Otoacoustic emission in patients with neurological disorders who have auditory brainstem response abnormality

Otoacoustic emissions (OAEs) were evaluated in 51 ears of 30 patients with a severe auditory brainstem response (ABR) waveform abnormality. Thirteen ears showed no ABR to click sound of higher intensity than 100 dBSPL (group 1). Fourteen ears exhibited only wave V or a decreased amplitude pattern of ABR (group 2). Twenty-four ears showed a predominant wave I or no wave III pattern (group 3). Almost all the ears with absent ABR showed no OAE, which strongly suggested hearing loss of cochlear origin, although one patient with alternating hemiplegia of childhood exhibited definite OAEs and auditory reactions without ABR. One patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) and her mother in group 2 had OAE abnormalities, which also suggested mild to severe hearing impairment. When OAEs are present, an accompanying ABR abnormality may be produced by brainstem dysfunction of the underlying disorder such as Pelizaeus-Merzbacher disease. There was a significant relationship (chi-square test P<0.001) between the positivity of the distortion product OAE response and the clinical auditory reactions in 24 patients, although their ABR abnormalities did not reflect hearing impairment directly. Careful examination of both audiometry and OAEs might be necessary for further assessment of the hearing function in pediatric patients with neurological disorders and specific auditory nerve disease.     

前庭阵发症脑干听觉诱发电位的特点

目的探讨前庭阵发症(VP)脑干听觉诱发电位(BAEP)的特点。方法对51例VP患者的BAEP结果进行回顾性分析。结果 BAEP异常者40例,异常率为78.4%。与Ⅰ-Ⅲ波峰间期正常的患者相比,Ⅰ-Ⅲ波峰间期延长的患者男性比例高(χ2=4.763,P=0.029),病程显著延长(t=2.469,P=0.021),而平均年龄差异无统计学意义。与Ⅲ-Ⅴ波峰间期正常的患者比较,Ⅲ-Ⅴ波峰间期延长的患者男性比例、平均病程、平均年龄差异均无统计学意义。与Ⅰ-Ⅲ波峰间期耳间差正常的患者比较,Ⅰ-Ⅲ波峰间期耳间差延长的患者病程相对较长(P=0.055),男性有增多趋势(P=0.058),但差异无统计学意义。结论 VP患者BAEP异常以Ⅰ-Ⅲ波峰间期延长为主,且以男性更多见。病程越长,蜗神经越易受累。     

59例听神经瘤患者脑干听觉诱发电位分析

目的 分析听神经瘤(acoustic neuroma,AN)患者的脑干听觉诱发电位的变化特征及健侧耳峰间期改变.方法 对59例听神经瘤(AN)患者进行脑干听觉诱发电位(BAEP)检测,测定Ⅰ、Ⅲ、Ⅴ波潜伏期(PL)、峰间期(IPL),双耳PL、IPL之间差值(ILD)等数值.结合MRI、CT影象学资料进行分析,并与36例健康者对照.结果 AN组与正常对照组BAEP各波PL、IPL测值比较差异有极显著性(P＜0.01).AN患侧BAEP异常率98.3%(58/59);主要表现:①Ⅰ、Ⅲ、Ⅴ波缺失;②Ⅲ、Ⅴ波PL延长;③Ⅰ～Ⅲ、Ⅲ～Ⅴ、Ⅰ～Ⅴ波IPL延长.AN患者健侧BAEP的异常率69.5%(41/59),主要表现:①Ⅴ波PL延长;②Ⅲ～Ⅴ及Ⅰ～Ⅴ波IPL延长;③Ⅲ～Ⅴ/Ⅰ～Ⅲ波IPL比值＞1.肿瘤直径＞2cm,BAEP的异常率有显著提高.不同大小肿瘤组间健侧BAEP测值比较:健侧Ⅴ波PL差异有显著性(P＜0.05),Ⅲ～Ⅴ及Ⅰ～ⅤIPL差异有极显著性(P＜0.01).结论 BAEP对AN诊断具有重要意义,它为病变提供了定位诊断依据,尤其健侧Ⅲ～Ⅴ/Ⅰ～Ⅲ波IPL比值异常,是脑干受压的敏感指标.     

Electroencephalogram and brainstem auditory evoked potential in 539 patients with central coordination disorder

BACKGROUND： Electroencephalogram （EEG） and brainstem auditory evoked potential （BAEP） are objective non-invasive means of measuring brain electrophysiology. OBJECTIVE： To analyze the value of EEG and BAEP in early diagnosis, treatment and prognostic evaluation of central coordination disorder. DESIGN, TIME AND SETTING： This case analysis study was performed at the Rehabilitation Center of Hunan Children＇s Hospital from January 2002 to January 2006. PARTICIPANTS： A total of 593 patients with severe central coordination disorder, comprising 455 boys and 138 girls, aged 1-6 months were enrolled for this study. METHODS： EEG was monitored using electroencephalography. BAEP was recorded using a Keypoint electromyogram device. Intelligence was tested by professionals using the Gesell scale. MAIN OUTCOME MEASURES： （1） The rate of abnormal EEG and BAEP, （2） correlation of abnormalities of EEG and BAEP with associated injuries, （3） correlation of abnormalities of EEG and BAEP with high risk factors. RESULTS： The rate of abnormal EEG was 68.6% （407/593 patients）, and was increased in patients who also had mental retardation （P 〈 0.05）. The rate of abnormal BAEP was 21.4% （127/593 patients）. These 127 patients included 67 patients （52.8%） with peripheral auditory damage and 60 patients （47.2%） with central and mixed auditory damage. The rate of abnormal BAEP was significantly increased in patients who also had mental retardation （P 〈 0.01 ）. Logistic regression analysis showed that asphyxia （P 〈 0.05）, jaundice, preterm delivery, low birth weight and the umbilical cord around the neck were closely correlated with abnormal EEG in patients with central coordination disorder, lntracranial hemorrhage, jaundice （P 〈 0.05）, low birth weight and intrauterine infection （P 〈 0.05） were closely correlated with abnormal BAEP in patients with central coordination disorder. CONCLUSION： Central coordination disorder is often associated with abnormal EEG an     

听神经瘤患者BAEP特征和术中面听神经保留情况分析

目的 分析听神经瘤(AN)患者脑干听觉诱发电位(BAEP)特征和显微外科手术中面、听神经保留情况.方法 安徽医科大学附属省立医院神经外科自2006年5月至2009年5月共采用肿瘤切除术治疗76例AN患者,术前术中均进行BAEP检查,分析患者术前、术中BAEP异常情况及AN直径大小与和术后面、听神经保留情况的关系.结果 AN患者术前患侧BAEP异常76例(76/76,100%),健侧BAEP异常63例(63/76,82.9%),两侧比较差异有统计学意义(P<0.05);术中健侧BAEP异常70例(70/76,92.1%),与术前健侧BAEP异常率比较差异有统计学意义(P<0.05);术中健侧BAEP异常与面、听神经的保留分别呈正相关关系(r=0.912,P=0.000;r=0.613,P=0.000);而AN直径与面、听神经的保留分别呈负相关关系(r=-0.869,P=0.000;r=-0.738,P=0.000).结论 术前BAEP检测对AN病变提供诊断依据,术中进行BAEP监测可提高面听神经的保留率,且AN直径越大,面、听神经越难保留.     

伴与不伴脑干听觉诱发电位异常的孤独谱系障碍患儿听觉统合训练的疗效比较

目的:探究听觉统合训练(AIT)对孤独谱系障碍(ASD)患儿的疗效,及脑干听觉诱发电位(BAEP)正常与否对疗效的影响。方法:采用方便抽样法抽取90例年龄2~6岁首诊的ASD患儿,以BAEP检测结果分为BAEP异常组(60例)及BAEP正常组(30例),并均给予AIT治疗,每日2次,20次(10 d)为1疗程;BAEP异常组每疗程结束后复查BAEP,直至检测结果恢复正常或不再有明显变化。BAEP正常组患者接受2疗程AIT。治疗前及每疗程结束后对入组者进行儿童孤独症评定量表(CARS)及孤独症治疗评定量表(ATEC)评估。结果:BAEP异常组患儿平均接受AIT(2.2±0.7)个疗程,其中32例(53%)BAEP恢复正常;BAEP异常组治疗前后CARS总分差值(4.3±3.5)及ATEC的语言因子分差值(4.2±2.4)、感知觉因子分差值(1.4±3.4)、行为因子分差值(1.7±4.7)显著高于BAEP正常组[(2.4±3.0),(1.0±2.2),(1.1±5.4),(0.8±7.0);F=66.583,F=89.562,F=6.635,F=4.131;P均0.05]。结论:AIT能够改善部分ASD患儿的核心症状和脑干听觉传导通路功能,对于BAEP异常的ASD患儿效果更显著。     

Evaluation and graduation of brain stem auditory evoked potentials in patients with neurological diseases

Based on normative data from 40 subjects, criteria for evaluating brain stem auditory evoked potentials have been elaborated. This system contained six grades from normal to diffusely abnormal, and was used in evaluating recordings from 142 patients, including 38 patients with definite and 48 with probable and possible multiple sclerosis, 18 patients with brain stem infarctions, six patients with other organic brain stem lesions, six patients with a postcommotional syndrome and 26 patients without any brain stem involvement. A statistically significant difference was found between normal persons and patients without evidence of brain stem lesions versus patients with such evidence, as well as between subgroups of slightly and severely affected patients. The system was found useful as a way of describing recordings, in company groups of patients and in a follow up of a few patients.     

脑干听觉诱发电位对癫(癎)患儿检测的意义

目的探讨脑干听觉诱发电位在癫患儿检测中的意义。方法对2000-01~2008-01我院38例癫患儿BAEP的检测结果进行总结。结果BAEP正常15例(39.5%),异常23例(60.5%),其中21例BAEP改变形式多样,异常指标相混出现。结论癫患者存在脑干功能异常。     

Lesion detection in MS patients with and without clinical brainstem disorders: magnetic resonance imaging and brainstem auditory evoked potentials compared

The findings of the present study can be summed up in the following points: (1) brainstem auditory evoked potentials (BAEP), as compared with magnetic resonance imaging (MRI), has a greater capacity and a lower cost in disclosing brainstem plaques both in MS patients with symptoms or signs of actual brainstem involvement and in clinically silent ones. This makes BAEP a useful technique for the neurologist, who can confirm the clinical suspicion of a brainstem lesion and follow the evolution of the disease in the patient. (2) The sensitivity of BAEP is lower than that of MRI as far as the anterior lesions of the brainstem are concerned. (3) MRI is more specific than BAEP, inasmuch as several types of injuries can alter the BAEP, while the demyelinating plaque has a specific image and can only be confused with little lacunar infarcts. (4) Plaques that produced symptoms or signs in the past can eventually disappear and be no longer detected by a subsequent MRI.     

Evaluation of ipsilateral and contralateral brainstem auditory evoked potentials in multiple sclerosis patients

Brainstem auditory evoked potentials (BAEP) were recorded simultaneously between the vertex and the mastoid ipsilateral and contralateral to the ear stimulated in 30 patients with multiple sclerosis (MS) and compared with the responses in a control group of 30 normal hearing adults. The control group showed that significant latency differences exist between ipsilateral and contralateral recording. Definitions of abnormalities were based on interwave separation and the wave V amplitude ratio. No case was found among the MS patients with an abnormal contralateral but normal ipsilateral response.     

动态脑电图和脑干听觉诱发电位在昏迷预后判断中的应用

目的探讨昏迷病人动态脑电图（AEEG）和脑干听觉诱发电位（BAEP）表现及其对预后判断的临床意义。方法对96例昏迷病人行动态脑电图和脑干听觉诱发电位检查,并根据结果进行预后判断,与病人随访3个月时结果进行比较。结果AEEG＋BAEP对预后的评估敏感性为92．3％,特异性95．5％,准确率93．8％,错误率为2．1％,Glasgow评分敏感性为76．9％,特异性77.7％,准确率77．3％,错误率为10．4％,经x^2检验,差异有统计学意义（P〈0．05）。结论动态脑电图和脑干听觉诱发电位联合应用对昏迷病人临床顸后的判断有重要意义。     

婴儿痉挛症脑干听觉诱发电位中的γ频带

目的 观察婴儿痉挛症患者脑干听觉诱发电位中γ频带成分。方法 用子波变换分别对正常婴儿、婴儿痉挛症患儿、经过一段时间治疗后的婴儿痉挛症患儿脑干听觉诱发电位信号进行多尺度分析。观察脑干听觉诱发电位信号多尺度成分的分尺度功率随尺度的分布。结果 婴儿痉挛症患儿的脑干听觉诱发电位信号的γ频带的成分缺损严重，其分尺度功率远低于正常婴儿，婴儿痉挛症患儿经过一段时间治疗后脑干听觉诱发电位信号的γ频带成分明显改善，其分尺度功率接近正常婴儿。结论 γ频带对信启在脑干中的接收、传输、加工、综合、反馈等高级功能和人脑的认知活动具有重要作用，γ频带成分缺损导致的信息传导阻滞是引起患儿智能发育迟滞的原因。     

Clinical diagnoses of pediatric patients without detectable auditory brainstem response (ABR)

One hundred and forty-seven out of 1,600 infants and children showed no detectable ABR. ABRs were evoked by clicks. As to hearing acuity, 121 out of 147 patients had sensorineural hearing loss and 19 had no hearing impairment of high frequency (3-4 kHz) and high sound intensity (85-90 dBnHL). Eight patients could not be diagnosed whether they had hearing impairment or not. Among 19 patients who did not have sensorineural hearing loss in those ranges described above, 5 patients had cerebral palsy with severe mental retardation, 4 had mitochondrial encephalomyopathy, 3 had degenerative disease of unknown etiology, 2 patients had Down syndrome, 1 was a low birth weight infant with respiratory distress syndrome, 1 was a neonate born to a diabetic mother, 1 experienced severe neonatal asphyxia, 1 patient had acute lymphoblastic leukemia and 1 infant was later confirmed as a healthy girl. Of these, four patients showed transiently negative response in ABR. Nine patients remained undiagnosed as to hearing because they were in deep coma, had profound brain damage, or died in early neonatal period. They had major neurological diseases: severe neonatal asphyxia, holoprocencephaly, microcephaly, degenerative diseases of the central nervous system of unknown origin, metachromatic leukodystrophy, trisomy of chromosome 18, anoxic encephalopathy, small for gestational age newborn with intracranial bleeding and posthemorrhagic hydrocephalus. When ABR cannot be recorded using high sound intensity clicks, it usually suggests presence of hearing impairment of high frequency range. However, there are patients who have no hearing impairment in those sounds.(ABSTRACT TRUNCATED AT 250 WORDS)     

Somatosensory and brainstem auditory evoked potential studies in nontraumatic coma

We believe that somatosensory and brainstem auditory evoked response studies help in the understanding of the dysfunction of the ascending sensory pathyways at various levels. In some patients where EEGs showed a significant contamination of muscle and background noise, the SEP studies helped to identify the level of dysfunction. The severity of the clinical condition (GCS score) correlated significantly (p = 0.003) with the prolongation of the CCT. Asymmetries in CCTs were more frequent in the stroke group than in the other groups. The presence of asymmetries in CCT in diffuse encephalopathies indicated a variable degree of dysfunction in the ascending sensory pathways, which clinically were not easily identifiable. This fact raised the possibility of either pre-existing lesion(s) or recent insult(s) such as ischemia. The presence or absence of N20 appeared to influence the duration of survival in subgroups. Some degree of difference in duration of survival was noted among the metabolic group with and without N20 potential. The subset of patients with N20 potential survived relatively longer than the group without it. A suggestion of influence was seen in the stroke group, but caution must be exercised because the absence of N20 was compatible with survival. The hypoxic group did not show any difference. A combination of prolonged interpeak EP-N13 and N13-N20 indicated a poor prognosis. A distinct absence of Wave I in BAER limited its usefulness on some occasions. A combination of abnormal interpeak III-V and abnormal CCT seemed to suggest a poor prognosis. Although death generally occurred earlier in the stroke group, age did not seem to influence the mortality in the first 10 days. Similarly, the cause of death also did not seem to influence the course in those 10 days. None of the adult patients survived.     

The brainstem auditory evoked potential in infants and children.

The brainstem auditory evoked potential (BAEP) is a neurophysiological study that provides functional information about the auditory system and brainstem. It provides information that is different from any other form of evaluation. This report tries to summarize technical and clinical information that will make the BAEP more meaningful for the practicing technologist and clinician. Attention is focused on the BAEP as it is used in infants and children, starting with a brief review of auditory physiology, then progressing to normative data, clinical utility, physical setup, preparing the patient, comments on technique, and comments on interpretation. This is not an exhaustive review of the topic, and many important elements of clinical utility had to be left out. This review tries to focus on recent references and on practical issues, and it relies on the experience and opinions of the author. Emphasis has been placed on the use of the BAEP by the neurologist, but an attempt has been made to acknowledge the importance of audiology and otolaryngology as well as speech and language science. Particular emphasis has been placed on the use of the BAEP in assessing the premature infant and young infant at risk for neurological and audiological injury. An enormous amount of published data is available in the literature, and much of it had to be left out of this review. However, the reader is encouraged to develop an enthusiasm for the BAEP and to further explore the broad range of its clinical uses.     

脑干听觉诱发电位及体感诱发电位在重型颅脑损伤患者预后评估中的作用

目的 分析探讨脑干听觉诱发电位(BAEP)及体感诱发电位(SEP)与重型颅脑损伤病人预后的关系.方法 对33 例重型颅脑损伤患者早期行BAEP 及SEP 测定并进行动态监测,同时行头颅CT 检查并记录GCS 评分.结果 BAEP 及SEP 预测预后的敏感性、特异性、准确性均较高.BAEP、SEP 异常程度低,则预后较好;异常程度高,则预后不良.结论 BAEP、SEP 可以比较准确地评估重型颅脑损伤患者的预后.     

Brainstem auditory evoked potential (BAEP) abnormalities in brucellosis

Twelve patients with neurobrucellosis and 17 patients with systemic brucellosis without neurological involvement underwent a brainstem auditory evoked potentials (BAEP) study. All neurobrucellosis patients (100%) showed abnormalities in their BAEP recordings, suggestive of brainstem lesions at various levels. On the other hand, only 5 (29%) of the 17 patients with systemic brucellosis had mild unilateral BAEP abnormalities, while the remaining 12 had normal responses. Comparison of pooled data between the systemic brucellosis and neurobrucellosis groups showed highly significant differences in all BAEP parameters. The recording of BAEP is thus considered a sensitive supplementary method to reveal CNS lesions in patients with neurobrucellosis.     

Positive auditory cortical responses in patients with absent brainstem response

ObjectiveTo compare the detectability of the different auditory evoked responses in patients with retrocochlear lesion.MethodsThe 40-Hz auditory steady state response (ASSR) and the N1m auditory cortical response were examined by magnetoencephalography in 4 patients with vestibular schwannoma, in whom the auditory brainstem response (ABR) was absent.ResultsApparent N1m responses were observed despite total absence of the ABR or absence except for small wave I in all patients, although the latency of N1m was delayed in most patients. On the other hand, clear ASSFs could be observed only in one patient. Very small 40-Hz ASSFs could be detected in 2 patients (amplitude less than 1 fT), but no apparent ASSFs were observed in one patient, in whom maximum speech intelligibility was extremely low and the latency of N1m was most prolonged.ConclusionThe N1m response and 40-Hz ASSR could be detected in patients with absent ABR, but the N1m response appeared to be more detectable than the 40-Hz ASSR.SignificanceCombined assessment with several different evoked responses may be useful to evaluate the disease conditions of patients with retrocochlear lesions.     

Evaluation of brainstem auditory evoked responses in congenital hydrocephalus

Brainstem auditory evoked responses (BAER) were studied in 20 children with clinically diagnosed and CT-confirmed congenital hydrocephalus before and after shunt surgery. Ninety-five percent of the children showed abnormal responses preoperatively. Prolonged wave V latency was the most common abnormality, followed by increased interwave latencies. Total absence of evoked responses was more common in children with communicating hydrocephalus. Following shunt surgery 50% of cases returned to normal and 20% showed a significant improvement. Abnormalities persisted in 10% of cases. BAER abnormalities referrable to caudal brainstem dysfunction recovered first, following CSF diversion. Study of BAER is useful for identifying physiological brainstem abnormalities in hydrocephalic children and promises to be a sensitive non-invasive diagnostic tool for the detection of non-infective complications of shunt surgery, if performed serially during follow up.     

脑干听觉诱发电位在血管性痴呆的应用价值

目的 探讨脑干听觉诱发电位(BAEP)在血管性痴呆患者应用价值.方法 选择200例血管性痴呆患者进行脑干听觉诱发电位(BAEP)检测分析.结果 脑干听觉诱发电位显示研究组III、V波潜伏期(PL)和I～III、III～V、I～V波峰间潜伏期(IPL)及(III～V)/(I～III)比值均高于对照组,差异有统计学意义(P<0.01).结论 脑干听觉诱发电位可客观的反映听觉系统和脑干功能状况,预防疾病进一步发展,对血管性痴呆的临床诊疗及预后均有价值.