Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

Background

Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis.

Case presentation

A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma.

Conclusions

Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology.     

Coalescent mastoiditis in a case of congenital aural atresia

A case of unilateral congenital aural atresia presenting as acute coalescent mastoiditis with superiosteal abscess in presented. The literature regarding aural atresia and otitic infection is reviewed, and the recommended preoperative evaluation of a patient with coalescent mastoiditis in an atretic ear is outlined.     

Congenital aural atresia

This report is based on 25 years' experience in the surgical management of 69 ears with congenital aural atresia. There were 7 ears with meatal atresia, 11 with partial atresia, 50 with total atresia, and 1 with hypopneumatic total atresia. Meatoplasty was performed in 7 ears, canaloplasty was performed in 22 ears, canaloplasties with strust in 8 ears, and mastoidectomy with stapediopexy was performed in 26 ears. Of the 62 ears that had partial or total atresia, five (8%) had hidden cholesteatomas that were destined to create future complications by expansion and erosion had they not been removed. In five of the 62 ears, temporary facial palsy resulted from exposing the facial nerve in the atresia plate, and in another five ears, attempts to improve hearing were abandoned intraoperatively because of surgically insurmountable anomalies. A reasonable criterion for success in an operation for bilateral atresia is that the need for a hearing aid is obviated; whereas, for unilateral atresia, a successful procedure should largely eliminate the disadvantages of unilateral hearing loss. These criteria seem to be satisfied if the threshold of hearing can be brought to a level of 20 dB or better. This criterion was met in 30% of the group that underwent canaloplasty and in 8% of the group that underwent mastoidectomy with stapediopexy.     

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.     

先天性外耳道狭窄或闭锁的治疗

目的探讨先天性外耳道狭窄、闭锁的手术治疗及其疗效。方法回顾性分析1996年5月～2011年8月我科手术治疗先天性外耳道狭窄、闭锁患者116耳。结果所有病例均有不同程度的听骨链畸形,其中锤砧融合者67耳,锤骨或砧骨缺如者31耳,前庭窗闭合着9耳,足板上结构异常29耳,面神经畸形或其他异常80耳,记录不全者15耳。术后55耳(47.4%)听力提高。结论手术治疗先天性外耳道狭窄、闭锁患者是有效的治疗方法。     

Microtia and congenital aural atresia

Microtia and congenital aural atresia (CAA) are congenital anomalies that are so common that every otolaryngologist should be familiar with the initial evaluation and care of the patient. When one ear hears normally, speech and language development should be normal. The gross and fine motor development of the baby or child is not expected to be affected in isolated cases of microtia and CAA. Current technologies allow for reconstruction or habilitation of the microtic ear when the child is several years of age. The hope is that tissue engineering can eliminate donor site morbidity. Temporary prosthetic ears will remain an option. Aural atresia work continues to be very dependent on the patient anatomy and the need or desire for better hearing in the affected ear.     

Surgical treatment of congenital aural atresia

The divergent views concerning indications, ideal age for surgery, and surgical approach to achieve better hearing illustrate the complexity of cases of congenital aural atresia. Whether the anomaly is unilateral or bilateral also influences the criteria on which the surgical decision is based. In the thirty-six ears operated on between 1972 and 1983 using the canal plasty technique, the average hearing gain was 20 dB, with 18 ears having an air conduction threshold of 35 dB or better after a long follow-up.     

The risk of cholesteatoma in congenital aural stenosis

Congenital aural stenosis, as compared to congenital aural atresia, carries a much greater risk of cholesteatoma. In a review of over 600 patients with major congenital ear malformations, 50 patients (54 ears) were found to have congenital aural stenosis. Ninety-one percent of the ears in patients 12 years and older with a stenosis of 2 mm or less presented with cholesteatoma. Based on this review, the following opinions are given: 1. A bony ear canal opening of 2 mm or less puts the patient at risk of cholesteatoma formation; 2. the cholesteatoma is slow-growing; and 3. undiagnosed cholesteatoma will cause extensive damage to the ear by early adulthood. We recommend surgery for patients with stenosis of the external ear canal measuring 2 mm or less. The appropriate time for surgery is late childhood or early adolescence, before irreversible damage has occurred.     

Surgical management of congenital aural atresia

Congenital aural atresia is a relatively common anomaly in otology. It requires an early assessment of hearing status and an early evaluation of the degree of atresia. Its surgical management is aimed at obtaining hearing gain and at establishing appropriate auditory canal status for the application of hearing aids. The authors analyzed the preoperative otologic findings, surgical findings, postoperative complications, and postoperative hearing results of 25 ears in 21 patients with congenital aural atresia. From January 1987 to February 1992, 24 of these ears were surgically corrected by anterior approach; the remaining 1 was corrected by transmastoid approach. Postoperative audiologic follow-up ranged from 8 months to 4 years (N = 21). A new modification of anterior approach is introduced. The final average air-bone gap was 30 dB, in contrast to preoperative 53 dB (N = 21). The final air-bone gap was improved to a level within 30 dB in 52% of the patients. Meatal stenosis was the most frequent postoperative complication and was seen in 12 patients in primary operation (N = 25). There was no postoperative facial nerve palsy and no sensorineural hearing loss. There was little difference in postoperative hearing among the various types of congenital aural atresia and the type of tympanoplasty that had been performed.     

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。     

影像导航在骨性外耳道闭锁手术的应用

目的 观察影像导航(image-guided surgery,IGS)下行外耳道骨性闭锁手术的应用效果.方法 分析2001～2004年患C型(Schuknecht分类)先天性小耳畸形病例36耳.试验组(IGS组)18耳,年龄12～29岁,术后随访6个月～1年;对照组(非IGS组)18耳,年龄10～27岁,术后随访6个月～3年.两组均采用鼓窦径路听力重建,由同一手术组完成;比较两组手术时间、术者感觉和听力学改变.结果 所有患者外耳道完全骨性闭锁,骨链畸形,鼓室发育不全,面神经畸形.术前颞骨CT未充分显示畸形的水平半规管,术中导航得以证实.IGS组手术准备时间增加20分钟(15～30分钟),实际手术时间(2小时32分钟)减少25分钟,总时间IGS组则减少5分钟.IGS配准率在0.6 mm～1.3 mm之间,平均0.84 mm.确保术野所见与IGS影像完美结合,使术者感觉更安全.IGS组1例,非IGS组3例术后出现部分外耳道再狭窄.两组均未出现面瘫和术后全聋,均获得明显的听力改善(平均听阈:IGS=31.8 dB,非IGS=30.5 dB).结论 IGS可用于C型外耳道骨性闭锁手术,对术者是一种有益的辅助手段,尤其当解剖标志变异,手术入路受限时,有助于明确手术方向,减少并发症;但IGS仅能明确术腔情况,其技术本身对提高听力无明显作用.     

先天性外耳道畸形伴胆脂瘤的临床分析

目的 探讨先天性外耳道畸形伴胆脂瘤的临床特点及预后.方法 收集2013年8月～2019年10月于福建医科大学附属第一医院手术的先天性外耳道畸形伴胆脂瘤19例(20耳)患者的临床资料,复习相关文献,分析其临床特点及预后.结果 本组病例中,发病年龄为2～30岁,平均9.4岁.主要表现为听力下降(20/20)、耳痛(17/2...     

Congenital aural cholesteatoma: Results of surgery in 60 cases

From 1978 to 1993, 59 patients (60 ears) with congenital middle ear cholesteatoma were treated at the House Ear Clinic. The median patient age at presentation was 5 years, and the period of postoperative follow-up was 4.8 years. An intact canal wall was maintained in 58 of 60 cases and a closed middle ear space in all cases. In 12 operations, lateral graft tympanoplasty eradicated the cholesteatoma in one stage; 32 patients required a second-stage surgery to rule out recurrence, and the remaining 16 cases required three or more operations to eradicate disease and reconstruct the hearing mechanism. Thirty-five (63%) of 56 patients had a postoperative air-conduction threshold pure-tone average (PTA) within 10 dB of the best bone-conduction PTA; 91% were within 20 dB. Average speech reception threshold improved from 32 dB hearing level (HL) preoperatively to 20 dB HL postoperatively.