Colesteatoma congénito: revisión de la literatura / Congenital cholesteatoma: literature review

El colesteatoma congénito es una entidad clínica única y desafiante, que se caracteriza por acumulación anormal de queratina en el oído medio, medial a la membrana timpánica. Se presenta, mayoritariamente, en el género masculino, con una incidencia estimada de 0.12 por 100.000 habitantes, representando el 4% a 24% de los colesteatomas en población pediátrica y un 2% a 5% del total de colesteatomas. Su origen aún es controversial, siendo la teoría más aceptada, la del arresto epitelial. Su diagnóstico es clínico, variando la sintomatología según severidad del compromiso, presentándose desde hallazgo incidental, hipoacusia de conducción, hasta presentar otalgia y perforación timpánica. Las imágenes se consideran un apoyo complementario preoperatorio. El tratamiento es quirúrgico, con diferentes técnicas disponibles, las cuales se deben definir de manera individual en el caso de cada paciente. Es fundamental su diagnóstico y manejo precoz, para lograr un tratamiento oportuno con menor tasa de complicaciones y compromiso a largo plazo. A continuación, se presenta una revisión de la literatura respecto de esta patología, para difusión en nuestro medio.

Congenital cholesteatoma (CC) is a unique and challenging clinical entity characterized by abnormal accumulation of keratin in the middle ear, medial to the tympanic membrane, being more frequent in the male gender, with an estimated incidence of 0.12 per 100,000 inhabitants. It represents 4% to 24% of cholesteatomas in the pediatric population and 2% to 5% of all cholesteatomas. Its cause is still controversial, the most accepted theory being epithelial arrest. The diagnosis is clinical, varying the symptoms according to the severity of the compromise, from incidental finding, through conduction hearing loss, to presenting otalgia and tympanic perforation. Images are considered additional preoperative support. Treatment is predominantly surgical, with different techniques available, which must be defined individually. Its early discovery and management are essential to achieve proper treatment with a lower rate of complications and long-term commitment. We present a review of the literature regarding CC to provide information relevant to our area of expertise.

A case report of middle ear cholesteatoma complicated with labyrinthine fistulaand delayed endolymphatic hydrops / 临床耳鼻咽喉头颈外科杂志

Delayed endolymphatic hydrops （DEH） is a rare disease that causes vertigo and is often misdiagnosed as other vertigo diseases. This article reports on a patient with vertigo who was easily misdiagnosed. The patient was a middle ear cholesteatoma complicated by labyrinthine fistula （LF）; however, his vertigo was episodic vertigo, which could not be explained solely by LF causing labyrinthitis. The possibility of endolymphatic hydrops was suspected, which was confirmed by inner ear magnetic resonance gadolinium imaging. This is the first reported case of middle ear cholesteatoma complicated by LF and DEH. The patient underwent surgical resection of the cholesteatoma and three semicircular canal obstructions at the same time. During two years postoperative follow-up, the patient did not experience a recurrence of vertigo. When diagnosing vertigo diseases, a careful history of vertigo is of utmost importance.

The Analysis of Postoperative Curative Effects of Facial Paralysis Caused by Middle Ear Cholesteatoma / 听力学及言语疾病杂志

Objective To study the effects of the duration and degree of the peripheral facial paralysis due to middle ear cholesteatoma on the recovery of postoperative nerve functions.Methods The clinical data of 30 cases (single ear) of patients with cholesteatoma complicated with facial paralysis were retrospectively analyzed.The mean age was 54.7±8.9 years old (range 21~77years old) including 17 males and 13 females.The duration of facial paralysis, degree and location of facial nerve injury,surgical timing of facial nerve decompression,recovery of facial paralysis after surgery were followed up.According to the course of paralysis, the patients were divided into ≤2 months group (14 cases) and >2 months group (16 cases).According to the degree of paralysis, the patients were divided into the incomplete facial paralysis group (III-IV grade,14 cases) and the complete facial paralysis group (V-VI grade,16 cases).The influence of the course and degree of paralysis on the postoperative recovery of neurological function were analyzed by the Fisher exact probability test.Results Three cases underwent open radical surgery with no facial nerve damage confirmed by intraoperative exploration, and their postoperative facial nerve functions were fully restored.Facial nerve canal damage was found in 27 cases, accompanied by facial nerve congestion, edema or granulation formation.Among them, the facial nerve damage location was the tympanic segment in 20 cases.The open radical operation and local facial nerve decompression were carried out, and the postoperative facial paralysis recovery rate was 46.67%(14/30).The facial nerve function recovery rate in less than 2 months group was higher than the >2 months group (P<0.05).The facial nerve function recovery rate of the incomplete paralysis group was higher than the complete facial paralysis group (P<0.05).Conclusion This study suggests that facial nerve damage most occurred in the tympanic segment of the facial nerve.Radical mastoidectomy and local facial nerve decompression are effective for the treatment of middle ear cholesteatoma complicated with facial paralysis.The shorter course and lighter degree of paralysis lead to the better postoperative recovery of neurological function.

Epitympanoplasty with Cartilage Obliteration in the Canal Wall Up Technique to the Middle Ear Cholesteatoma / 听力学及言语疾病杂志

Objective To report the curative effect of the epitympanoplasty with cartilage obliteration in the treatment of middle ear cholesteatoma .Methods This study retrospectively analysed 123 cases ,with middle ear cholesteatoma treated in our department from November 2009 to July 2012 .The operation included mastoidectomy with posterior canal wall preserved ,lateral wall of attic resected ,facial recess opened to the epitympanum ,epitym-panum obliterated with cartilage and mastoid cavity blocked with bone dust .All cases were followed up on complica-tions and hearing improvement .Results All cases were followed up 6~38 months .In 123 cases ,there were 2 ca-ses had cholesteatoma recurrence in the middle ear cavity ,6 cases had infection of post auricular incision after opera-tion ,3 cases had residual marginal tympanic membrane perforation ,2 cases had ossicular replacement prosthesis ex-clusion ,and 6 cases had canal wall skin swelling or defect .The rest cases acquired full -recovery after 2 or 3 wound dressing changes .Out of 123 cases ,83 cases had the whole hearing document ,the average AB gap at 0 .5 ,1 ,2 kHz reduced from 32 .2 ± 11 .2 dB pre-operation to 20 .7 ± 12 .4 dB post -operation .The AB gap of pre - and post -operation had significant difference (P<0 .001) .Conclusion The mastoidectomy with posterior canal wall preserved greatly shortened the healing time .The resection of lateral wall of epitympanum can exposed operating field more completely and remove lesions thoroughly .The cartilage obliteration in the attic can prevent the retraction pocket formation effectively .This technique provides alternative in treating middle ear cholesteatoma .

Galectin-1, -3, -7 Expressions in Congenital and Acquired Pediatric Cholesteatomas Compared to External Auditory Canal Skin

OBJECTIVES: There is a classical distinction based on clinical criteria between acquired and congenital cholesteatomas. To determine if these two types of lesions show different immunohistochemical features, we have studied the expression patterns of three distinctive galectins (animal lectins implied especially in cellular proliferation and apoptosis) in both types of cholesteatomas and compared it to their expression patterns in external auditory canal skin. METHODS: Our study is based on nine acquired and eight congenital cholesteatomas, obtained from children during ear surgery. Six specimens of normal adult auditory meatal skin served as control. Specimens were analyzed by immunohistochemistry using monoclonal antibodies with galectin-1 and galectin-3, and a polyclonal antibody with galectin-7. RESULTS: We did not observe any differences in the galectin distribution pattern between congenital and acquired pediatric cholesteatomas. Compared to the control group, cholesteatomas present some particular features. There was no expression of galectin-1 and a lower expression of galectin-3 in the epithelium. Furthermore, we observed a preferentially nuclear distribution of galectin-7 in cholesteatomas, whereas it is essentially cytoplasmic in the control group. CONCLUSION: The data reported in this study suggest, on the basis of a lesser marked galectin-3 in cholesteatomas epithelium compared with an external auditory canal skin, that an immature keratinocytes population is at the origin of these lesions and that galectin-3 and galectin-7 play a part in the capacity as apoptosis modulators. Our study does not establish a difference in the galectin expressions of congenital and acquired cholesteatomas, but it constitutes however an additional argument in favor of the "undifferentiated" origin of keratinocytes in cholesteatomas.

Achados bacterianos encontrados na secreção da otite média cronica: estudo comparativo entre o colesteatoma (OMCC) e a otite média cronica simples (OMCS) / Bacterial findings found in the chronic otitis media secretion: comparative study between cholesteatoma (OMCC) and simple chronic otitis media (SCOM)

Introdução: Foi feita uma comparação na frequencia com que os patogenos infectantes foram detectados na supuração causada pelo colesteatoma e pela otite crônica simples no período de 2006 a 2008. Objetivo: Fazer um estudo comparativo entre os achados bacterianos encontrados na secreção da otite crônica simples e a colesteatomatosa. Método: Foram estudados a bacterioscopia de 83 pacientes (125 orelhas) portadores de otite média crônica, sendo 43 (52 orelhas) com colesteatoma e 40 (73 orelhas) com otite crônica simples, com predominância de idade dos 16 aos 20 anos. A duração da otorreia variou entre 2 meses e 10 anos. Para a colheita do material utilizamos um equipamento bastante pratico com caldo de tioglicolato dentro e fora enviados ao laboratório por um período máximo de até 18 horas. Resultados: O S. aureus foi mais frequente na otite crônica simples, enquanto que os anaeróbios foram mais frequentes no colesteatoma. A P. aeruginosa foi mais frequente na otite crônica simples e o Corynebacterium sp. apresentou maior frequencia no colesteatoma. o S. epidermidis apareceu com frequencias iguais em ambas as doenças otológicas. Conclusão: Não encontramos mudanças notáveis na bacteriologia dessas duas doenças. Na otite crônica simples os achados mais frequentes foram S. aureus, Pseudomonas sp. e fungos. No colesteatoma os achados mais frequentes foram os Anaeróbios e o Corynibacterium sp. A frequencia de aparecimento para S. epidermidis, Klebisiela sp. e Streptococcus sp. foi igual em nosso estudo.

Introduction: This study carried out a comparison in the frequency with which the infecting pathogens were detected in the suppuration caused by cholesteatoma and simple chronic otitis media in the period from 2006 to 2008. Objective: To carry out a comparative study between the bacterial findings found in the simple and cholesteatomatous chronic otitis media secretion. Method: We studied the bacterioscopy of 83 patients (125 ears) with chronic otitis media, 43 (52 ears) with cholesteatoma and 40 (73 ears) with simple chronic otitis, and age prevalence from 16 to 20 years. The duration of otorrhea ranged between 2 months and 10 years. For collection of the material we used very practical instrument with tioglicolate broth inside and outside sent to the laboratory for a maximum period of until 18 hours. Results: The S. aureus was more frequent in the simple chronic otitis, and the anaerobic were more frequent in the cholesteatoma. The P. aeruginosa was more frequent in the simple chronic otitis and the Corynebacterium sp. presented a higher frequency in cholesteatoma. The S. epidermidis appeared with the same frequencies in both otologic diseases. Conclusion: We did not find any critical changes in the bacteriology of either disease. In the simple chronic otitis, the most frequent findings were S. aureus, Pseudomonas sp. and fungi. In the cholesteatoma, the most frequent findings were the Anaerobios and Corynibacterium sp. The frequency of S. epidermidis, Klebisiela sp. and Streptococcus sp. was the same in our study.

Efeito do ácido trans-retinóico na inibição de colesteatoma em cobaias / Effect of trans-retinoic acid in the inhibition of cholesteatoma in guinea pigs

O colesteatoma de orelha média atingia mais de 5 milhões de pessoas até a década de 80. Vários modelos animais já foram utilizados para alternativas de tratamento do colesteatoma sem sucesso. OBJETIVO: Estudar os efeitos do ácido trans-retinóico, uso tópico na orelha externa em cobaias, na inibição da formação do colesteatoma de orelha média induzido pelo propilenoglicol. Estudo experimental prospectivo. MATERIAL E MÉTODOS: 25 cobaias foram submetidas à aplicação de propilenoglicol a 100 por cento na bula timpânica bilateralmente e uma solução de ácido trans-retinóico foi aplicada topicamente (total de 5 aplicações) na orelha externa, região justa-timpânica, na orelha direita, enquanto na orelha esquerda aplicou-se solução fisiológica (orelha controle). As cobaias foram sacrificadas após 6 semanas do procedimento inicial e os ossos temporais foram separados, fixados e descalcificados, para análise macroscópica e histológica. RESULTADOS: Os achados macroscópicos evidenciaram a presença e suspeita de colesteatoma em 25 por cento das orelhas direitas e 85 por cento das orelhas esquerdas (P=0,0003*). Os achados histológicos dos 40 ossos temporais evidenciaram a presença de colesteatoma em 30 por cento das orelhas direitas e 75 por cento das orelhas esquerdas (P=0,0104*). CONCLUSÃO: O uso tópico do ácido trans-retinóico é efetivo na inibição da formação de colesteatoma induzido pelo propilenoglicol em cobaias.

Middle ear cholesteatoma affected more than 5 million people until the 80`s. Many animal models were used, unsuccessfully, to study an alternative therapy to cholesteatoma. AIM: observe the effect of the trans-retinoic acid in the inhibition of middle ear cholesteatomas induced by propylene glycol. STUDY DESIGN: Clinical and Experimental. METHODS: 25 guinea pigs were submitted to the application of a 100 percent propylene glycol solution in their bulla bilaterally and a solution of trans-retinoic acid was applied locally in the external right ear, while in the left ear saline solution was applied (control ear). The guinea pigs were slaughtered and their temporal bones were prepared for macroscopic and histological analysis. RESULTS: The macroscopic findings had evidenced the presence of cholesteatoma in 25 percent of the right ears and 85 percent of the left ears (P=0.0003 *). The histological study had evidenced the presence of cholesteatoma in 30 percent of right ears and 75 percent of the left ears (P=0.0104*). CONCLUSION: The local use of the trans-retinoic acid is effective in inhibiting the induced formation of cholesteatomas in guinea pigs.

Congenital Middle Ear Cholesteatoma in Children / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a rare entity that may go undiagnosed for years. Aims of this study were to assess the characteristic features and recurrence of CMEC in pediatric patients of different stages and to determine the value of preoperative CT scan in CMEC. SUBJECTS AND METHOD: Thirty cases of CMEC under 15 years old that had been treated at the hospitals of the Catholic University from 1995 through 2005 were reviewed retrospectively. The age range was from 2 to 13 with the mean age of 6.2. The main outcome measures were CT findings, surgical findings, recurrence rate and hearing assessment. RESULTS: Preoperative CT scan accurately predicted the extent of the cholesteatoma seen during surgery in 25/30 (83.3%). The recurrence rate of CMEC was 6.7% (2/30) and all of recurrent cases were belonged to stage IV. In the recurrent cases, cholesteatomas were extended to sinus tympani and facial recess at revisional operation as well as at the initial operation. CONCLUSION: Preoperative CT scan is essential in defining the extent of existing pathology. The intra-operative CMEC extension and location influence the outcome of surgery. In the higher stages, careful eradication of disease, particularly in the region of sinus tympani and facial recess, are recommended.

Congenital Middle Ear Cholesteatoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a keratinous mass behind an intact tympanic membrane. CMEC does not have a history of instrumentation and is less common than acquired one. Many theories have been put forward to explain the pathophysiology of CMEC, however, none of these so far have been convincingly proven. This clinical study was performed to investigate the characteristic features of CMEC and to evaluate the correlation between pathophysiology and CMEC by retrospectivly reviewing the cases. MATERIALS AND METHOD: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic university from January 1993 to September 1998 have been reviewed. They were ten males and four females, ranging in age from 4 to 59 (mean age 18). RESULTS: Three of the 14 patients had the lesions isolated to the anterosuperior quadrant of the mesotympanum which were cystic, easily removed and did not affect hearing. The others had more serious condition with extension into the posterior mesotympanum, which were large, often too extensive to indicate a formative site, and causing ossicular damage. CONCLUSION: CMEC presents in two distinctive forms according to the site of formation: the anterosuperior and posterior mesotympanum. The review suggest that the pathophysiology of posterior lesions may be different from anterior ones. For early diagnosis of CMEC, screening program should be carried out in children to prevent the more extensive diseases.

Expression of Transforming Growth Factor-Beta(TGF-beta) in Middle Ear Cholesteatoma / 대한이비인후과학회지

The role of transforming growth factor-beta(TGF-beta) in the proliferation and progression of the epithelium of middle ear cholesteatoma was studied. We eluciated the site and degree of expression of the TGF-beta mRNA in middle ear choleasteatoma using in situ hybridization method and compared with that in epidermis of external auditory canal skin, cholesterol granuloma and cervical lymph node. TGF-beta mRNA was intensely expressed in lymphatic cells of cervical lymph nodes while canal skin and cholesterol granuloma did not express TGF-beta mRNA. Expression of TGF-beta mRNA was detected in the basal layer of epithelium of cholesteatoma in 12(83%) and in all layer of epithelium of cholesteatoma in 7(50%) of 14 samples even though it was more intense in the basal layer of epithelium. The results of the present study suggest that TGF-beta may play a role of immunoregulator in a mechanism of epithelial basal cell proliferation in middle ear cholesteatoma.