谈烟酸等药物对感音性耳聋的治疗

听觉系统中传音、感音或综合分析部分的功能异常,致使听力不同程度的减退,称耳聋;耳聋按病变发生的部位可分为传音性聋、感音神经性聋;螺旋器发生病变引起的聋称感音性聋,神经传导径路发生病变引起的聋称神经性聋,两者合称为感觉一神经性聋,由于临床上简单的测听法不易将     

传导性耳聋

外耳与中耳属传音结构 ,传音结构的病变可使声波传入内耳受到障碍 ,引起听力减退 ,而感音器官的耳蜗和耳蜗以上的功能是正常的。传导性聋的听力损失一般不超过 6 0ｄＢ。环境噪声对传导性聋患者的听力干扰轻微 ,这种患者在噪声较大的环境中接受语言的能力往往和正常人相仿。传导性聋可见于以下疾病。先天性疾病 :外耳畸形如耳廓畸形、外耳道闭锁 ;中耳畸形如鼓膜发育不全、缺如或呈骨板样 ,鼓室发育不全呈裂隙状 ,听骨链畸形、中断、发育不全或融合成块状并有固定 ,前庭窝缺如等。由于先天性遗传因素所致的传导性聋还见于以下综合征 :颅面骨…     

感音神经性聋治疗现状

在发展中国家，目前有超过1亿感音神经性聋患者受到听力丧失的折磨，在美国和欧共体，估计有超过9000万中重度感音神经性聋的患者，其中，超过6500万患者没有得到有效的治疗。随着人们对中耳机制和内耳生理认识的不断深入以及科学技术的不断发展，对感音神经性聋的治疗取得了显著的进展。我国目前治疗感音神经性聋的方法主要是让患者佩戴助听器。而在欧美国家，植入式听力辅助设备、毛细胞的再生及干细胞的转化等相关研究已广泛展开。本文就欧美国家治疗感音神经性聋的现状作一综述。     

耳腭指(趾)(OPD)综合征的颢骨病理改变

OPD综合征主要表现为传导性耳聋、腭裂及骨骼(指趾常见)结构异常。自1962年Taybi报告首例以来。迄今已逾25例临床资料。本文为Schuknecht教授收集的颞骨标本,为世界文献中首例OPD综合征的颞骨病理报告。死者为2岁半的男孩,生后即发现有典型的OPD综合征表现,语言测听及一般听力检查为传音性聋而脑干电反应测听(BSER)除证实双耳听力重度下降外,还显示右耳轻度感音性聋。本例有OPD综合征家族史。     

多导人工耳蜗植入在6例语前聋儿童的临床应用

目的：探讨多导人工耳蜗植入对重度感音性语前聋儿童的治疗效果。方法：对6例极重度感音性语前聋儿童植入澳大利亚Cochlear公司生产的Nuclus24型人工耳蜗，术后1个月开机，结果：6例语前聋儿在接受人工耳蜗植入后均获得听力，听阈平均提高到33．33分贝听力级（dBHL)，双音节词言语辨别率（SDS）术后6个月听平均28．33％，听加唇读平均61．6％，术后12个月（SDS）听平均51％，听加唇读平均73．8％，比术前有明显改善，结论：多导人工耳蜗植入对恢复极重度感音性语前聋儿童的听觉是有效的，植入年龄越小，效果越好。     

慢性化脓性中耳炎对骨导听力影响的初步观察

对慢性化脓性中耳炎117例骨导听力的研究结果表明，慢性化脓性中耳炎的中耳损伤不仅会引起传导性聋，也会导致感音神经性聋，并可累及各个频率，尤以语言频率高频段骨导听力损伤较明显。     

124例以耳鸣为首发症状的分泌性中耳炎诊断体会

对１２４例１９９耳首灰耳鸣的分泌性中耳炎进行分析，发现上呼吸道感染占５１．３５％，是发病的主要因素。诊断的关键在详细病史，鼓膜象及听力学检查。耳鸣性质依次为吹风样、流水音、搔抓声、难以表达、汽笛声、哨音、蝉鸣音。低音调耳鸣主要表现在急性期及浆液性、混合性积液中，听力损失呈中低频区传导性聋；高音调耳鸣主要在慢性期粘液性积液耳，听力损失呈混合性、感音性聋。说明：耳鸣性质及听力损失程度取决于病程及中耳积     

听力损失

<正>听力损失是指听觉器官不能感受到正常水平声音强度的一种状态。是听觉功能障碍的表现,轻者称重听或听力减退,重者称耳聋或全聋。一般临床上把听力损失分为传导性、感音神经性和混合性听力损失。传导性听力损失病变在外耳或中耳,使声波传入内耳受到障碍;感音神经性听力损失病变在耳蜗、听神经或听觉中枢,引起对声音感觉和认知功能障碍的听力损失;混合性听力损失是传导性听力损失和感音神经性听力损失的因素同时存在,兼有传导性听力损失和感音神经性听力损失特点。     

临床庆大霉素耳中毒的组织病理学所见

作者报告的病例,系48岁女性患者,因长期滥用非那西汀而导致慢性肾功衰竭,以出现血尿及高血压于1970年2月入院。主诉:进行性听力下降已12月之久,无耳痛、耳漏及眩晕史,亦无家族耳聋史。检查及经过:两侧鼓膜正常,左耳后疤痕系5岁时行乳突皮质切除术所遗留。瑞内氏试验,双侧阳性;韦伯氏试验正中。纯音侧听示双侧对称性感音神经性耳聋,左侧伴轻度传音聋成份。言语识别率得分结果与感音聋一致。1970年10月和1971年11月所测听力曲线无显     

短增量敏感指数测试的临床应用

对23名听力正常者和10名临床诊断为蜗性聋者作了短增量敏感指教对比测试.结果表明,这种方法对诊断蜗性聋即感音性聋有较高的价值,具有测试简便迅速.结果可靠等优点.     

中耳传音机构炎性病损及其听力状态

本文分析鼓室成形术125耳的病变与听力状态。105 耳鼓膜大穿孔听骨受累者占62％,鼓室硬化病变占 12.8％。鼓膜大穿孔而听骨链完整者气导平均损失 47.1dB,若听骨链中断则损失达60.8dB;鼓膜中等穿孔而听骨链完整者损失41.9dB,若听骨链中断则损失达53.9dB。16例鼓室硬化症平均损失 60.4dB。本研究有助于认识中耳病变与听力损失的关系及分析临床听力图。     

耳内镜下手术治疗单纯先天性中耳畸形疗效分析

目的：探讨单用耳内镜手术治疗单纯先天性中耳畸形的疗效。方法：回顾性分析2017年1月至2021年6月在温州医科大学附属第一医院收治的27例单纯先天性中耳畸形患者的临床资料,对术前高分辨率计算机断层扫描结果、术中所见、手术方式及术前后听力学资料进行分析。结果：27例患者均为单耳发病,24例患者为传导性聋,3例混合性聋且以传导性为主。Teunissen-Cremers分型I型5例,II型6例,III型15例,IV型1例。术中分别使用PORP 8例、TORP 7例和Piston 11例行听骨链重建术。1例IV型患者系前庭窗闭锁,听骨及面神经严重畸形,行加长的7.0 mm Piston锤骨前庭桥接术。27例患者术前平均气导为（62.1±8.3）dB HL,术后6个月平均气导为（30.6±7.4）dB HL（t=19.47,P＜0.001）。15例（55.6%）气导听力提高超过30 dB HL。术前平均气骨导差为（43.1±9.3）dB HL,术后6个月为（13.4±5.2）dB HL（t=16.75,P＜0.001）。结论：单纯先天性中耳畸形主要表现为听骨链畸形,单用耳内镜诊治先天性中耳畸形具有视野清晰、操作准确、无需外耳道或耳后切口、创伤小、并发症少、术后听力恢复好的优点。     

单耳单纯听骨链畸形的临床特点及听骨链重建术

目的研究单耳单纯听骨链畸形的临床特点,探讨钛质人工听骨在听骨链重建手术中的应用。方法回顾分析9例单纯听骨链畸形的病例资料。患者均为单耳病变,术前纯音测听检查为传导性耳聋,气骨导差距为30—65dB。所有患者均在全麻下行鼓室探查术,根据探查结果使用相应的钛质人工听骨进行听骨链重建。结果术中发现,5例为砧骨长突发育不良,行Ⅲa鼓室成形术;2例为砧、镫骨发育不良,行Ⅲb鼓室成形术;1例为锤砧关节不连,行锤砧融合术;1例为镫骨、前庭窗发育不良,术中终止手术。8例患者术后2个月复查听力,ABG缩小为8．8—22．5dB,1例听力无改善。1例患者术后出现外耳道狭窄。随访0．5～5年,无听骨脱出、听力下降。结论单耳单纯听骨链畸形不易早期发现,易被忽视。畸形以砧骨长突发育异常较多见。根据术中探查结果使用钛质人工听骨进行听骨链重建,手术疗效确切。     

交叉镫骨肌声反射客观评价鼓室成形术后听力改变

应用交叉镫骨肌的交叉特性,客观评价了67例单侧慢性中耳炎患者行鼓室成形术后听力改变情况。结果显示鼓膜修补术后,听力提高了17.4dB左右,(?)型鼓室成形术后听力提高超过了25dB;交叉镫骨肌声反射测试与纯音听阈测试结果间差异无显著性意义,但交叉镫骨肌测试结果有客观性,可推广应用于儿童患者和某些特殊情况,此外,交叉镫骨肌声反射较脑干听反应等客观听力检查有明显优越性,故具有重要的临床意义。     

鼓室成形术中听骨链重建的研究进展

鼓室成形术是现代耳科学的重要组成部分,听骨链重建作为鼓室成形术的一部分,其目的是恢复中耳的传导机制,提高听力。一般听骨链异常最多见的是锤骨与镫骨之间的连接中断,其原因可能是病变腐蚀或外伤等所致,也可以是术中清除中耳病变而人为将其中断的结果。听骨链是维持正常听力所必需的重要传音结构,它的残缺将导致传导性耳聋。慢性化脓性中耳炎患者多数存在不同程度的听力障碍,其原因除了鼓膜穿孔外．多伴有听小骨的破坏和缺失,因而听骨链重建鼓室成形术可有效恢复听力。     

耳道—鼓室成形、异种异体听骨链重建术治疗先天性耳道闭锁

为了解决先天性耳道闭锁重度传导性聋的生理缺陷，开展耳道—鼓室成形、异种异体听骨链重建术。4例先天性耳道闭锁，3例伴中耳畸形和耳廓缺如，1例伴中耳畸形和悬雍垂畸形。术中见鼓室狭小，鼓膜缺如，代之坚硬骨板，听骨链严重畸形，面神经走行明显变异，中颅窝硬脑膜下垂，乙状窦前移。在手术显微镜下行乳突轮廓化；重建鼓室、耳道；钛质听骨重建听骨链；移植筋膜行鼓膜成形。术后随访46～164日，听力较术前提高20～45dB。就手术适应证，术中面神经保护及听骨链重建进行讨论。     

Modification of closed tympanoplasty in middle ear cholesteatoma

Background Classic mastoidectomy and modified mastoidectomy are traditional surgical procedures for middle ear cholesteatoma with goals of eradicating diseases, creating dry ears and preventing severe complications. However, the drawback of these procedures is the lack of hearing improvement. Therefore, our study aimed to investigate the effects and safety of a modification of closed tympanoplasty for middle ear cholesteatoma. Methods Eighty-three patients were recruited in this study based on the following two criteria： each patient had middle ear cholesteatoma in one ear; the affected ears had a functional eustachian tube and had neither intracranial nor extracranial complications. All the patients received a modification of closed tympanoplasty which included ossicular reconstruction with total ossicular replacement prosthesis （TORP） or partial ossicular replacement prosthesis （PORP） and membrane repair with conchal cartilage-perichondral complex. Results All the 83 cases had dry ears with membranes healed within 4-6 postoperative weeks. After 6 postoperative months, there were 3 cases with re-perforation at the tympanic membrane center and after 1.5 postoperative years, there were 5 cases with cholesteatoma recurrence （6.02%）. Function tests after one postoperative year exhibited an improvement of pure tone audiometry （PTA） in 27 cases that was more than 30 dB, in 33 cases between 20-29 dB, 14 cases with improvement between 10-19 dB, and in 9 cases there was no improvement. Conclusions The modified closed tympanoplasty procedure for middle ear cholesteatoma in the present study has all the advantages of both close-cavity and open-cavity procedures. It has low recurrence rate and good hearing improvement.     

Auditory cortical responses evoked by pure tones in healthy and sensorineural hearing loss subjects: functional MRI and magnetoencephalography

Background Blood oxygen level dependent functional magnetic resonance imaging （fMRI） and magnetoencephalography are new techniques of brain functional imaging which can provide the information of excitation of neurons by measure the changes of hemodynamics and electrophysiological data of local brain tissue. The purpose of this study was to study functional brain areas evoked by pure tones in healthy and sensorineural hearing loss subjects with these techniques and to compare the differences between the two groups. Methods Thirty healthy and 30 sensorineural hearing loss subjects were included in this study. In fMRI, block-design paradigm was used. During the active epoch the participants listened to 1000 Hz, sound pressure level 140 dB pure tones at duration 500 ms, interstimulus interval 1000 ms, which presented continuously via a magnetic resonance-compatible audio system. None stimulus was executed in control epoch. In magnetoencephalography study, every subject received stimuli of 1000 Hz tone bursts delivered to the bilateral ear at duration 8 ms, interstimulus intervals 1000 ms. Sound pressure level in healthy subjects was 30 dB; in sensorineural hearing loss subjects was 20 dB above everyone＇ s hearing threshold respectively. All subjects were examined with 306-channel whole-scalp neuromagnetometer. Results In fMRI, all subjects showed significant activations in bilateral Heschl＇s gyri, anterior pole of planum temporale, planum temporale, precentral gyri, postcentral gyri, supramarginal gyri, superior temporal gyri, inferior frontal gyri, occipital lobes and cerebellums. The healthy subjects had more intensive activation in bilateral Heschl＇s gyri, anterior pole of planum temporale, inferior frontal gyri, left superior temporal gyri and right planum temporale than the hearing loss subjects. But in precentral gyri, postcentral gyri and occipital lobes, the activation is more intensive in the hearing loss subjects. In magnetoencephalography study, both in the hearing loss and the healthy subjects, the most evident audio evoked fields activated by pure tone were N100m, which located precisely on the Heschl＇s gyrus. Compared with the hearing loss subjects, N100m of the healthy subjects was stronger and had longer latencies in right hemisphere. Conclusions Under proper pure tone stimulus the activation of auditory cortex can be elicited both in the healthy and the sensorineural hearing loss subjects. Either at objective equivalent stimuli or at subjectively perceived equivalent stimuli, the auditory responses were more intensive in healthy subjects than hearing loss subjects. The tone stimuli were processed in a network in human brain and there was an intrinsic relation between the auditory and visual cortex. Blood oxygen level dependent fMRI and magnetoencephalography could reinforce each other.     

中耳胆固醇肉芽肿的诊断和手术治疗

目的探讨中耳胆固醇肉芽肿（CG）的发病机制、临床特点及治疗效果。方法回顾性分析43例CG患者的临床资料，其中42病例经手术及病理证实。主要临床表现包括听力下降43／43例，耳闷感29／43例，咖啡样耳漏12／43例，耳呜26／43例。蓝鼓膜为典型体征36／43例。颞骨高分辨CT（HRCT）主要表现为中耳乳突软组织阴影，可伴有听骨链破坏（17/43），手术中发现（13／43）与术前符合率76．47％（13／17）。结果手术方法选择完璧式乳突切除＋鼓室探查＋鼓室置管术31例，完璧式乳突切除＋鼓室探查＋听骨链重建术11例，单纯鼓室置管术1例。术后随访0．5～3．0年，无复发。术后1．2个月鼓膜颜色恢复正常，听力均有不同程度提高，骨气导间距平均减少（25．0±3．5）dB。结论cG的主要临床表现为听力下降、耳闷感、耳呜、咖啡样耳漏和蓝鼓膜。HRCT对中耳胆固醇肉芽肿诊断及手术有重要参考价值。诊断明确应采取手术治疗，清除病变，建立鼓室及乳突的通气、引流，并重建听力。     

学龄前儿童综合听力评估120例临床应用分析

目的探讨耳声发射、中耳声导抗、ABR相结合的综合听力评估方法,在学龄前儿童的临床应用。方法选择120例无法配合行为测听的儿童,检测瞬态诱发耳声发射、畸变产物耳声发射以及中耳声导抗,结合短声以及有频率特异性的短纯音刺激的ABR测试来评估听力。结果120例受检者中,上述3项检查都通过者51例,诊断为感音神经性聋64例,混合性耳聋2例。传导性耳聋1例,神经性耳聋1例,听神经病1例。结论采用有频率特异性的短纯音刺激的ABR。结合耳声发射、中耳声导抗能较好地评估5岁以下无法配合行为测听的儿童的听阈。