一組聽神經(jīng)病病人的聽力學特征研究
發(fā)布時間：2018-08-06 18:18
【摘要】：從上個世紀80年代開始,國內(nèi)外臨床聽力學家、聽覺生理學家、耳鼻咽喉科臨床醫(yī)師以及分子遺傳學家們開始關注一種具有特殊臨床表現(xiàn)及聽力學特征的疾病——聽神經(jīng)病,但各家命名不一,直到1996年Starr將其正式命名為聽神經(jīng)病,沿用至今。 聽神經(jīng)病(Auditory neuropathy,AN)是一種表現(xiàn)為聲音可以通過外耳、中耳正常的進入到內(nèi)耳但是聲音信號不能同步地從內(nèi)耳傳輸?shù)酱竽X的聽功能異常性疾病,定義為第Ⅷ腦神經(jīng)的聽神經(jīng)支受損而引起的一種臨床表現(xiàn)特殊的神經(jīng)性聾。其特點包括：聽性腦干反應(auditory brainstenm responses, ABR)缺失或嚴重異常,誘發(fā)性耳聲發(fā)射(evoked otoacoustic emission, EOAE)正常,言語識別率下降,且不成比例的明顯差于純音聽閾,聽力圖多以低頻聽閾升高為主,鐙骨肌聲反射消失或閾值升高和EOAE對側(cè)抑制消失等。這是一組不同于一般感音神經(jīng)性聾及中樞性聾、具有獨特聽力學特點的聽功能障礙的癥侯群,近年來已引起廣泛關注,但對其認識仍處在摸索階段,其病因及發(fā)病機制尚不清楚,命名和病變部位也存在不同意見,治療亦無十分有效措施。正是由于聽神經(jīng)病存在上述諸多不確定性,目前對于聽神經(jīng)的診斷尚處在功能性診斷層次上,這就突顯了聽力學檢查做為診斷標準的重要性。隨著聽力學的發(fā)展及人們生活水平的提高,越來越多聽神經(jīng)病人被檢出,已遠遠超于預期。 目的：本課題旨在總結(jié)聽力學檢測(PTA、AIM、ABR、DPOAE、CM、SRS)在聽神經(jīng)病診斷與鑒別診斷過程的意義,進一步探討聽神經(jīng)的臨床聽力學特征,以提高對AN的診斷和進一步加深對AN的認識。 方法：回顧性調(diào)查分析我院2010-09至2011-03耳鼻咽喉門診根據(jù)其主訴、臨床表現(xiàn)、初步的臨床聽力學檢查疑為AN的16例患者的臨床資料,對此組患者進行一組聽力學檢查(PTA、AIM、ABR、DPOAE、CM、SRS),并對其結(jié)果進行總結(jié)分析,探討聽力學檢查對AN診斷與鑒別診斷的意義及聽神經(jīng)病的聽力學特征。 結(jié)果： 1.對象及臨床表現(xiàn) 本組病例選我院2010-09至2011-03耳鼻咽喉門診主訴、臨床表現(xiàn)及初步的聽力學檢查疑為AN的16例病人,均以雙耳漸進性聽力減退、辨音障礙,尤其在喧鬧嘈雜環(huán)境中明顯就診；其中2例(4耳)伴有間斷性或持續(xù)性低音調(diào)耳鳴；2例伴有四肢乏力,走路不穩(wěn),無其他明顯不適,神經(jīng)內(nèi)科確診有周圍神經(jīng)�。贿�有2例有可疑家族耳聾遺傳性疾病史(母親與胞弟均有同樣的臨床表現(xiàn))。本組患者均可用普通話交流,并有不同程度的日常言語交流障礙。所有病例行CT和MRI檢查排除聽神經(jīng)瘤、顱內(nèi)占位等器質(zhì)性病變、無噪聲接觸史及耳毒性藥物應用史。 2.聽力學檢查結(jié)果 2.1 PTA：本組病人中聽力圖12例(24耳)為低頻上升型,2例(4耳)為低頻下降為主高頻下降為輔的覆盆形,2例(4耳)為全頻下降的平坦型。其中前14例為輕、中度感音神經(jīng)性聾,聽力圖中250Hz、500Hz下降明顯。后2例為中、重度感音神經(jīng)性聾,全頻下降。16例病人32患耳純音聽閾測試結(jié)果氣、骨導均下降,符合感音神經(jīng)聽力損失的聽力學特征。 2.2 AIM：本組病例16例(32耳)耳均為“A”型曲線。鐙骨肌聲反射15例(30耳)同側(cè)及對側(cè)聲反射均未引出,1例(2耳)的單項或多項頻率鐙骨肌聲反射可引出,但鐙骨肌聲反射閾值均升高,且反射誘出耳聲衰減試驗陽性,2例(4耳)有響度重振現(xiàn)象其余均無。 2.3 DPOAE:本組病人中14例(28耳)可引出DPOAE,不能被對側(cè)白噪聲抑制,2例未引出。 2.4 ABR：本組病人中14例(28耳)ABR不能引出(97dBnHL);2例(4耳)僅出現(xiàn)波V (90dBnHL),波分化可,波潛伏期延長。 2.5 CM：本組病人中14例(28耳)CM波形基本正常,2例(4耳)無正常波形可見。 2.6 SRS：本組病人中(安靜狀態(tài)下)14例言語識別率均差,范圍在0%-58%之間,與純音聽閾明顯不成比例,隨聲音強度增加,言語分辨率不升反降；2例言語識別率分別為86%,88%,且隨聲音強度增加言語識別率增加。 結(jié)論： 1.聽神經(jīng)病具有獨特的聽力學特征,聽力學檢查為診斷的主要標準。 2.本組病例中14例為聽神經(jīng)病,2例為一般感音性耳聾。 3.各聽力學檢查之間互相彌補、層層遞進,共用避免漏診誤診。 4.必要時行影像學及其它系統(tǒng)檢查。
[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.
【學位授予單位】：中南大學
【學位級別】：碩士
【學位授予年份】：2011
【分類號】：R764
本文編號：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.
【學位授予單位】：中南大學
【學位級別】：碩士
【學位授予年份】：2011
【分類號】：R764
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