相反极性短声所记录的耳蜗微音电位在小儿听神经病诊断中的应用

目的比较听力正常和ABR缺失儿童相反极性短声所记录的耳蜗微音电位(cochlear microphonics,CM)和DPOAE,以探讨它们在小儿听神经病中的诊断价值。方法根据短声ABR阈值、DPOAE和声导抗测试等结果,将受试儿分为听力正常及ABR缺失两组。听力正常组由短声ABR阈值≤30dB nHL、DPOAE正常的54名受试儿(91耳)组成,年龄为1~43月龄,平均为8月龄(中位数4月龄)。ABR缺失组由短声ABR100dB nHL无反应的93名(158耳)受试儿组成,年龄为2~67月龄,平均22月龄(中位数18月龄)。测试状态为水合氯醛镇静睡眠。ABR的刺激声为短声,极性为疏波和密波,刺激速率为19.3次/秒,耳机为ER-3A插入式耳机。听力正常组ABR的分析强度为80dBnHL,ABR缺失组为100dBnHL。结果听力正常组除6耳(6.6%)在80dBnHL未记录到明显的CM外,其余85耳(93.4%)均记录到CM,其时程为0.89±0.19ms。ABR缺失组中24耳记录到CM,其中有12耳(6人)(7.6%)DPOAE正常,有12耳(5人双耳,2人单耳)(7.6%)未记录到DPOAE,该组CM时程为2.61±0.72ms。听神经病在所有ABR缺失病例中的发病率为13.98%。结论CM是一种可靠的评价内耳外毛细胞功能的指标,与DPOAE相比,不易受到其它病理状态的影响。如果不进行CM的测试,可能会将一部分听神经病误诊为耳蜗性听力损失,因此建议对于双耳ABR最大输出无反应、而未记录到DPOAE的婴幼儿病例行CM检查,以避免听神经病的漏诊。     

儿童单侧听神经病附三例分析

目的 探讨儿童单侧听神经病的听力学特征。方法 对3例单侧听神经病患儿进行纯音听阈(PTT)、声反射(AR)、听性脑干反应(ABR)和畸变产物耳声发射(DPOAE)检查，并对其结果进行综合分析。结果 3例患儿的PTT均呈1侧耳听力正常，另1侧耳听力丧失。健耳的同侧和交叉AR能引出，患耳的同侧和交叉AR未能引出。健耳的ABR各波潜伏期正常，患耳的ABR各波未能引出。双耳DPOAE各频率反应幅值正常。结论：DPOAE正常或基本正常，PTT、AR和ABR异常是听神经病的重要特征。单侧听神经病的听力学特征与双侧听神经病基本一致。对儿童表现为单侧感音神经性聋者宜进行系统的听力学检查，以期作出正确的诊断。     

蜗神经发育不良患者的听力学特征分析

目的探讨蜗神经发育不良(cochlear nerve deficiency,CND)患者的听力学特征。方法以30例(54耳)(男14例,女16例,年龄6月~29岁,平均4.9岁)内听道斜矢状位磁共振成像示蜗神经发育不良的患者为研究对象,回顾性分析患者的纯音测听或行为测听、声导抗、耳声发射、听性脑干反应(ABR)、听性稳态反应等听力学检测结果及颞骨高分辨CT(HRCT)结果,分析蜗神经发育异常者的听力学特征。结果 30例54耳蜗神经发育不良患者中,双侧24例(80%),单侧6例(20%),其中蜗神经缺如33耳,蜗神经细小21耳。54耳中,共有25耳(46.29%)伴内耳畸形,而单侧蜗神经发育不良者的对侧6耳中,50%(3耳)伴内耳畸形。纯音测听或行为测听和听性稳态反应结果均提示蜗神经发育不良患耳呈重度-极重度听力损失,且蜗神经缺如耳较蜗神经细小耳的听力损失程度更重。11耳(20.37%,11/54)蜗神经发育不良耳可在80~100 dB nHL高强度声刺激下引出听性脑干反应;6例双侧蜗神经发育不良者(20.0%,6/30)中7耳(1例双耳,5例单耳)全部频率或部分频率引出DPOAE,但其中5耳未引出ABR,2耳分别于100和90 dB nHL刺激声强下引出ABR。结论蜗神经发育不良患者常表现为重度-极重度听力损失,近一半伴内耳畸形,部分患耳可引出DPOAE,而ABR严重异常或缺失。     

397例听神经瘤听力特征分析

目的 分析听神经瘤患者听力学特征,为筛查听神经瘤提供参考依据。方法 回顾性分析2019年8月—2022年4月诊治的397例单侧听神经瘤患者临床资料,所有患者均行纯音测听、声导抗测试、言语识别率(SDS)、畸变产物耳声发射(DPOAE)、听性脑干诱发电位(ABR)和颅脑增强MRI。结果 以典型症状就诊者312例,非典型症状就诊者85例。以突发性聋就诊者25例;患耳听力正常者54例,包含大型甚至特大型肿瘤,99例全聋者亦包含内听道和中型肿瘤。5例全聋者言语识别能力未完全消失。DPOAE一致型245例,耳蜗型72例,蜗后型79例。ABR阳性率98.0%(389/397),8例ABR波形正常者中3例为双侧非对称听力损失。结论 非典型症状就诊者占比21.4%,以突发性聋就诊、听力正常或ABR正常者不能排除听神经瘤;DPOAE和ABR可作为筛查听神经瘤的重要检查方法。     

小儿类听神经病的临床表现

目的:报道1组小儿类听神经病(ANSD)的临床特征,以加深对该病的认识。方法:回顾性分析84例(151耳)ANSD患儿病史、影像学、听力学表现及人工耳蜗植入效果。结果:①高胆红素血症11例,家族史2例,缺氧3例,早产1例,基底核病变1例,先天性脑发育落后2例,脑瘫1例,蜗神经发育不全13例;②79.8%(67/84)为双侧发病,20.2%(17/84)为单侧发病;③短声ABR:70.2%(106/151)在最大输出(100dB nHL)无反应波形引出,29.8%(45/151)在非常高的刺激强度仅有分化不良的Ⅴ波;④行为听力测试:23例有测试结果者中,1例为轻度感音神经性聋,2例重度,20例为极重度;⑤影像学:29例有内耳MRI者中,外、中、内耳结构正常者16例,蜗神经发育不良12例,蜗神经发育不良伴双脑室周围白质髓鞘形成不良1例;⑥人工耳蜗植入效果:5例患耳植入人工耳蜗,其中3例术后1年婴幼儿有意义听觉整合量表得分与蜗性聋儿相当,1例稍差于蜗性聋儿,另1例无效。结论:ANSD是听力学表现相同的一大类疾病,在病史、听力学、影像学及人工耳蜗植入效果方面存在多样性,因此对此类患儿应进行全面、详细的临床检查,以便制...     

小儿蜗神经发育不良

目的报道一组蜗神经发育不良(cochlear nerve deficiency,CND)小儿的临床特征,以提高对本病的认识。方法回顾性分析2007年1月到2008年4月就诊的、内耳MRI及听力学资料完整的20例(37耳)CND小儿(其中男13例,女7例,年龄10个月到4岁)的影像学、听力学表现及人工耳蜗植入效果。结果 85%(17/20)的患儿为双侧发病,15%(3/20)为单侧发病;16.22%(6/37)合并前庭神经发育异常,8.11%(3/37)合并面神经发育异常。根据是否伴有内耳畸形分为三组:32.43%(12/37)伴耳蜗畸形或同时伴有前庭畸形(第一组);13.51%(5/37)仅伴前庭畸形(第二组);54.05%(20/37)独立发病,不伴内耳畸形(第三组)。听力学测试结果:86.49%(32/37)ABR最大输出(100 dB nHL)无反应,13.51%(5/37)在非常高的刺激强度仅有分化不良的波Ⅴ;9耳有行为听阈结果者均为极重度感音神经性聋;第一组12耳DPOAE和CM均未引出,第二组60%(3/5)DPOAE和CM均未引出,40%(2/5)DPOAE和/或CM引出;第三组45%(9/20耳)DPOAE和CM均未引出,55%(11/20耳)DPOAE和/或CM引出。人工耳蜗植入效果:2例术前、术后资料完整者中1例术后开机一年婴幼儿有意义听觉整合量表(infant and toddler meaningful auditory integration scale,IT-MAIS)评估得分与蜗性聋儿相当,另1例无效。结论 CND患儿多无特殊病史,根据是否伴有内耳畸形及内耳畸形的种类,其听力学表现多样,诊断主要依据影像学检查,应注意鉴别诊断,以便制定合理有效的干预措施。     

严重高胆红素血症新生儿的听力学特征

目的探讨严重高胆红素血症新生儿的听力学特点及其与血清总胆红素浓度峰值的相关性。方法对100名严重高胆红素血症新生儿(研究组)和30名正常新生儿(对照组)进行畸变产物耳声发射(DPOAE)、听性脑干反应(ABR)和1 000 Hz探测音声导抗检查,于3月龄和6月龄时对研究组进行复查。比较两组结果并分析患儿血清总胆红素浓度峰值与ABR反应阈的相关性。结果①对照组ABR、DPOAE、声导抗检查均正常;②研究组中所有患儿鼓室导抗图均为A型,15人22耳有不同程度的听力损失(ABR异常),其中6人(40%,6/15)9耳DPOAE不能引出,9人(60%,9/15)13耳DPOAE引出。3月龄复查时,15人中听力损失改善3人(4耳),加重1人(2耳),6月龄复查时与3月龄时无改变;③研究组血清总胆红素浓度峰值与ABR反应阈呈浓度依赖性正相关,血清总胆红素>500μmol/dl是高胆红素致听力损失的风险因素。结论严重新生儿高胆红素血症导致的听力损失表现为单耳或双耳感音神经性聋,部分表现为听神经病的听力学特点,3～6月龄部分听力损失可逆转,部分听力损失可继续加重。     

小儿蜗后听觉神经损害并耳蜗受损的听力学特征分析

目的 探讨听性脑干反应(auditory brainstem response,ABR)严重异常、畸变产物耳声发射(distortion product otoacoustic emission,DPOAE)轻微异常为特征的小儿蜗后听觉神经功能损害同时合并耳蜗功能受损的临床听力学特征.方法 选取2002～2006年听力专科中ABR严重异常、最大强度声刺激(103 dB nHL)时才出现波Ⅴ或无波分化、排除了中耳传导功能异常组,镫骨肌声反射消失、DPOAE轻微异常患儿30例(47耳)作为研究对象,详细登记围产期病史,并与同龄听力正常对照组、蜗性病变听力损害组比较DPOAE幅值及引出率的异同.结果 ①30例蜗后病变合并耳蜗功能轻度受损的患儿中,23例(76.67%)新生儿期有高胆红素血症病史,其中13例(56.52%)血中间接胆红素水平达重度标准,曾经"换血"治疗和/或遗留核黄胆所致脑性瘫痪;②蜗后病变合并耳蜗功能轻度受损耳, DPOAE幅值较正常对照组出现一定程度的下降,而与ABR波V阈值为60 dB nHL的蜗性病变耳DPOAE幅值相近;③蜗后病变合并耳蜗功能轻度受损耳DPOAE幅值异常的频率点分布主要在F9、F10和F11三个频点.结论 以蜗后病变为主的听力损害患儿中,存在一定比例的耳蜗同时受累现象,受累的部位主要在耳蜗的基底部,以高频听力损失为主;新生儿期重度高胆红素血症是导致这一现象的最主要致病因素.     

听性脑干反应严重异常和耳声发射正常的小儿听力障碍的临床特征分析

目的探讨包含小儿听神经病在内的听性脑干反应(auditory brainstem response，ABR)严重异常，畸变产物耳声发射(distortion product otoacoustic emission，DPOAE)正常为特征的听功能障碍症侯群的病因学特征、病损部位与临床听力学特点。方法选取2002～2004年听力专科中ABR严重异常、DPOAE正常，排除中耳传导功能异常的患儿98例(168耳)入选为研究组。根据神经康复科医师的评估，作出是否伴有精神运动发育迟缓及其相关病因的诊断，统计伴有精神运动发育迟缓的研究组中听力损失高危因素的类型与比例；选择ABR严重异常、DPOAE异常、排除中耳病变的患儿46例(82耳)作为蜗性病变对照组，比较研究组与蜗性病变患儿ABR的异同点；选择健康同龄小儿40例(72耳)作为正常对照组，观察研究组与正常听力小儿ABR和DPOAE的异同点。结果①研究组98例患儿中，83.67％患儿伴有精神运动发育迟缓的临床症状，其相关的致病因素中，以新生儿高胆红素血症所占的比例最高；②103 dB强度刺激声(听力级)刺激时，无波分化的为53耳、仅能记录到波Ⅴ的7耳，90dB强度刺激声仅能记录到Ⅴ波的9耳，80dB仅能记录到Ⅴ波的4耳，共73耳(43．45％)，病损部位主要在听神经远端；103dB强度刺激声仅能记录到波Ⅰ，其他各波缺失的28耳(16．07％)，病损部位主要在低位脑干；可同时记录到波Ⅰ 波Ⅲ和(或)波Ⅴ的67耳(39．88％)，其中有16耳能引出1kHz纯音同侧镫骨肌声反射，提示耳蜗、第Ⅷ颅神经听支、低位脑干或有可能均存在不同程度的受累；③高频区DPOAE幅值正常或轻微下降，中高频区DPOAE引出率与正常对照组一致。结论新生儿高胆红素血症是包含小儿听神经病在内的ABR严重异常DPOAE正常为特征的听功能障碍症侯群的最主要致病因素，临床上应积极预防和及时治疗，以减少小儿听神经病的发生；包含小儿听神经病在内的ABR严重异常DPOAE正常为特征的听功能障碍症侯群的听力学改变具有多样性，在临床诊断上应加以注意。     

听神经病的临床与听功能特征

目的:探讨听神经病的临床与听功能特征。方法:总结分析54例听神经病患者的临床资料、听力学测试及电生理检查情况。结果:纯音听力图呈上升型70耳,覆盆型25耳,平坦型5耳,下降型4耳;低频、中频及高频平均阈值为(67.63±15.30,43.61±16.28,32.25±14.80)dB HL。声导抗鼓室图全部正常,77耳镫骨肌声反射消失,31耳声反射阈部分增高。听性脑干反应(ABR)全部未引出。畸变产物耳声发射(DPOAE)正常引出,26例行对侧声抑制未受影响。16例言语识别率差,与纯音听阈不成比例。23例颞骨CT或MRI未见异常。10例伴有周围神经病。结论:ABR自波Ⅰ起缺失而DPOAE正常引出,言语分辨力差与纯音听阈不成比例,镫骨肌声反射及OAE交叉抑制异常,纯音听力图多呈上升型以低频损失为主,是听神经病听功能的重要特征。提示病损主要位于耳蜗内听神经纤维。应与一般的感音神经性聋和中枢性聋相鉴别。     

Auditory brainstem response and otoacoustic emission assessment of hearing-impaired children of mothers who contracted rubella during pregnancy

CONCLUSIONS: Based on the auditory brainstem response (ABR) results obtained in this study, the incidence of deafness in children whose mothers had rubella during pregnancy is high (29.5%), and deafness is profound in most cases (80%). Vaccinating women of childbearing age against rubella is essential to reduce the number of cases of childhood sensorineural hearing loss caused by gestational rubella. OBJECTIVE: It has been shown in the literature that, in Brazil, gestational rubella is responsible for approximately 21% of cases of deafness in children. The purpose of this study was to assess the incidence of deafness in children whose mothers had rubella during pregnancy. MATERIAL AND METHODS: Between February and July 2001, we conducted a prospective study assessing hearing status in 17 children (mean age 6 months). The mothers had serologically (ELISA) confirmed gestational rubella. We recorded ABRs and analysed distortion product otoacoustic emissions (DPOAEs). RESULTS: In 5 children (29.5%), ABRs revealed sensorineural hearing loss, which was moderate to severe in 1 (20%) and profound in 4 (80%). The hearing loss was bilateral in 3 children (60%) and asymmetrical in 4 (80%). Regarding DPOAEs, 7 children (41%) presented no response, and this occurred bilaterally in 4 (57%). All children with abnormal ABRs also presented abnormal DPOAEs. Two children with normal ABRs presented abnormal DPOAEs.     

High-frequency hearing impairment assessed with cochlear microphonics

Abstract Conclusion: Cochlear microphonic (CM) measurements may potentially become a supplementary approach to otoacoustic emission (OAE) measurements for assessing low-frequency cochlear functions in the clinic. Objective: The objective of this study was to investigate the measurement of CMs in subjects with high-frequency hearing loss. Currently, CMs can be measured using electrocochleography (ECochG or ECoG) techniques. Both CMs and OAEs are cochlear responses, while auditory brainstem responses (ABRs) are not. However, there are inherent limitations associated with OAE measurements such as acoustic noise, which can conceal low-frequency OAEs measured in the clinic. However, CM measurements may not have these limitations. Methods: CMs were measured in human subjects using an ear canal electrode. The CMs were compared between the high-frequency hearing loss group and the normal-hearing control group. Distortion product OAEs (DPOAEs) and audiogram were also measured. Results: The DPOAE and audiogram measurements indicate that the subjects were correctly selected for the two groups. Low-frequency CM waveforms (CMWs) can be measured using ear canal electrodes in high-frequency hearing loss subjects. The difference in amplitudes of CMWs between the high-frequency hearing loss group and the normal-hearing group is insignificant at low frequencies but significant at high frequencies.     

Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy

OBJECTIVE: To define both auditory nerve and cochlear receptor functions in subjects with auditory neuropathy (AN). DESIGN: We tested 33 AN subjects (66 ears) and compared them with 21 healthy subjects (28 ears). In AN subjects, the average pure-tone (1, 2, and 4 kHz) threshold loss was 57 dB HL. Click stimuli were used to elicit transient evoked otoacoustic emissions (TEOAEs), cochlear microphonics (CMs), and auditory brain stem responses (ABRs). Both cochlear and ABR potentials were recorded from surface electrodes (vertex-ipsilateral mastoid) using averaging procedures. The amplitudes and latencies of CMs and ABRs and the amplitude of the TEOAEs were analyzed. RESULTS: CM amplitudes recorded from normal ears decreased as a function of subject age. CMs recorded from AN subjects fell within the normal age-adjusted range in 60% of the subjects and were >2 SEEs (standard error of estimate) above the age-adjusted normal regression in 40% of the subjects. TEOAEs were absent in 19 (30%) AN ears (bilaterally in eight, and unilaterally in three subjects) and were present in 44 ears. In AN subjects, correlations among CM amplitude, TEOAE amplitude, and pure-tone average thresholds were not significantly related. CM amplitudes were not significantly different whether TEOAEs or ABRs were present or absent. The ABR was present in 21% of AN subjects and consisted of a low-amplitude Wave V without a preceding Wave I. Measures of CM amplitude and PTA hearing loss were not significantly different in those AN ears with a preserved ABR compared with ears with absent ABRs. Summating potentials to transient click stimuli were of small amplitude (<0.1 microV) and detectable in approximately 50% of the AN and healthy control subjects limiting formal analysis of summating potentials. CONCLUSIONS: In a significant proportion of AN subjects, we found abnormalities of cochlear receptor function, including elevated CM amplitudes and absence of TEOAEs. These two abnormalities occurred independently of each other. A low amplitude Wave V of the ABR was found in approximately one-fifth of AN subjects, evidence that neural synchrony can be partially preserved in some subjects with this disorder.     

Auditory brainstem response and otoacoustic emission assessment of hearing-impaired children of mothers who contracted rubella during pregnancy

Conclusions Based on the auditory brainstem response (ABR) results obtained in this study, the incidence of deafness in children whose mothers had rubella during pregnancy is high (29.5%), and deafness is profound in most cases (80%). Vaccinating women of childbearing age against rubella is essential to reduce the number of cases of childhood sensorineural hearing loss caused by gestational rubella.

Objective It has been shown in the literature that, in Brazil, gestational rubella is responsible for ≈21% of cases of deafness in children. The purpose of this study was to assess the incidence of deafness in children whose mothers had rubella during pregnancy.

Material and methods Between February and July 2001, we conducted a prospective study assessing hearing status in 17 children (mean age 6 months). The mothers had serologically (ELISA) confirmed gestational rubella. We recorded ABRs and analysed distortion product otoacoustic emissions (DPOAEs).

Results In 5 children (29.5%), ABRs revealed sensorineural hearing loss, which was moderate to severe in 1 (20%) and profound in 4 (80%). The hearing loss was bilateral in 3 children (60%) and asymmetrical in 4 (80%). Regarding DPOAEs, 7 children (41%) presented no response, and this occurred bilaterally in 4 (57%). All children with abnormal ABRs also presented abnormal DPOAEs. Two children with normal ABRs presented abnormal DPOAEs.     

Assessment of cisplatin-induced ototoxicity using derived-band ABRs.

Ototoxicity is an adverse side effect of numerous therapeutic agents (amino-glycoside antibiotics, blood chelating agents, diuretics and oncologic drugs) used in treatment of both adult and pediatric patients. Recently, there has been increasing interest in using the auditory brainstem response (ABR) to detect both short-term effects of ototoxicity in adults and long-term effects of drug administration on neonates and children. Since click ABRs have relatively poor frequency selectivity they best approximate the pure-tone hearing threshold in the 2000-4000 Hz frequency range. Hearing loss above or below that frequency range can be present without producing significant abnormalities in the ABR waveform parameters. Frequency-specific ABRs can be obtained using the derived response technique. The purpose of this study was to investigate early cisplatin ototoxicity using both the broadband click and derived ABR and to monitor progressive hearing loss with repeated drug trials in 18 patients studied over a 2-year period. ABRs were obtained serially prior to and following intravenous administration of cisplatin. Derived ABRs were found to be more sensitive than broadband click ABR in detecting early high-frequency hearing loss. For click ABRs, the cumulative dosage of cisplatin at age of ABR examination was correlated with hearing loss in only those patients under 3 years of age. No significant correlation was found between cumulative cisplatin dosage when tested and degree of hearing loss in those patients over 3 years of age.     

Audiological and electrocochleography findings in hearing-impaired children with connexin 26 mutations and otoacoustic emissions

We recorded cochlear potentials by transtympanic electrocochleography (ECochG) in three hearing-impaired children with GJB2 mutation who showed otoacoustic emissions. Pure tone thresholds, distortion product otoacoustic emissions (DPOAEs) and, auditory brainstem responses (ABRs) were also obtained. Subjects 1 (35delG/35delG) and 3 (M34T/wt) had profound hearing loss and showed the picture of auditory neuropathy (AN) as DPOAEs were detected with absent ABRs in both ears. The hearing impairment found in subject 2 (35delG/35delG) was profound in the right ear and moderate in the left ear. Both DPOAEs and ABRs with normal latencies and morphology were recorded only from the left ear. On the ECochG recording the cochlear microphonic was obtained from all children. No compound action potential (CAP) was detected in subject 1. A neural response was recorded only from the left ear in subject 2 with a threshold corresponding to the audiometric threshold while no CAP was detected on the right side. The ECochG obtained from subject 3 showed a low-amplitude broad negative deflection which was identifiable down to low stimulus levels. This response decreased in amplitude and duration when utilizing a high-rate stimulation paradigm. The amount of amplitude reduction was close to that calculated for normal ears, thus revealing the presence of an adapting neural component. These findings indicate that patients with GJB2 mutations and preserved outer hair cells function could present with the picture of AN. The hearing impairment is underlain by a selective inner hair cell loss or a lesion involving the synapses and/or the auditory nerve terminals. We suggest that neonatal hyperbilirubinemia may play a role in protecting outer hair cells against the damage induced by GJB2 mutations.     

畸变产物耳声发射在唇腭裂婴幼儿听力检查中的应用

目的探讨唇腭裂婴幼儿畸变产物耳声发射（distortion product otoacoustic emission,DPOAE）的特点,并将其与听性脑干反应（auditory brainstem response,ABR）阂值检查的结果加以对照,以探讨DPOAE在这类患儿听力检查中的应用价值。方法DPOAE检查63例（126耳）,年龄2个月-42个月,平均11.83个月。其中单纯腭裂组（以下简称腭裂组）23例（46耳）,腭裂并发唇裂及牙槽裂组（以下简称唇腭裂组）30例（60耳）,单纯唇裂组（以下简称唇裂组）10例（20耳）,每耳均检查8个频率,若2-5kHz4个频点有≥3个频点通过即为该耳通过。在上述患儿中,ABR阈值检查腭裂组17例（34耳）,唇腭裂组10例（20耳）,唇裂组6例（12耳）,以能重复引出V波的最小刺激强度为ABR阈值。结果DPOAE检查：腭裂组通过7耳,未通过39耳,通过率为15.22％;唇腭裂组通过6耳,未通过54耳,通过率为10.00％;唇裂组通过18耳,未通过2耳,通过率为90％。统计分析腭裂组与唇腭裂组无显著差异,而腭裂组与唇裂组,唇腭裂组与唇裂组均有显著差异。ABR阈值检查统计分析结果与DPOAE一致。将各组DPOAE通过率与ABR正常率进行比较,腭裂组及唇腭裂组中二者无差异（P〉0.05）,虽然唇裂组中二者有差异（P〈0.05）,但唇裂组ABR阈值反应的听力下降较轻（均≤50dB nHL）。从总体趋势上说,DPOAE与ABR阈值检查在检测的结果上是一致的。结论唇腭裂患儿DPOAE和ABR检测结果一致,与ABR相比,DPOAE具有快速、简便、易实施等特点,因此DPOAE可以作为唇腭裂婴幼儿听力检查的手段,但仍需进一步结合ABR及其他相关的听力检查,以明确听力损害的程度和类型。     

Correlation of transiently evoked to distortion-product otoacoustic emission measures in healthy children

OBJECTIVE: Transiently evoked (TEOAEs) and distortion-product otoacoustic emissions at the 2f1-f2 frequency (DPOAEs) are being used as a clinical tool for diagnosis of peripheral auditory pathology. Because both tests are fast and non-invasive, they may be an excellent method for hearing screening in infants and children. The purpose of this study was to compare the TEOAE and DPOAE measures obtained in a group of healthy children. METHODS: Sixty-six school-aged children with normal hearing were included in the study. Subjects with recent otologic disease or abnormal tympanograms were excluded. TEOAEs and DPOAEs were performed using a DP Echoport ILO 292 Otodynamics analyzer connected to a portable personal computer. Correlation between TEOAE amplitudes and DPOAE levels was estimated. RESULTS: Correlation between TEOAE amplitudes and DPOAE levels was highly significant across all measured frequencies. Correlation was more significant at the middle frequencies than at the low and high frequencies. CONCLUSIONS: Although frequency specific information may be obtained by both tests, most reliable results were obtained at the middle frequencies. TEOAE values were more prominent at low frequencies, whereas DPOAEs were more effective at high frequencies. Both methods are reliable, objective, fast and useful tests of the cochlear status and should be included in the standard audiological diagnostic work-up of children.     

Auditory sensitivity in children using the auditory steady-state response

OBJECTIVE: To determine the effectiveness of auditory steady-state response (ASSR) as a measure of hearing sensitivity in young children suspect for significant hearing loss. DESIGN: Within-subject comparisons of click auditory brainstem response (ABR) thresholds and ASSR thresholds. SUBJECTS: The study population comprised 42 children suspect for hearing loss and subsequently referred for hearing assessment using electrophysiologic techniques. MAIN OUTCOME MEASURES: Electrophysiologic threshold responses for click ABR and ASSR stimuli (0.5, 1, 2, and 4 kHz) for right and left ears. RESULTS: Based on ABR and ASSR thresholds, 50% of the subjects demonstrated significant hearing loss in the severe to profound range. In some subjects, ASSRs were present at higher stimulus levels when click ABRs were absent. Significant correlations (P<.05) were found between high-frequency ASSR and click ABR thresholds for this study sample. For some subjects, ASSR findings suggested differences between ears that were not observable from the no-response click ABR results. CONCLUSIONS: Auditory steady-state response testing may provide additional information for children who demonstrate hearing levels in the severe to profound range. This information may be helpful when selecting the ear for cochlear implantation for a young hearing-impaired child. Multiple objective methods, such as ABR and ASSR testing, may be needed to determine accurate hearing sensitivity for young children being considered for sensory devices, and in particular, cochlear implants.     

obstructive sleep apnea-hypopnea syndrome and hearing in children

Objective To explore the relationship between hypoxemia and hearing in children with obstructive sleep apnea-hypopnea syndrome. Methods Auditory brainstem responses (ABRs) were recorded in 68 ears and distortion product otoacoustic emissions (DPOAEs) in 60 ears in children with OSAHS and type "A" tympanograms, and in 30 ears in normal children. Results ABR latencies of waves Ⅰ, Ⅲ and Ⅴ, and Ⅰ-Ⅲ, Ⅲ-Ⅴ and Ⅰ-Ⅴ intervals were not statistically different between OSAHS and normal children. Wave Ⅰlatency was delayed in children with OSAHS compared to normal children3 (P<0.05). DPOAE amplitudes in children with mild OSAHS were lower than normal children at 8 kHz (P<0.05). DPOAEs were lower at 6 kHz and 8 kHz in children with moderate/severe OSAHS than normal children (P<0.05). Conclusion Cochlear function was affected when AHI was at or greater than lO/hour. ABR and DPOAE can be used to detect early changes in auditory function in children with OSAHS.