they attended both a rotation and an impulse test. All 14 children cooperated in the impulse test, and 12 completed the vestibular rotation test successfully. Three out of 14 children tested so far have been confirmed to have a bilaterally pathological vestibulo-ocular reflex confirmed both in the rotation test and the impulse test. Our results show that both the rotation test and the vestibular impulse test can be successfully performed on small children at a regular outpatient appointment.     

Osteopathia striata with cranial sclerosis and hearing loss

Osteopathia striata is a manifestation of several bone dysplasias. In association with cranial sclerosis, it represents a separate entity that is not limited to the bones, but may also affect other structures, leading to an abnormal face, cleft palate, deafness, heart defects and vertebral anomalies. Neurological findings range from normal development to marked retardation with hydrocephalus, cranial nerve deficiencies and deafness. So far, only a few patients have been described in the literature, demonstrating autosomal dominant inheritance. Here, we report a spontaneous mutation of inheritance. The parents and three sisters of the affected child are healthy. In 78.6% of the reported cases with osteopathia striata and hearing loss, a conductive hearing loss was described. Surgical attempts to mobilize the middle-ear bones were unsuccessful because of swelling and augmented growth of the bones in the middle ears. Here the mobilization of the eardrum was not attempted because the middle ear cavities were epidermized. This is the second case in the literature in which a myringotomy was not done because the middle ear was completely epidermized. As a possible alternative, children with osteopathia striata and cranial sclerosis with hearing loss should be fitted with hearing aids because surgery of the middle ear probably will be difficult or perhaps not possible.     

急性双耳感音神经性聋19例病因及临床特点分析

目的 探讨急性双耳感音神经性聋的病因及其相应的临床特点,为临床诊疗提供借鉴.方法 回顾性分析2000年11月至2013年12月期间共19例以急性双耳感音神经性聋为主要首发症状患者的临床资料,分别对其临床特征、全身系统检查、实验室检查、听力学及影像学特征进行整理分析,归纳病因,并追踪临床诊疗及预后.结果 19例患者中非耳科系统疾病15例,占78.9％.大部分病例呈现多系统、多器官受累:3例为中枢神经系统疾病,包括真菌性脑膜炎、结核性脑膜炎以及病毒性脑炎各1例,耳聋为双侧进行性加重,伴有发热、头痛、头晕,恶心、呕吐及精神状态的改变等,言语识别率下降,言语识别阈不成比例地明显差于纯音听阈;5例为免疫系统疾病,包括抗中性粒细胞胞质抗体相关小血管炎、复发性多软骨炎、类风湿性关节炎和系统性红斑狼疮,耳聋累及双侧、进展快,同时存在自身免疫性疾病的特点;5例为血液内分泌系统疾病,包括糖尿病、白血病和甲状腺功能低下,听力下降多为双侧对称性、进展性;4例为耳科系统疾病,包括大前庭水管综合征和突发性聋;另有2例为药物性聋.在经过针对病因治疗后,19例患者中,痊愈1例,显效3例,有效7例,无效(亦包括死亡及放弃治疗的病例)8例,总有效率为57.9％.结论 急性双耳感音神经性聋多与全身系统性疾病相关,临床诊治上应详细分析病史、临床表现,并完善实验室检查、听力学及影像学等检查以明确诊断,针对病因积极治疗.     

Utility of a multidisciplinary approach to pediatric hearing loss

Introduction

Because management of hearing loss (HL) often requires multiple specialists, a multidisciplinary clinic, Pediatric Hearing Management Clinic, (PHMC) was established to coordinate care for children with newly diagnosed HL.

急性低频感音神经性聋的临床研究

目的探讨急性低频感音神经性聋（acute low-frequency sensorineural hearing loss,ALHL）的临床特征和疗效,提高对该疾病的认识和鉴别能力。方法回顾性分析42例（42耳）ALHL的临床表现、听力学检测结果和2～5年的随访情况,总结ALHL的临床发病特征。结果本组42例（42耳）ALHL呈急性发病,年龄27～44岁。男16例（38.10%）,女26例（61.90%）。20例（20耳）伴低调耳鸣,17例（17耳）伴耳部堵闷,11例（11耳）伴听觉过敏现象,6例（6耳）伴有头昏。0.125～1kHz平均听阈为（38.74&#177;4.62）dBHL,2～8kHz平均听阈为（13.52&#177;3.86）dBHL,治疗后上述平均听阈分别为（21.05&#177;9.74）dBHL和（19.85&#177;9.44）dBHL。所有病人的听性脑干反应（auditory brainstem response,ABR）各波潜伏期及波间期正常。畸变产物耳声发射（distortion product otoacoustic emissions,DPOAE）在低频区引出率为16.67%,高频区引出率为85.71%。结论ALHL以单侧发病为主,常伴低调耳鸣、耳部堵闷及胀满感。该病多见于青年女性。纯音听阈以低频听阈升高为特征,ABR正常。病变可能位于耳蜗顶周。