急性低频感音神经性听力损失的临床研究

目的　探讨急性低频感音神经性听力损失(acutelow tonesensorineuralhearingloss,ALHL)的病因病理、临床特点和诊治方法。方法　选择30例ALHL患者为研究对象,在详细采集病史和耳科检查的基础上,行系统的听力学检测,包括纯音测听、声导抗测试、听性脑干反应(auditorybrainstemresponse, ABR)和耳声发射(otoacousticemissions,OAE)。所有患者接受为期15天的皮质类固醇激素试验性治疗,疗程结束后随访6~14个月(平均10 2个月)。结果　本组患者以青年为主,临床主诉多为低音调耳鸣、耳闷和听力下降,耳科检查未见异常。所有患者(30例31耳)纯音测听显示轻到中度的低频感音神经性听力损失;鼓室图呈“A”型, 26耳引出镫骨肌声反射,其中14耳Metz试验阳性。受检的20耳中,ABR均正常;初诊时14耳瞬态诱发性耳声发射未通过,畸变产物耳声发射听力图表现为0 .5及0 .75kHz两个频率点上异常。经激素治疗后, 24耳听力完全恢复, 4耳部分恢复, 3耳无变化,总有效率90. 3%。结论　ALHL以突发的低音调耳鸣、听力下降和耳闷为主要表现,常单耳发病,听力学定位诊断提示为蜗性病变,仅累及低频区。本病尚无特效疗法,皮质类固醇激素可能是一种有希望的治疗药物。     

急性低频感音神经性聋76例临床分析

目的探讨急性低频感音神经性聋(acute low-frequeacy hearing loss,ALHL)的临床特点及疗效。方法回顾性分析76例急性低频感音神经性聋患者的临床特征、听功能检查情况及以皮质类固醇激素、扩血管药物、神经营养药及高压氧治疗的疗效。结果 76例患者均以耳闷为主要症状,纯音听力曲线表现为低频下降型,中高频正常,单耳多发,女性多见,不伴眩晕且预后良好;治疗前后平均听阈值(125、250、500Hz)分别为30.5和14.7dB HL,治疗后较治疗前下降15.8dB;治愈率87.50%(70/80),有效率95.00%(76/80),仅2例(4耳)无效。结论 ALHL患者主要表现为耳闷,纯音听阈曲线为低频下降型,皮质类固醇激素及扩血管药物、神经营养药治疗有效。     

单侧低频感音神经性聋：附40例分析

文中对40例单侧低频感音神经性聋患者进行分析,发现患者主诉中有耳鸣的最多(87.5%)、耳聋次(75%)、眩晕和耳闷者各为35%及25%.纯音测听均为低频感音神经性聋,其低频与高频听阈均值之差为26.32 dB.40例中36例听性脑干反应正常.并讨论了产生低频感音神经性聋可能的原因,耳鸣、低频听力损害与内淋巴积水的关系,并提出对低频感音神经性自应有统一的划定标准.     

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±4.62）dBHL,2～8kHz平均听阈为（13.52±3.86）dBHL,治疗后上述平均听阈分别为（21.05±9.74）dBHL和（19.85±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。     

听神经病的临床及基础研究

关于“听神经病”的命名经历几个不同的认识阶段。我国学者顾瑞将其称之为“中枢性低频感音神经性听力减退”,依据是：①纯音听力正常或轻度减退而言语听力显著减退;②镫骨肌反射不能引出或反射衰减阳性;③多数耳蜗电图（electrocochleogram,ECochG）可引出SP,AP振幅降低或消失;④听性脑干反应（auditory brainstemre．sponse,ABR）引不出或不能重复,特别是不能引出Ⅰ～Ⅴ波,提示病变在听觉传导的中枢通路。提出“Ⅰ型传入神经元病”概念,其依据的是患者有双侧进行性听力损失,严重的言语听力障碍,纯音测听以低频为主的上升型听力图,言语测听和ABR改变比纯音听阈改变严重,耳声发射多正常且幅度增大,不被对侧抑制。     

急性低频感音神经性耳聋的临床观察

目的探讨急性低频感音神经性耳聋(acute low-tone sensorineural hearing loss,ALHL)的临床特点和疗效,提高对该疾病的诊断和认识。方法回顾性分析62例ALHL患者的临床表现、听力学检查和治疗情况,总结其临床发展规律。结果发病年龄以青中年为主,女性明显多于男性,多为单耳发病,表现为耳闷或伴耳鸣,听力下降,不伴眩晕,所有患者纯音听阈均表现为轻中度低频感音神经性耳聋,治疗前后分别为(38.71±6.82)dB和(20.56±9.44)dB,两者比较差异具有统计学意义(P<0.05)。鼓室图"A"型,49例(80.9%)镫骨肌反射引出,40例(64.5%)Metz试验阳性,62例ABR均正常。62例患者治疗前DPOAE在0.5～1 kHz的引出率仅为18.7%,反应幅值明显降低,治疗后DPOAE在0.5～1 kHz的引出率提高至43.8%,幅值亦有所提高。结论 ALHL以突发的耳闷和(或)伴耳鸣为主要表现,常单耳发病,青中年女性为主,听力学定位诊断为蜗性聋,仅累及低频区,皮质类固醇激素治疗有较好的疗效。     

听神经病

目的介绍一种特殊的感音神经性听力疾患-听神经病,探讨其临床特征及听力学特点.方法报道5例听神经病患者,2例为成人;3例为儿童.记录患者的临床资料,并对患者进行纯音测听、脑干电反应测听、耳声发射、耳蜗电图及语言辨别率等听力学检查.结果5例均主诉听力下降,听力学检查纯音听阈为轻、中度感音神经性聋,与纯音测听不相符的语言辨别率明显下降,不能引出脑干诱发电位(ABR),耳蜗电图基本正常,畸变产物耳声发射(DPOAE)基本正常.提示外毛细胞功能正常,病变可能在听神经.结论听神经病是一种主诉听力下降,纯音听阈为轻、中度感音神经性聋,不能引出脑干诱发电位(ABR),畸变产物耳声发射正常的听力疾患,临床上应与其他感音神经性聋区别.     

感音神经性聋耳听阈级与短声诱发听性脑干反应潜伏期的关系

分析感音神经神经性聋512耳的0．5k、1k、2k和4kHz纯音听阈级与短声诱发ABRⅠ、Ⅴ波潜伏期的关系，结果表明，各频率的有效刺激级（ECL）与Ⅰ、Ⅴ波潜伏期均有很好的相关性。与正常耳比较。显示感音神经性聋耳在能引出ABR时，就有重振现象。4kHz纯音主观听阈大于50dBHTL的感音性听力减退耳，ABR波Ⅴ潜伏期比同一有效刺激级的正常组短，Ⅰ－Ⅴ波间期与正常组一致。     

低频感音神经性听力损失的病因分析

目的分析低频感音神经性听力损失的病因,以避免漏诊和误诊。方法对56例低频感音神经性听力损失患者详细了解其病史,进行仔细的耳科常规检查及纯音听阈（PTT）、声导抗、听性脑干反应（ABR）、畸变产物耳声发射（DPOAE）、耳蜗电图（ECochG）及CT和/或MRI检查,综合分析各项结果。结果在56例患者中病因不明的急性低频感音神经性听力损失38例,梅尼埃病9例,听神经病6例,听神经瘤1例,多发性硬化1例,小脑半球旁蛛网膜囊肿1例。结论低频听阈升高的上坡型感音神经性听力损失可见于多种疾病。对低频感音神经性听力损失应采用多项组合的听力学检测方法进行检查和综合分析,必要时辅以CT和/或MRI检查可以及时、有效地作出可靠的诊断和鉴别诊断。     

急性低频感音神经性听力损失临床分析

目的探讨急性低频感音神经性听力损失(acute low-frequency sensorineural hearing loss,ALHL)的发病特点、听力学特征及治疗方法。方法回顾性分析47例ALHL患者的临床症状、体征、听力学检查及随访结果。结果 47例ALHL患者平均发病年龄为36.91±10.18岁,女性24例(51.06%),男性23例(48.94%)。单耳发病43例(91.49%,43/47),31例(65.96%)患者伴耳鸣,10例(21.28%)伴耳闷。全部患者纯音听阈均表现为低频听力下降,鼓室导抗图均为A型,ABR均正常。经脱水剂或利尿剂及类固醇激素等治疗后总有效率为85.11%(40/47),1例自愈。随访4～25个月,37例无复发(90.24%,37/41),4例复发(9.76%,4/41)。结论 ALHL一般单耳发病,中青年居多,伴随症状以耳鸣最为常见,其次是耳闷;听力学检测主要提示耳蜗受累;脱水剂或利尿剂及类固醇激素疗效显著;该病有复发的可能,应加强随访。     

Analysis of audio-vestibular assessment in acute low-tone hearing loss

Conclusion This study demonstrated excellent hearing recovery following the combined treatment of diuretic and oral steroid, and electrocochleography (ECoG) was significantly higher than normal side. This study reports characteristics of acute low-tone hearing loss (ALHL) that show the greater low-tone hearing loss, the higher ECoG, and excellent recovery, even-though low-tone hearing loss is worse, which can be different compared with sudden deafness. Objective To analyze ALHL without vertigo, this study compared the ALHL group with all patients exhibiting low-tone hearing loss and ear fullness. Hearing changes and vestibular functions were analyzed. Materials and methods ALHL was defined as a mean hearing loss of?≥?30?dB at 125, 250, and 500?Hz, and?≤?20?dB at 2, 4, and 8?kHz. From 156 cases of low-tone hearing loss of more than 10?dB without vertigo, 31 met the ALHL criteria and were subjected to audio-vestibular assessments including PTA, ECoG, vestibular evoked myogenic potential (VEMP) testing, and caloric testing. Results In ALHL, low-tone hearing loss was 42.7?±?9.5?dB, and 83.9% of ALHL significantly recovered by more than 10?dB. The ECoG in ALHL was 0.334?±?0.11 (higher than 0.25?±?0.08 on the normal side) and ECoG abnormality was 35.5% (the greater low-tone hearing loss, the higher ECoG value).     

Clinical observations of acute low-tone sensorineural hearing loss considered as cochlear hydrops

Acute low-tone sensorineural hearing loss (ALHL) has the following three criteria; obscure origin, acute onset and sensorineural hearing loss limited to low frequencies. Sixteen cases of ALHL which were considered as cochlear hydrops using glycerol test and electrocochleogram were studied. All patients visited our department within two weeks after onset and were followed up for one year or more after initial examination. The subjective symptoms, the character of vestibular and hearing impairment and prognosis of 16 cases with ALHL were also investigated. The results were as follows. 1. Patients complained of ear fullness rather than hearing impairment. Four patients were unaware of hearing loss. 2. Recruitment phenomenon was found in all of 15 cases examined. Vestibular findings were mostly normal, except that spontaneous nystagmus was found in two cases and head-shaking nystagmus in one. 3. Recurrence and fluctuation of hearing impairment occurred in 14 cases. Three cases had an attack of rotatory vertigo once and two has diagnosed as Meniere's disease later. 4. During three months prior to last examination, hearing was stabilized in nine cases and continued to fluctuate in seven cases. In the former, hearing was improved in four cases, unchanged in three, and worsened in two. 5. Two patients underwent an endolymphatic sac operation and have had a good prognosis. 6. The authors suggest that most of ALHL should be considered as transient cochlear hydrops because the subjective symptom and audiological and vestibular findings of our cases are similar to those of cases which were reported as ALHL by other investigators. According to the findings of glycerol test and electrocochleogram, endolymphatic hydrops in ALHL is considered to exist in all turns of cochlea.     

急性低频感音神经性聋镫骨肌声反射消失的病因及临床意义探讨

目的 探讨部分急性低频感音神经性聋(acute low-tone sensorineural hearing loss,ALHL)镫骨肌声反射(acoustic stapedius reflex,AR)消失的原因及临床意义.方法 分析21例(21耳)镫骨肌声反射消失的ALHL患者患耳的声顺值、共振频率、耳蜗电图、DPOAE及ABR的改变,与其健耳以及镫骨肌声反射正常的ALHL患者相应观察值进行对照,并比较患耳上述观察值治疗前后的变化.结果 ALHL镫骨肌声反射消失耳的病变在内耳,其共振频率略高于健耳及对照组,但无显著性差异.患耳耳蜗电图-SP/AP值高于健耳及对照组,差异显著.糖皮质激素及利尿剂治疗后,听力恢复正常16耳,其中仅4耳AR可引出,12耳仍不能引出;其余5耳听力仅部分恢复,AR仍不能引出.结论 膜迷路积水导致镫骨底板活动受限是AR消失的重要原因之一,膜迷路积水同时引起传声系统质量及劲度的改变,其对共振频率的影响取决于积水对镫骨底板活动度的影响程度.     

Bilaterality in acute low-tone sensorineural hearing loss

Bilaterality in acute low-tone sensorineural hearing loss (ALHL) is more generally recognized than that in idiopathic sudden sensorineural hearing loss. Subjects were 274 patients diagnosed with ALHL based on criteria of a study group of the Ministry of Health, Labor and Welfare of Japan, i.e., total of 3 low tone hearing of 70dB or more and, a total of 3 high-tone hearing of 60dB or less, and treated at the departments of otolaryngology at Yamanashi University and Suwa Central Hospital. ALHL involving bilateral ears symptoms and/or bilateral hearing impairment conforming to diagnostic criteria was selected and summarized. Clinical ear symptoms, clinical test results, and hearing levels (total 3 low tone hearing, 1kHz, and total of 3 high-tone hearing) were statistically analyzed. We also reviewed Japanese clinical reports of ALHL that include bilateral cases. In 32 cases (11.7%) of 274 cases, both ear symptoms and hearing impairment were bilateral. In 22 (8.0%) of the 274, bilateral ear symptoms were present, but showed unilateral hearing loss conforming to diagnostic criteria. Another 22 (8.0%) out the 274 reported unilateral ear symptoms, but hearing tests indicated bilateral ALHL. A total of 76 cases (27.7%) of the 274 had bilaterality in either ear symptoms or hearing loss. Our review indicated that 9.0% (162 of 1803) ALHL patients were bilaterally affected, possibly indicating that AIHL includes a larger number of bilateral cases than currently assumed, if the opposite side were given a especially detailed clinical interview. Statistical analysis (Mann Whitney test, P<0.01) of hearing of unilateral cases indicated that 3 low tone hearing was more affected than 3 high-tone hearing, even on the normal side. These results indicate that ALHL tends to be bilateral, possibly due to the mechanism of pathogenesis, and also that the mechanism may include both local and general conditions. This more closely resembles Meniere's disease than idiopathic sudden sensorineural hearing loss. Both sides of bilateral cases were not usually the same in hearing patterns, glycerol test results, or prognosis. A statistically significant difference (Mann Whitney test, P<0.01) in total of 3 low tone hearing was seen between worse and better sides in bilateral cases. The degree of disease on both sides in bilateral cases thus was not always the same. Bilateral cases may result from both the influence of general conditions such as fatigue, stress, and lack of sleep and local conditions such as pathogenesis of endolymphatic hydrops that may cause differences in both ears. No clear difference was seen in clinical symptoms, hearing levels, and clinical examination, e.g., Schellong and glyceol tests, between unilateral and bilateral cases. Bilateral cases had a poorer prognosis (lower complete recovery ratio; chi2 test P<0.01) than unilateral cases. Our results indicated that cautious evaluation of opposite ear is necessary in diagnosis, treatment, and prognostication of ALHL.     

不同剂量糖皮质激素在急性低频下降型感音神经性聋治疗中的疗效差异

目的:探讨不同剂量糖皮质激素在治疗急性低频下降型感音神经性聋(ALHL)中的疗效差异。方法:选取54例(54耳)ALHL患者,随机分为无泼尼松组(10例)、低剂量泼尼松组(22例)和高剂量泼尼松组(22例),治疗10d,并随访6个月。结果:无泼尼松组、低剂量泼尼松组和高剂量泼尼松组痊愈率分别为30.0%、59.1%、90.9%,高、低剂量组比较差异有统计学意义(P<0.05);改善率分别为40.0%、86.4%、95.5%,低剂量、高剂量泼尼松组与无泼尼松组比较均差异有统计学意义(均P<0.05)。结论:用糖皮质激素治疗ALHL疗效显著,高剂量优于低剂量,提示该病与内淋巴水肿和患者自身免疫机制可能相关。     

Alteration of frequency range for binaural beats in acute low-tone hearing loss

The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.     

Idiopathic sudden sensorineural hearing loss and acute low-tone sensorineural hearing loss: a comparison of the results of a nationwide epidemiological survey in Japan

Abstract

Objectives: The aim of this study was to investigate the differences between idiopathic sudden sensorineural hearing loss (SSNHL), and acute low-tone sensorineural hearing loss (ALHL) using the results of a nationwide survey database in Japan and to analyze the variables associated with their clinical features and the severity of hearing impairment, treatment, and prognosis.

Methods: Participants were patients registered between April 2014 and March 2016 in a multicenter epidemiological survey database involving 30 university hospitals and medical centers across Japan. Statistical analysis was performed to clarify the factors associated with their clinical characteristics and the severity of hearing impairment, treatment, and prognosis.

Results: Idiopathic SSNHL and ALHL differed significantly in terms of male-to-female ratio, age distribution, and time from onset to start of treatment. The treatment methods and hearing prognosis also differed markedly between the two diseases. A majority (92%) of idiopathic SSNHL patients were administered some type of corticosteroid, while half of the ALHL patients received corticosteroids and a diuretic agent.

Conclusion: The results suggested that idiopathic SSNHL and ALHL belonged to different categories of inner ear disease.     

Audio-vestibular functions in patients with hemifacial spasm

Audio-vestibular functions and its laterality were evaluated preoperatively on the affected side and the non-affected side of 57 patients operated upon to relieve hemifacial spasm (HFS). One patient had hearing impairment and tinnitus on the affected side and showed abrupt high tone sensorineural loss, directional preponderance and ABR abnormalities. On another patient, the latencies for wave III and V from the affected ear were prolonged, while there was no audiovestibular symptoms. However, there was no significant difference in audio-vestibular symptoms, ABR findings, pure tone thresholds and caloric results between the affected ears and the non-affected ears of the remainder. We believe that HFS and the microvascular compression of the eighth cranial nerve are two different diseases.