先天性耳廓、中耳畸形及外耳道闭锁的整复和鼓室成形术——附52例临床分析

目的观察Ⅰ期整复治疗先天性外、中耳畸形和外耳道闭锁并存患者的临床疗效和并发症。方法回顾性分析2000年1月～2007年1月间我科收治52例先天性外耳道闭锁并存中耳畸形患者。其中Ⅰ期全耳廓再造 外耳道成形 听骨链成形19例,耳后置水囊皮肤扩张器 外耳道成形 听骨链成形9例,单纯外耳道成形 听骨链成形20例,单纯外耳道成形 人工听骨安装2例,单纯Ⅰ期全耳廓再造 外耳道成形2例。结果听力重建50例,术后1月听力均有提高,语言频率平均提高25dB以上;其中术后随访6月～5年的38例,29例(76%)语言频率听力平均提高5～40dB。再造耳廓全部成活,其中1例出现耳廓挛缩;外耳道再造52例中,出现外耳道狭窄4例,外听道再闭锁者2例,均经及时处理得到缓解。未出现面瘫及迷路漏管等并发症。结论Ⅰ期整复治疗先天性外、中耳畸形和外耳道闭锁并存患者,并利用自体肋软骨再造耳廓可获得满意效果。     

先天性外中耳畸形的Ⅰ期手术整复

目的 :探讨 期整复术治疗先天性外耳、中耳畸形和外耳道闭锁并存的患者的疗效。方法 :耳廓成形术 :取自体肋软骨作支架 ,患耳颞部超薄皮瓣及皮下组织瓣包裹软骨支架形成再造耳廓。鼓室成形术 :根据鼓室畸形情况作 型或大鼓室 型成形术 ;外耳道再造 :用全厚皮片移植。结果 :术后随访 4～ 6年 ,全部病例外耳道宽畅 ,成形鼓膜完整。语言频率听阈降低均达到应用水平 ;再造耳 11耳成活 ,8耳外形满意。结论 : 期整复术是治疗外耳、中耳畸形和外耳道闭锁并存的有效方法。     

CT定位行外耳道闭锁手术21例

目的：探讨使用CT定位打开骨性外耳道闭锁板行外耳道成形术治疗先天性小耳并外耳道闭锁的有效性和安全性。方法：对21例(22耳)先天性小耳并外耳道闭锁进行术前高分辨率CT检查,术前在CT定位和指导下打开骨性外耳道,一期进行耳廓、外耳道、中耳成形。结果：术后随访8个月～4年,听力平均提高28dB,耳廓外观满意,无外耳道再狭窄或闭锁。结论：高分辨CT定位、指导下打开骨性外耳道安全、快捷,一期进行耳廓、外耳道、中耳成形能达到满意的手术效果。     

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。     

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。     

先天性外耳中耳畸形的一期听力重建整形术

耳廓、中耳先天性畸形常同时与外耳道闭锁并存,导致传导性耳聋。听力重建术包括外耳道与鼓室成形术,通常需分次进行手术。自199S年3月～2002年3月我们对10例(10耳)患者一期进行耳廓、外耳道再造,鼓室成形。取得了满意的效果。现报告如下:     

大腿内侧Thiersch皮瓣在小耳畸形耳廓成形并外耳道重建术中的应用

目的探讨小耳畸形患者采用大脚内侧Thiersch皮瓣在耳廓成形并外耳道重建术中的应用。方法 总结2000年1月-2012年5月先天性小耳畸形并外耳道闭锁的患者105例（115耳）临床资料,所有患者在全麻下行耳廓成形并外耳道重建术,在重建的外耳道内采用大腿内侧Thiersch皮瓣移植,术后10-12 d局部换药,3-4周后外耳道用透明质酸和膨胀海绵填塞,3-6个月后取出填塞物。结果耳廓外形满意,外耳道皮肤上皮化良好,112耳外耳道口直径6-10 mm,外耳道口宽敞;3例患者因未定期复诊出现外耳道狭窄。结论大腿内侧Thiersch皮瓣可满足于先天性小耳畸形术后外耳道植皮需要,术后定期复查可有效预防外耳道狭窄。     

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.     

耳科学

971990全耳廓再造术与听力重建术同步进行/冷同嘉//中华整形烧伤外科杂志一1997,13(1)一5~8 为使先天性小耳畸形外耳道骨性闭锁患者同步取得增进听力并改善耳廓外形一举两得的双重效果.巧8例(16。耳)同步完成听力重建及耳廓再造术。结果:听力增进率(20一45dB)132例(133耳)占83.1%。全耳廓再造成活率155例(157耳)占98.2%,耳廓外形优良(解剖轮廓清楚) 137耳(85.6%)。认为在严格选择病例的情况下可获增进听力及良好外耳形态的效果。图2参9(蓝锋),71,91先天性耳廓畸形耳廓再造术40例小结/周其友…//临床耳鼻咽喉科杂志一1 997.11(4)一176~177,7…     

先天性外耳道闭锁听骨链的虚拟耳镜观察

目的：探讨虚拟耳镜对先天性外耳道闭锁听骨链畸形术前评估的作用。方法：23例（28耳）先天性外耳道闭锁、中耳畸形患者,经高分辨率螺旋CT轴位、冠状位颞骨扫描,应用仿真内镜软件行图像三维重建。术前虚拟耳镜结果与手术探查结果进行对比分析。结果：28耳外耳道闭锁、中耳畸形,虚拟耳镜提示25耳听小骨发育不全,3耳狭小鼓室未见听骨。24耳行手术治疗,术中发现锤、砧骨发育不全19耳,镫骨畸形11耳,镫骨缺失3耳,前庭窗闭锁1耳。术前虚拟耳镜观察与术中探查符合率为100％。结论：虚拟耳镜为先天性外耳道闭锁听骨链畸形的术前评估提供了可靠的影像学信息。     

Navigation-assisted construction of an external ear canal using an autogenous foreskin graft

Classic microtia is combined with external ear canal atresia and middle ear malformation. In order to evaluate whether an operation to improve hearing ability and the use of computer-assisted surgery are indicated, preoperative high-resolution navigation CT is mandatory. We combined atresia surgery and tympanoplasty with auricular reconstruction in the case of an 8-year-old boy with bilateral microtia, aural atresia and malformation of the middle ear. After creating an auricle framework with rib cartilage and transplanting it under the skin of the mastoid plane, we shifted it forward in the second step, and the new auditory canal was drilled under computer-assisted navigation and facial nerve monitoring. In the same operation, tympanoplasty was accomplished, and a silastic cylinder, wrapped into pieces of rib cartilage, was inserted into the constructed canal and removed 2 months later. For lining the new auditory canal, we used the patient's prepuce, harvested by elective circumcision.     

Combined reconstruction of congenital auricular atresia and severe microtia

OBJECTIVES: Due to their embryologic developments, auricular atresia and severe microtia are in most cases combined malformations. The aims of this study were to develop a surgical technique for combined esthetic and functional reconstruction with a minimum of operations and to evaluate the results. STUDY DESIGN: Prospective clinical evaluation. PATIENTS AND METHODS: Fifty-two patients with third-degree microtia and congenital aural atresia with a sound-conducting block of about 50 dB were treated as described here. In the first operation, autogenous cartilage is harvested and the auricular framework fabricated and implanted. In addition, the tympanic membrane and the external ear canal are prefabricated and stored in a subcutaneous pocket. In the second step, the elevation of the new framework is combined with the operation for atresia utilizing the prefabricated tympanic membrane and external ear canal. In the third step, the cavum conchae is deepened and the external ear canal opened and covered with a skin graft. RESULTS: Seventy-six percent of the patients had a final conducting hearing loss of 30 dB or less. No restenosis of the new external ear canal was observed. The esthetic results of the constructed auricles are shown. CONCLUSION: With this combination of plastic surgery for the auricle and functional surgery for the middle ear, no additional operations are necessary, and the prefabrication of the external ear canal and the tympanic membrane gives stable and reliable results. Therefore, we think that this combined technique offers the best chance for an optimal esthetic and functional rehabilitation of patients with these malformations.     

Value of CT scan in the diagnosis of temporal bone diseases in children

CT scan of the temporal bone has become a frequently ordered test for diagnosis of temporal bone pathology. We retrospectively examined our experience with CT scans of the temporal bones in 124 consecutive children from January 1983 to September 1984 in an attempt to assess its usefulness in diagnosis and treatment of ear disease in children. Patients were divided into six categories according to their pre-scan diagnosis, (trauma, congenital aural atresia, dizziness, facial nerve paralysis, middle ear disease, congenital sensorineural hearing loss) and CT findings were compared to data found by physical examination, by otologic studies such as audiogram and BSER, by other x-ray studies and ultimately compared to findings at surgery when applicable. CT was found to be instrumental in diagnosing middle ear disease; gave necessary preoperative information in children with aural atresia; delineated most temporal bone fractures; ruled out gross inflammatory, neoplastic, or traumatic etiologies in dizzy patients and in facial nerve abnormalities; and provided anatomic information about the inner ear in patients with sensorineural hearing loss. In our group of patients with sensorineural hearing loss, a 6.8% incidence of anatomic malformations was found by CT. However, CT had major limitations in stapes and oval window areas, especially in cases of perilymph fistulas. CT use was also limited in children because of the difficulty in achieving projections that require active patient cooperation.     

Disregard of cholesteatoma in congenital aural stenosis

Congenital aural stenosis (CAS) is one type of congenital aural atresia (CAA) and is assumed to be a relatively mild type. Although CAS may be associated with cholesteatoma in the external ear canal, little attention has been paid to this association. We present two cases of CAS with middle ear infection due to destructive cholesteatoma. Both had been followed for microtia by a plastic surgeon. These patients had already exhibited cholesteatoma on CT examination, although it had been missed before the emergence of ear symptoms, otorrhea, and otalgia. We present our cases with successive changes in CT findings over time and discuss the problems of CAS with cholesteatoma.     

Pneumatization of perpendicular plate of the ethmoid bone and nasal septal mucocele

Congenital aural stenosis (CAS) is one type of congenital aural atresia (CAA) and is assumed to be a relatively mild type. Although CAS may be associated with cholesteatoma in the external ear canal, little attention has been paid to this association. We present two cases of CAS with middle ear infection due to destructive cholesteatoma. Both had been followed for microtia by a plastic surgeon. These patients had already exhibited cholesteatoma on CT examination, although it had been missed before the emergence of ear symptoms, otorrhea, and otalgia. We present our cases with successive changes in CT findings over time and discuss the problems of CAS with cholesteatoma.     

钛金属管网状支架在外耳道重建术中的应用

目的观察钛金属管网状支架在先天性外耳道闭锁外耳道重建术中的作用,评估钛金属管网状支架预防外耳道重建术后外耳道再度狭窄或闭锁的疗效。方法 16例（16耳）先天性外耳道闭锁患者同期行外耳道和中耳成型术,在成形的外耳道内置钛金属管网状支架支撑;术后14 d取出外耳道内填塞物,保留内置的支撑钛金属管网支架,并于1年后取出。结果术后随访2年,全部病例新成形的外耳道形态良好,局部无感染与肉芽组织增生,无再度狭窄或闭锁。结论外耳道成形术中应用钛金属管网状支架支撑成形的外耳道,对预防术后外耳道再度狭窄或闭锁具有良好效果。     

Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience

Aims

Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear.Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved.The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia.

Methods

We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7).The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4 kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Children's Benefit Inventory; rate and type of surgical complications.

Results

The mean preoperative air- and bone-conduction thresholds were 51.2 ± 12.5 and 14.1 ± 6.3 dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3 ± 7.2 dB HL, and the mean postoperative threshold with the Baha system was 18.1 ± 7.5 dB HL. Quality of life improved for all operated patients.

Conclusions

The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted in quality of life, while the rate of complications was very low.Therefore, we are convinced that the Baha system is the treatment of choice for hearing loss due to bilateral congenital aural atresia.     

Sound lateralization test in patients with unilateral microtia and atresia after reconstruction of the auricle and external canal and fitting of canal-type hearing aids

Conclusion: In patients with unilateral microtia and atresia after reconstruction of the auricle and external canal and fitting of a canal-type hearing aid for the operated ear, the ability to discriminate the inter-aural intensity difference (IID) was acquired in all of the patients, whereas that to discriminate inter-aural time difference (ITD) was acquired in one-half of the patients. Objective: To study the post-operative sound lateralization ability in patients with unilateral microtia and atresia after reconstruction surgery of the auricle and external canal and fitting of a canal-type hearing aid of the operated ear. Methods: Eighteen patients with unilateral microtia and atresia ranging from 13–24 years of age were recruited in this study. All of them underwent reconstruction of the auricle and external canal and were fitted a canal-type hearing aid for the operated ear. The sound lateralization test was conducted to determine IID and ITD using a self-recording apparatus. The test stimulus was a continuous narrow-band noise at 500 Hz and 50 dBHL presented to the right and left ears through the air conduction receivers. Results: IID could be measured in all of the patients, whereas ITD could be measured in only nine out of the 18 patients. Post-operative binaural hearing could be acquired in all the patients.     

Advances in the treatment of microtia

PURPOSE OF REVIEW: Creating the fine details of the ear in a patient with a congenital absent ear is extremely challenging. Each component of the multidisciplinary team that manages the ear reconstruction, hearing restoration, and associated craniofacial anomalies of these patients has seen recent progress. RECENT FINDINGS: Population studies have provided new insights into the etiology of microtia. Novel techniques for costal cartilage harvest, implantation, and positioning add to the techniques of Brent and Nagata, which remain the gold standard for microtia repair. Advances in the use of alloplasts and tissue-engineered cartilage appear promising. SUMMARY: Technical advances in combined aural atresia/microtia reconstruction, bone-anchored prosthetics, bone-anchored hearing aides, and use of alloplastic implants provide numerous options to patients and practitioners. Implantable, tissue-engineered auricular frameworks appear to be a promising option for the future.