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Cholesteatoma surgery: residual and recurrent disease. A review of 1,024 cases.

This is an analysis of 1,024 primary cases of mastoid surgery for cholesteatoma operated upon during a ten-year period at the Otologic Medical Group, Inc. Our philosophy of management of the mastoid in these cases has been as follows: 1) avoid an open mastoid cavity when possible; 2) perform the operation in two stages if necessary; 3) reexplore the mastoid and middle ear for residual cholesteatoma when indicated. One-third of 380 revised cases had residual cholesteatoma, disease left by the surgeon. In 260 cases in which the surgeon felt it unlikely that there was residual disease he found it in 23%. In 4% this residual cholesteatoma was found in the mastoid. The incidence of residual cholesteatoma was higher in children and in planned, as opposed to unplanned, revisions. Residual cholesteatoma was detected in the middle ear more frequently than in the epitympanum, and in the epitympanum more frequently than the mastoid. Indications for, and timing of, the reexploration are discussed. Recurrent cholesteatoma refers to a retraction pocket and must be differentiated from residual cholesteatoma; the causes, prevention and treatment are different. Recurrent cholesteatoma was detected in 5%. The most common complication of the disease was a labyrinthine fistula (10%). Operative facial nerve damage occurred in one case. The most common postoperative complication was graft failure (3%). Intact canal wall tympanoplasty with mastoidectomy should be performed as a two-stage procedure in most cases when used in the treatment of aural cholesteatoma.

Adolescent

Identification of miRNA expression profile in middle ear cholesteatoma using small RNA-sequencing.

BACKGROUND: The present study aims to identify the differential miRNA expression profile in middle ear cholesteatoma and explore their potential roles in its pathogenesis. METHODS: Cholesteatoma and matched normal retroauricular skin tissue samples were collected from patients diagnosed with acquired middle ear cholesteatoma. The miRNA expression profiling was performed using small RNA sequencing, which further validated by quantitative real-time PCR (qRT-PCR). Target genes of differentially expressed miRNAs in cholesteatoma were predicted. The interaction network of 5 most significantly differentially expressed miRNAs was visualized using Cytoscape. Further Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genome (KEGG) pathway enrichment analyses were processed to investigate the biological functions of miRNAs in cholesteatoma. RESULTS: The miRNA expression profile revealed 121 significantly differentially expressed miRNAs in cholesteatoma compared to normal skin tissues, with 56 upregulated and 65 downregulated. GO and KEGG pathway enrichment analyses suggested their significant roles in the pathogenesis of cholesteatoma. The interaction network of the the 2 most upregulated (hsa-miR-21-5p and hsa-miR-142-5p) and 3 most downregulated (hsa-miR-508-3p, hsa-miR-509-3p and hsa-miR-211-5p) miRNAs identified TGFBR2, MBNL1, and NFAT5 as potential key target genes in middle ear cholesteatoma. CONCLUSIONS: This study provides a comprehensive miRNA expression profile in middle ear cholesteatoma, which may aid in identifying therapeutic targets for its management.

Humans

[Operative therapy for chronic otitis media and middle ear cholesteatoma with preservation of the posterior auditory canal wall ("intact wall technique") (author's transl)].

The late results of two different procedures for middle ear surgery are compared 2-10 years following surgery. A total of 478 ears were operated, of which 367 had cholesteatomas and 111 chronic granulating otitis without cholesteatoma. a) In conservative radical operations, tympanoplasties, meatoplasties with fascia and obliteration with muscle (298 ears) gave satisfactory and stable results: 8% required re-operation, 2.8% had residual cholesteatoma, 0.9% had recurrent cholesteatoma, 15% had perforations, and 90% had dry operative cavities. b) In modified "Intact Wall Technique" with small cortical mastoidectomies and epitympanic control openings, 7% had residual cholesteatomas, 2% recurrent cholesteatomas, and 11% required re-operation. The following conclusions were made: The "Intact Wall Technique" is a safe method for the surgical management of granulating otitis media. However, the procedure is not considered to be safe when disease involves cholesteatoma because of the significant frequency of recurrent disease or the development of retractions which can then form cholesteatoma. Under such circumstances, combination of the "Intact Wall Technique" with obliteration may give more stable results.

Cholesteatoma

Congenital middle ear cholesteatoma: two unusual cases and a review of the literature.

Forty-three patients with congenital middle ear cholesteatoma have been described since the first case in 1953. In these patients ten cholesteatomas were confined to the anterior mesotympanum in young children who had no ossicular damage. Involvement of the posterior mesotympanum (8 ears), meso and epitympanum (21 ears), and antrum and middle ear (7 ears) was associated with an 81% incidence of ossicular erosion in addition to other abnormalities. Anterior mesotympanic cholesteatomas were readily identified by the appearance of a white mass behind a translucent drum. The other congenital cholesteatomas had a more varied appearance with a mass and a whitish appearance each noted in about half. In almost half there was a bulging of the TM. Two new cases are reported: a 4-year-old with an extensive epi and mesotympanic cholesteatoma initially misdiagnosed as non-suppurative otitis media and a 23-year-old with the third reported case of bilateral congenital cholesteatoma.

Adolescent

Cholesteatoma in children.

The 65 cholesteatomas operated on in children showed a more expansive and rapid growth than those in adults. In one fifth of the cases cholesteatoma filled the whole air-cell area, which was wide in half of the children. Fifty-two ears of these children had an attic or a posterosuperior perforation. One case was complicated by a fistula in the horizontal semicircular canal, and the ossicular chain was unbroken in 23 cases (35%). Thus, the findings support the idea of the primary soft-tissue spread of cholesteatoma in children. Five ears (8%) discharged postoperatively, and three ears (5%) were reoperated on and showed residual tympanal cholesteatoma. Cavity obliteration with canal wall down technique proved safe, even in the cases of the most extensive and active cholesteatoma.

Adolescent

Management of otitic cholesteatomas.

When otitic cholesteatomas are removed by a surgical method that preserves or reconstructs the bony canal wall and tympanic membranes, there is a risk of imcomplete removal and possible regrowth of the lesion. To study the incidence of this complication, 52 patients with cholesteatoma had elective repeaated explorations at various postoperative intervals; eight (15.4%) had early cholesteatoma regrowth even though there was no clinical evidence of it. Seven of these regrowths were small and were again srugically removed. It is advocated that conservative surgery for cholesteatoma be followed up in two years by another elective surgical exploration to check for and dispose of possible cholesteatoma regrowth. This would provide safety comparable to that achieved with radical mastoidectomy, with far better function and hearing. The method does necessitate patient understanding and cooperation and I suggest methods of achieving this.

Aged

Growth rate and recurrence of residual epidermoid cholesteatoma after tympanoplasty.

One hundred and forty-one consecutive patients with chronic otitis media and cholesteatoma treated mainly by open surgical techniques between 1965 and 1972 were studied to investigate the phenomenon of residual cholesteatoma. The average rate of emergence of residual cholesteatoma was 0.25% of the cases per month during the first 5 years, after which the rate of discovery almost ceased. Observed cumulative frequencies of recurrence were 1.4% at 10 months, 14.9% at 5 years and 17.0% at 8.3 years postoperatively. The null hypothesis of no apparent predilection of cholesteatoma for recurrence at any one site was retained. Parameters of growth rates of epidermoid cysts arising from residual cholesteatoma were estimated. Epitympanic cysts grew fairly rapidly, doubling in diameter every 10 months. Mastoid cysts were slower, doubling in diameter every 25 months. Results for the mesotympanum were inconclusive. The practical implications of this information for the management of future cases is discussed.

Cholesteatoma

Intact canal wall tympanoplasty in the management of cholesteatoma.

There are presently two schools of thought on the proper surgical management of cholesteatoma associated with chronic ear disease: one feels that the ear should be exteriorized and left "open"; the other takes the position that the disease can be removed, and the ear left in its normal anatomical and functional state. The latter group are known to favor a "closed" or more conservative procedure. The purpose of this paper is to review a series of patients who have had cholesteatoma managed by an intact canal wall procedure that prevents a postoperative cavity. Out of a series of 590 chronic ear surgeries performed between January 1, 1970, and December 31, 1974, there were 179 done for cholesteatoma (30 percent). There were 153 patients, 26 of whom had bilateral disease. Twenty-three patients were lost to follow-up, giving an overall total of 154 surgeries with from one to five-year information. The overall follow-up rate was 85 percent. Recurrent cholesteatoma was the most bothersome complication and occurred in 14 percent of the series. Residual cholesteatoma was managed by doing all procedures in two stages. The authors feel that the intact canal wall tympanoplasty is a procedure that will gain acceptance and will be more widely used in the future.

Adolescent

[Residual-and recurrent cholesteatoma at preservation of the external auditory bony canal (author's transl)].

During the last four years we controlled 351 operations in order to check the indication of preservation of the posterior canal wall on patients with cholesteatoma. The aim of this study was to make a comparison between operative technique and the frequency of residual and recurrent diseases. It has shown that the consistent reconstruction of the lateral attic wall by endogenous conchal cartilage reduces the number of recurrence on a level of 3%. Since we haven't got yet a follow-up period of five years for all patients, the percentage of recurrent cholesteatomas was forecasted with 16% from the sample of patients with a follow-up period of two years, mixed with and without reconstruction of the lateral attic wall. The percentage of residual cholesteatoma was calculated with 25% from 104 revision operations. In future, a planned revision operation seems to be necessary for each patient with a posterior canal wall being preserved in cholesteatoma surgery. Osteoplastic management of the bony canal wall should lower the rate of residual cholesteatoma.

Bone Conduction

[Paralayrinthine cholesteatoma and tympanoplasty (author's transl)].

Closed tympanoplasty techniques greatly increase the risk of recurrent cholesteatoma. This is obvious as many surgeons using these methods insist on a necessary obligatory control operation as a "second look". In this paper cholesteatomata are classified according to their tendency to extend within the petrous pyramid and the subsequent danger of invading semicircular canals, cochlea or cranial fossae. The predisposition to this type of growth is present in the well defined "medial" type of epitympanic cholesteatoma, and more in the anteriorly than the posteriorly placed ones. As infection becomes less prominent in countries with advanced medical services, especially otology, the cholesteatoma hidden behind a small dry epitympanic perforation and those even with no perforation termed "hidden primary cholesteatoma" become more frequent. They cause their own special symptomatology, not infrequently a slowly progressive facial palsy. Every cholesteatoma requires mandatory prophylactic surgery. This means the complete exposure of the danger area, the epitympanum, and nowadays the possibility of the syncronous complete reconstruction of a normal middle ear and external canal, i.e. osteoplastic epitympanotomy.

Cholesteatoma

External auditory canal cholesteatoma.

Cholesteatoma of the EAC is a rare otologic problem, particularly when keratosis obturans otica is excluded. The predominant features of EAC cholesteatoma are acute external symptoms, severe pain, recurrent physician visits, and paucity of X-ray findings. Poorly responding otitis externa should always alert the physician to the possibility of neoplasm, diabetes, or some other underlying condition which will not respond to just topical treatment. Cholesteatoma of the external auditory canal should also be considered in refractory cases of otitis externa. Three patients with EAC subperiosteal cholesteatoma are reviewed.

Adult

Some considerations on middle ear cholesteatoma in 'foreign workers'.

UNLABELLED: The treatment of cholesteatoma in patients without a permanent residence or in medically underdeveloped countries presents a serious problem in middle ear surgery. Methods which erradicate disease safely and reduce the risk of recurrence to a minimum are necessary. We differentiate 3 clinical types of cholesteatoma: 1 Retraction of Shrapnell's membrane or in the postero-superior quadrant with very little cholesteatoma lateral to the ossicles. A transcanal technique is used to evert the cholesteatomatous pocket into the meatus intact, followed by reconstruction of the outer attic wall. 2 Circumscribed cholestatoma lateral and often also medial to the ossicles. Combined approach tympanoplasty is used in such cases. 3 Marginal perforations with large cholesteatomas, situated medial to the ossicular chain. For these ears, classical radical mastoidectomy is the method of choice. (Radical mastoidectomy also has to be performed in all cases of type II, where luxation of the matrix in toto has failed.) RESULTS: No recurrence has been detected in cases where follow-up was possible.

Cholesteatoma

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.

Adolescent

[Clinical, pathological and therapeutic aspects of cholesteatoma in children (author's transl)].

Middle ear cholesteatoma in childhood is discussed on the basis of 117 operated cases. Early diagnosis was rare, even after fetid otorrhea of long duration; occassionally they presented as acute mastoiditis. The cholesteatoma occurred mostly in older children (90%) and extended to the antrum (73%) or beyond it (54%), with destruction of the ossicles (77%); the hearing often remained normal. Tympanic membrane perforations were usually small, relatively often they were central. More than 50% of the children had normal mastoid pneumatization, usually there were no typical radiological signs of cholesteatoma. Tympanoplasty was most frequently type III (68.5%), the postoperative hearing gain in this group was 47.3%. Residual or recurrent cholesteatoma occurred in 8.5%.

Age Factors

Cholesteatoma and the middle ear cleft: a review of pathogenesis.

The advent of antibiotics has not reduced the incidence of cholesteatoma resulting from chronic otitis media. This incidence remains high due to continued Eustachian tube problems and to other poorly understood mechanisms. Because the otologist will continue to encounter cholesteatoma in clinical practice, he should be prepared to judge the extent and severity of disease and its potential for complications based on the initial office examination. This judgment is best accomplished through an understanding of the anatomy and embryology of the middle ear cleft--the compartments which encourage growth of cholesteatoma and the membranes which limit its growth. This paper reviews the anatomy and embryology of the compartments of the middle ear cleft in an attempt to understand the pathogenesis of cholesteatoma and its potential for complication.

Cholesteatoma

[Experimentally produced cholesteatoma (author's transl)].

Application of various mildly irritant substances to the posterosuperior and anteroinferior aspects of the external auditory canal of the rabbit, with intact ear drum and middle ear, produced cholesteatomas in the skin of the auditory canal, and the tympanic membrane particularly in the pars flaccida. At the anteroinferior insertion of the tympanic membrane, however, cholesteatoma growth could not be induced in these animal experiments. In severe diffuse otitis externa, secondary to the operative closure of the external auditory canal, cholesteatomas also preferably develop in the pars flaccida, the relatively thick, loose intermediate layer of connective tissue allows a rapid expansion of epithelial ridges thus favouring the formation of cholesteatoma in this region.

Animals

[Problems of autologic incus interposition in cholesteatoma operations (author's transl)].

Our clinical study proves whether or not the warning is justified not to reimplantate autologic ossicula in cholesteatoma operations in order to avoid recidives. After interposition of the autologic incus in 18 patients we found cholesteatome recidives--originating from the incus--in two cases. According to histological studies, even the most careful preparation cannot avoid that some cells of the epithelium remain on the ossicula, from which a cholesteatoma recidive may develop. Clinical studies show rather low rates of recidives compared with the histological findings. Possibly remaining squamous epithelium cells in autologic ossicles need not always cause recidives. Clinical and histological studies lead to the demand that because of the possibility of cholesteatoma recidives autologic ossicula should not be reimplated in cholesteatoma operations.

Cholesteatoma

[Behavior of the middle ear mucosa in cholesteatoma and following tympanoplasty. An experimental histological study in rabbits].

After operation three consecutive phases can be observed: 1. thickening of epithelial cell layer with development of edema in the submucosa, 2. metaplasia of epithelium with formation of ciliated and goblet cells, concomitantly round cells are found in the submucous tissue, 3. regression of inflammation and epithelial metaplasia. After 6 months goblet and ciliated cells disappear and eventually a slightly irregular monolayer epithelium is seen. If cholesteatoma is present the second phase persists. Remarkably epithelial and subepithelial changes are not concentrated in the vicinity of cholesteatoma but are seen in the whole middle ear. By autoradiography it was demonstrated, that the subepithelial tissue of cholesteatoma has a normal metabolic rate. It can be concluded that high metabolic activity postulated by other authors cannot be responsible for inflammation.

Animals