虚拟耳镜在先天性外耳道闭锁术前评估中的价值

目的：探讨虚拟耳镜在先天性外耳道闭锁术前评估中的价值。方法：选取20例先天性外耳道闭锁患者行颞骨薄层CT检查,其中8例接受外耳道成形术、鼓室成形术。螺旋CT获取原始图像数据,经软件三维重建后,以虚拟耳镜方式观察中耳等结构。结果：虚拟耳镜能够清晰显示镫骨、锤砧复合体、面神经（仅限鼓室段）、砧镫关节、圆窗龛及中耳腔等结构,与手术探查结果的符合率分别为87．5％、100．0％、i00．0％、75．0％、100．0％和100．0％。结论：虚拟耳镜有助于先天性外耳道闭锁的术前评估,使手术方案达到个体化。     

Facial nerve course in congenital aural atresia – identified by preoperative CT scanning and surgical findings

Conclusions. The facial nerve courses shown by preoperative CT imaging coincide with the surgical findings in most congenital aural atresia cases. CT scanning is critical in evaluation of a patient's candidacy for atresia surgery. Objectives. To evaluate the diagnostic value of preoperative CT scanning of the facial nerve course in congenital aural atresia. Subjects and methods. All inpatients (135 cases) with congenital aural atresia had routine preoperative CT examination. The facial nerve courses were observed in the operation and compared with the preoperative CT imaging. Results. CT imaging showed that the mastoid portions of the facial nerves in 57% of cases (77/135) were located at the level of the round window, and 17% (23/135) were located at the levels of the oval window and the cochlea. Differential overlapping of the oval window by the facial nerve could also be shown in most of the cases. In the majority of cases (75%, 61/81) the tympanic portions of the facial nerve on CT imaging could be confirmed by surgical findings. In severe overlapping of the oval window or cases with anterior displacement, stapedectomy or tympanoplasty, respectively, could not be performed as usual.     

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.     

Korner 隔与外耳道骨性闭锁

的：分析外耳道骨性闭锁并发Korner隔(KS)的解剖特征及临床意义。方法：外耳道骨性闭锁并发KS异常的50例(耳)患者均行同步性听力重建术及全耳再造术,术前均行高分辨颞骨CT检查,常规采用轴、冠状位CT扫描。结果：39例KS较为完整,与鼓窦有明显分界;11例KS不完整,术中可见KS明显延长,分隔乳突气房与鼓窦,仅有极细小孔隙与乳突窦相通。41例于术中经解剖定向后终于破“隔”进入鼓窦;9例因脑膜低位、髁突后位等解剖障碍而中止手术,未探及到KS。结论：术前CT检查对观察KS异常有重要意义,对临床手术具有指导作用。     

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年间收治的 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 型 80耳、加高 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 2 0 d B以上者 80耳 (85 .1% ) ,提高 2 5 d B以上者 49耳(5 2 .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。     

Grading system for the selection of patients with congenital aural atresia.

It is generally recognized that surgery for congenital aural atresia is difficult. In an effort to select those patients who have the greatest chance of success, we have developed a grading scheme based on the preoperative temporal bone CT scan and the appearance of the external ear. Patients are graded on a possible best score of 10. The stapes is assigned the highest rating (2 points), while all other entrees on the scale are 1 point. The grade assigned preoperatively has been shown to correlate well with the patient's chance of success, herein defined as a postoperative speech reception threshold of 15 to 25 dB. A patient with a preoperative grade of 8/10 would, therefore, have a 80 percent chance of achieving this threshold. Patients with scores of 5/10, or less, are not considered surgical candidates, because the risk of the operation would outweigh the potential benefits. We have found that the grading system allows us to avoid impossible surgical cases while allowing for a reasonable prediction of the hearing outcome.     

The location of the mastoid portion of the facial nerve in patients with congenital aural atresia

In order to investigate the location of the mastoid portion of the facial nerve in patients with congenital aural atresia and to assess its effect on the round window middle ear implant (MEI) transducer implantation approach, 70 patients with unilateral congenital aural atresia were examined by computer tomography (CT). The patients were divided into two groups based on their ages: 44 patients in Group A (2–12 years) and 26 patients in Group B (13–29 years). CT scans were reviewed for each patient. Based on the CT findings, the mastoid portion of the facial nerve’s spatial configuration with respect to the oval and round windows was qualitatively recorded. Additionally, the exact location of the facial nerve was measured quantitatively. The results suggested that of the 70 deformed ears, 57 had facial nerves located at the round window, six at the oval window, and seven at the normal site. Of the 70 normal opposite ears, 63 had facial nerves located at the normal site, and the other seven had facial nerves located at the round window. Based on the quantitative measurements, the mastoid portion of the facial nerve was more anteriorly positioned in the deformed ears: 3.44–6.09 mm more anteriorly located in Group A and 4.35–7.41 mm more anteriorly located in Group B. In conclusion, in patients with congenital aural atresia, the dislocation of the facial nerve could have significant effects on the surgical approach to round window MEI transducer implantation.     

Application of a rapid-prototyped temporal bone model for surgical planning

CONCLUSION: This 3D prototyped model constructed using a selective laser sintering method serves as a good material for surgical simulation of a malformed ear. OBJECTIVE: In order to perform surgery of the malformed ear, a thorough understanding of the anatomy of the ear is required, together with sufficient experience. The aim of this study was to investigate the validity of a prototyped temporal bone model for surgical simulation. MATERIAL AND METHODS: Simulated 3D models of two cases of congenital aural atresia were prototyped using a selective laser sintering method. Conventional surgical instruments were used to dissect the models. RESULTS: Abnormal structures of the middle ear, such as the ossicles, labyrinth and facial nerve, were identified by dissecting the model. Presurgical dissection informed the surgeon of the optimum orientation for surgery. The model was disinfected and could be handled by the surgeon in the operating room. Surgery could be carried out safely although in one case it was canceled because of the highly abnormal location of the middle ear cleft and facial nerve.     

先天性外耳道骨性闭锁患者人工耳蜗植入的手术分析

目的探讨先天性外耳道骨性闭锁行人工耳蜗植入患者的听力学检查、影像学特征及手术径路的选择。方法收集2015年7月—2019年1月诊治的5例先天性外耳道骨性闭锁行人工耳蜗植入术的患者,回顾性分析其病史、听力学检查、影像学特征及手术径路。结果5例患者听力均表现为重度感音神经性聋。影像学表现为外耳道骨性闭锁,伴听骨链畸形,面神经走形异常,均无内耳畸形。5例患者均采用鼓窦径路,其中2例患者经面神经后下植入人工耳蜗。所有患者均为圆窗膜植入,植入电极过程顺利,术后开机反应佳。结论针对外耳道骨性闭锁畸形需行人工耳蜗植入的患者,应采用外耳道骨性闭锁的鼓窦径路的手术方式,开放部分乳突及鼓窦,取出畸形的听骨链,在此基础上,进一步暴露圆窗龛。对于面神经乳突段前移,完全遮挡圆窗龛的患者,则可转经面神经后下暴露圆窗龛。     

Congenital cholesteatoma in a case of congenital aural atresia.

OBJECTIVE: A congenital cholesteatoma arising in the setting of congenital aural atresia is described. This represents the first case reported in the literature. STUDY DESIGN: Case report. SETTING: Tertiary otologic referral center, University of Virginia Health System, Charlottesville, Virginia. PATIENT: Eight-year-old patient with unilateral congenital aural atresia. INTERVENTIONS: Preoperative computer tomographic scanning and surgical atresia repair with removal of cholesteatoma. MAIN OUTCOME MEASURES: Recurrence of cholesteatoma; epithelialized ear canal; hearing improvement. RESULTS: A congenital cholesteatoma located medial to the ossicular mass in a child with congenital aural atresia was removed, with no evidence of recurrent disease at a revision operation 1 year later. Hearing was improved to a speech reception threshold of 25 dB. CONCLUSION: Congenital cholesteatoma can arise medial to the ossicular mass in congenital aural atresia. This finding lends support to the epidermoid rest theory for the cause of congenital cholesteatoma.     

Results of tympanoplasty for congenital aural atresia and stenosis,with special reference to fascia and homograft as the graft material of the tympanic membrane

The surgical management of congenital aural atresia is a challenging, complex procedure, and the risks are great. The otologic surgeon is responsible for keeping a patent external auditory canal and for achieving satisfactory hearing. The present report studied hearing changes that occurred after tympanoplasty and the long-term results of tympanoplasty in 12 cases of congenital aural atresia or stenosis. Patients were followed for more than 2 years after surgery. In four ears, an allograft of the tympanic membrane with an attached malleus was used, with a good graft take and hearing results. An autograft of temporal fascia was used in eight ears. Hearing acuity decreased in six of the eight ears and was maintained in two ears. Three primary surgery patients required revision surgery for postoperative restenosis of the external auditory canal. Some comments have been made with regard to this problem. Emphasis is also placed on the selection of patients for surgery using a thorough audiologic and roentgenologic evaluation.     

Identification of 2.3-Mb gene locus for congenital aural atresia in 18q22.3 deletion: a case report analyzed by comparative genomic hybridization.

OBJECTIVE: 18q deletion syndrome is a multiple-anomaly mental retardation syndrome associated with congenital aural atresia. The purpose of this study was to determine the frequency of the congenital aural atresia phenotype in 18q deletion syndrome patients and to delineate a potential critical region for congenital aural atresia at the 18q22.3-18q23 region. STUDY DESIGN AND PATIENTS: The study describes one 18q deletion syndrome clinical report (Patient 15) with an overview of 19 other selected 18q deletion syndrome patients presenting congenital aural atresia from 18 published articles and one presented poster on 18q deletion syndrome. RESULTS: Our investigation, together with the results of published 18q deletion syndrome reports, shows that the average frequency of congenital aural atresia is approximately 52%. A combination of three 18q deletion syndrome probands defines a chromosomal deletion site for congenital aural atresia at 18q22.3-18q23 in the region between markers D18S489 and D18S554. These polymorphic markers outline a putative critical interval of approximately 2.3 Mb, including the genes ZNF407, ZADH2, SDCCAG33, ZNF516, FLJ44881, ZNF236, MBP-Golli, and GALR1. The haploinsufficiency of these genes is suggested to be a primary cause of congenital aural atresia phenotype in 18q deletion syndrome individuals. CONCLUSION: Congenital aural atresia is a relevant diagnostic clue and a major recognizable feature of 18q deletion syndrome. Early diagnosis of 18q deletion syndrome may enable application of hearing aids. Knockout studies on the congenital aural atresia mouse gene homolog may add further insight into the genes responsible for this condition.     

Diagnosis and management of salivary fistula after surgery for congenital aural atresia.

OBJECTIVE: Salivary fistula is an uncommon and unreported yet meaningful complication associated with the repair of congenital aural atresia. The capsule of the parotid gland may be violated during two steps of the operation: the initial dissection around the glenoid fossa or while aligning the auricle with the bony canal at the end of the procedure. We present the first described series of patients with salivary fistula after repair of congenital aural atresia. STUDY DESIGN: Retrospective case review from 1985 to 2004. SETTING: Tertiary referral center. PATIENTS: We included all patients who were diagnosed with a salivary fistula after congenital aural atresia repair. MAIN OUTCOME MEASURE: The diagnosis of a salivary fistula or salivary tissue in the external auditory canal after atresia repair was based on one of the following criteria: 1) identification of a fistula tract or salivary tissue in the external auditory canal, 2) otorrhea positive for amylase, or 3) intermittent otorrhea associated with eating. RESULTS: Of 1,500 patients operated on for aural atresia, we identified 6 with salivary fistula after atresia repair. Salivary fistulas were diagnosed from 15 days to 10 years postoperatively, and the duration ranged from 6 months to 14 years. Treatment included observation, medical management, and surgical intervention. CONCLUSION: Salivary fistulas in the external auditory canal may present with granulation tissue, persistent crusting, or persistent otorrhea; it is therefore necessary to consider salivary fistula when managing these findings in postoperative congenital aural atresia patients. Salivary fistula secondary to repair of congenital aural atresia may be managed conservatively or surgically.     

手术治疗外耳道闭锁或狭窄15例

目的 探讨手术治疗外耳道闭锁或狭窄的更好方法。方法 回顾性分析15例接受外耳道成形术的外耳道闭锁或狭窄患者的病历资料,术后随访6个月～2年。结果 15耳中术后出现再闭锁1耳,其余患者外耳道均获得扩大。结论 外耳道闭锁或狭窄可根据先天性或后天性,伴或不伴中耳乳突病变,采用不同的手术方式。手术关键在于成形外耳道和外耳道口,移植皮片覆盖缺损区并成活。     

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。     

Diagnosis and management strategies in congenital atresia of the external auditory canal. Study Group on Otological Malformations and Hearing Impairment

This consensus report represents a distillation of current opinion regarding diagnosis and management of congenital aural atresia. It also takes into account the philosophical differences which exist in Europe. Congenital aural atresia requires prompt diagnosis, genetic counselling and an early assessment of hearing. In bilateral atresia, early amplification with a bone conduction hearing aid is essential for proper speech development. Further rehabilitation in bilateral cases is managed with surgical reconstruction in selected patients or by implantation of a bone-anchored hearing aid. Atresia repair surgery is worthwhile if proper patient selection is made by use of stringent audiological and radiological criteria and state of the art surgery is performed. The divergent views concerning indications, ideal age for surgery and surgical approach to achieve better hearing are discussed. Review of the literature demonstrated that even in the hands of the best surgeons a mean hearing gain of only 20-25 dB is achieved in atresia Type II, with 30-35 dB in Type I. Therefore, surgical reconstruction should only be done in the more favourable cases where post-operative hearing of <25-30 dB is attainable. Less favoured patients should be helped with bone-anchored hearing aids, as this type of surgery does not interfere with the future use of new techniques.     

Congenital malformation of the oval window: experience of radiologic diagnosis and surgical technique

The aim of this study was to introduce and discuss the method of preoperative radiological diagnosis to the congenital absence or atresia of the oval window (OW), and the method of surgical treatment. From July 2010 through August 2014, patients with normal external canal and tympanic membrane but conductive hearing loss underwent high resolution CT scan (HRCT). The multi-planar reformation (MPR), a post-processing protocol, was used. The patients with diagnosis of OW atresia and malformed stapes preoperatively underwent surgical treatment. The vestibular drilled-out and promontory drilled-out technique was used to reconstruct the ossicular chain. In the preoperative radiological diagnosis, six patients (ears) were noted to have congenital absence or atresia of the oval window with malformed facial nerve (class 4) and two patients (ears) were found to have footplate fixation (class 2). In the surgical treatment of eight ears, the malformed structure was identified and the ossicular chain reconstruction was made in six ears. The coronal HRCT CT imaging and the MPR post-processing technique can provide us practical and definite information for surgical treatment, especially in the discrimination of OW atresia and the fixed stapedal footplate. The promontory drill-out technique, fenestration in the bottom of the basal turn, provides us a new method in the hearing reconstruction when the area of OW was fully covered by malformed facial nerve. This technique was first reported in the literature.     

Congenital aural atresia. A new subclassification and surgical management

The four surgical methods currently in use to improve hearing in patients with congenital aural atresia are (i) fenestration of the lateral semicircular canal, (ii) type III tympanoplasty, (iii) canal plasty and (iv) canal plasty with homograft tympanoplasty. Because the surgery is difficult and the functional results are not always good, the criteria for surgery are also influenced as to whether the anomaly is unilateral or bilateral and what the cosmetic goal is. When aural rehabilitation and cosmetic surgery are contemplated, co-ordinated planning by the otological and plastic surgeon is necessary to attain the best end result. Of the 36 ears with a type II anomaly operated on at the University of Nijmegen between 1972 and 1983 by the technique of canal plasty, the average hearing gain was 20 db, with 18 patients having a 35 dB or better air conduction threshold. The functional results seem to be related to the degree of malformation involved. Classification of the malformations has proved to be useful in discussing the surgical results.     

Congenital aural atresia A new subclassification and surgical management

The four surgical methods currently in use to improve hearing in patients with congenital aural atresia are (i) fenestration of the lateral semicircular canal, (ii) type III tympanoplasty, (iii) canal plasty and (iv) canal plasty with homograft tympanoplasty. Because the surgery is difficult and the functional results are not always good, the criteria for surgery are also influenced as to whether the anomaly is unilateral or bilateral and what the cosmetic goal is. When aural rehablitation and cosmetic surgery are contemplated, co-ordinated planning by the otological and plastic surgeon is necessary to attain the best end result. Of the 36 ears with a type II anomaly operated on at the University of Nijmegen between 1972 and 1983 by the technique of canal plasty, the average hearing gain was 20 db, with 18 patients having a 35 dB or better air conduction threshold. The functional results seem to be related to the degree of malformation involved. Classification of the malformations has proved to be useful in discussing the surgical results.