非耳疾就诊患者的听力筛查意愿与筛查结果分析

目的 调查非耳疾就诊患者的听力筛查意愿及分析筛查结果,提高医护人员与患者对听力健康的重视程度,尽可能避免可控因素致听力损害.方法 记录参与调查患者的临床资料,对其中愿意行听力筛查者行纯音听阈测定并分析结果;对拒绝行听力筛查者完成问卷以明确其拒绝原因.结果 280例参与调查的患者中,72例愿意行听力筛查,208例拒绝听力筛查;拒绝筛查的主要原因为自觉听力正常、自觉听力与本次就诊疾病无关;年龄大于或等于60岁、家属或身边重要人士有听力疾患者较对照组更愿意接受听力筛查(均P＜0.05);约40.00％长期服药患者存在听力障碍,较对照组更多(P＜0.05);自觉听力状况与纯音听阈测定结果之间差异有统计学意义(P＜0.05).结论 听力障碍在非耳疾就诊患者中有极高的患病率,高龄、长期服药等患者应特别重视听力健康状况;自觉听力正常者仍应警惕潜在的听力障碍.     

山东省5 664例听力障碍患者听力检测联合失聪基因检测结果分析

背景失聪是影响人类健康和造成人类残疾的常见疾病,其发病率一直在各类残疾中高居首位。引起失聪的原因很多,其中遗传因素约占60%,通过失聪基因筛查和家系分析明确是否为遗传性失聪,为失聪患者提供相应的遗传咨询服务,以阻断失聪的代代相传。目的了解山东省听力障碍患者听力损失情况和失聪基因突变频率,明确听力障碍致病原因。方法对2016—2020年参加山东省听力障碍人士失聪基因检测项目的5 664例持听力残疾证或持听力诊断证明的听力障碍患者进行遗传性失聪基因筛查检测,通过纯音测听检测听力障碍患者听力损失情况,应用常见的4个基因15位点遗传性失聪基因芯片进行基因检测。结果5 664例听力障碍患者中,听力残疾一级3 891例,听力残疾二级1 463例,听力残疾三级188例,听力残疾四级73例,其余49例（小耳畸形38例,外耳道封闭11例）。5 664例听力障碍患者检测出失聪基因突变者2 503例,其中GJB2基因突变1 227例（携带率为21.66%）,SLC26A4基因突变975例（携带率为17.21%）,线粒体12SrRNA基因突变97例（携带率为1.71%）;GJB3基因突变158例（携带率为2.79%）;双基因杂合突变46例（携带率为0.81%）。GJB2、SLC26A4基因突变在听力等级中比较一致,属于热点突变。携带GJB2基因、SLC26A4基因突变患者听力一级比例高于听力二级（P<0.05）。结论山东省听力障碍患者中常见的4个遗传性失聪基因热点突变主要集中在GJB2基因和SLC26A4基因上;失聪相关基因尚存有许多未知的领域,有待于进一步研究。通过对不同基因型个体进行婚育指导,可以降低聋-聋婚配中聋病的垂直传递,减少本地区新生听力障碍儿童的出生。     

听力障碍婴幼儿语言、语音及认知的早期干预

目的探讨听力障碍婴幼儿语言、语音及认知早期干预的效果和早期干预的临床模式。方法将56例10个月~2岁的听力障碍婴幼儿分为两组,20例配戴助听器并接受系统语言康复的患儿为干预组,36例未接受治疗的患儿为未干预组。采用Gesell发育量表对56例患儿及28例正常对照组儿童进行语言及认知评估;采用普通话语音测试对56例患儿的语音发育状况进行评估,比较干预组、未干预组和正常儿童的语言、语音和认知发育水平。结果(1)干预组患儿的言语能DQ(92.25±17.32)高于未干预组(70.44±29.99),差异有统计学意义(P=0.00),干预组患儿与正常儿童言语能DQ(100.29±8.86)的差异无统计学意义(P=0.07)。未干预组患儿的言语能DQ低于正常儿童,差异有统计学意义(P=0.00)。等级相关分析显示,干预组患儿的语言康复水平与听力损失程度的相关性无统计学意义(P=0.27)。(2)干预组患儿应人能DQ(101.40±7.66)高于未干预组(83.94±22.09),差异有统计学意义(P=0.00),干预组患儿与正常儿童应人能DQ(101.68±13.83)的差异无统计学意义(P=0.93);未干预组患儿的应人能DQ低于正常儿童,差异有统计学意义(P=0.00);粗动作、细动作和应物能DQ3组比较,差异均无统计学意义。(3)干预组除1例极重度听力障碍患儿的/d/发音异常外,其他患儿辅音发音正常。未干预组中重度、重度患儿各有1例表现为/d/发音异常,极重度患儿中/d、m/发音异常6例,其他患儿正常。干预组除2例极重度患儿声调异常外,其他患儿的声调正常。未干预组中度、中重度、重度和极重度患儿中声调异常分别为2例、1例、1例和12例。结论未干预听力障碍婴幼儿的语言、语音和认知水平明显落后于正常儿童,早期干预能显著改善听力障碍患儿的语言、语音和认知能力。已经建立有效的早期干预临床模式。     

Exploration between shen and ear by auditory determination in patients with renal disease

Hearing determination of 51 cases had been made to observe and explore the TCM pathogenic relation between the collateral channels of Shen and ears. Model 51A-T72N pure tone auditional determinator was used. For each case, routine auditional determination was made after rest in bed quietly for an hour after breakfast. The determinating results were divided into normal (average hearing loss less than 15dB), low frequency, high frequency and plain-typing hearing loss. Results: Among the 51 cases, 8 cases were normal hearing (15.7%), the hearing loss cases were 43 (84.3%); 30 cases were double-ear hearing loss (58.8%); 13 cases were single ear hearing loss (25.5%). Cases of renal diseases frequently occurred among the young (in this group, average age was 29.5 years). Such high incidence was obviously non-physiological, but related to the renal disease itself. More serious hearing damage were seen and were in high frequency. Whether or not it was caused by hypoalbuminaemia, oedema and deprivation in blood vessel leading to disorder of circulation of sound sensitizen, or accumulation of toxin owing to renal dysfunction, or changing composition of endolymph influencing the hearing bristle cells, further research should be done in the near future.     

400例耳鸣患者听力减退情况的临床分析

目的 研究耳鸣与听力减退的相关性,为探索耳鸣的病因、治疗方法及预后提供临床证据。方法 通过对门诊耳鸣为第一主诉的400例患者的临床资料进行分析,根据耳鸣与听力减退出现的部位、时间先后关系及听力减退情况,研究耳鸣与听力减退的相关性。结果 400例耳鸣患者中,61.00%与其听力不相关（其中52.05%听力正常,47.95%听力减退）, 39.00%与其听力相关（其中70.51%明确相关,29.49%可能相关）。结论 耳鸣与听力减退之间没有绝对相关性,二者之间并非互为因果关系,在临床工作中,不宜将耳鸣与听力减退进行同等看待和治疗。     

Post meningitic sensori-neural hearing loss in children--alterations in hearing level

Post meningitic sensori-neural hearing loss was studied in forty new cases of bacterial meningitis and ten cases of viral meningitis treated at the Pediatric Institute, Kuala Lumpur Hospital from April 1991 to March 1992. Hearing assessment at 2 weeks, 3 months and 6 months following the diagnosis of meningitis using Brain Stem Evoked Response Audiometry showed that hearing loss was prevalent only in patients with bacterial meningitis. Hearing loss was detected in 32.5% of these patients during the acute phase of the disease, 22.8% after 3 months and 24.2% after 6 months. In 63.6% of the affected cases, hearing loss was bilateral. In 61.5% of the patients who had hearing loss during the acute phase of the disease, it was permanent, 16.7% had either partial or complete recovery and, 15.4% had deterioration in hearing level. In 2 cases the subsequent hearing level was unknown. The risk of developing sensori neural hearing loss was found to be significantly higher in patients who developed other neurological sequelae. The study highlights the importance of performing repeated hearing assessment in children with bacterial meningitis and the difficulty in appropriate selection of hearing aids in the early stages.     

糖尿病人听阈分析

木文对33名糖尿病患者和30名正常对照进行听阈检查。所有受试者年龄均小于60岁,并排除耳部疾病及其他代谢疾病。病人按年龄、病程及并发症等分组。结果显示:(1)糖尿病患者听阈高于正常对照;(2)糖尿病患者>50岁组与≤50岁组间听阈有显著差异;(3)糖尿病病程与听力减退无关;(4)有并发症患者听力减退发生率高于无并发症患者;(5)伴有高血压的患者与无并发症患者相比听阈有显著差异;(6)高血脂与听力减退无关。     

When the diagnosis of deafness is wrong.

OBJECTIVE: To describe three children in whom there had been major errors in the diagnosis of hearing loss. CLINICAL FEATURES: In three children (two developmentally delayed, one not developmentally delayed) hearing thresholds obtained by behavioural testing were later proven wrong. This resulted in significant family distress and inappropriate educational approaches. INTERVENTION AND OUTCOME: Electrocochleography and brainstem audiometry were performed, demonstrating normal cochlear function. Simultaneous microinspection of the ears gave information about current or old middle ear disease and the likelihood of past conductive hearing loss. In each case hearing aids could be discarded, enabling parents and teachers to concentrate on one rather than multiple problems. CONCLUSION: Electrocochleography and brainstem audiometry should be used more frequently to check the diagnosis of hearing loss in children who are developmentally delayed, hyperactive or autistic and who do not give consistent responses to behavioural testing. It should also be considered if parents are firmly convinced that the diagnosis of deafness is wrong.     

A Case-control Study on High-risk Factors for Newborn Hearing Loss in Seven Cities of Shandong Province

To investigate the high-risk factors for newborn hearing loss and to provide information for preventing the development of hearing loss and delaying its progression, from May 2003 to June 2006, neonates who failed to pass the universal newborn hearing screening (UNHS) were referred to Jinan Newborn Hearing Screening and Rehabilitation Center from 7 newborn hearing screening cen- ters in seven cities of Shandong province. One-to-one pair-matched case-control method was em- ployed for statistical analysis of the basic features of definitely identified cases. High-risk factors re- lating to the bilateral hearing loss were evaluated by univariate and multivariate Logistic regression analysis. Our results revealed that 721 transferred newborns who didn't pass the hearing screening received audiological and medical evaluation and 367 were confirmed to have hearing loss. Of them, 177 neonates with hearing loss who met the matching requirements were included in the study as subjects. Univariate analysis showed that high-risk factors related to hearing loss incuded age of fa- ther, education backgrounds of parents, parity, birth weight, gestational weeks, craniofacial deformity, history of receiving treatment in neonatal intensive care unit (NICU), neonatal disease, family history of otopathy and family history of congenital hearing loss. Multivariate Logistic regression analysis revealed that 4 independent risk factors were related to bilateral hearing loss, including parity (OR=16.285, 95% CI 3.379-78.481), neonatal disease (OR=34.968,95% CI 2.720-449.534), family history of congenital hearing loss (OR=69.488,95% CI 4.417-1093.300) and birth weight (OR=0.241, 95% CI 0.090-0.648). It is concluded that parity, neonatal disease and family history of hearing loss are the promoting factors of bilateral hearing loss in neonates and appropriate interven- tion measures should be taken to deal with the risk factors.     

Noise-induced hearing loss and blood pressure.

From a group of industrial workers who had a noise-induced hearing loss of at least 30 dB at 4000 Hz 62 were randomly selected after stratification. Controls matched for age and duration of employment were also selected. Resting blood pressures were measured and audiometry was repeated. The findings are presented by age group and for the two groups as a whole. No relation between systolic or diastolic blood pressure and hearing loss was found, and equal proportions of each group had blood pressures exceeding 140/90 mm Hg. Persons with greater hearing loss (more than 60 dB at 4000 Hz) did not have significantly higher blood pressures than their matched controls.     

自身免疫性感音神经性聋豚鼠的子代内耳生理功能研究

目的 :观察自身免疫性感音神经性聋 (ASHL)母豚鼠所产子代内耳生理功能的变化 ,探讨针对内耳的自身免疫因素对子代内耳生理功能的影响及其改变特点。方法 :同种内耳抗原 (CIEAg)持续免疫孕豚鼠 ,采用耳蜗电图 (记录cAP、CM )和眼震电图仪 (记录自发性眼震和冷热空气试验 )测试母鼠和子鼠的听觉和前庭功能 ,并检测血清特异性体液免疫反应。结果 :ASHL模型母豚鼠所产子鼠血清中发现有特异性抗体水平升高 ,部分 (3 /9)出现听觉损伤。非ASHL母鼠和对照组母鼠所产子代未见明显异常。结论 :ASHL雌鼠所产子代可出现感音神经性聋 ,其内耳损伤和功能障碍极可能与针对内耳组织的自身免疫反应 (尤其是体液免疫 )有关 ,从而提示内耳自身免疫因素可能为部分先天性非遗传性感音神经性聋的病因之一。     

突发性耳聋预后的相关因素探讨

目的：探讨影响突发性耳聋预后的相关因素。方法286例突发性耳聋患者治疗前和治疗后进行纯音听阈测试对比,探讨影响预后的相关因素。结果突发性耳聋预后与患者的发病时间、年龄大小、听力损失情况、有无眩晕、高血压、糖尿病及焦虑情绪有关,而与性别无关。结论突发性耳聋患者发病时间越长、年龄越大、听力损失越重,治疗预后越差。听力图中,高频型和水平型比中频型和低频型预后差,全聋型最差。不伴眩晕、无高血压高血脂糖尿病、无焦虑情绪的突发性耳聋患者疗效明显好于伴眩晕、有高血压高血脂糖尿病、有焦虑情绪患者。     

Hearing loss in elderly patients in a family practice.

OBJECTIVE: To investigate hearing loss in elderly patients. DESIGN: Cohort study. SETTING: Family practice. PATIENTS: All ambulatory patients 65 years of age or older who attended the practice from June to August 1989. OUTCOME MEASURES: The Hearing Handicap Inventory for the Elderly--Screening Version (HHIE-S) and the Welch-Allyn Audioscope. Patients who failed one or both of the screening tests were referred to a speech and hearing clinic for audiologic assessment and treatment recommendations. Those with hearing aids were excluded from the main study but were given the opportunity to have them assessed at the clinic. MAIN RESULTS: Of 157 eligible patients 42 were excluded: 16 refused to participate, 13 already had hearing aids, and 13 could not be contacted. Of the remaining 115, 34 failed one or both of the tests (14 failed the HHIE-S, 9 failed the audioscope test, and 11 failed both). Of the 34, 25 completed the audiologic assessment at the clinic. Fifteen were found to have severe hearing impairment; the recommendation was hearing aids for 12, further assessment for 2 and no treatment for 1. Of the remaining 10 patients it was thought that 6 would benefit from hearing aids. Ten of the 11 patients with hearing aids who agreed to undergo testing at the clinic were found to need an adjustment or replacement of their devices. CONCLUSIONS: Hearing loss is a significant problem in elderly patients in primary practice. Further study is required to determine which of the two screening tools is most effective. Most elderly patients with hearing aids may require modification or replacement of their devices.     

脑干听觉诱发电位在小儿语言发育障碍病因探讨中的应用价值

作者对368例语言发育障碍患儿和116例各年龄组健康儿童，进行脑干听觉诱发电位（BAEP）的检查。结果，健康儿童BAEP全部正常，在368例语言障碍患儿中，BAEP正常78例，异常290例。患儿中90例有氨基糖甙类抗生素注射史，55例有宫内窘迫或窒息史、围产期重症感染或缺血缺氧性脑病，35例为脑性瘫痪患儿，41例有不同程度的智力障碍，12例有各种急性中枢神经感染史，7例有家族史。作者认为，BAEP方法简便、无创伤性、客观性强，结果正确、可靠，可作为早期发现语言发育障碍患儿是否存在听力损害的一种常规筛选手段。     

畸变产物耳声发射在社区糖尿病患者听力监测中的应用

目的 了解社区2型糖尿病（type 2 diabetes mellitus,T2DM）患者听力损失知晓率及听力现状,探讨畸变产物耳声发射（distortion products otoacoustic emission,DPOAE）在社区T2DM患者听功能监测中的应用。方法 2018年4月至2018年10月,因糖尿病就诊于北京市东城区天坛社区卫生服务中心和北京市东城区永外社区卫生服务中心T2DM患者156例,其中,男性69例,女性87例,年龄20~79岁,平均年龄（62.74 ±6.54）岁。对所有患者进行问卷调查、纯音听力检查、声导抗检查和DPOAE检查。结果 听力下降组的DPOAE幅值在各频率较听力正常组降低,其中在1.5 kHz差异有统计学意义（P=0.014）。312耳中,主诉听力下降98耳（31.41%）,DPOAE检查1.5 kHz下降242耳（77.56%）（P=0.000）;老年组230耳,主诉听力下降67耳（29.13%）,DPOAE检查1.5 kHz下降190耳（82.61%）（P=0.000）;中青年组82耳,主诉听力下降31耳（37.80%）,DPOAE检查1.5 kHz下降52耳（63.41%）（P=0.001）。312耳经纯音听力检查发现听力下降216耳（69.23%）。其中主诉听力正常214耳中,纯音听力检查发现130耳（60.75%）存在不同程度的听力下降（P=0.000）。老年组纯音听力检查耳听力下降165耳（71.74%）（P=0.000）;中青年组纯音听力检查证实51耳（62.20%）听力下降（P=0.002）。结论 社区糖尿病患者听力损失发生率高,但知晓率较低,DPOAE检查可在主诉听力下降之前发现耳蜗外毛细胞功能状态的改变,社区糖尿病患者应尽早进行听力保健的健康教育并择时进行听力检查。     

Phenotype and genotype analysis of a Chinese family with prelingual X-linked hereditary hearing impairment

Background X-linked hearing impairment is clinically and genetically a heterogeneous disease. Although many disorders manifest with hearing loss, a limited number of sex-linked loci and only one gene （POU3F4） have been shown to be implicated in X-linked non-syndromic hearing impairment. In the present study, we have performed a clinical and genetic analysis of a Chinese family with X-linked non-syndromic hearing loss, with emphasis on audiological findings and genomic mapping.
Methods The clinical features of Family JX01 were evaluated by physical and audiometric examination in eighteen family members. Mutation screening of POU3F4 was identified by polymerase chain reaction （PCR） amplification and sequencing. Molecular evaluation consisted of X-chromosome wide genotyping by microsatellite makers （STR）, followed by analyzing using MLINK computer program.
Results Five affected males demonstrated bilateral, symmetrical sensorineural and profound hearing loss. The hearing impairment started prelingual. The female carriers did not have any complain of hearing loss, however, two of them were tested with milder loss with high frequency. No causative mutations in POU3F4 gene were detected by DNA sequencing. Linkage analysis indicated that the responsible gene was linked to locus DXS1227 （maximum Iod score=2.04 at θ=0）.
Conclusions The affected males in Family JX01 have profound prelingual sensorineural hearing impairment. In addition, two female carriers showed mild to moderate hearing losses. However, none of females complained of any hearing loss. Analysis of hereditary deafness in this family mapped most compatibly to the Xq27.2.     

Relationship between the external aperture and hearing loss in large vestibular aqueduct syndrome

The otologists are usually puzzled by sensorineural hearing loss (SNHL) due to unknown cause in children. Large vestibular aqueduct syndrome (LVAS) is one of the common causes of SNHL in children. Sometimes, most audiologists are unware of LVAS or do not suspect it to be the enlargement of the vestibular aqueduct (EVA).1 EVA is the most usual abnormality of the inner ear, which can be diagnosed with CT. Valvassori et al2 first reported the Meniere’s-like disturbances associated with…     

Audiological evaluation in Chinese patients with mitochondrial encephalomyopathies

Background Hearing impairment has been reported to be common in patients with mitochondrial disorders,a group of diseases characterized by pleiomorphic clinical manifestations due to defects in oxidative phosphorylation of mitochondria.This study aimed to investigate the audiological characteristics in a large cohort of patients with mitochondrial disease.Methods Comprehensive audiological evaluations,including pure tone audiometry,tympanometry,speech audiometry,otoacoustic emissions,electrocochleography and auditory brainstem evoked potentials,were performed in 73 Chinese patients with mitochondrial encephalomyopathy and with confirmed mitochondrial DNA （mtDNA） defects.Results Among the patients,71％ had hearing impairment.However,the incidence rate and severity of hearing impairment were much less in the chronic progressive external ophthalmoplegia （CPEO） subtype than in the mitochondrial encephalomyopathy,lactic acidosis,and stroke-like episodes （MELAS）,myoclonic epilepsy with ragged red fibers （MERRF） and Kearns-Sayre syndrome （KSS） subtypes.While most of our patients had a predominantly cochlea origin for the hearing deficit,five patients had an auditory neuropathy spectrum disorder and three patients had impairment of both cochlea and auditory codex.Conclusions Various portions of the auditory system could be involved in patients with mitochondrial diseases,including cochlea,auditory nerve,auditory pathway and cortex.Hearing loss was more associated with multisystem involvement.Genotype,mutant load of mtDNA and other unknown factors could contribute to heterogeneity of hearing impairment in mitochondrial disease.     

某电子厂噪声作业工人听力损失调查研究

目的了解某电子厂噪声作业工人听力损害的情况。方法回顾性分析我院对某电子噪声作业工人连续3年的纯音听力检查结果,并对听力损害与工龄、年份的关系分别进行统计学分析。结果有效调查2436人次,高频听力损失检出率为33．9％（825／2436）,显著高于语频听力损失检出率4．8％（117／2436）。高频及语频听力损失率在各年份及各工龄组间差异无统计学意义。结论企业应加强建立听力保护计划,防止职业性噪声聋的发生。     

Ear disease in three aboriginal communities in Western Australia

Surveys of ear disease amongst Aboriginal people in two isolated bush communities (Wiluna and La Grange) and one urban community (Kwinana) in Western Australia were undertaken in 1988 or 1989. The age-adjusted prevalence odds ratio (relative risk) of perforations of the tympanic membrane for Wiluna compared with Kwinana was 5.0 (95% confidence interval [CI] 2.7-12.2) and 6.8 (95% CI 3.5-13.9) for La Grange compared with Kwinana. The relative risk of mild hearing loss, in comparison with Kwinana, was 2.5 (95% CI 1.5-4.3) for Wiluna and 3.2 (95% CI 2.0-5.0) for La Grange. There was no significant difference in the relative risk of moderate or severe hearing loss or impedance pattern B, usually interpreted as "glue ear", in any of the three communities. Overall, the urban Aboriginal community had less ear disease and hearing loss than either of the isolated bush communities, but even this community did not approach the much lower levels of prevalence in Australia as a whole.