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 in resident cholesteatoma surgery: a review of 85 cases

A review of all patients with cholesteatoma operated on by the residents of the New York Eye and Ear Infirmary under the attending supervision of the Otology Service between 1980 and 1984 was done. Of 130 resident cases, 85 had documented follow-up 1 year or longer and were included in the study. Recurrence rates were 18% with canal wall down (CWD) and 43% with canal wall up (CWU) techniques. Considering other forms of failure (such as precholesteatoma, tympanic membrane perforation, and chronic infection), overall failure rates were 33% (CWD) compared with 82% (CWU). Cholesteatoma recurrence failure rates were higher in patients under the age of 20 (33%) than over the age of 20 (19%). Overall failure rates were 61% and 37% for younger and older patients, respectively. Due to the high failure rate with canal wall up techniques in resident hands, it is recommended that resident service cases be treated with canal wall down technique, especially in the younger age group.     

Congenital middle ear cholesteatoma: experience in 48 cases

We studied 48 patients (48 ears) with congenital cholesteatoma who underwent surgery at our department from 1979 to 2000, and investigated symptoms at initial onset, tympanic membrane findings, cholesteatoma configuration and site, type of surgical procedure, and surgical outcome. Patients were from 2 to 62 years old (mean: 16.7 years), with 60.4% aged 15 years or younger. The symptom at initial onset was hearing loss in most (58.2%). Hearing loss was the main symptom in all with open type cholesteatoma, and most of these patients had normal tympanic membrane findings. The cholesteatoma was located mainly in the superior posterior portion of the tympanic cavity in many patients. The site of involvement was the tympanic cavity in 12 (25.0%), mastoid cavity in 2 (4.2%) and the petrous apex in 1 (2.1%). In many of (31 ears, 64.6%), the cholesteatoma was advanced and extended from the tympanic cavity to the mastoid antrum. For 23 of the 48 ears, treatment was completed in one operation. The remaining 25 ears required staged surgery. Loss of the structure of the upper part of the stapes was seen in 58.3% of patients, so most underwent type IV ossiculoplasty, with types III and I next most common.     

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.     

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.     

Congenital aural atresia surgery: Transmastoid approach,complications and outcomes

Repair of complete congenital aural atresia (CAA) could be a challenging procedure due to complications reported with CAA surgery such as facial nerve palsy, canal stenosis, graft lateralization, sensorineural hearing loss or the difficulty involved in the surgical technique. From 2006 to 2009, we used a one stage-modified transmastoid approach for surgical repair of 33 ears with complete CAA via a non-randomized controlled clinical trial. Some modifications in the technique of mastoidectomy, ossiculoplasty, fascia and skin grafting and meatoplasty have been described. Patients were followed up for 12 months to assess audiometric results and post-operative complications. Changes in air-bone gap and need for revision surgery or hearing aids were assessed at follow-up. There were no cases of facial weakness, dead ear or bony canal stenosis. Hearing success in 2 months follow-up was achieved in 72.7% of all patients. Success rate increased to 92.3% in patients with Jahrsdoefer’s scores of 8 and above. Overall success rate decreased to 63.6% at 12 months follow-up. There were no significant difference in Jahrsdoerfer score of patients with successful first surgical attempt and those who needed revision surgery (P value >0.056). Also patients of lower age (less than 5-years-old) did not have more need for revision surgery when compared with older patients (P value >0.36). However, being a syndromic patient did increase the need for revision surgery (P value <0.04). Age was not a predictor of meatal/canal stenosis and patients with lower Jahrsdoerfer scores could also achieve good results.     

Congenital middle ear cholesteatoma: report of 3 cases.

In this report, we presented 3 cases of congenital middle ear cholesteatoma which occurred in a 12-year-old girl, a 4-year-old boy, and a 6-year-old boy. In all 3 cases, there was a whitish mass behind a normal tympanic membrane. Congenital middle ear cholesteatoma is not a rare disease. In the early stage, it is asymptomatic. But when it progresses, this disorder can destroy conductive systems of the middle ear and cause many symptoms. One patient (Case 1) had a complaint of hearing impairment. She underwent mastoidectomy and tympanoplasty; however, the cholesteatoma recurred. The other 2 patients had no symptoms. The abnormal appearance of their tympanic membrane was found by chance at their local otologists. We performed tympanotomies and removed cholesteatomas without aftereffects. When otologists note an abnormal appearance behind a normal tympanic membrane, with or without symptoms, tympanotomy should be done due to the possibility of congenital middle ear cholesteatoma.     

Immunologically activated cells in aural cholesteatoma.

In this immunohistochemical study, we characterized the cells infiltrating the stroma of acquired aural cholesteatomas in detail, using a panel of monoclonal antibodies directed against immune cell type-specific antigens, HLA class II antigens, and interleukin-2 receptor. For all antibodies used, normal ear skin was stained for comparison. The vast majority of the infiltrating cells was CD45-positive, ie, derived from bone marrow. Reactivity with anti-CD3 and anti-CD6 antibodies revealed an abundant infiltration of T lymphocytes beneath the squamous epithelium of cholesteatoma. The B lymphocyte-specific anti-CD19 and anti-CD22 antibodies detected only occasional positive cells. Hence, the cellular infiltrate in the stroma of aural cholesteatoma is made up primarily of T cells with macrophages scattered between them. Expression of HLA-DR was almost as high as that of CD45, whereas CD25-positive cells were detected in lower amounts. We infer that the majority of T cells and macrophages in the stroma of cholesteatoma are in an immunologically activated state. The characteristics of the infiltrating cell population suggest an antigen-driven process in cholesteatoma.     

Pathogenesis of experimental aural cholesteatoma in the chinchilla

Histopathological observation of celloidin serial sections of the chinchilla middle ear after treatment with propylene glycol disclosed the development of severe inflammation of the middle ear mucosa and tympanic membrane, papillary proliferation of the epidermis of the tympanic membrane and external auditory meatus, and retraction and adhesion of the tympanic membrane. The findings for the tympanic membrane, impedance testing and histopathological examination suggested that there were two types of acquired cholesteatoma formation, probably with a difference in the pathogenesis. In one type, the proliferated epidermal layer of the tympanic membrane penetrated into the middle ear cavity making tympanic perforations. In the other type, there was progressive retraction of the tympanic membrane forming a retraction pocket. We discuss the two different patterns of cholesteatoma development.     

Plasminogen activators in tissue extract of aural cholesteatoma

Using a biochemical technique, the authors characterized and identified the plasminogen activator (PA) derived from tissue extracts of six aural cholesteatomas. The results of fibrin zymography indicated that the tissue extracts of two cholesteatomas demonstrated two lytic zones on fibrin-agarose plates. One of the lytic zones was at about 72 kd, while the other zone was at about 64 kd. Using various goat immunoglobulin G (IgG)-containing antibodies (anti-human uterine tissue type PA (t-PA), anti-human low-molecular-weight (LMW) urokinase, and nonspecific goat IgG) and plasminogen-free fibrin-agarose plates, we confirmed that the cholesteatoma tissue extracts contained 72 kd t-PA and 64 kd urokinase type PA (u-PA). Furthermore, we measured the t-PA and u-PA activities in the tissue extracts selectively by parabolic rate assay. In order to estimate the PA activity, we developed optimal conditions for this assay. The specific t-PA activity ranged from 0.03 to 0.43 mIU/μg-protein and the specific u-PA activity ranged from 0 to 0.35 mIU/μg-protein. The highest percentage of u-PA with respect to the total PA activity was 44.9%. However, in four of the six cases, we failed to detect u-PA activity. In the present study, we thus clarified the presence of PAs in tissue extracts of aural cholesteatomas. Furthermore, we confirmed that measurable u-PA occurred in some tissue extracts. We anticipate that the u-PA in inflammatory tissues plays an important role in the degradation of the extracellular matrix via the formation of plasmin and collagenases.     

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.     

Results of conservative surgery for middle ear cholesteatoma

A retrospective study was carried out to analyze treatment results for cholesteatoma at the University of Iowa Hospitals and Clinics. All patients undergoing primary surgical treatment from January 1, 1969 to December 31, 1973 were followed through October 1976. Treatment failures were based on the occurrence of postoperative cholesteatoma. The probability of being disease free for periods up to five years after original surgery was then estimated for each type of operation. Postoperative cholesteatoma occurred after atticotomy in 17%, intact canal wall mastoidectomy in 35%, and modified radical mastoidectomy in 9%. The postoperative cholesteatoma rate in the group having intact canal wall mastoidectomy was more than twice as high for those patients under age nine. The likelihood of being disease free five years after intact canal wall surgery was estimated to be 36% using the life table method. Disease recurrence was significantly higher after intact canal wall surgery compared to other surgical methods. The effectiveness of this method should be evaluated in a randomized, prospective manner to minimize patient selection and treatment bias.     

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 cholesteatoma of the middle ear-a report of 10 cases

Objectives To study clinical, imaging features and treatment outcomes of congenital cholesteatoma of middle ear (CCME). Methods This is a retrospective review of 10 CCME cases selected from 952 cholesteatoma cases treated between January 1995 and December 2005 at the Department of Otolaryngology-Head and Neck Surgery, Chinese PLA General Hospital. The main outcome measures were the site of origin, clinical features, surgical findings, imaging characteristics and hearing results. Results The mean age of the 10 patients was 16 years(ranged from 10 to 24 years), with 6 being older than 18 years. There were 7 males and 3 females. The average delay to diagnosis was longer than 2 years. The mean preoperative PTA was 55 dB HL, with a mean ABG of 45 dB. Typical cholesteatomas were seen behind the tympanic membrane in the superoposterior quadrant on otoscopy only in 2 patients. High resolution CT was completed in all patients. Most of the patients(8/10) were diagnosed with otosclerosis or ossicular abnormality before operation. All patients underwent a one-stage tympanoplasty following transmeatal explorative tympanotomy and complete cholesteatoma removal, except one, who underwent a CWU mastoidectomy due to extensive cholesteatoma involvement. The choleasteatoma lesion was confined to the superoposterior mesotympanum in all patients. The mean postoperative PTA was 20 dB HL. All patients were followed-up for at least 1.5 years postoperatively. Revision procedures were performed in 2 patients for hearing deterioration. No residual or recurrence of cholesteatoma was found. Conclusion CCME is a rare disease that often gets delayed diagnosis. Residual lesions and the prognosis mainly depend on the extent of the lesion.     

Congenital aural atresia: Stability of surgical results

Objectives/Hypothesis: To compare early (<1 y) and longer-term (1.0–7.5 y; mean follow-up, 2.8 y) hearing results following surgery for congenital aural atresia and to analyze revision cases for cause of failure, time of occurrence, and outcome. Study Design: Retrospective study of 55 consecutive patients (59 ears) undergoing surgery during an 11-year period for congenital aural atresia. Methods: The best speech reception threshold (SRT) during the first postoperative year was compared with the most recent SRT beyond the first postoperative year. The complication rate and long-term hearing results (>1 y) for initial and revision surgeries were compared. Results: In the early postoperative period, an SRT of ≤25 dB was achieved in 60% and an SRT of ≤30 dB in 70%. With longer follow-up (mean, 2.8 y), 46% of patients maintained an SRT ±25 dB. Revision surgery was necessary in approximately one third of patients. Longer-term hearing results following initial surgery only or after revision surgery showed an SRT ±25 dB in 53% and an SRT ±30 dB in 64%. For primary and revisions surgeries the incidence of temporary facial paralysis was 1.5% and for significant hearing loss, 3.0%. Conclusions: Some degradation in hearing does occur as patients are followed beyond the first postoperative year. Revision surgery will be necessary in approximately one third of cases. With revisions, an SRT ±25 dB can be expected in half of cases and an SRT ±30 dB in two thirds of cases. The ability to provide these hearing results supports this surgery in unilateral cases.     

Aberrant expression of epidermal growth factor receptor in aural cholesteatoma

A monoclonal antibody recognizing an epitope of the external domain of the human epidermal growth factor (EGF) receptor was used in an alkaline phosphatase-antialkaline phosphatase (APAAP) technique to compare the distribution of this protein in normal human skin and aural cholesteatoma. EGF receptors appear to be highly expressed on the basal layer of the epidermis, in hair follicle apocrine sweat glands, and in the capillary system of normal skin. Cholesteatoma epithelium showed increased positive reactions in the suprabasal layers. A heterogeneity in the expression was found in different parts of the cholesteatoma. These results suggest the presence of an aberrant regulation and persistence of EGF receptors in cholesteatoma and confirm the hyperproliferative character of the cholesteatoma epithelium.     

Bone destruction due to the rupture of a cholesteatoma sac: A pathogenesis of bone destruction in aural cholesteatoma

Severe bone destruction in a cholesteatoma is one of the characteristic clinical features. To clarify the mechanism of bone destruction in cholesteatoma, the matrix of cholesteatoma and the attached bone, obtained during middle ear surgery, was observed by light microscope. Rupture of the epithelial lining in a cholesteatoma and the escaping contents (keratin), which gave rise to intense characteristic granulations in subepithelial tissue, were found. Furthermore bone destruction was always found at the site of subepithelial tissue of cholesteatoma. From these facts, the escape of contents from the sac of cholesteatoma into the subepithelial layer is considered to be an important factor in the mechanism of bone destruction.