Anterior and posterior middle ear congenital cholesteatomas in children.

Congenital cholesteatomas arise from embryonic epithelial cell rests in the middle ear cleft. We report our series of 19 cases observed in 18 children between 1985 and 1990. Clinical data and surgical observations are analyzed. In regard to the location of the lesions, our findings are consistent with most of what has been reported in the literature. Sixty-eight percent of the lesions originated from the anterosuperior part of the mesotympanum. Only 10% were posterosuperiorly located. Pathogenic mechanisms are discussed with respect to the lesion's primary site.     

Keratinization of middle ear cholesteatomas

Summary A histochemical study was performed to determine the involvement of epidermal transglutaminase (ETgase) in the keratinization of middle ear cholesteatomatous lesions, and to compare it with its role in the middle ear mucosa and epidermis. In a first assay, we localized the (E)Tgase activity in situ. A second immunohistochemical assay revealed the distribution of the particulate form of ETgase, which is involved in cross-linked envelope formation. A remarkable difference between strongly keratinized epidermal tissues and the cholesteatoma matrix is the frequent observation in the latter of the remnants of (E)Tgase activity in cytosol, even in advanced stages of differentiation. As a consequence, the cell-membrane-associated ETgase activity, and thus the extent of cross-linking within the envelope, is at a lower level than expected. This aspect is reminiscent of the keratinization phenomenon manifested by thin epidermal tissues. In addition, our findings are the first to show that ETgase is a substantial marker of middle ear mucosa.This paper is dedicated to the memory of Prof. E. Gillis, deceased 3 August 1988, aged 51. His guidance will be sorely missed by those who knew and worked with him.     

Keratinization of middle ear cholesteatomas

A histochemical study was performed to clarify further the role played by epidermal transglutaminase (ETgase) in the keratinization of aural cholesteatoma. Weakly and strongly keratinized epidermal tissues and healthy middle ear mucosa were included as references. A first assay revealed the distribution of non-specified acyl donor substrates. In a second assay, the topography of involucrin was assessed immunohistochemically. In both epidermal and cholesteatoma matrix tissues, the presence of acyl donors was not restricted to the sites of (E)Tgase activity, but was almost uniformly extended throughout living layers. In reference tissues, residual acyl donors were poorly detected in horny layers, while they were more abundant in the stratum corneum of the cholesteatomas studied. The presence of involucrin along the cell membrane was observed at varying distances throughout the spinous and granular layers, depending upon the epidermal and matrix configurations. In thick epithelia, involucrin rapidly became concentrated at the cell periphery (in spinous keratinocytes), while in thin epithelia it was usually associated with cell flattening. This latter staining profile was observed more frequently in cholesteatomatous tissues. In addition, we regularly noticed an immediately suprabasal accumulation of involucrin, suggesting a locally hyperproliferative state of the matrix. An insufficient availability of acyl donors, especially involucrin, could not be used to explain the defective ETgase-mediated cross-linking of cholesteatoma cell membranes during terminal stages of differentiation. The present investigation may be the first to demonstrate the presence of involucrin in middle ear mucosa.     

Bilateral congenital middle ear cholesteatomas

Congenital middle ear choleslcatomas remain of interest because of their relative rarity and unknown origin. A 4-month-old child presenting with bilateral congenital middle ear cholestcatomas forms the basis for this report. The nature of the epithelial debris found suggests an external origin. An extended transcanal tympanotomy approach for removal, based upon an operative experience of 11 similar ears, will be discussed.     

Congenital cholesteatoma of the middle ear in children

Congenital cholesteatoma, once considered to be a rarity, has shown an exponential increase in its incidence as evidenced by numerous clinical studies over the past decade. No explanation has been offered for this phenomenon. A review of the literature indicates that the definition of "congenital cholesteatoma" has strayed from the original one formulated by Derlacki and Clemis, which included a cholesteatoma medial to an intact tympanic membrane, without prior history of aural infections. Allowing patients with recurrent otitis media into studies may have contributed to a "false positive" rise in the incidence of this condition. This article reviews 19 cases of cholesteatomas behind intact tympanic membranes, treated at the Montreal Children's Hospital over a 10-year period. They represent 22% of the entire cholesteatoma population. There appears to be two groups of patients, each with a distinct clinical presentation that correlates well with the surgical outcome. Whether this implies different pathogenetic mechanisms remains unclear.     

Langerhans cells in human middle ear cholesteatomas

Summary Langerhans cells have been found in cholesteatomas for many years. It is believed that they are immunocompetent cells and have the same role in cell-mediated immunologic mechanisms in cholesteatoma as well as in skin. This study used the transmission electron microscope to observe the cellular characteristics of Langerhans cells and the apposition phenomenon of Langerhans cells with lymphocyte-like cells in human middle ear cholesteatomatous tissue. These findings are evidence for cell-mediated immune responses in middle ear cholesteatomas. In vitro Langerhans cells conditioned medium prepared from Lewis rat skin was used to show its effects on protein synthesis and the differentiation of basal cells. Since the cellular behaviour of basal cells is important in the development and pathogenesis of cholesteatoma, the present study shows that Langerhans cells may have some role in the clinical formation of a cholesteatoma. Since cells extracted from rat skin may have a different response from that of cells from human middle ear cholesteatoma, further investigations are necessary to compare the biological effects of both tissues. Correspondence to: W.-Y. Chao     

Congenital and acquired cholesteatoma middle ear in children

Cholesteatoma of the middle ear in children may cause hypoacusis. Early diagnosis and optimal treatment is neccessary for good functional effect. We present 57 children (58 ears) with cholesteatoma treated in ENT Department of Medical University in Gdańsk in 1991-2002. The age of patients ranged between 3 and 16 years, the most common 11-15 years. In 52 (89.6%) cases acquired cholesteatoma and in 6 (10.4%) congenital cholesteatoma was diagnosed. Epitympanal cholesteatoma was found in 32 children (55.1%) whereas in posterior part of tympanic cavity--in 20 children (34.5%). In 6 cases (10.4%) intact tympanic membrane was found. Mean air-bone gap in acquired cholesteatoma before treatment was 18.7 dB, after treatment 15.7 dB. In congenital cholesteatoma mean air-bone gap before treatment was 13.6 dB, after treatment 14 dB. The most frequent symptom was hearing loss (98.3%) and purulent otorrhea (85.4%). Positive bacteriological culture was obtained in 43.1% of the cases. X-ray revealed sclero-pneumatic mastoid in 26 (34.5%) cases, sclerotic in 25 (43.1%) and pneumatic mastoid in 7 (12.1%) cases. Intracranial complications were found in 2 cases, intratemporal in 2 cases and extracranial complication in 1 case. Radical surgery was performed in 23 cases (39.7%) and in each case, which required reoperation, modified radical mastoidectomy in 20 cases (34.4%), in another 15 children (25.9%) tympanoplasty was done. The most frequent failure was purulent otorrhea in 21 (36.2%) cases. Reoperation in cholesteatoma recurrence was performed in 17 children (29.3%). Improvement or the same as preoperatively hearing level was obtained in 35 (60.3%) ears, hearing loss was revealed in 23 (39.7%) ears. Treatment of temporal bone cholesteatoma in children is difficult due to silent beginning, aggressive growth and frequent recurrence. The best treatment results in children cholesteatoma are obtained in early clinical stage and with open tympanoplasty procedure.     

Congenital cholesteatoma of the middle ear in children]

In a series of 16 middle ear cholesteatomas of a congenital type are reported in children; the youngest (18 months) presented with a bilateral case. Whereas a simple tympanoplasty could cure a localized pearl, typically anterosuperior in the mesotympanum, the stapes is fast eroded (7 cases) if progression goes on. Intact canal wall technique in 2 stages was the typical procedure. Good hearing results were generally achieved (except in one case of fixed footplate): 9 cases/14 with an ABG within 20 dB and an AC level within 30 dB.     

Congenital cholesteatomas in children: an embryologic correlation

The clinical findings in 37 children with congenital cholesteatoma of the middle ear, 17 of which have not been previously reported, are presented. Clinical findings and surgical observations are correlated with recent developmental studies. It is hypothesized that congenital cholesteatoma may originate from an epidermoid formation, which has been identified in the anterior superior lateral tympanic cavity adjacent to the anterior annulus during fetal development, and which normally is present early in development, involuting by 33 weeks' gestation. It is proposed that the epidermoid formation may not always involute, and could serve as an embryologic anlage of congenital cholesteatomas.     

Stromal expression of matrix metalloprotease-9 in middle ear cholesteatomas

BACKGROUND: Matrix metalloprotease-9 (MMP-9) plays an important role in the degradation of extracellular matrix (ECM). In cholesteatoma there are significant changes in the composition of the ECM, supporting the view of a disturbed cell-ECM interaction. It has been reported that in cholesteatoma tissues, MMP-9 is expressed in epithelial cells but not in stromal cells. In this study, we report conflicting results regarding the identity of the cholesteatoma cells that express MMP-9. METHODS: An immunohistochemical assay was performed to examine the expression of MMP-9 in 25 cases (formalin-fixed paraffin-embedded sections) of cholesteatoma, using an MMP-9-specific monoclonal antibody. RESULTS: In all cholesteatomas examined, MMP-9 was expressed in the stromal cells but not in the epithelial cells. MMP-9 immunostaining was undetectable in external auditory meatal skin (n = 5) and tympanic membrane (n = 3). CONCLUSIONS: These results suggest that the MMP-9 expressed in stromal cells may be involved in the disturbed cell-ECM interaction associated with the process of cholesteatoma formation.     

Overexpression of ErbB-2 protein in human middle ear cholesteatomas

OBJECTIVE: The purpose of this study is to verify the hypothesis that ErbB-2 protein is overexpressed in human middle ear cholesteatomas and to elucidate the relationship between overexpression of ErbB-2 protein, cell proliferation, and apoptosis. STUDY DESIGN: Prospective review of 20 patients between 2001 and 2003 with middle ear cholesteatoma. METHODS: Middle ear cholesteatoma matrix and retroauricular skin were immunostained with anti-ErbB-2, Ki-67, and single-stranded DNA (ssDNA) antibody. The distribution of immunoreactivity to these antibodies and labeling indices were compared between cholesteatoma and retroauricular skin. RESULTS: In matrix of middle ear cholesteatoma, ErbB-2 and ssDNA were expressed in the keratinocytes of all layers and Ki-67 was expressed in the keratinocytes of the basal, lower spinous, and occasionally granular layer. In retroauricular skin, ErbB-2 and Ki-67 were expressed in the keratinocytes of the basal and occasionally lower spinous layer and ssDNA was expressed in the keratinocytes of all layers. Labeling indices against anti-ErbB-2, Ki-67, and ssDNA antibody were significantly greater in cholesteatoma as compared with retroauricular skin. CONCLUSIONS: In cases of cholesteatoma, ErbB-2 protein was overexpressed and cell proliferation and apoptosis of keratinocytes were accelerated. ErbB-2 protein could modulate terminal differentiation and apoptosis in the keratinocytes of all layers in cholesteatoma matrix and cell proliferation in the keratinocytes of the basal and lower spinous layer in normal skin.     

Congenital cholesteatoma of the middle ear in children: A clinical and histopathological report

Forty-one children with congenital cholesteatoma of the middle ear seen from 1978 through 1989 are reviewed. The most common presentation was that of an asymptomatic white mass behind a normal intact tympanic membrane. Computed tomography (CT) scan was useful in documenting extension beyond the mesotympanum. Surgical removal was performed using an extended tympanotomy for lesions in the middle ear and tympanomastoidectomy for those that had extended into attic and mas-toid air cells. Observation over an average 3.1-year period indicated that 80% of children were free of disease after initial surgery. Residual disease that required further surgery was present in 20%. The importance of early diagnosis of congenital cholesteatoma is strongly advocated. The prognosis is better when the cholesteatoma is confined to the anterosuperior quadrant of the middle ear. Seventeen patients in this study had such a lesion, and extended tympanotomy allowed removal of an encapsulated closed cholesteatoma with normal postoperative hearing and no residual cholesteatoma. The average age was 2.3 years. Temporal bone histopathological studies of three cases of congenital cholesteatoma demonstrate two distinct pathological types of congenital cholesteatoma. A “closed” keratotic cyst in the anterior mesotympanum, which is easily removed, and an “open” infiltrative type in which there is no containment of the keratotic debris and the cholesteatoma matrix is in direct continuity with middle ear mu-cosa. Surgical extirpation of the “open” type is difficult and more likely to be associated with residual disease.     

Symposium: Congenital anomalies of the middle ear. III. Congenital anomalies of the middle ear.

Congenital anomalies of the middle ear have been considered rare. The tremendous volume of middle ear surgery performed during the last 15 years has brought attention to numerous anomalies of the facial nerve, ossicles, and middle ear cleft, suggesting that middle ear anomalies are not as rare as once believed. This paper discusses anomalies of the descending and horizontal facial nerve which have been previously reported. The literature of anomalies of the ossicles is reviewed. A very rare anomaly of the carotid artery presenting in the middle ear is described and its surgical treatment outlined. Two anomalies of the middle ear associated with other branchial arch anomalies are reported and their treatment results discussed.     

先天性中耳畸形

先天性中耳畸形是耳科常见畸形之一,发病率为1/15 000,男女比例为3:1,单侧比双侧多3～5倍,以右侧多见[1].近年来随耳显微外科的广泛开展和颞骨高分辨率CT(high resolution computed tomography,HRCT)的应用,国内外有关报道日益增多.我们就先天性中耳畸形作一综述.