Cases Of Cholesteatoma Research Articles

How we do it: the Farrior‐Olaizola mastoidectomy technique in the management of squamous chronic otitis media

Keypoints• In the Farrior‐Olaizola mastoidectomy the connection between the antrum and attic is closed with a cartilage plate –‘the antrum exclusion and attic elimination on demand technique’. This prevents future tympanic membrane retraction and recurrent disease.• It is a suitable treatment for most cases of cholesteatoma, including children.• As a canal wall up technique residual disease occurred in 18% of adults and 22% of children in our series. These have been discrete encapsulated epithelial pearls easily removed at the second look stage.• Recurrent disease was uncommon (3.5%).

Pediatric external canal cholesteatoma with extensive invasion into the mastoid cavity

Cholesteatoma in the external auditory canal (EAC) is an uncommon situation, and is especially rare in pediatric patients. We report two pediatric cases of external canal cholesteatoma with extensive invasion into mastoid cavity. Both cases had otalgia and poor hearing as the initial symptoms, and received operation according to the extent of the lesions. Since external canal cholesteatoma with extensive invasion into the mastoid cavity has the propensity to involve the vertical segment of the facial nerve, extreme care should be taken when performing any procedure in this area. Through thorough pre-operative evaluation and adequate surgical procedures, good outcomes can be achieved and hearing as well as facial nerve function can be preserved.

실험적으로 유발된 중이진주종에서 Telomerase의 발현

Background and Objectoves: Telomerase is a marker of cell proliferation in malignant tissues and also can be expressed in benign disease such as cholesteatoma. There are two opposite opinions about the expression of telomerase in cholesteatoma. The purpose of this study is to evaluate whether telomerase is expressed in expreimentally induced cholesteatoma. Materials and Methods: Cholesteatoma was induced by ligation of EAC of ten Mongolian gerbils. The telomerase activity was detected by the telomerase repeat amplification protocol (TRAP) assay method in both cholesteatoma and normal control retroauricular skin. Results : 10 of 10 (100%) cholesteatoma cases expressed telomerase activity, whereas 3 of 10 (30%) retroauricular normal skin detected telomerase activity. Conclusions: The high expression of telomerase in experimentally induced cholesteatoma suggests activation of telomerase may be related to the proliferative nature of cholesteatoma as in human tissues. (J Clinical Otolaryngol 2004;15:250–255)

Jugular foramen syndrome caused by choleastatoma

Unilateral paresis of 9th–11th cranial nerves together is defined as jugular foramen (Vernet's) syndrome. A cholesteatoma case that penetrated into intracranial area after eroding temporal bone and led to jugular foramen syndrome is presented here, since such a case has not been reported in the literature hitherto. A 46-year-old male patient was evaluated for hoarseness. It was learnt from his anamnesis that he had been operated for otitis media nearly 20 years ago. Uvula deviated towards right. Loss of gag reflex was remarkable on the left. Paresis was found on the left vocal cord. There was weakness in rotation of the head to the right. Computerized tomography (CT) scan revealed that the temporal bone lost its integrity and that there was an extra-axial hypodense mass neighboring pontocerebellum. Post-contrast magnetic resonance imaging showed that the mass, which showed thin, regular circular contrasting and which was hypointense in T1-weighted MR and hyperintense in T2-weighted MR, extended to the left jugular foramen. The mass was totally removed with left sub-occipital approach in the treatment. After the treatment, hoarseness, weakness in the rotation movement of the head and loss of gag reflex recovered totally, while deviation in the uvula was permanent. Cholesteatomas can extend to posterior fossa and cause jugular foramen syndrome. Early surgery is important to completely reverse the lost nerve functions in treatment.

Biphasic ceramics and fibrin sealant for bone reconstruction in ear surgery.

Bone reconstruction is still a matter of concern in middle ear surgery despite the large number of surgical techniques proposed. A composite made of biphasic calcium phosphate ceramic granules mixed with human fibrin sealant, forming a moldable material that is easy to set for bone reconstruction, has been used with success over a 16-year period for reconstruction of the posterior canal wall or for mastoid obliteration. In a retrospective series of 72 ears with long-term follow-up (average follow-up of 46 months), regular microscopic examination, and computed tomographic controls, we have evaluated this bone reconstruction technique for radical mastoidectomy cavities or the closed technique. The bone reconstruction material provided an immediate anatomic reconstruction that remains stable and is well tolerated over the long term. A slight leakage of granules in the meatus was initially observed in several patients with transient aseptic otorrhea. Fifty-seven ears were followed up for 1 year or more. Satisfactory clinical results with complete regression of the mucous membrane disease were found in 82.4% of the patients, with perfect stability of the material in 65%. We did not observe any cases of recurrent cholesteatoma in or behind the filling material. Eighteen biopsies were performed between 8 and 84 months after surgery during a second surgical step performed for functional purposes or for recurrent cholesteatoma; 17 of the 18 cases showed osteointegration of the filling material. After a 16-year experiment, we consider this technique to be a highly satisfactory procedure that could be extended to other situations of bone reconstruction.

Otologic manifestations of ectodermal dysplasia.

To determine the range and prevalence of otologic disorders in patients with ectodermal dysplasia (ED) and provide a general review of its multiple otolaryngological manifestations. Case series. Ectodermal dysplasia family conference. Sixty-nine individuals with ED were evaluated. The average age was 11 years (range, 1-56 years). Most were male patients (44 [64%]), and most had Christ-Siemens-Touraine syndrome/hypohidrotic ED (42 [61%]), with the remaining having Hay-Wells, Clouston, and ectrodactyly ED clefting syndromes and unclassified types of ED. Questionnaire including a quality-of-life assessment modeled after the Otitis Media 6 instrument, physical examination, screening audiogram, and tympanometry. Of the 69 patients, 15 (22%) had a known history of hearing loss, and over half reported some level of difficulty processing verbal information (30 patients [43%] reported speech problems and 4 patients [6%] required hearing aids). A history of otitis media was common, with 15 patients (21%) presenting with tympanostomy tubes in place. The mean +/- SD quality-of-life rating was 2.0 +/- 1.1 (range 1-7), with lower scores suggesting less of a problem. On physical examination, 18 patients (26%) had pinna anomalies. One case of advanced cholesteatoma and 2 cases of external auditory canal stenosis were identified. Of the 24 patients who received 4-tone screening audiogram, 2 (8%) had a highest pure-tone average threshold of 50 to 65 dB, whereas 5 patients (21%) had a 30- to 45-dB threshold, with the remaining having a 0- to 25-dB threshold. In our study, which is, to our knowledge, the largest reported collection of ED patients evaluated for otologic disease, most patients were found to have 1 or more otologic abnormalities, ranging from auricular anomaly to complications of otitis media to profound hearing loss.

ｏｎｌｙ ｈｅａｒｉｎｇ ｅａｒ の手術

The management of only hearing ears remains controversial because of the risk of postoperative hearing deterioration. On the basis of our recent series of 12 only hearing ears at Hyogo College of Medicine from 1999 to 2002, we reviewed the literature and discussed indications for surgery on only hearing ears. The incidence of only hearing ears was less than 2% of ear surgeries in the literature. They comprised mainly cholesteatoma cases in English papers, and otitis media and cholesteatoma cases in Japanese papers. The causes of profound hearing loss on the contralateral ear were mainly former surgery and otitis media in English papers, and otitis media in Japanese papers. Sensorineural hearing loss was a rare cause in both papers. In our recent series, 7 ears (58.3%) showed hearing improvement (of more than 15dB), 4 ears (33.3%) remained unchanged (change within 10dB), and 1 ear (8.3%) showed hearing deterioration (of more than 15dB), which were better results than those in the previous papers. Our surgical concepts are the following: (1) In cases of chronic otitis media, we first recommend simple underlay myringoplasty, and secondly, tympanoplasty without mastoidectomy. (2) In cases of cholesteatoma, we recommend the canal wall down method, leaving matrix on the fistula. (3) When an only hearing ear shows severe combined hearing loss, we recommend informing the patient of the possible necessity of cochlear implant if hearing deteriorates after surgery. Finally, it is needless to say that skillfull surgeons should perform surgery on only hearing ears after special consideration.

Bilateral Cholesteatomas: The Hospital for Sick Children Experience

The purpose of this article is to review the cases of bilateral cholesteatomas in a tertiary care pediatric otolaryngology department. We compare the presenting signs and symptoms and timing of ipsilateral and contralateral cholesteatomas and the initial audiometric findings. Retrospective chart review. Department of Otolaryngology, The Hospital for Sick Children, Toronto, ON. Twenty-two patients with bilateral cholesteatomas were accrued. Nine patients had bilateral congenital cholesteatomas and presented at a substantially younger age than those with acquired disease (average of 5 years, 9 months vs 11 years). Sixteen of the 22 patients were initially diagnosed with bilateral cholesteatomas. The majority of the remaining patients had evidence on initial assessment of contralateral middle ear disease. A particular subset of patients with severe disease requiring numerous bilateral revision surgeries was also identified. These patients also underwent revision surgery after a substantially shorter follow-up period. Bilateral cholesteatomas in the pediatric population are extremely rare. Bilateral congenital cholesteatomas are more common in males, whereas bilateral acquired cholesteatomas are more common in females. The outcome is generally poor. A small subset with particularly aggressive disease requires many more bilateral revision surgeries at an earlier period when compared with the remainder of the group.

Epidermal Growth Factor Expression in Middle Ear Cholesteatoma

Middle ear cholesteatoma is destructive to auditory ossicles and temporal bone, and treatment usually requires surgical removal of all epithelial content. Epidermal growth factor (EGF) can stimulate the growth and differentiation of a variety of mammalian cells, including epithelial cells. Our study used the avidin-biotin complex technique to evaluate the expression of EGF in 40 cases of middle ear cholesteatoma (active cholesteatoma, 31 cases; inactive cholesteatoma, 9 cases) and 34 normal postauricular skin samples. In middle ear cholesteatoma, EGF was expressed in squamous epithelium in 21 cases (53%), fibroblasts in two cases (5%), and cholesteatoma endothelium in two cases (5%). In normal postauricular skin, EGF was expressed in squamous epithelium in 14 samples (41%), fibroblasts in one sample (3%), and endothelium in none. No statistical difference in EGF expression was found between cholesteatoma and normal postauricular skin samples. These results show that the distribution of EGF in middle ear cholesteatoma is not deranged and that the progression of cholesteatoma might be induced by the release of factors from the cholesteatoma matrix via autocrine stimulation, or by inflammatory cells of the subepithelial tissue through paracrine stimulation, or in both of these ways.

Surgical findings in revision tympanoplasty

A retrospective study of 135 patients (152 ears) with the surgical findings in revision tympanoplasty of their cases will be reported. No mastoidectomy was performed.Temporalis fascia graft was used in all cases. Cases of recurrent cholesteatoma or persistent otorrhea and planned second stage operations were excluded. Underlay technique was used in 138 cases, mediolateral in 9 cases and in 5 cases overlay.The findings were failure of anterior adaptation, atrophy of the graft and infection and in some cases, no cause could be found. Hearing results after revision surgery were assessed and closure of air bone gap within 10 to 25 dB was accomplished in most of the cases. Thirty-nine ears (25.7%) showed no improvement, seven ears (4.6%) had mixed hearing loss and three (2%) developed sensorineural hearing loss at high frequency. Details of the surgical findings and causes of failure will be presented and discussed.

Expression of Epidermal Growth Factor Receptor in Human Middle Ear Cholesteatoma

Middle ear cholesteatoma is destructive to auditory ossicles and temporal bone, and treatment usually includes surgical removal of all epithelial content in the tympanomastoid cavity. Epidermal growth factor receptor (EGFR) is a 170 kd to 180 kd transmembrane glycoprotein and its distribution density is related to the ability of the keratinocytes to differentiate and their state of differentiation. We used the avidin-biotin complex technique and EGFR monoclonal antibody to evaluate the expression of EGFR in 29 cases of cholesteatoma and 34 samples of normal postauricular skin. Of patients with cholesteatoma, 79% (23 cases) had EGFR-positive cells in the basal layer, 66% (19 cases) in the parabasal layer, and 62% (18 cases) in the upper layer of the epithelial tissue. Among patients with normal postauricular skin, 85% (29 cases) had EGFR-positive cells in the basal layer, 79% (27 cases) in the parabasal layer, and 79% (27 cases) in the upper layer of the epithelial tissue. No statistical difference in EGFR expression between each layer of cholesteatoma and postauricular skin was noted. However, there was an intensity gradient of positive EGFR immunoreactivity from the basal to the higher layers in cholesteatoma. Our results showed that the distribution of EGFR in middle ear cholesteatoma is not deranged, but is similar to that in normal skin tissue.

Results of cholesteatoma surgery: the influence of age

Hypothesis: There is a difference in clinical characteristics, type of surgery and outcome between pediatric and adult cholesteatoma. Background: Pediatric cholesteatoma is a chronic disease with many challenges concerning diagnosis and treatment. There are no universally accepted opinions among otologists about the course, consequences and outcome of surgery for cholesteatoma in pediatric patients. There is also a difference in opinion concerning the choice of surgical technique. Methods: The patients were divided in three groups: children younger than 9 years (17), adolescents—10 to 16 years (25), and adults (265 cases). For all the children, closed tympanoplasty was performed, and reoperation or conversion to open tympanoplasty was done later if needed. Results: Post-operative audiometry had good audiological results, comparable to cholesteatoma surgery in adults. An air–bone gap up to 20 dB was achieved in 60% of children under 9 years, in 65% of children aged between 10 and 16, and in 65% of adults. During a post-operative course, retraction of neomembrane was found in 30% of younger children, 29% of adolescents, and in 9% of adults. Also, residual and recurrent cholesteatoma were more than twice as frequent in children. Reoperation was performed in 40% of children younger than 9, in 28% of children aged 10 to 16, and in 13% of adults. In half the cases of pediatric cholesteatoma reoperations, conversion to open tympanoplasty was done because of extensive disease. Conclusion: Adequate preoperative medicamentous and local treatment of cases with pediatric cholesteatoma are needed because of different sensitivity of bacteria and potential ototoxicity of antibiotics. Post-operatively functional and audiological results are comparable to adults, but during further follow-up, retraction pockets (30%) and recurrent cholesteatoma (20%) are found. Reoperation was performed in 32% children and conversion to open tympanoplasty in 25% of pediatric cholesteatoma cases. The age of the patient is a very important factor that determines the type of surgical procedure and the results of surgery for middle ear cholesteatoma.

Possible Involvement of Keratinocyte Growth Factor and Its Receptor in Enhanced Epithelial-Cell Proliferation and Acquired Recurrence of Middle-Ear Cholesteatoma

Middle-ear cholesteatoma is characterized by enhanced proliferation of epithelial cells and granular tissue formation. However, the molecular mechanism underlying these pathological changes is largely unknown. Keratinocyte growth factor (KGF) is a mesenchymal cell-derived paracrine growth factor that specifically stimulates epithelial cell proliferation. In the present study, we investigated the possible involvement of KGF and its receptor, KGFR, in the pathogenesis of cholesteatoma using in situ hybridization and immunohistochemistry, respectively. We examined 56 cholesteatoma specimens, and 8 normal skin areas as control. KGF and KGFR expression was examined by immunohistochemistry using rabbit anti-human KGF and anti-human KGFR polyclonal antisera raised in our laboratories against synthetic peptides corresponding to parts of human KGF and KGFR, respectively. KGF protein and mRNA were detected exclusively in stromal fibroblasts and infiltrating T lymphocytes in 80% of cholesteatoma cases, whereas KGFR protein and mRNA were localized in the epithelium in 72% of cases. Assessment of the proliferative activity of cholesteatoma using the labeling index for Ki-67 showed a significantly higher Ki-67 labeling index (66%) in KGF+/KGFR+ cases than other cases. There was a significant correlation between KGF+/KGFR+ expression and recurrence. Our results indicate the possible involvement of both KGF and KGFR in enhanced epithelial cell proliferative activity and recurrence of cholesteatoma.

A retrospective study on cholesteatoma otitis media coexisting with cholesterol granuloma.

To investigate the etiology and pathogenesis of cholesteatoma otitis media accompanied by cholesterol granuloma and the relationship between cholesteatoma and cholesterol granuloma, 63 cases of middle ear cholesterol granuloma treated in our hospital during the period from March 1988 to May 2000 were retrospectively reviewed. All cases were surgically and pathologically verified. 15 cases of cholesteatoma coexisting with cholesterol granuloma were found among the 63 patients. All 15 cases had a long-term history of otitis media, such as otorrhea (sanguine purulent otorrhea and bloody otorrhea in 8 cases) and perforation of the eardrum (perforation of pars flaccida in 8 cases). Temporal bone CT scans showed cholesteatoma in 11 cases. All patients were treated surgically, and cholesteatoma and cholesterol granuloma were found coexisting alternately, the latter lying mainly in the tympanic antrum, attic and mastoid air cells. Chocolate-colored mucus was accumulated in well-developed mastoid air cells, and glistening dotty cholesterol crystals were also found. In most cases, enlarged aditus, destruction of lateral attic wall, erosion of ossicular chain, exposure of horizontal segment of facial nerve and tegmen of attic were observed. Occlusion of Eustachian tube was noted in 6 cases, and occlusion of tympanic isthmus was revealed in all cases. A post-operative dry ear was achieved in all patients, and hearing improvement was achieved in all 12 cases following tympanoplasty. Cholesteatoma and cholesterol granuloma in middle ear may share a common pathophysiological etiology: occlusion of ventilation and disturbance of drainage. The diagnosis should be considered when patients presented with chronic otitis media with bloody otorrhea. CT and magnetic resonance imaging are useful for the diagnosis before operation. The surgical approach depends on the location, extension and severity of the lesion. The purpose of surgery is to remove the lesion and create an adequate drainage.

초기 중이 진주종의 치료

Chronic otitis media with cholosteatoma had been one of the most prevalent forms of otological disease. However, recently, the incidence of chronic otitis media have decreased owing to better economic status and health care. Thus, cases of middle ear surgery have also decreased relatively in number. So it seems that otologists nowadays should be engaged in revision surgery of the middle ear and management of early cholesteatoma. While the incidence of extensive or complicated cholesteatoma has dramatically decreased for last decades, the early staged or less extensive cholesteatoma and retraction pockets are more frequently found. Especially localized adhesive otitis media, usually posterior tympanic membrane, is one of the most difficult dilemmas for the otologist. There are several controversial issues for managing patients with early cholesteatoma : 1 ) conservative care or surgical treatment 2) techniques of surgery 3) whether or not mastoidectomy. So surgery for early cholesteatoma is thought to be reconstructive rather than destructive and it can be a prophylactic operation. I proposed a strategy for the management of early cholesteatoma which was based on safe marsupialization of retraction pocket or cholesteatoma, preservation of the mucosa, adequate ventilation, and prevention of retraction pocket. In case of attic retraction pocket or cholesteatoma, atticotomy with scutumplasty was made. On the other hand, the procedure to remove posterior annular bone and posterior scutum to allow access tympanic sinus and posterior attic space designated as posterior sinusectomy with reconstruction were applied to sinus retraction pocket or cholesteatoma. Early tensa retraction cholesteatoma would be managed as the combination of atticotomy and posterior sinusectomy. So I preferred the term “atticosinoplasty” which includes attic reconstruction with scutumplasty after atticotomy and posterior sinusectomy. If the disease seems to extend to mastoid, endoscopes were introduced transmeatally. If cholesteatoma occupies in mastoid, complete mastoidectomy is made, and sometimes mastoid cavity is obliterated to eliminate the cavity problems. During the last 7 years, I managed surgically 208 cases of attic or sinus cholesteatoma, so-called early cholesteatoma, among which there were 45 cases of atticosinoplasty. I analysed 45 cases with atticosinoplasty with special reference to period of postoperative care and hearing result, as compared to cases with mastoid obliteration and open cavity mastoidectomy. In conclusion, I personally think that middle ear cholesteatoma has a possibility of different clinical entity rather than a kind of chronic otitis media, eustachion tube dysfunction can not be always a prerequisite of middle ear cholesteatoma but it can be a secondary phenomenon, and finally surgury for early cholesteatoma is reconstructive rather than destructive ant it can be a prophylactic operation. (J Clinical Otolaryngol 2002;13:13–19)

Staging and management of primary cerebellopontine cholesteatoma.

Primary cerebellopontine angle (CPA) cholesteatoma grows slowly and silently in the subarachnoidal spaces. The diagnosis is often late, when the lesion has reached large dimensions. Surgical removal is the only available therapy. Fifteen consecutive cases of CPA cholesteatoma managed at a tertiary otoneurosurgical referral unit between September 1985 and April 1999 were reviewed. The study population, consisting of seven males and eight females, had a mean age of 44 years of age (range 21-69) at the time of surgery. The clinical, audiological and radiological presentations were examined. The tumours were classified according to the Moffat classification of CPA cholesteatomas. In 67 per cent of cases the presenting symptom was related to the vestibulo-cochlear nerve. The average duration of symptoms was 23 months (ranging from one month-10 years). The hearing preservation approaches were utilized the most (11 cases), while the translabyrinthine approach alone, or in association with a middle fossa craniotomy, was performed in four cases. Tumour removal was total in 12 cases and subtotal in three cases. In cases undergoing hearing preservation surgery the mean pre-operative pure tone average (PTA) for the frequencies 0.5, 1, 2, and 3 kHz was 19.3 dB HL (SD 13.84) and the mean pre-operative speech discrimination score (SDS) was 89.8 per cent (SD 5.97). In 44.4 per cent of patients the hearing was preserved and the mean post-operative PTA was 20.29 dB HL (SD 15.84). In five patients post-operative complications occurred. No peri- or post-operative death occurred in this series, one patient developed a recurrence 15 years after the initial surgery.

Posterior and attic wall osteoplasty: hearing results and recurrence rates in cholesteatoma.

To assess the efficacy of a surgical technique, the temporary attic and posterior canal wall osteoplasty, in preventing cholesteatoma recurrence. The records of 94 patients submitted to tympanoplasty for cholesteatoma in the ear, nose, and throat department of a main city hospital between 1989 and 1997 were retrospectively reviewed. Intact canal wall procedure with attic and posterior canal wall temporary removal was performed in 47 cases. A canal wall down operation was performed in the 47 remaining cases. The follow-up ranged from 22 to 84 months. The patients from one group could be exactly matched for the main prognostic factors (type of cholesteatoma, ossicular status, and preoperative auditory status) with the patients from the other group. Auditory results were defined according to the Committee on Hearing and Equilibrium Guidelines. A 1-way analysis of variance was used to determine group differences. A probability value of p < 0.05 was the level of significance selected. All of the patients in the 2 groups were cholesteatoma free at the last follow-up, and significantly better hearing results were observed in the osteoplasty group. The osteoplasty with temporary removal of the posterior and attic canal wall is a useful adjunct to tympanoplasty in cholesteatoma cases as far as the hearing results and recurrence rates are concerned. This method combines the functional advantages of canal wall up operations with the safety yielded by canal wall down procedures.

３Ｄ‐ＣＴにてとらえた小児先天性真珠腫例

A case of congenital cholesteatoma identified on multi-slice computed tomography and 3D-CT is reported. The patient was 1-year 9-month-old female infant, who did not demonstrate any otological syndrome. When she brought to an ENT physician for nasal obstruction, a white round mass could be seen through the right tympanic membrane and she was referred to our out-patient clinic. There was no history of otitis media.Computed tomography was performed with an Aquilion by TOSHIBA. Then 3D-CT was reconstructed using the volume rendering method. The linkage of the ossicles was clearly differentiated on the healthy side by 3D. Images of the pathological side confirmed a coin lesion on the ossicular chain of the incus-stapes. Surgery was performed under the general anesthesia. Removed cholesteatomas were about 3mm in diameter, and appeared similar to a pearl. The stapes had bean dislocated by the cholesteatoma mass. In this case, it was possible to recognize the location of the cholesteatoma preoperatively by 3D-CT, it was considered that 3D-CT is a useful technique for determines the cholesteatoma location preoperatively. This is thought to be the first report in which the site of congenital cholesteatoma was confirmed preoperatively using 3D-CT imaging.

外傷性外耳道乳突蜂巣内真珠腫の２例

外傷性外耳道乳突蜂巣内真珠腫の２例

Renal Cholesteatoma: Two Cases Report

Cholesteatoma of the urinary tract is an extremely rare disease occurring in the bladder and renal pelvis. We report the radiologic findings in two cases of renal cholesteatoma involving the renal pelvis, calyces and ureter, and correlate these with the histopathologic findings.