Audiological evaluation of the middle ear implant--speech discrimination under noise circumstances

Speech discrimination scores under noise circumstances were studied by use of the middle ear implant (MEI) and the conventional hearing aid (HA, HA-33, RION, Co, Ltd). The studies were performed in 10 patients implanted with the MEI and in 12 adult volunteers with normal hearing as a control. The tests were carried out using Japanese monosyllabic lists from 57S-speech discrimination test list as a test sound and multi-talker noise as a noise source. A speaker was placed in front of a subject in one meter apart. Sound characteristics of HA were adjusted as far as possible to those of the MEI by use of a sound equalizer. The intensity of speech sound pressure was adjusted at 65dB SPL, while that of the noise was changed to 65, 70 and 75dB SPL in the position of patients. Audiological evaluation of the MEI, the HA and control obtained by percentages of correct answers to 50 words in speech discrimination test with and without noise (65, 70, 75dB SPL). When the test was performed without noise, speech discrimination scores by the MEI and the HA were either 96.8 +/- 3.6% and 94.8 +/- 4.1%. However, under noise circumstances (65dB SPL) that by both devices were either 81.6 +/- 9.1% and 66.8 +/- 10.6% (P less than 0.001). When the intensity of the noise increased to 70 and 75dB SPL, speech discrimination scores by both devices deprecated together with consistent difference (P less than 0.01). Moreover speech discrimination scores by the MEI were almost same as control.(ABSTRACT TRUNCATED AT 250 WORDS)     

听神经病的听力学评估和病因学研究

听神经病(auditory neuropathy)是一种听功能异常性疾病,表现为声音可以通过外耳、中耳正常地进入到内耳,但是声音信号不能同步地从内耳传输到大脑,患者主诉为可以听到声音但是对言语的辨别及理解能力异常.     

Audiological tests for evaluation of central auditory disorders.

Three audiological tests (Competing Sentences, Rapidly Alternating Speech, Staggered Spondaic Words) currently being used to evaluate central auditory dysfunction are discussed. Audiological and neurological findings on three patients with central auditory lesions are presented, with special reference to their performance on these central auditory tests. Results suggest the tests are of value in identifying and/or corroborating central lesions, and in providing a more complete explanation of patient communicative difficulties.     

外耳道及中耳恶性肿瘤的临床分析

目的 探讨外耳道及中耳恶性肿瘤的临床表现、诊断要点及治疗原则和方法,以期提高患者生存率,改善生活质量.方法 回顾性分析2003年6月～2009年6月我院确诊的25例外耳道及中耳恶性肿瘤患者的临床资料.结果 按Still系统临床分期,T1期5例,T2期15例,T3期5例,2年生存率87%,5年生存率67%,单纯手术治疗易...     

Amyloidosis of the external auditory canal and middle ear: unusual ear tumor

Amyloidosis of the ear is rare. We describe the case of a 41-year-old man who had localized amyloidosis that involved the external auditory canal and middle ear. To the best of our knowledge, only 4 other cases of amyloidosis involving the external auditory canal have been previously reported; in none of these cases was the middle ear involved. We also discuss the clinical importance of this condition and its treatment.     

听神经病听力学分析

目的　探讨听神经病的听觉电生理特点。方法　总结了 10例听神经病患者的病史、纯音测听、镫骨肌反射、听性脑干反应 (auditorybrainstemresponse ,ABR)、耳蜗电图、畸变产物耳声发射(distortionproductotoacousticemission ,DPOAE)及对侧白噪声抑制试验、中潜伏期反应和慢皮层反应。 5例患者作了颅脑CT或磁共振成像 (magneticresonanceimaging ,MRI)。结果　患者男女兼有 ,平均年龄为 2 0 3岁 ,10余岁的青少年占多数。 9例主诉双耳听力下降 ,1例双耳鸣。纯音测听示 19耳为轻度至中度的低频下降型感音神经性听力损失 ,听力损失最严重的频率为 0 5kHz或 (和 ) 0 2 5kHz;1耳听力正常。 19耳镫骨肌反射消失 ,1耳纯音测听为上升型曲线者反射阈提高。ABR不能引出或仅出现波Ⅴ或 (和 )波Ⅰ。全部病例均可记录到DPOAE ,但不能被对侧噪声抑制。耳蜗电图示大部分病例动作电位 (actionpotential,AP)消失或振幅很小 ,负总和电位 (negativesummatingpotential,-SP)振幅绝对平均值为 0 5 95 μV ,慢皮层反应皆正常 ,6例测中潜伏期反应 5例正常。CT或MRI无异常发现。 结论听神经病早期纯音测听可正常 ,但其镫骨肌反射和ABR消失或阈值升高 ,其确切病变部位尚不明了 ,可能位于脑干平面以下的听觉系统     

13例外耳及中耳恶性肿瘤临床分析

目的分析外耳及中耳恶性肿瘤的类型、治疗方法及疗效。方法回顾性分析北京大学第一医院1993年至2010年经治的13例外耳及中耳恶性肿瘤患者的临床表现和手术结果。其病理类型分别为鳞状细胞癌8例,腺癌3例,神经内分泌癌1例,恶性黑色素瘤1例。3例行单独手术治疗,8例行手术治疗联合术后放射治疗,2例行放、化疗。结果全组总5年生存率60%,手术或综合治疗者5年生存率为75%,切缘阳性者5年生存率为40%。结论对外耳道及中耳恶性肿瘤的患者采取手术或综合治疗可提高生存率,术后病理切缘阳性可能是预后不良的因素。     

Radiotherapy in carcinoma of the middle ear and auditory canal.

In the decade 1961 to 1970, 89 patients with epidermoid carcinoma of the middle ear or auditory canal were seen. Seventy-eight were treated with 5-year survival figures of 22 per cent for middle ear, 42 per cent for auditory canal and 31 per cent for the total group. The majority of failures occurred within 18 months of diagnosis. The author suggests that radiotherapy and surgery must be combined to improve results. Eleven patients in the series developed second primary tumours.     

听神经病听力学分析

目的 探讨听神经病的听觉电生理特点。方法 总结了10例听神经病患者的病史、纯音测听、镫骨肌反射、听性脑干反应（auditory brainstem response,ABR)、耳蜗电图、畸变产物耳声发射（distortion product otoacoustic emission,DPOAE)及对侧白噪声抑制试验、中潜伏期反应和慢皮层反应。5例患者作了颅脑CT或磁共振成像（magnetic resonance imaging,MRI)。结果 患者男女兼有，平均年龄为20．3岁，10余岁的青少年占多数。9例主诉双耳听力下降，1例双耳鸣。纯音测听示19耳为轻度至中度的低频下降型感音神经性听力损失，听力损失最严重的频率为0．5kHz或（和）0．25kHz；1耳听力正常。19耳镫骨肌反射消失，1耳纯音测听为上升型曲线者反射阈提高。ABR不能引出或仅出现波V和（和）波1。全部病例均可记录到DPOAE，但不能被对侧噪声抑制。耳蜗电图示大部分病例动作电位（action potential,AP)消失或振幅很小，负总和电位（negative summating potential,-SP)振幅绝对平均值为0．595μV，慢皮层反应皆正常，6例测中潜伏期反应5例正常。CT或MRI无异常发现。结论 听神经病早期纯音测听可正常，但其镫骨肌反射和ABR消失或阈值升高，其确切病变部位尚不明了，可能位于脑干平面以下的听觉系统。     

先天性外耳道及中耳畸形的治疗

先天性外耳道与中耳畸形在临床上并不少见,通常会合并有耳廓的畸形.一般来讲,耳廓与耳道的畸形越重,则提示中耳的畸形也就越重,有些病例甚至合并有内耳或者身体其他部位的畸形,如Treacher Collins综合征等[1].除外形上的改变外,外中耳畸形主要表现为传导性听力障碍,合并内耳畸形的病例还会有混合性聋,但罕有全聋者.     

Clinical evaluation of congenital cholesteatoma of the middle ear

We conducted a retrospective study to identify the clinical features and surgical observations of congenital cholesteatoma. Sixty patients were diagnosed and underwent surgery for congenital cholesteatoma between April 1987 and May 2002. All diagnoses were made on the basis of two operative findings: 1. the tympanic membrane manifested neither retraction, perforation, nor granulation. 2. the tympanic membrane was not continuous with the cholesteatoma. In this series, congenital cholesteatoma accounted for 7% of all cholesteatomas (853 ears). The patient age ranged from 2 to 48 years. The male to female ratio was 4:1. Seventeen patients had multiple cholesteatoma. Fifty-three patients exhibited closed-type cholesteatomas, while the remaining 7 patients had open-type cholesteatomas that had formed as a flat surface of the epidermis. Patients with open-type cholesteatomas presented with a much more pronounced conductive hearing loss and ossicular erosion or malformation. Twenty-two patients with relatively small cholesteatomas were analyzed to estimate the origin of their cholesteatomas. Of the 22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at the time of detection was older in the PSQ-type group than in the ASQ-type group and the frequency of ossicular erosion or malformation was more prominent in the PSQ-type group than in the ASQ-type group. The primary site of origin was thought to be the portion between the tympanic ostium of the auditory canal and the semicanal for tensor tympani in the ASQ-type group and near the incudostapedial joint in the PSQ-type group. A planned staged procedure was performed in 29 patients, 15 patients (52%) had residual lesions situated mostly on the oval window, the round window, an exposed facial nerve or an exposed lateral semicircular canal. The frequency of residual lesions in patients who presented with extended, multiple cholesteatoma and those with ossicular malformation was comparable to the frequency of patients who did not present with these features.     

外耳道、中耳恶性肿瘤治疗方案的探讨

目的探讨外耳道、中耳恶性肿瘤的治疗方案及相关问题。方法回顾性分析1990—2005年我科收治的34例外耳道、中耳恶性肿瘤的临床资料,其中6例为复发病例,面神经受累7例。34例中4例行单纯放射治疗;30例根据肿瘤的不同部位和侵犯范围行不同的手术治疗,其中26例行术后放射治疗,放疗剂量为50～60GY。结果本组34例。术前面神经未受侵犯的27例中,术后6例发生不同程度的面瘫,其中5例术后3个月逐渐恢复,1例不全恢复。4例行单纯放射治疗者,1例早期病例存活61个月,其余3例分别在治疗后12、23以及31个月复发死亡。4例行单纯手术治疗者,2例早期病例分别存活62、72个月;另2例拒绝术后放疗,分别存活24、61个月。行手术加术后放疗的26例中,3年生存率为53.2%,5年生存率为43.5%,复发多在2～5年内。结论外耳道、中耳恶性肿瘤针对不同的病例选择合适的治疗方案,对提高疗效、保留功能和外观等有重要意义。     

听神经病患者的临床听力学特点

目的探讨听神经病患者的临床听力学特点。方法自2003年1月～2007年5月共收集到22例听神经病患者的病例。总结听神经病患者的病史、纯音测听、鼓室曲线图、镫骨肌声反射、言语测听、听性脑干反应（auditory brainstem response，ABR）、耳蜗电图（electrocochleogram，ECochG）、畸变产物耳声发射（distortion product otoacoustic emission，DPOAE）及对侧白噪声抑制试验特点。其中10例患者行颅脑核磁共振成像（magnetic resonance imaging，MRI）检查。结果男性患者9例，女性患者13例，发病年龄为10～20岁，表现为进行性双耳听力下降，7例患者伴有耳鸣，最突出的表现是言语识别率差，无噪声接触史和耳毒性药物应用史，无家族史。22例患者听力曲线包括上升型13例，平坦型7例，高频听力下降型2例；鼓室曲线图均为A型；镫骨肌声反射均消失。所有听神经病患者ABR不能引出；全部病例均可记录到DPOAE，但不能被对侧白噪声抑制。耳蜗电图显示17例患者-SP／AP〉0．4，5例患者动作电位（action potential，AP）消失或振幅很小，颅脑MRI检查无异常发现。结论听神经病患者听力学检查有以下共同特点：①双耳进行性听力下降，青少年发病，言语辨别能力甚差，听力损失程度不同，多为轻度到重度，少数为极重度，纯音听力大多为低频下降为主的感音神经性聋，一部分表现为平坦型、甚至高频下降型；②声导抗为“A”型鼓室曲线，镫骨肌声反射引不出；③DPOAE均可引出，耳声发射对侧抑制消失；④ABR引不出或严重异常；⑤影像学检查无任何阳性发现。听神经病的确定目前仍停留于功能性诊断，尚不能确定其病因、病变部位，详尽的听力学检查、典型的听力学表现有助干临床诊断。     

先天性外耳道狭窄伴外耳道或中耳乳突胆脂瘤的手术治疗

目的 :探讨先天性外耳道狭窄及伴中耳畸形外耳道、中耳乳突胆脂瘤病人的手术治疗方法。方法 :31例病人中 9例为单纯先天性外耳道狭窄伴发外耳道胆脂瘤 ,进行外耳道胆脂瘤根治术 ,并行外耳道成形术 ,其中 7例行外耳道成形 +鼓室成形术 ;2 2例为先天性外耳道狭窄伴发中耳畸形和中耳乳突胆脂瘤 ,在根治胆脂瘤的同时 ,行外耳道成形术 ,其中 13例进行鼓室成形术 ,随访 3个月到 1年。结果 :7例外耳道成形 +鼓室成形术者 ,成功率为 85 .7% (6 / 7) ;13例进行乳突根治术 +鼓室成形术者 ,成功率为 76 .9% (10 / 13)。凡鼓室成形术成功者听力均提高 15 d BHL(语言频率 5 0 0～2 0 0 0 Hz)以上。结论 :先天性外耳道狭窄伴发外耳道胆脂瘤或中耳胆脂瘤病人 ,在彻底清除胆脂瘤的基础上进行鼓室成形术 ,可以提高病人的听力 ;术中发现中耳畸形常有面神经遮盖卵圆窗或无卵圆窗者 ,对该类病人应在控制感染后再行前庭或半规管开窗术