先天性外中耳畸形耳廓再造与听力重建手术的远期疗效观察

目的探讨先天性外中耳畸形患者全耳廓再造与听力重建手术的远期听力效果和并发症。方法对1984年1月至2001年1月期间，北京同仁医院耳鼻咽喉科住院的675例(700耳)行全耳廓再造与听力重建手术的外中耳畸形患者进行随访，其中40耳未行听力重建术，实际听力随访635例(660耳)。随访3～19年，平均7．9年。结果外耳道狭窄120耳，外耳道再闭锁2耳，发生率18．5％(122／660)。外耳道感染6耳。术后听力改善(听力级，下同)20dB以上者512耳，占77．6％；其中改善30dB以上者231耳，占35％。随访结果：术后听力稳定者450耳(68、2％)；随访听力比术后3周时听力不同程度下降者160耳(24．2％)，其中，感音神经性聋2耳，听力下降20dB以上者35耳，123耳为听力下降10～15dB，但仍然比术前听力好。结论68．2％(450／660)耳廓再造与听力重建手术的患者可长期保持稳定的术后听力效果；患者听力下降主要发生于术后半年内，与外耳道狭窄及感染关系密切，少数患者的听力下降与鼓室内粘连、后下壁骨质增生、听骨固定或移位有关。     

先天性外-中耳畸形听力重建疗效观察

目的观察听力重建术治疗先天性外-中耳畸形的临床效果。方法回顾性分析2001年1月～2006年6）1间收治的71例（72耳）先天性外耳道闭锁伴中耳畸形患者治疗结果。所有病例均采用鼓窦径路手术，术中用Fisch技术重建外耳道；听力重建术式则视听骨链畸形程度而定。71耳完成了听力重建手术，其中Ⅰ型6耳，Ⅱ型50耳，Ⅲ型12耳，外半规管开窗3耳。72耳中，59耳（81．94％）随访1～5年。结果术后1-3个月进行听力测试，语频区平均提高15dB以上者58耳（80．56％），25dB以上者41耳（56．94％），35dB以上者21耳（29．16％）。听力重建手术类型与听力提幅程度明显相关。随访的59耳中，49耳（83．05％）术后听力维持长期稳定，10耳（16．95％）因成形鼓膜增厚而听力下降（均大干15dB）。结论听力重建手术是先天性外-中耳畸形患者获得听力提高的良好方法。术中采用Fisch技术重建外耳道，术后定期随访并加强炎症控制是提高术后听力的重要手段。     

先天性外耳和中耳畸形患者的听力学分析

目的探讨不同程度的先天性外、中耳畸形患者的听力学表现。方法对55例(双耳15例,单耳40例,共70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CT检查,其中45耳行外耳或中耳成形术,10耳单纯行耳廓成形术,并结合术中所见按不同程度的畸形分组,比较其听力学改变。结果63耳纯音测听显示为传导性聋,骨导听阈正常,70耳ABR反应阈升高,耳廓和听骨链畸形严重者纯音听阈值大于70dB(其中镫骨和卵圆窗畸形严重者纯音听阈大于80dB),与畸形程度较轻者差异有统计学意义(P<0.05)。乳突气化差、外耳道骨性闭锁者纯音听阈值大于70dB(其中外耳道口处无骨性小凹者纯音听阈值大于70dB),较外耳道、乳突发育好者差异有统计学意义(P<0.05)。结论对先天性外、中耳畸形患者术前进行纯音测听、ABR检查,并结合临床和颞骨CT检查,可初步评估外耳、中耳发育程度,进而指导手术治疗。     

小儿先天性外耳畸形的听力评估

目的：了解小儿先天性外耳畸形的临床听力学特点。方法：将110例先天性外耳畸形患儿分为耳廓畸形加外耳道完全闭锁畸形（闭锁组），耳廓畸形加外耳道狭窄（狭窄组）和仅耳廓畸形，外耳道及鼓膜正常（仅耳廓畸形组）3组。患侧耳和健侧耳均进行听性脑干诱发电位（ABR）测试，健侧耳及外耳道狭窄的患儿还进行畸变产物耳声发射和鼓室导抗测试。结果：闭锁组94例（101耳）中，79耳（78．22％）ABR表现为重度异常；健侧87耳中，8耳（9．20％）ABR表现为中、重度异常。狭窄组8例（8耳）中，5耳（62．50o／4）ABR表现为重度异常；对侧健耳中ABR未出现中、重度异常。仅耳廓畸形组8例（9耳）中，4耳（44．44%）ABR表现为重度异常；对侧健耳中ABR未出现中、重度异常。结论：先天性外耳畸形是导致小儿听力障碍的重要影响因素，并存在听觉神经损害。     

先天性外耳道闭锁症手术方法的选择

目的探讨先天性外耳道闭锁症的中耳畸彤特点、治疗方法及其效果。方法回顾性总结分析1999年1月-2003年12月在我科接受外耳道及鼓室成形术，且有完整随访资料的先天性骨性外耳道闭锁64耳。结果41耳采取直入式径路即前上径路，23耳采崩乳突径路。64耳中，有3耳未找到鼓室。61耳中，鼓室及听小骨存在不同程度的发育异常；同时，术中发现而神经畸形或异常共40耳（65．57％）：术后随访1～5年。61耳中，49耳术后（语频平均）听力提高〉30dBHL（80-33％1．气骨导差缩小至15dBHL以内者32耳（52．46％1。61耳中有6耳于术后3月～2年出现外耳道狭窄而需行冉次外耳道成形术，4例术后效果良好，2例拒绝再次手术；2例术后出现鼓膜外侧愈合，听力提高不理想，均因另耳听力尚可亦拒绝再次手术；术后有3耳出现面瘫，2—6月后完全恢复。术后2例出现术腔感染。结论外耳道区内为骨化型和松质型者可作为直入式径路的适应证，而混合型或气房型最好采用乳突径路。手术步骤无固定术式，须视术中所见，灵活机动处理．     

鼓窦径路手术治疗先天性外耳道闭锁中耳畸形附89例临床分析

目的：探讨鼓窦径路治疗先天性外耳道闭环锁中耳畸形的效果及重建耳道再闭锁的预防方法。方法：回顾性分析1993－1998年5月间收治的89例（94耳）先天性外耳道闭锁中耳畸形病人,均采用鼓窦径路重建外耳道,鼓室成形术,其中Ⅱ型80耳、加高Ⅲ型11耳、内耳开窗3耳。康宁克通A注射、扩张子扩张等预防耳道再闭锁。结果：94耳均顺利找到鼓窦,开放鼓室,行听力重建,术后语言频率平均听力提高20dB以上者80耳（85．1％）,提高25dB以上者49耳（52．1）。再造耳道有闭锁征象者经康宁克通A注射、扩张子扩张取得良好效果。结论：鼓窦径路治疗先天性外耳道闭锁具有易掌握、安全、省时、效果可靠等特点,再造耳道有闭锁征象时应及时处理。     

先天性外中耳畸形的听力重建与同步耳廓再造

目的 总结同步进行外耳道、鼓室成形术与应用多孔高密度聚乙烯（MEDPOR）再造耳廓技术治疗先天性外、中耳畸形的手术经验.方法 25例（25耳）外、中耳畸形患者,采用Ⅰ期外耳道、鼓室成形术联合耳后皮下皮肤扩张器埋置术,Ⅱ期应用MEDPOR作支架进行耳廓再造.术后随访1～5年,观察疗效并总结临床经验.结果 术后1个月语频气导听力提高15dB HL以上者21耳（84%）,其中听力提高30dB HL以上、气骨导间距少于15dBHL者10耳（40%）,仍能保持听力稳定者17耳（68%） MEDPOR耳廓再造18耳一期愈合,外形良好,7耳支架外露,需要再次手术修复.结论 听力重建与MEDPOR耳廓再造同步进行可获得较满意的听力提高水平和耳廓外形.     

先天性耳畸形的手术治疗(附24例报告)

目的探讨治疗先天性耳畸形的不同手术方式及疗效，评估同期行耳廓成形及听力重建术的可行性。方法回顾性分析1985～2003年本科收治的24例(24耳)先天性耳畸形的患者，分别为单纯外耳道成形术6耳，外耳道-鼓室成形术10耳，同期行耳廓耳道成形及鼓室成形术8耳。结果行听力重建术的18耳，术后1个月纯音测听言语频率平均气骨导差缩小10～30dB；外耳道成形24耳中，5耳耳道狭窄；耳廓再造8耳均成活，近期耳轮、对耳轮出现，但远期效果较差，耳廓有不同程度的缩小变形。结论外耳道成形及听力重建术是治疗先天性耳畸形有效的手段，而同期行耳廓成形是可行的；以扩张后的薄皮瓣行耳廓成形效果较好，耳后带蒂皮瓣修复外耳道能有效防止外耳道再闭锁。     

Medpor与膨体聚四氟乙烯在耳廓和外耳道一期重建术中的应用

目的：探索先天性小耳并外耳道闭锁行耳廓和中耳一期成形术的效果。方法：对6例（耳）采用Medpor聚乙烯材料作为对耳轮支架，膨体聚四氟乙烯（PTFE）作为耳轮支架，一期进行耳廓，外耳道，中耳成形。结果：术后随访8-23个月，听力平均提高28dB，耳廓外观满意，无再狭窄或闭锁。结论：Medpor作为对耳轮支架，PTFE作为耳轮支架，一期进行耳廓，外耳道，中耳成形，可以达到满意的术后效果。     

先天性外中耳畸形的诊断和治疗

目的：探讨先天性外、中耳畸形的特点,术前评估和治疗效果。方法：分析2000年6月～2005年6月接受手术治疗的先天性外耳道闭锁中耳畸形患者21例（22耳）的临床资料,全部患者均行外耳道鼓室成形术。结果：术中发现鼓室腔狭小16耳（72．7%）;听骨链畸形20耳（90．9％）,其中锤砧骨畸形19耳（86．4％）,镫骨畸形7耳（31．8％）;面神经畸形8耳（36．4％）;鼓室隔板1耳（4．5%）;垂直外耳道1耳（4．5％）。术后1个月平均语频听力提高〉15dB15耳（68．2％）,其中提高≥30dB9耳（40．9％）。随访6个月～5年,听力保持在术后水平或有轻度提高者17耳,下降3耳,失访2耳。并发外耳道闭锁1耳,狭窄3耳,鼓膜外侧愈合2耳。结论：先天性外、中耳畸形表现复杂多样,术前详细的影像学和听力学评估是保证手术成功的前提,成形一个宽敞的外耳道、预防术后感染是防止外耳道再闭锁和狭窄的关键。     

Congenital malformations of the middle ear with an intact external ear: a review of 38 cases

In patients with middle ear malformations, one can expect an improvement in hearing following ear surgery. Thus, it is crucial for the ear surgeon to have an increased awareness of this disease. For a better understanding of this condition, 38 patients who underwent ear surgery were studied. All patients had congenital ossicular malformations but with an intact external ear. On the basis of our intra-operative observations, 15 cases were assigned to group A (single malformation) and 23 cases to group B (multiple malformations). The pre-operative air-conduction threshold in group B patients was higher than that in group A. Pre-operatively in group A 2 out of 20 ears had mild, 17 had moderate and 1 had severe hearing loss (HL). In group B, out of 25 ears, 3 had mild, 13 had moderate and 9 had severe HL. Post-operatively in group A, 2 had normal hearing, 16 had mild, 2 had moderate and none had severe HL. In group B, postoperatively 1 had normal hearing, 18 had mild, 4 moderate and 2 severe HL. Therefore, in patients with multiple middle ear malformations, the surgeon should know that the prospect of an improvement in the hearing threshold is rather low even after surgical reconstruction.     

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。     

Congenital perilymphatic fistula and associated middle ear abnormalities

To specifically determine the frequency and type of middle ear abnormalities associated with perilymphatic fistula (PLF), a retrospective chart review was performed of 94 patients (117 ears) who underwent exploratory tympanotomy for PLF from 1980 to 1989. Of these 94 patients, 60 (63.8%) had a PLF identified. Of the 117 ears explored, 80 (68.4%) had a PLF, and in 65 (81.3%) of these ears, a middle ear malformation was associated with the PLF. Of these 65 ears in which a congenital middle ear abnormality was observed, a malformed stapes was the most common abnormality seen (39 ears, 60%), followed by a deformed round window (20 ears, 30.8%), a deformed incus (11 ears, 16.9%), and a deformed promontory (2 ears, 3%). Often these malformations coexisted amongst themselves or with inner ear abnormalities. Sixteen children (25 ears) had an inner ear malformation identified on computed tomography (CT); all of these children had a PLF found at the time of surgery. This study demonstrated that 86.3% of the ears found to have a PLF had a deformity of the middle ear, inner ear, or both. A malformation of the stapes, most frequently identified as a deformity of its superstructure (and presumably also the anterior footplate), was the most common congenital middle ear abnormality found to be associated with PLF in children.     

先天性内耳畸形并中耳畸形患者多通道人工耳蜗植入术3例报告

目的：探讨先天性内耳畸形并中耳畸形患者行多通道人工耳蜗植入术的方法及效果。方法：1995年5月-2002年5月我院为3例罕见的先天性内耳畸形并中耳畸形患者经乳突进路行人工耳蜗植入术,分别植入27、28、32个电极,植入后3个月行声场测听。结果：3例患者均成功地行人工耳蜗植入,术中、术后无严重并发症发生,术后声场测听听阈达35-40dBHL。结论：罕见的先天性内耳畸形并中耳畸形患者也可行人工耳蜗植入术,术后效果满意。     

One hundred and seventy-five surgically treated malformations of the external and middle ear: findings and results

During 10 years 222 patients suffering from malformation of the external and/or middle ear came for treatment to our clinic. One hundred and fifty-seven have been operated on, 139 on one side, 18 bilaterally. This report is based therefore upon 175 cases and deals especially with findings seen during operation, methods of reconstruction and results achieved. Thirty-six patients showed a stenosis of the external meatus, 78 had a partial or complete aplasia. In 61 ears exclusive malformation of middle ear structures was the cause of hearing loss (anomalies of malleus 58.2%, of incus 58.8% and of the stapes nearly always: 172 out of 175 ears). Combinations were frequent. Three structures may affect the development of the ossicular chain, when showing abnormal development themselves: 1) facial nerve, 2) a persistent stapedial artery and 3) the chorda tympani. Anomalies of the course of the facial nerve have been found in 87 ears. In 60 cases these deviations of the course caused obviously a malformation of the ossicular chain, too. A persistent stapedial artery has been seen in six ears. The stapes footplate was always fixed. In four patients a lateral deviation of the chorda tympani's course caused an interruption of the ossicular chain, mainly between the head and the handle of the malleus. The long process of the incus was involved, too, in different degree. The ratio between one-sided and bilateral aplasias was about 2:1. Our method to reconstruct the external meatus is described in detail. The functional results were very satisfying in exclusive anomalies of the ossicular chain.(ABSTRACT TRUNCATED AT 250 WORDS)     

Classification of facial nerve aberration in congenital malformation of middle ear: Implications for surgery of hearing restoration

Objectives

Facial nerve aberration is the most troublesome situation in congenital malformations of middle ear. The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement.

Analysis of surgical reconstructions of canal and middle ear via "direct entrance" approach in 53 ears with congenital aural atresia]

OBJECTIVE: To recognize the indication of surgical reconstruction via the "direct entrance" surgical approach (superoanterior surgical approach) for congenital atresia of the external acoustic canal and malformations of the middle ear. METHODS: A series of 53 ears operated on via the superoanterior surgical approach during 10 years from January 1988 to December 1997 was analyzed. RESULTS: The tympanic cavity was encountered without difficulty in 52 ears. Facial paralysis occurred postoperatively in one ear, which recovered after two months. The hearing improvement was observed in 45 ears (84.9%), of which 24 ears had hearing improvement greater than 30 dB and the air-bone conduction gap was within 15 dB (45.3%). A long-term (1-9 years) follow-up of 25 ears demonstrated that the hearing levels in 18 ears were kept unchanged. Postoperative stenosis of the canal occurred in one ear of a 2 years-old child. CONCLUSION: Based on the scanning images of the temporal bone by high-resolution computed tomography before the operation, the authors classified the bony structures of the paths of external acoustic canals into four types. Our results indicated that the selerotic and the diploetic paths of external acoustic cmeati could be indications for the "direct entrance" surgical approach, whereas the mixed type, either containing a little small cells or diploetic bone in sclerotic bone, might be suitable for this surgical approach also.     

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.