先天性外中耳畸形听力重建

先天性外中耳畸形是耳科常见疾病,主要造成两大生理缺陷：容貌缺陷和听力障碍。由于约90%外中耳畸形患者的内耳正常,所以他们的听力障碍以传导性聋为主,耳显微外科手术,如外耳道成形加鼓室成形术及人工中耳植入手术等可改善患者听力。1常规听力修复手术——外耳道成形加鼓室成形术Kiesselbach于1883年首次尝试对先天性外中耳畸形患者行听力修复手术,但不幸的是患者术后出现了面瘫。     

先天性外中耳畸形听力与颞骨畸形的临床探讨

目的探讨先天性外中耳畸形患者的听力与颞骨畸形的相关性。方法对50例(60耳)先天性外中耳畸形患者行纯音测听、听性脑干反应(ABR)和颞骨HRCT检查,比较其听力与颞骨畸形的相关性。结果先天性外中耳畸形患者听力下降主要以传导性聋为主,平均纯音阈值为64.08±13.07 dB HL,平均气骨导差为53.28±12.05 dB HL,ABR检查结果示平均阈值为76.86±11.89dB HL,先天性外中耳畸形患者听阈与耳廓畸形分级呈正相关,与HRCT总分呈负相关。听力与听骨链、中耳发育情况和面神经发育有关,而与蜗窗关系尚待进一步研究。结论先天性外中耳畸形患者术前进行听力检查和颞骨CT检查可初步评估外耳、中耳发育程度,进而指导手术治疗。     

先天性外中耳畸形听力植入技术研究进展

先天性外中耳畸形是面部最常见的出生缺陷之一,临床表现多样。除了面部缺陷,患者通常伴有中重度的传导性听力损失。这类患者需要早期干预和治疗以减少对言语发育和社会适应的不良影响。随着科学技术发展及人工听觉植入设备的出现,先天性外中耳畸形患者的听力重建有了更多选择和听力获益。本文对先天性外中耳畸形听力植入技术研究进展进行综述。     

先天性中耳畸形的诊断与治疗

先天性中耳畸形在临床上较为常见，既可与耳廓畸形、外耳道畸形合并存在，也可单独发生，主要表现为传导性听力损失，少数为混合性或感音神经性听力损失。诊断主要依据病史（自幼听力障碍或伴有外耳畸形）、听力学检查（传导性或以传导性为主的混合性听力损失）及颞骨高分辨率CT 检查结果进行综合判断。除了合并先天性外耳道狭窄者（因其容易并发胆脂瘤，手术以切除胆脂瘤、预防并发症为主）外，先天性中耳畸形治疗的目的为改善患者听力；治疗方式包括：常规听骨链重建术（伴或不伴外耳道成形）、人工听觉植入术（BA HA、振动声桥及骨桥）及佩戴助听器（以软带骨导助听器为首选）。     

先天性外中耳畸形的听力重建策略

先天性外中耳畸形也称小耳畸形,是耳科最常见的一种畸形,主要表现为外中耳形态结构异常及听力异常,治疗方案包括耳廓再造及听力重建两部分。本文针对先天性外中耳畸形患者的听力重建策略问题进行讨论,为临床提供参考。     

先天性外中耳畸形的耳廓再造

先天性外中耳畸形是耳科常见的先天性畸形之一,治疗以手术为主。本文就先天性外中耳畸形的耳廓再造手术时机、手术方式、耳廓支架及其各自的优缺点、听力重建及未来的发展趋势等进行综述。     

先天性小耳畸形全耳廓成形及听力重建术

目的:探讨先天性小耳畸形的临床特点及全耳廓成形及听力重建术的方法和效果。方法:统计、整理2005-01-2010-10期间在我科住院治疗的58例(62耳)小耳畸形患者的资料,本组患者均为先天性耳廓畸形伴外耳道闭锁、听骨链畸形、重度传导性聋。所有患者术前行颞骨CT检查并三维重建,听力学检查。手术分2期进行,一期行耳廓一次成形,外耳道重建,中耳重建手术;二期行耳后植皮,颅耳角再造,对术前、术后的资料进行回顾性分析。结果:耳廓成形加外耳道、中耳重建术后再造耳廓外形良好,耳廓和外耳道口位置接近正常,大部分患者术后听力获得改善。结论:对先天性耳廓畸形并外耳道闭锁的患者,术前应精心设计,根据多层螺旋CT三维重建结果确定听力重建入路及中耳畸形程度,全耳廓成形及听力重建术后可以同时改善耳廓外形和提高听力。     

先天性外中耳畸形

先天性外中耳畸形是耳科常见的先天性畸形之一,治疗以手术为主,近年来随着耳显微外科的广泛开展,国内外有关报道日益增多,本文就外耳道闭锁的分型、手术时机、术前评估、手术方式、术后听力效果和并发症等作一综述.     

先天性小耳畸形手术治疗进展

先天性小耳畸形是由第一鳃弓和第二鳃弓发育异常引起的,为耳郭发育不全且较正常者为小,常伴有外耳道闭锁、中耳畸形.手术治疗是小耳畸形治疗的最主要手段,本文就小耳畸形耳郭成形术、听力重建以及相关手术进展做一综述.     

不同类型先天性中耳畸形的手术治疗

探讨先天性中耳畸形的临床分型及其手术策略。方法选取2016年6月—2019年12月收治的23例（33耳）先天性中耳畸形患者进行中耳畸形临床分型及不同的手术策略进行回顾分析，观察术后疗效和听力的恢复情况。术前对23例（33耳）中耳畸形患者均行耳内镜检查、听力学检查及颞骨CT并听骨链三维重建检查。结果23例患者中18例为术前明确畸形，5例为术中探查发现畸形。其中15例为锤、砧骨畸形，选择人工听骨赝复物（TORP或PORP）或自体听骨植入修复；6例为镫骨畸形，选择人工镫骨赝复物（Piston）植入；1例为鼓膜穿孔伴有先天性中耳畸形，先行鼓膜成形术，二期行听骨链重建手术；1例畸形程度比较复杂，面神经走形异常，在前庭窗处分叉，遮挡前庭窗，未能行进一步治疗。术后气导阈值提高至正常的有15例（65.2%）；骨气导差（ABG）恢复至30 dB以内的有16例（69.6%），ABG术后仍超过30 dB的有5例（21.7%），1例听力未改善（4.3%），1例无法行听骨重建（4.3%）。结论先天性中耳畸形术前诊断比较困难，CT检查很难精确评估，手术探查为最有说服力的诊断标准，明确中耳畸形临床分型后选择最佳的手术方式，是治愈疾病并提高患者术后听力最佳选择。     

虚拟耳镜对鼓室成形术前后鼓室结构的观察

目的 探讨虚拟耳镜在鼓室成形术中的临床评估作用.方法 应用虚拟耳镜观察耳病患者102例(204耳),72例(75耳)完成鼓室成形术和外耳道鼓室成形术,其中慢性中耳炎53例(55耳),先天性外耳道闭锁1 9例(20耳).结果 23耳慢性化脓性中耳炎胆脂瘤型,虚拟耳镜观察19耳听骨链破坏,手术证实23耳均有听骨腐蚀;32慢性化脓性中耳炎骨疡型,虚拟耳镜观察29耳听骨链不同程度破坏,手术证实23耳锤骨和砧骨侵蚀,11耳镫骨头或镫上结构缺失;20耳先天性外耳道闭锁,中耳畸形,术前提示18耳听骨畸形,2耳小鼓室无听骨链,手术证实17耳外耳道闭锁,听骨严重畸形,2耳镫骨缺失,1耳前庭窗闭锁.2耳术后突然听力下降,复查虚拟耳镜发现移植听骨与鼓膜脱离.虚拟耳镜与手术探查符合率,中耳炎为92%,先天性外耳道闭锁、中耳畸形为100%.结论 虚拟耳镜为鼓室成形术术前病变程度及术后疗效评估提供可靠的影像学依据.     

Die Therapie der Tympanosklerose

Tympanosclerosis is a scarring process with a remarkable variability in its localization within the middle ear. It can lead to conductive hearing loss in many cases. It is usually caused by recurrent chronic inflammation of the middle ear. It is generally accepted that functionally relevant tympanosclerotic findings of the middle ear structures should be treated by surgery. Depending on the extent of the surgical resection, the tympanic membrane and the ossicular chain must be reconstructed individually. In cases of isolated myringosclerosis with no hearing loss, no surgery is required. When the ossicular chain is affected by the sclerosis, sound conduction can often be restored only by interposition of grafts. Stapes footplate fixation can be treated by a stapesplasty. Tympanosclerosis of the oval window combined with other chronic inflammation usually requires two-stage surgery. Tympanosclerotic findings without any clinical symptoms should not be removed. The fitting of conventional or implantable hearing aids is the only treatment if chain reconstruction fails or is impossible.     

Treatment of tympanosclerosis

Tympanosclerosis is a scarring process with a remarkable variability in its localization within the middle ear. It can lead to conductive hearing loss in many cases. It is usually caused by recurrent chronic inflammation of the middle ear. It is generally accepted that functionally relevant tympanosclerotic findings of the middle ear structures should be treated by surgery. Depending on the extent of the surgical resection, the tympanic membrane and the ossicular chain must be reconstructed individually. In cases of isolated myringosclerosis with no hearing loss, no surgery is required. When the ossicular chain is affected by the sclerosis, sound conduction can often be restored only by interposition of grafts. Stapes footplate fixation can be treated by a stapesplasty. Tympanosclerosis of the oval window combined with other chronic inflammation usually requires two-stage surgery. Tympanosclerotic findings without any clinical symptoms should not be removed. The fitting of conventional or implantable hearing aids is the only treatment if chain reconstruction fails or is impossible.     

单纯中耳畸形的临床分析

目的 :分析单纯中耳畸形的临床和听力学特征 ,手术探查明确畸形形式 ,避免和减少对该病的误诊、误治。方法 :9例 11耳单纯性中耳畸形患者均行鼓室探查术 ,部分行听骨链重建术 ,其中 ,行镫骨切除加钢丝结绨组织栓人工听骨重建 4耳 ,前庭开窗加人工听骨重建 2耳 ,砧镫关节松解术 1耳 ,高分子材料TORP重建听骨1例 ,蜗窗不明确的 3耳 ,仅做鼓室探查。结果 :术中发现砧骨畸形最多 (10 / 11) ,豆状突细小或缺失 ,砧骨游离 ,长突缺失、变细或变粗 ;镫骨缺如 (2 / 11) ;其他 (7/ 11)包括镫骨不完整或游离、固定、前后脚融合 ;砧镫关节粘连(1/ 11) ;面神经和前庭窗无法辨认 (4 / 11) ;鼓岬表面凹凸不平 ,无法辨认圆窗 (3/ 11)。 3例并发其他全身异常。听骨重建后患者听力满意 ,纯音测听示低频比高频提高明显 ,言语频率平均提高 2 4dB。无并发症发生。结论 :单纯先天中耳畸形最终确诊靠鼓室探查术 ,一定条件下镫骨切除或前庭开窗加钢丝结绨组织栓人工听骨重建术可满意提高听力     

Congenital auditory ossicle malformation without external ear abnormality]

In 29 ears of 24 patients with auditory ossicle malformation, but without external ear abnormalities, who were operated on at our clinic in the 10 years between June 1993 and June 2002, we studied the condition of ossicles, preoperative examination, surgical procedures, and postoperative hearing improvement. Our study did not include patients with cholesteatoma or those with only fixed stapes but without ossicular deformity. Samples were 12 ears of 11 men and 17 ears of 13 women. We clinically distinguished 3 major categories: (i) discontinuity between the incus and stapes with mobile stapes, (ii) congenital fixation of the stapes with ossicular deformity, and (iii) congenital fixation of the malleus and deformity of the incus with mobile stapes. We classified each category into minor abnormalities based on the condition of the long process of the incus, the superstructure of the stapes, and the stapes footplate. We conducted auditory ossicle reconstruction by mobilization of the malleus and incus in 2 ears, by type III tympanoplasty in 5 ears, by type IV tympanoplasty in 13 ears, and by stapes surgery in 8 ears. Improvement in hearing after the operation was observed in 25 of the 29 ears treated. In the classification of the auditory ossicle malformation without external ear abnormalities, we should evaluate discontinuity or fixation of the ossicles and their deformity. This requires that varied ossicular reconstruction be prepared for surgery, but the possibility of hearing improvement is high, so intensive treatment is needed in many cases of auditory ossicle malformation.     

Congenital defects of the middle ear - uncommon cause of pediatric hearing loss

IntroductionIn children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects.ObjectiveThis study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear.MethodsThis was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented.ConclusionMiddle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.     

耳内镜下听骨链畸形手术疗效分析

目的 探讨耳内镜下听骨链手术的可行性及疗效.方法 回顾性分析2017年6月至2019年9月耳内镜手术探查确诊为先天性听骨链畸形15例(17耳),其中单纯镫骨底板固定3耳,伴有其他听骨链畸形的镫骨底板固定5耳,镫骨底板活动伴听骨链畸形8耳,前庭窗或蜗窗发育不良或闭锁1耳,依据其畸形类型分别行耳内镜下鼓室探查术+鼓室成型I...     

Surgery for congenital anomalies of the middle ear with mobile stapes

Summary In a series of 104 patients with congenital middle ear anomalies operated on from 1964 to 1986, 27 cases were found in which the stapes footplate was mobile and the conductive deafness was due to an anomaly in the remaining part of the ossicular chain. In 8 cases the middle ear anomaly was caused by discontinuity of the ossicular chain owing to congenital malformation. In the other 19 ears, epitympanic fixation of the ossicular chain was observed, whether or not in combination with malformation of the stapes, incus or malleus. Ossicular chain reconstruction produced an improvement of at least 15 dB in 6 of the first 8 cases with discontinuity of the ossicular chain. The mean gain was 31 dB. Epitympanotomy with exposure of the fixed ossicle and if necessary ossicular chain reconstruction led to an improvement of at least 15 dB in 12 of the 19 ears, with a mean gain of 28 dB. Correspondence to: E. Teunissen     

15例先天性中耳畸形临床特点及治疗

目的分析单纯中耳畸形的临床特点,探讨先天性单纯中耳畸形的诊断治疗方法。方法先天性中耳畸形患者15例（15耳）。男11例,女4例。年龄11～37岁,平均年龄（20.4±6.21）岁,单侧发病7例,双侧发病8例。平均气骨导差（50.33±7.73）dB HL,均行颞骨薄层CT扫描。显微镜下行鼓室探查及鼓室成形术,根据不同的畸形情况采用相应的听骨链重建技术。结果 15例中11例颞骨CT显示不同程度的听骨链异常。术中进行镫骨足板开窗或足板全切除4例（4耳）、前庭开窗2例（2耳）、外半规管开窗2例（2耳）、镫骨上结构存在、足板活动正常,行IIIa型鼓室成形术7例（7耳）。结论影像学检查对先天性中耳畸形的诊断十分重要,手术是主要治疗手段,多数病例可通过手术重建听骨链,恢复中耳传音功能,提高听力。但手术涉及内耳比例高,病例选择要慎重同时要做好充分术前准备。