一组听神经病病人的听力学特征研究
发布时间：2018-08-06 18:18
【摘要】：从上个世纪80年代开始,国内外临床听力学家、听觉生理学家、耳鼻咽喉科临床医师以及分子遗传学家们开始关注一种具有特殊临床表现及听力学特征的疾病——听神经病,但各家命名不一,直到1996年Starr将其正式命名为听神经病,沿用至今。 听神经病(Auditory neuropathy,AN)是一种表现为声音可以通过外耳、中耳正常的进入到内耳但是声音信号不能同步地从内耳传输到大脑的听功能异常性疾病,定义为第Ⅷ脑神经的听神经支受损而引起的一种临床表现特殊的神经性聋。其特点包括：听性脑干反应(auditory brainstenm responses, ABR)缺失或严重异常,诱发性耳声发射(evoked otoacoustic emission, EOAE)正常,言语识别率下降,且不成比例的明显差于纯音听阈,听力图多以低频听阈升高为主,镫骨肌声反射消失或阈值升高和EOAE对侧抑制消失等。这是一组不同于一般感音神经性聋及中枢性聋、具有独特听力学特点的听功能障碍的症侯群,近年来已引起广泛关注,但对其认识仍处在摸索阶段,其病因及发病机制尚不清楚,命名和病变部位也存在不同意见,治疗亦无十分有效措施。正是由于听神经病存在上述诸多不确定性,目前对于听神经的诊断尚处在功能性诊断层次上,这就突显了听力学检查做为诊断标准的重要性。随着听力学的发展及人们生活水平的提高,越来越多听神经病人被检出,已远远超于预期。 目的：本课题旨在总结听力学检测(PTA、AIM、ABR、DPOAE、CM、SRS)在听神经病诊断与鉴别诊断过程的意义,进一步探讨听神经的临床听力学特征,以提高对AN的诊断和进一步加深对AN的认识。 方法：回顾性调查分析我院2010-09至2011-03耳鼻咽喉门诊根据其主诉、临床表现、初步的临床听力学检查疑为AN的16例患者的临床资料,对此组患者进行一组听力学检查(PTA、AIM、ABR、DPOAE、CM、SRS),并对其结果进行总结分析,探讨听力学检查对AN诊断与鉴别诊断的意义及听神经病的听力学特征。 结果： 1.对象及临床表现 本组病例选我院2010-09至2011-03耳鼻咽喉门诊主诉、临床表现及初步的听力学检查疑为AN的16例病人,均以双耳渐进性听力减退、辨音障碍,尤其在喧闹嘈杂环境中明显就诊；其中2例(4耳)伴有间断性或持续性低音调耳鸣；2例伴有四肢乏力,走路不稳,无其他明显不适,神经内科确诊有周围神经病；还有2例有可疑家族耳聋遗传性疾病史(母亲与胞弟均有同样的临床表现)。本组患者均可用普通话交流,并有不同程度的日常言语交流障碍。所有病例行CT和MRI检查排除听神经瘤、颅内占位等器质性病变、无噪声接触史及耳毒性药物应用史。 2.听力学检查结果 2.1 PTA：本组病人中听力图12例(24耳)为低频上升型,2例(4耳)为低频下降为主高频下降为辅的覆盆形,2例(4耳)为全频下降的平坦型。其中前14例为轻、中度感音神经性聋,听力图中250Hz、500Hz下降明显。后2例为中、重度感音神经性聋,全频下降。16例病人32患耳纯音听阈测试结果气、骨导均下降,符合感音神经听力损失的听力学特征。 2.2 AIM：本组病例16例(32耳)耳均为“A”型曲线。镫骨肌声反射15例(30耳)同侧及对侧声反射均未引出,1例(2耳)的单项或多项频率镫骨肌声反射可引出,但镫骨肌声反射阈值均升高,且反射诱出耳声衰减试验阳性,2例(4耳)有响度重振现象其余均无。 2.3 DPOAE:本组病人中14例(28耳)可引出DPOAE,不能被对侧白噪声抑制,2例未引出。 2.4 ABR：本组病人中14例(28耳)ABR不能引出(97dBnHL);2例(4耳)仅出现波V (90dBnHL),波分化可,波潜伏期延长。 2.5 CM：本组病人中14例(28耳)CM波形基本正常,2例(4耳)无正常波形可见。 2.6 SRS：本组病人中(安静状态下)14例言语识别率均差,范围在0%-58%之间,与纯音听阈明显不成比例,随声音强度增加,言语分辨率不升反降；2例言语识别率分别为86%,88%,且随声音强度增加言语识别率增加。 结论： 1.听神经病具有独特的听力学特征,听力学检查为诊断的主要标准。 2.本组病例中14例为听神经病,2例为一般感音性耳聋。 3.各听力学检查之间互相弥补、层层递进,共用避免漏诊误诊。 4.必要时行影像学及其它系统检查。
[Abstract]:From the 80s of the last century, clinical audiologists, audiologists, otolaryngologists, clinicians and molecular geneticists began to pay attention to a disease with special clinical and audiological characteristics - Acoustic neuropathy, but different names were named as auditory neuropathy in 1996 Starr. So far.
Auditory neuropathy (AN) is a kind of abnormal neurogenic deafness, defined as a special neurogenic deafness caused by the damage of the auditory nerve branch of the eighth brain nerve, as the sound can pass through the external ear and the middle ear normally enters the inner ear but does not transmit the sound signals synchronously from the inner ear to the brain. The points included: auditory brainstem response (auditory brainstenm responses, ABR) deletion or severe abnormalities, evoked otoacoustic emission (evoked otoacoustic emission, EOAE) normal, speech recognition rate decreased, and disproportionate difference between pure tone threshold and low frequency audiometry, stapes reflex disappearance or threshold increase and E OAE disappearance of contralateral inhibition. This is a group of symptoms different from general sensorineural deafness and central deafness, with unique audiological characteristics of hearing impairment. In recent years, it has attracted extensive attention, but its understanding is still in the exploratory stage, its etiology and pathogenesis are still unclear, naming and diseased parts have different opinions, treatment and treatment. There is no very effective measure. It is due to the many uncertainties of auditory neuropathy. At present, the diagnosis of auditory nerve is still at the level of functional diagnosis, which highlights the importance of audiological examination as a diagnostic standard. With the development of Audiology and the improvement of people's living standards, more and more patients have been detected. It's far more than expected.
Objective: the purpose of this study is to summarize the significance of audiological detection (PTA, AIM, ABR, DPOAE, CM, SRS) in the diagnosis and differential diagnosis of auditory neuropathy, and to further explore the clinical audiological features of the auditory nerve, so as to improve the diagnosis of AN and further deepen the understanding of AN.
Methods: a retrospective study was conducted to analyze the clinical data of 16 patients suspected of AN in our hospital from 2010-09 to 2011-03 otorhinolaryngology. A set of audiological tests (PTA, AIM, ABR, DPOAE, CM, SRS) were carried out in this group of 16 patients who were suspected to be suspected to be in the clinical audiology. The significance of diagnosis and differential diagnosis and audiological features of auditory neuropathy.
Result:
1. objects and clinical manifestations
16 cases of 2010-09 to 2011-03 otorhinolaryngology in our hospital were selected, clinical manifestations and preliminary audiological examination were suspected to be 16 patients with doubtful hearing loss, dysarthria, especially in noisy and noisy environment; 2 cases (4 ears) were accompanied by intermittent or persistent bass tinnitus; 2 cases were accompanied with extremities. Power, walking instability, no other obvious discomfort, neurology confirmed peripheral neuropathy, and 2 cases of suspected family deafness hereditary disease history (both mother and brother have the same clinical manifestation). This group of patients can communicate in Mandarin and have different degrees of daily verbal communication disorder. All cases of routine CT and MRI examination exclude auditory nerve. Tumor, intracranial occupying and other organic lesions, no noise exposure history and application history of ototoxic drugs.
2. results of audiological examination
2.1 PTA: 12 cases (24 ears) were low frequency ascending type of hearing (24 ears), 2 cases (4 ears) were low frequency descent as the auxiliary raspberry, 2 cases (4 ears) were flat type of full frequency descent. Among them, the first 14 cases were light, moderate sensorineural deafness, and 250Hz and 500Hz decreased in the hearing map. In the last 2 cases, severe sensorineural deafness and full frequency decreased. 32 of the 16 patients had pure tone hearing threshold test. The results showed that gas and bone conduction were all decreased, which accords with the audiological characteristics of sensorineural hearing loss.
2.2 AIM: 16 cases (32 ears) of the group were all "A" curve. Acoustic reflex of stapes muscle was not elicited in 15 cases (30 ears) on the same side and contralateral acoustic reflex. 1 cases (2 ears) had a single or multiple frequency stapes reflex, but the acoustic reflex threshold of stapes muscle increased, and the reflex induced ear attenuation test was positive, and 2 cases (4 ears) had loudness rejuvenating phenomenon. The rest is not.
2.3 of the 14 patients (28 ears) in the DPOAE: group, DPOAE could not be induced, and could not be suppressed by contralateral white noise, and 2 cases were not induced.
2.4 ABR: 14 cases (28 ears) could not induce ABR (97 dBnHL); 2 cases (4 ears) only had wave V (90 dBnHL), wave differentiation was possible, wave latency was prolonged.
2.5 CM: of the 14 patients (28 ears), the CM waveforms were basically normal, and 2 (4 ears) had no normal waveforms.
2.6 SRS: in this group of patients (quiet state), the recognition rate of 14 examples is equal, the range is between 0%-58%, and it is not proportional to the pure tone hearing threshold. With the increase of sound intensity, speech resolution is not rising, and the recognition rate of 2 examples is 86%, 88% respectively, and the rate of speech recognition increases with the increase of sound intensity.
Conclusion:
1. auditory neuropathy has unique audiological characteristics. Audiological examination is the main criterion for diagnosis.
2. in this group, 14 cases were auditory neuropathy and 2 cases were general sensorineural hearing loss.
3. each audiological examination is complementary to each other, progressively, sharing to avoid misdiagnosis.
4. necessary imaging and other systematic examination.
【学位授予单位】：中南大学
【学位级别】：硕士
【学位授予年份】：2011
【分类号】：R764
本文编号：2168585
[Abstract]:From the 80s of the last century, clinical audiologists, audiologists, otolaryngologists, clinicians and molecular geneticists began to pay attention to a disease with special clinical and audiological characteristics - Acoustic neuropathy, but different names were named as auditory neuropathy in 1996 Starr. So far.
Auditory neuropathy (AN) is a kind of abnormal neurogenic deafness, defined as a special neurogenic deafness caused by the damage of the auditory nerve branch of the eighth brain nerve, as the sound can pass through the external ear and the middle ear normally enters the inner ear but does not transmit the sound signals synchronously from the inner ear to the brain. The points included: auditory brainstem response (auditory brainstenm responses, ABR) deletion or severe abnormalities, evoked otoacoustic emission (evoked otoacoustic emission, EOAE) normal, speech recognition rate decreased, and disproportionate difference between pure tone threshold and low frequency audiometry, stapes reflex disappearance or threshold increase and E OAE disappearance of contralateral inhibition. This is a group of symptoms different from general sensorineural deafness and central deafness, with unique audiological characteristics of hearing impairment. In recent years, it has attracted extensive attention, but its understanding is still in the exploratory stage, its etiology and pathogenesis are still unclear, naming and diseased parts have different opinions, treatment and treatment. There is no very effective measure. It is due to the many uncertainties of auditory neuropathy. At present, the diagnosis of auditory nerve is still at the level of functional diagnosis, which highlights the importance of audiological examination as a diagnostic standard. With the development of Audiology and the improvement of people's living standards, more and more patients have been detected. It's far more than expected.
Objective: the purpose of this study is to summarize the significance of audiological detection (PTA, AIM, ABR, DPOAE, CM, SRS) in the diagnosis and differential diagnosis of auditory neuropathy, and to further explore the clinical audiological features of the auditory nerve, so as to improve the diagnosis of AN and further deepen the understanding of AN.
Methods: a retrospective study was conducted to analyze the clinical data of 16 patients suspected of AN in our hospital from 2010-09 to 2011-03 otorhinolaryngology. A set of audiological tests (PTA, AIM, ABR, DPOAE, CM, SRS) were carried out in this group of 16 patients who were suspected to be suspected to be in the clinical audiology. The significance of diagnosis and differential diagnosis and audiological features of auditory neuropathy.
Result:
1. objects and clinical manifestations
16 cases of 2010-09 to 2011-03 otorhinolaryngology in our hospital were selected, clinical manifestations and preliminary audiological examination were suspected to be 16 patients with doubtful hearing loss, dysarthria, especially in noisy and noisy environment; 2 cases (4 ears) were accompanied by intermittent or persistent bass tinnitus; 2 cases were accompanied with extremities. Power, walking instability, no other obvious discomfort, neurology confirmed peripheral neuropathy, and 2 cases of suspected family deafness hereditary disease history (both mother and brother have the same clinical manifestation). This group of patients can communicate in Mandarin and have different degrees of daily verbal communication disorder. All cases of routine CT and MRI examination exclude auditory nerve. Tumor, intracranial occupying and other organic lesions, no noise exposure history and application history of ototoxic drugs.
2. results of audiological examination
2.1 PTA: 12 cases (24 ears) were low frequency ascending type of hearing (24 ears), 2 cases (4 ears) were low frequency descent as the auxiliary raspberry, 2 cases (4 ears) were flat type of full frequency descent. Among them, the first 14 cases were light, moderate sensorineural deafness, and 250Hz and 500Hz decreased in the hearing map. In the last 2 cases, severe sensorineural deafness and full frequency decreased. 32 of the 16 patients had pure tone hearing threshold test. The results showed that gas and bone conduction were all decreased, which accords with the audiological characteristics of sensorineural hearing loss.
2.2 AIM: 16 cases (32 ears) of the group were all "A" curve. Acoustic reflex of stapes muscle was not elicited in 15 cases (30 ears) on the same side and contralateral acoustic reflex. 1 cases (2 ears) had a single or multiple frequency stapes reflex, but the acoustic reflex threshold of stapes muscle increased, and the reflex induced ear attenuation test was positive, and 2 cases (4 ears) had loudness rejuvenating phenomenon. The rest is not.
2.3 of the 14 patients (28 ears) in the DPOAE: group, DPOAE could not be induced, and could not be suppressed by contralateral white noise, and 2 cases were not induced.
2.4 ABR: 14 cases (28 ears) could not induce ABR (97 dBnHL); 2 cases (4 ears) only had wave V (90 dBnHL), wave differentiation was possible, wave latency was prolonged.
2.5 CM: of the 14 patients (28 ears), the CM waveforms were basically normal, and 2 (4 ears) had no normal waveforms.
2.6 SRS: in this group of patients (quiet state), the recognition rate of 14 examples is equal, the range is between 0%-58%, and it is not proportional to the pure tone hearing threshold. With the increase of sound intensity, speech resolution is not rising, and the recognition rate of 2 examples is 86%, 88% respectively, and the rate of speech recognition increases with the increase of sound intensity.
Conclusion:
1. auditory neuropathy has unique audiological characteristics. Audiological examination is the main criterion for diagnosis.
2. in this group, 14 cases were auditory neuropathy and 2 cases were general sensorineural hearing loss.
3. each audiological examination is complementary to each other, progressively, sharing to avoid misdiagnosis.
4. necessary imaging and other systematic examination.
【学位授予单位】：中南大学
【学位级别】：硕士
【学位授予年份】：2011
【分类号】：R764
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