儿童感音神经性聋听觉稳态诱发反应阈与听性脑干反应阈比较

目的研究听觉稳态诱发反应(ASSR)和听性脑干反应(ABR)阈与纯音听阈的差别和相关性。方法选择74例儿童感音神经性聋患者(118耳)分别进行ASSR、ABR和电测听检查,比较ASSR、ABR反应阈及纯音听阈,同时就ASSR、ABR反应阈与纯音听阈进行相关性分析。结果 ASSR和ABR反应阈与纯音听阈均有良好的相关性。ABR的反应阈与纯音听阈阈值接近,而ASSR反应阈与纯音听阈间差值较大。ASSR反应阈与纯音听阈间的相关性要优于ABR反应阈与纯音听阈间的相关性。结论 ASSR和ABR均为较好的评估行为听阈的客观测听方法。     

伴有和不伴有眩晕的突发性聋纯音测听检测结果研究

目的 检测伴有和不伴有眩晕的突发性聋患者的纯音听阈表现,分析突发性聋发病时伴有眩晕或不伴有眩晕时纯音听阈的特点,研究两者纯音听阈检测结果的差异性。方法 将2015年1月~2017年1月我科诊治的突发性耳聋116例患者分为眩晕组和不伴眩晕组,分别详细询问病史后,对患者进行电耳镜检查,纯音听阈测听,声导抗测听,耳声发射及内听道MRI检查,排除外耳,中耳及中枢性病变,记录患者检测数据后进行统计分析。结果 突发性聋伴有眩晕患者纯音测听检查结果多提示有高频听阈的提高,而不伴有眩晕的突发性聋患者纯音测听无特异性及规律性。结论 在临床治疗过程中,伴有眩晕的患者恰好听阈改变多在高频区。针对性治疗耳蜗底部受损这一特点所致突发性耳聋患者,可能对我们医学科研及临床的诊断和治疗工作有参考价值。     

Audiological findings in Williams syndrome: a study of 69 patients

The aim of this study was to investigate, in a clinical setting, the auditory function of a group of individuals affected by Williams syndrome (WS). Sixty-nine patients with WS, aged 2-30, underwent comprehensive audiological testing including air/bone conduction behavioral audiometry, speech audiometry, tympanometry and measurement of the acoustic reflex, transient evoked otoacoustic emissions and brainstem auditory evoked responses. Hearing loss, defined by a pure-tone average above 15?dB HL, affected 22.6% of the patients studied with traditional audiometry and was mostly slight in severity. Hearing loss was conductive in 9.4% of patients, mainly children with otitis media with effusion, and sensorineural in 13.2% of patients. However, 30% of the ears studied had a hearing impairment in the high frequency range (high-frequency pure-tone audiometry above 15?dB HL), higher in participants above 15 years (46.15%) than in the younger ones (23.45%). Contralateral stapedial reflexes were present in all patients with A-type tympanograms. Transient otoacoustic emissions were absent in 44% of the ears of patients with normal hearing. Brainstem auditory evoked responses fell within normal ranges thus confirming the absence of retrocochlear dysfunction. Although hearing loss does not seem to be frequent, a cochlear fragility, especially in the high frequency range, related to outer hair cells is characteristic of WS. Therefore we strongly recommend monitoring patients affected by WS using annual audiometric tests and performing otoacoustic emissions in order to identify a subclinical cochlear dysfunction which might benefit from an audiological follow up before the possible onset of hearing loss.     

A novel missense mutation in a C2 domain of OTOF results in autosomal recessive auditory neuropathy

Screening of 12 Turkish families with apparently autosomal recessive nonsyndromic sensorineural deafness without GJB2 and mtDNA m.1555A > G mutations for 11 previously mapped recessive deafness loci showed a family in which hearing loss cosegregated with the DFNB9 (OTOF) locus. Three affected children were later found to carry a novel homozygous c.3032T > C (p.Leu1011Pro) mutation in the OTOF gene. Both parents were heterozygous for the mutation. p.Leu1011Pro alters a conserved leucine residue in the C2D domain of otoferlin. Pure tone audiometry of the family showed severe to profound sensorineural hearing loss (with U-shape audiograms) in children, and normal hearing in the parents. Otoacoustic emissions and auditory brainstem response (ABR) suggested the presence of auditory neuropathy in affected individuals.     

心理暗示在外伤性聋听力检测中的应用

目的 纯音测听时应用心理暗示，配合声导抗对外伤性鼓膜穿孔患者进行听力损失程度的评估。方法 对外伤性聋进行客观声导抗测试和主观纯音测试。如果两者不符，根据客观听力检查结果，对患者进行心理暗示，以取得真实的纯音听阈。结果 582例外伤后称听力下降病例中，358例第1次纯音测听与声导抗结果一致，伪聋和夸大性聋198例，后经纯音测听时的心理暗示才与声导抗结果一致。结论 应用心理暗示对外伤性聋进行听力检测获得较好效果。     

Pure tone audiometry: comparison of general practice and hospital services

Pure tone audiometry was obtained for both ears of 32 children by a general practitioner using a simple audiometer in his surgery, and by audiometricians in a hospital department on the same day. Comparing the worst hearing threshold at any of the three tested frequencies, the general practitioner did not find any ears to hear more than 10 dB better than the hospital (no false negatives). However, there were six false positives (9%) where the general practitioner identified an apparent hearing loss of greater than 15 dB. It is concluded that pure tone audiometry could be carried out accurately in the practice.     

BERA in children with hearing loss and delayed speech

OBJECTIVES: The brainstem evoked response audiometry (BERA) is an objective neurophysiological method for the evaluation of the hearing threshold and diagnosing retrocochlear lesions. The aim of the study was to investigate the hearing level in children with suspected hearing loss or pathological speech development. PATIENTS AND METHODS: The BERA diagnostic procedure was applied in 184 children ranging from 1 to 12 years of age at Ahmadi Hospital in Kuwait. RESULTS: We found profound hearing loss (deafness) in 13 children, severe hearing loss in 8 children, moderate hearing loss in 34 children, mild hearing loss in 34 children, and normal hearing level in 95 children. Out of the children suspected for hearing loss, 42% actually had some level (mild-moderate) of hearing loss. Out of the children with delayed speech, 63% had some level (mild-profound) of hearing loss which actually caused the delay in speech development; 37% had normal hearing, but inadequate verbal communication affected their language acquisition and speech development. CONCLUSIONS: These results illustrate the necessity to test children hearing even with the slightest suspicion by the parent or doctor of hearing loss. The results warrant the establishment of a hearing screening test of newborns in Kuwait to provide hearing aid to children with hearing loss.     

Audiological follow-up of 24 patients affected by Williams syndrome

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5–14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in most patients.     

Hearing loss in skeletal dysplasia patients

A hearing screening program was performed to determine the prevalence of hearing loss and abnormal tympanometry in individuals with short-stature skeletal dysplasias attending a national meeting. Behavioral audiometry, otoacoustic emission testing, and tympanometry were used to assess hearing. Failed hearing screen was defined as hearing ≥ 35 dB at one or more frequencies or by "fail" on otoacoustic emissions. One hundred ten of 112 subjects completed the screening. 58 (51.8%) were children. Seventy-three (65.2%) had achondroplasia, 34 (30.4%) had one of 11 other diagnoses, and 5(4.4%) were undiagnosed. 25.8% of children failed hearing screening in one or both ears, while 46.3% of adults failed in one or both ears. 55.1% of adults and 25.0% of children with achondroplasia failed screening. Abnormal hearing was also found in the some patients with spondyloepiphyseal dysplasia congenital (SEDC; 75%), diastrophic dysplasia (66%), and Morquio (66%). Hearing was normal in those with hypochondroplasia, pseudoachondroplasia, and microcephalic osteodysplastic primordial dwarfism. Tympanometry was abnormal in at least one ear in 53.3% of children and 38.5% of adults. Abnormal tympanometry in the absence of functioning tympanostomy tubes was associated with 9.5 greater odds of hearing loss in children and 2.8 greater odds of hearing loss in the total cohort. Only 3 (2.7%) respondents reported the use of hearing aids. Hearing loss and middle ear disease are common in both children and adults with skeletal dysplasia. Adults were more likely to fail hearing screening than children. Abnormal tympanometry is associated with hearing loss. Hearing screening with appropriate intervention is recommended for these patients.     

Limitation of Conventional Audiometry in Identifying Hidden Hearing Loss in Acute Noise Exposure

PurposeThe concept of hidden hearing loss can explain the discrepancy between a listener''s perception of hearing ability and hearing evaluation using pure tone audiograms. This study investigated the utility of the suprathreshold auditory brainstem response (ABR) for the evaluation of hidden hearing loss in noise-exposed ear with normal audiograms.Materials and MethodsA total of 15 patients (24 ears) with normal auditory thresholds and normal distortion product otoacoustic emissions were included in a retrospective analysis of medical records of 80 patients presenting with histories of acute noise exposure. The control group included 12 subjects (24 ears) with normal audiograms and no history of noise exposure. Pure tone audiometry and suprathreshold ABR testing at 90 dB peSPL were performed. The amplitudes and latencies of ABR waves I and V were compared between the noise-exposed and control groups.ResultsWe found no significant difference in the wave I or V amplitude, or the wave I/V ratio, between the two groups. The latencies of ABR wave I, V, and I-V interpeak interval were compared, and no significant intergroup difference was observed.ConclusionThe results suggest that either hidden hearing loss may not be significant in this cohort of patients with acute noise exposure history, or the possible damage by noise exposure is not reflected in the ABRs. Further studies are needed to inquire about the role of ABR in identification of hidden hearing loss.     

新生儿及疑似听力障碍儿童听力筛查的病例分析

目的1.收取本地区新生儿听力障碍发病资料;2.分析听力筛查未通过的因素及临床听力学特征。方法选取2006年5月至2007年5月出生的286例新生儿,在出院前进行新生儿听力筛查;96例转诊至我科的疑似听力障碍患儿进行诊断性听力学检查。对未通过者进行声阻抗、耳声发射、听性脑干反应、听觉稳态诱发反应全面的综合检查以确定听力损失的性质和程度,给予相应干预措施。结果286例新生儿,其中初筛有40例（13.99%）未通过筛查,42天复筛DPOAE,最后有3例需行ABR检查,经过听力学初步评估发现2例（发病率为6.99‰）有听力损失,其中正常新生儿1例（发病率为3.94‰）,高危新生儿1例（发病率为31.25‰）并作相应诊断和干预;转诊疑似听力损失患儿96例,确认听力损失78例（81.25%）,其中轻度2例（2.5%）、中度7例（9.0%）、重度25例（32.1%）、极重度44例（56.4%）,发现确诊年龄在6个月～3岁范围内。结论3个月内早期诊断和6个月内早期干预是必要的,以便尽早发现患儿及时给予干预。推行新生儿及婴幼儿听力损失的早期筛查和干预,加强耳聋知识的宣传,提高家长的防聋治聋意识是非常必要的。     

Biallelic p.V37I variant in GJB2 is associated with increasing incidence of hearing loss with age

PurposeThis study aimed to quantitatively assess the incidence of hearing loss in relation to age in individuals with biallelic p.V37I variant in GJB2.MethodsPopulation screening of the biallelic p.V37I variant was performed in 30,122 individuals aged between 0 and 97 years in Shanghai. Hearing thresholds of the biallelic p.V37I individuals and the controls were determined by click auditory brainstem response or pure tone audiometry.ResultsBiallelic p.V37I was detected in 0.528% (159/30,122) of the subjects. Of the biallelic p.V37I newborns, 43.91% (18/41) passed their distortion-product otoacoustic emissions–based newborn hearing screening or had hearing thresholds lower than 20 decible above normal hearing level. The older newborns had elevated hearing thresholds, with increasing incidence of 9.52%, 23.08%, 59.38%, and 80.00% for moderate or higher grade of hearing loss in age groups of 7 to 15 years, 20 to 40 years, 40 to 60 years, and 60 to 85 years, respectively. Their hearing deteriorated at a rate of 0.40 dB hearing level per year on average; males were more susceptible, and deterioration occurred preferentially at higher sound frequencies.ConclusionThe biallelic p.V37I variant is associated with steadily progressive hearing loss with increasing incidence over the course of life. Most of the biallelic p.V37I individuals may develop significant hearing loss in adulthood and, can benefit from early diagnosis and intervention through wide-spread genetic screening.     

Absence of Early Neuronal Death in the Olivocochlear System Following Acoustic Overstimulation

This study was conducted to examine possible effects of noise trauma on olivocochlear (OC) neurons. Anesthetized rats were exposed to a continuous 10 kHz pure tone at 120 dB sound pressure level for 2 hrs. The effects of treatment were verified by recordings of auditory brainstem response and distortion product otoacoustic emission. Three or 8 days after acoustic trauma, rats received unilateral injections of an aqueous solution of the retrograde neuronal tracer Fluorogold (FG) into the scala tympani to identify OC neurons (OCN). Five days after FG injection, brains were perfusion‐fixed, and brainstem sections were cut and analyzed with respect to FG‐labeled neurons. We found that, in both groups, numbers of OCN were similar to that of controls. The incubation of a second set of sections with antibodies against choline‐acetyltransferase (the enzyme responsible for acetylcholine synthesis) showed the cholinergic neurons of the brainstem, however, without suggesting differences between groups. Our study, the first to investigate noise trauma effects on identified OCN, revealed that no visible alterations occurred in 2 weeks following trauma, neither in identified OCN nor in choline‐acetyltransferase‐immunofluorescence. At this time, auditory brainstem response and distortion product otoacoustic emission measurements showed severe signs of hearing loss. The mechanisms leading to hearing loss upon noise trauma apparently do not involve degeneration of OCN. Anat Rec, 299:103–110, 2016. © 2015 Wiley Periodicals, Inc.     

Study of the ototoxicity of amikacin and netilmicin using provoked acoustic oto-emissions and high-frequency audiometry]

To compare the ototoxicity of amikacin and netilmicin, tone audiometry, high-frequency audiometry, early auditory evoked potentials, and evoked otoacoustic emission testing were used to evaluate 30 patients (15 under amikacin and 15 under netilmicin). Ototoxicity was not significantly different in the two groups.     

Functional effects of adult human olfactory stem cells on early-onset sensorineural hearing loss

Transplantation of exogenous stem cells has been proposed as a treatment to prevent or reverse sensorineural hearing loss. Here, we investigate the effects of transplantation of adult human olfactory mucosa-derived stem cells on auditory function in A/J mice, a strain exhibiting early-onset progressive sensorineural hearing loss. Recent evidence indicates that these stem cells exhibit multipotency in transplantation settings and may represent a subtype of mesenchymal stem cell. Olfactory stem cells were injected into the cochleae of A/J mice via a lateral wall cochleostomy during the time period in which hearing loss first becomes apparent. Changes in auditory function were assessed 1 month after transplantation and compared against animals that received sham injections. Hearing threshold levels in stem cell-transplanted mice were found to be significantly lower than those of sham-injected mice (p < .05) for both click and pure tone stimuli. Transplanted cells survived within the perilymphatic compartments but did not integrate into cochlear tissues. These results indicate that transplantation of adult human olfactory mucosa-derived stem cells can help preserve auditory function during early-onset progressive sensorineural hearing loss.     

Clinical interpretation of brainstem evoked response audiometry abnormalities in cochlear pathology

This investigation involved 45 patients with sensorineural hearing loss (SNHL): 24 with Meniere's disease, 18 with acoustic trauma, and 3 with SNHL due to ototoxic drugs. They all underwent pure tone audiometry and standard brainstem evoked response audiometry (BERA). In patients without wave I in auditory brainstem response, electrocochelography (ECochG) was performed. The findings are presented showing that cochlear lesions (beside threshold elevation) cause latency prolongation of wave I, III and V relative to normal latencies at the actual click hearing level. At high stimulation levels, this effect is almost completely compensated for by the fact that cochlear recruiting ears exhibit steeper latency-intensity curves than do normal ears. But, at the same time this pathology does not cause latency prolongation of central conduction time (CCT). Beside this, cochlear lesions will cause, in some cases, deterioration of replicability (poor waveform resolution) of waves preceding wave V. In such cases, the authors strongly recommend electrocochleography (ECochG) to make wave I visible, because they think that it is the best way to verify the diagnosis of cochlear lesion using BERA.     

腭裂患儿120例电反应测听法脑干听力阈值分析

目的：分析腭裂患儿脑干听力阈值特征。方法：使用电反应测听法检测120例腭裂患儿听力阈值，采用分组对照研究，进行Χ^2检验。结果：①240耳中141耳听力阈值异常，听力障碍发生率为58．8％；②Ⅱ°与Ⅲ°腭裂患儿听力障碍发生率有极显著性差异，P〈0．005；腭裂程度与听力障碍程度之间无相关性，P〉0．75；③3岁前后比较，听力障碍发生率差异极显著，P〈0．005，3—6岁与〉6岁比较无显著差异，P〉0．05。结论：①58．8％腭裂患儿存在不同程度听力障碍；②腭裂程度越重听力障碍发生率越高，但与听力障碍程度无相关性；③患病年龄越小听力障碍发生率越高，3岁前后有极显著性差异。     

耳聋患者及正常人GJB2基因的突变筛查

目的 检测常染色体隐性遗传耳聋患者GJB2基因突变情况,并分析其与临床表型的关系.方法 收集42例耳聋患者的临床资料,对患者进行纯音电测听检查、声阻抗检测、脑干听觉诱发电位检查;应用聚合酶链反应和直接测序法,对患者和9例患者的父母以及105名正常对照进行GJB2基因检测.结果 两例患者具有235delC纯合性突变,其中1例系感音神经性耳聋,另1例系混合性耳聋;1对混合性耳聋的双生子患者同时携带176de116bp杂合性突变.109G→A、79G→A和341A→G的纯合及杂合突变在患者及正常对照中均有出现.结论 235delC纯合性突变为致病突变,该突变可出现在混合性耳聋中;双生子患者的176de116bp杂合性突变考虑为宫内受到外界环境影响所致,或者由其它基因突变所致.109G→A、79G→A和341A→G考虑为是该基因的多态性,其临床意义仍需进一步探索.     

Prevalence of hearing impairment in the elderly living at home

Using pure tone audiometry, we assessed the prevalence of hearing impairment in a sample of elderly people living at home and aged 70 or more. Deafness was defined as an average loss over the speech frequencies at 1 kHz, 2 kHz and 4 kHz of 35 db or more in the better ear. We found that 60 per cent of the sample were deaf. This figure is substantially higher than previous estimates arrived at without the use of audiometry. We discuss some possible reasons for this discrepancy and consider the implications of this level of prevalence of hearing impairment for the health care of the elderly.     

Age-related changes in cochlear and brainstem auditory functions in Fischer 344 rats

Auditory function in Fischer 344 (F344) and Long Evans (LE) rats was monitored during their lifespan by evaluating hair cell loss, middle-ear compliance and the recording of otoacoustic emissions and auditory brainstem responses. The results revealed a faster deterioration of hearing function in F344 rats compared with LE rats, resulting in larger hearing threshold shifts, a decrease in the latency and amplitude of click-evoked auditory brainstem responses, diminution of the distortion product otoacoustic emissions and a decrease in middle-ear compliance. However, hair cell loss, observed only at the most basal and apical parts of the organ of Corti, was comparable in older individuals of both rat strains. The results suggest involvement of cochlear (stria vascularis) and extracochlear (middle-ear) pathological changes during ageing. Thus, F344 rats represent a complex mix of conductive hearing loss (with low-frequency threshold shift, declining parameters of the middle-ear admittance and asymmetric otoacoustic emissions) and sensorineural hearing loss (with a decrease in the amplitudes of auditory brainstem response and a high-frequency threshold shift).