Imaging analysis of dural exposure in cholesteatomas with a skull base defect.

PURPOSE: Dural exposure during cholesteatoma surgery can pose a risk of cerebrospinal fluid leakage or residual disease. Therefore, delicate handling of the area surrounding the bone defect in the cranial fossa is required. However, in small-sized defects, preoperative prediction of dural exposure can be challenging. This study aimed to evaluate the diagnostic value of computed tomography (CT) for preoperative prediction of cholesteatoma-related dural exposure in bone discontinuities in the skull base. METHODS: We evaluated serial high-resolution CT images showing bone density discontinuities in the middle cranial fossa (MCF) requiring mastoidectomy for cholesteatoma. The CT and intraoperative findings were analyzed retrospectively. We evaluated the length between the superior margins of the bone density discontinuities using coronal CT planes. Receiver operating characteristic (ROC) curves were constructed to determine the optimal cut-off values. RESULTS: We extracted data from 107 bone density discontinuities, among which 54 (50.5%) showed dural exposure intraoperatively. Discontinuities with dural exposure (n = 54) had significantly greater lengths than did those without (n = 53) (p < 0.001, Wilcoxon rank-sum test). The area under the curve was 0.9780 according to the ROC analysis, and the optimal cut-off value was determined to be 2.99 mm (sensitivity 92.59%; specificity 94.34%). CONCLUSION: A bone density discontinuity length of > 2.99 mm in the MCF on coronal CT plane is a reliable diagnostic marker for cholesteatoma-related dural exposure. Thus, preoperative high-resolution CT analysis can inform optimal surgical preparation and planning before manipulating the area surrounding the osteolytic lesion in the MCF.

Experimental cholesteatoma: a comparison between spontaneous and induced models.

OBJECTIVE: To compare the incidence and the histopathological aspect of spontaneous and two induced Mongolian gerbils' models of cholesteatoma: External Auditory Canal (EAC) obliteration model and the Auditory Tube (AT) cauterization model. METHODS: Fifty-four ears of 27 animals were divided into EAC obliteration, AT cauterization, and control groups and histologically assessed for cholesteatoma incidence and classification at intervals of 2, 4, 8, and 16 weeks. RESULTS: Cholesteatoma was diagnosed in 30 of the 53 ears evaluated with a significantly higher incidence in groups that received some type of intervention (p<0.0001). It was not possible to histologically distinguish cholesteatomas of the same stage between the study groups. CONCLUSION: Although we observed a significant increase in cholesteatoma incidence with the two methods used when compared to the control group, all developed cholesteatomas were apparently identical from a histological point of view.

Association of CARD8 Activating Polymorphism With Bone Erosion in Cholesteatoma Patients.

OBJECTIVES: We compared the incidence of polymorphisms activating the NLRP3 inflammasome between controls and patients with cholesteatoma and its potential association with bone erosion in patients with cholesteatoma. METHODS: This is a case-control study assessing the mutation rates in genes of interest in patients with and without cholesteatoma. A total of 133 saliva samples from control (n = 65) and cholesteatoma (n = 68) patients were collected for DNA extraction. Caspase recruitment domain family member 8 (CARD8) (AA: homozygous wild type, AT: heterozygous, TT: homozygous mutant polymorphism) and NLRP3 (CC: homozygous wild type, CA: heterozygous, AA: homozygous mutant) polymorphisms were analyzed with TaqMan single-nucleotide polymorphism (SNP) quantitative polymerase chain reaction (ThermoFisher Scientific, Waltham, MA). Mutation status was correlated with a novel bone erosion scoring model developed as a part of this study. Summary statistics, including frequencies (%) and median (Q1, Q3) were used to describe the sample. RESULTS: The presence of CARD8 and NLRP3 homozygous wild-type polymorphisms were generally similar for the control and cholesteatoma patient groups. CARD8 homozygous TT polymorphisms were an exception, occurring more frequently in patients who developed a cholesteatoma compared to the control group (29% vs. 10%, P = .009). Those patients with CARD8 homozygous TT polymorphism had higher median scores of bone erosion as compared to subjects with nonhomozygous mutant genotypes (median [interquartile range]: 4.0 [3.0, 5.5] vs. 2.5 [1.0, 3.5], P = .0142). CONCLUSION: Cholesteatoma patients have a significant, twofold higher incidence of CARD8 homozygous TT polymorphism. Furthermore, cholesteatoma patients with this homozygous polymorphism had greater bone erosion rates than controls. These findings suggest that genetic mutations may increase host susceptibility to cholesteatomas. Specifically, the CARD8 TT polymorphism may influence the severity of cholesteatoma-induced bone erosion. LEVEL OF EVIDENCE: 3B.

Keratinocyte growth factor signaling promotes stem/progenitor cell proliferation under p63 expression during middle ear cholesteatoma formation.

PURPOSE OF REVIEW: Middle ear cholesteatoma is an epithelial lesion that expands into the middle ear, resulting in bone destruction. However, the pathogenesis of this has been unknown. The purpose of this review is to understand the role of keratinocyte growth factor (KGF) during epithelial stem and/or progenitor cell proliferation in middle ear cholesteatoma. RECENT FINDINGS: Many researchers have investigated the molecular mechanism of middle ear cholesteatoma to establish a conservative treatment. Recently, some studies have focused on the stem cells of middle ear cholesteatoma and their detection, but the key molecules for stem cell formation were not shown. SUMMARY: We established an animal model for middle ear cholesteatoma and are showing the results of our studies. KGF expression accelerates the proliferation of stem/progenitor cells through the induction of transcription factor p63 expression in the epithelium of the tympanic membrane and mucosal epithelium overlying the promontory of the cochlea and within the attic. This is typical in middle ear cholesteatoma. Moreover, the partial epithelial-mesenchymal transition under the p63 signaling pathway plays an essential role in epithelial cell growth in middle ear cholesteatoma formation. Understanding p63 expression following KGF expression and associated signaling events can improve therapeutic outcomes in patients with middle ear cholesteatoma.

Rate of chronic otitis media operations and cholesteatoma surgeries in South Korea: a nationwide population-based study (2006-2018).

The aim of this study was to estimate the total number and rate of chronic otitis media (COM) operations and cholesteatoma surgeries in South Korea, using a nationwide survey which analysed a 13-year trend (2006-2018). This study also analysed the trend of COM operations and cholesteatoma surgeries according to year, sex, and age using a nationwide population-based database, and the 13-year trend was analysed according to age groups. This study used nationwide data from the National Health Information Database (NHID), which is a government-affiliated agency under the Korean Ministry of Health and Welfare that supervises all medical activities in Korea. Retrospective medical data of patients of all ages were extracted from the NHID from January 2006 to December 2018 (NHIS-2018). This study was conducted by the Research Committee of the Korean Society of Otorhinolaryngology-Head and Neck Surgery, and the Korean Audiological Society reviewed and confirmed the study. There was a 1.5 fold increase in COM operation rates in 2018, compared to 2007 figures. The annual total number of COM operations was 5,935 in 2007, 8,999 in 2012 (peak), and 8,870 in 2018 (17 in 100,000). Meanwhile, the total annual number of cholesteatoma surgeries decreased from 3,502 in 2006 to 3,199 in 2018 (6 in 100,000). The rate of COM operations was higher (1.27 fold) in the female population than in the males in 2018. However, cholesteatoma surgery rates were higher (1.2 fold) in the male population than in the females in 2018. According to the 2018 data, COM operations were most commonly performed in patients in their 50s. COM operation rates increased rapidly in patients aged 51-80. In other age groups however, rates were constant or showed a decrease in figures, especially in the 40s age group (1st rank in 2006 to 3rd rank in 2018). According to the 2018 data, cholesteatoma surgery was most commonly performed in patients in their 50s. Cholesteatoma surgery rates increased dramatically from 2006 to 2018 in patients aged 0-10 years due to congenital cholesteatoma. Cholesteatoma surgery rates also increased in patients aged 61-80 years due to ageing population. Cholesteatoma surgery rates decreased in patients aged 41-50 years, ranking 1st in 2006 and 4th in 2018. In conclusion, the annual rate of COM operations was 0.017%, and no longer increases, but stabilizes/decreased after a peak point in the advanced country. The mean rate of cholesteatoma surgery was 0.006%, and decreased annually. There was female dominance in COM operations, but male dominance in cholesteatoma surgery. Major age groups of patients who underwent COM/cholesteatoma surgery were the 50s and 60s, and congenital cholesteatoma (0-10 years) accounted for about 20% of all cholesteatoma surgery.

Management of acquired cholesteatoma in patients with craniofacial anomalies: An institutional experience.

PURPOSE: To analyze the surgical management of cholesteatoma in patients with craniofacial abnormalities, cleft lip/palate by reviewing the institutional experience. The secondary aim was to identify and describe the epidemiological profile of the collected data, and to relate the cleft palate and cholesteatoma. DESIGN AND METHODS: This retrospective chart review includes 97 patients with craniofacial abnormalities and acquired cholesteatoma with anatomopathological proven in 118 ears. The following data were collected from the medical records between 1994 and 2018. RESULTS: The first surgery performed on 76 of the 118 ears (64.4%) was the wall up mastoidectomy, while 42 of the 118 ears (35.5%) received the wall down technique. During the follow-up period of these patients, which ranged from 2 to 29 years, with an average of 13.4 years (±5.88), 77 wall up (40.3%) and 114 wall down (59.6%) mastoidectomies were performed. This brought the total to 191 mastoidectomy surgeries in 118 ears of 97 patients. Of the wall up mastoidectomies, 65 of the 77 (84.4%) presented with cholesteatoma recurrence. In the wall down mastoidectomies follow up, there were new surgical approaches in 15 of the 114 procedures (13.1%), with 6 patients (5.2%) having anatomopathologically proven cholesteatoma recurrences and 9 (7.8%) having clinical instability for cavity cleaning without identification of disease recurrence. CONCLUSIONS: Early approach with wall down/modified techniques guided by specific indication criteria may be more resolute, prevent multiple procedures, and preserve the bone pathway to facilitate possible future hearing rehabilitation in these patients.

Iatrogenic cholesteatoma originating from a misplaced tympanomeatal flap during tympanoplasty: a series of five patients.

PURPOSE: To report our experience of a sequence of events that resulted in an iatrogenic cholesteatoma originating from the external auditory canal (EAC) years after tympanoplasty that had included a tympanomeatal flap. METHODS: Data on the presentation and pathogenesis of iatrogenic cholesteatomas arising from misplaced tympanomeatal flaps during tympanoplasty without mastoidectomy were retrieved from the patients' medical records and analyzed. RESULTS: Five patients were identified with cholesteatomas involving the EAC. They all had recurrent ear infections and varying degrees of conductive hearing loss. Each patient's past surgical history included one or more tympanoplasties in which an ipsilateral tympanomeatal flap had been raised. None had undergone a mastoidectomy. Two patients presented with small cholesteatomas that had developed over an average of 6.5 years after surgery. Three patients had large cholesteatomas that had developed over an average of 33.7 years after surgery. Clinical presentations and imaging studies suggested a misplaced tympanomeatal flap as the most likely source of cholesteatoma. CONCLUSION: Tympanomeatal flap misplacement may cause iatrogenic cholesteatoma formation originating from the EAC during tympanoplasty even without mastoidectomy. These cholesteatomas can grow substantially before becoming symptomatic as they extend to and through the mastoid. They may not affect the sound conduction system until late in the course of the disease. Meticulous replacement of tympanomeatal flaps and exercising a high index of suspicion postoperatively can reduce the incidence of this complication.

Can endoscopic ear surgery replace microscopic surgery in the treatment of acquired cholesteatoma? A contemporary review.

Acquired cholesteatoma leads to significant morbidities while current surgical options remain a challenge. The principles of surgery include complete removal of disease, prevention of recurrence, and restoration of hearing function when possible. Traditionally, this has been performed using microscopes; however, a novel technique using endoscopes offers a new perspective on our understanding of anatomy, pathogenesis and surgical approaches. In recent years, various studies have demonstrated good outcomes with transcanal endoscopic ear surgery (EES) in cholesteatoma surgery. Nevertheless, the use of EES is not universal and remains controversial due to the efficacy of microscopes, specific limitations of endoscopes and the need to learn new skills. This review focuses on recent advances in EES for the treatment of acquired cholesteatoma, benefits, current challenges, and a discussion on the indications and contraindications of EES.

Subannular ventilation tubes in the pediatric population: Clinical outcomes of over 1000 insertions.

OBJECTIVES: Long-term transtympanic tube insertions for chronic middle ear disease are associated with high rates of complications. The objective of this study was to examine the clinical outcomes achieved with an alternate technique, the subannular tube insertion, by determining mean tube lifespan, cumulative incidence of post-operative events and complications, audiometric changes and risk factors associated with earlier tube extrusion in the pediatric population. METHODS: A retrospective chart review of all patients operated for subannular tube insertion between January 2007 and 2013 was conducted in a single pediatric tertiary care center. Exploratory Cox regression analysis was performed to identify potential risk factors. RESULTS: A total of 1014 tubes from 459 patients were included in the study. Mean subannular tube lifespan was 41.3 months with median time of 35.0 months. Cumulative incidence of post-operative events in decreasing frequency were otorrhea (21.7%), tube blockage (16.0%), tympanic membrane retraction (12.5%), otitis media with effusion (10.0%), acute otitis media (6.4%), perforation (4.6%) and cholesteatoma formation (1.1%). For patients with available pre- and post-operative audiograms, mean air-bone gap improved from 19.5 dB to 7.0 dB after subannular tube insertion (p < 0.01). Increasing age and previous subannular tube insertion carried hazard ratios of 1.029 (p < 0.01) and 1.749 (p < 0.01) for tube extrusion respectively, while craniofacial anomalies and concomitant tympanoplasty at the time of tube insertion had hazard ratios of 0.795 (p < 0.01) and 0.680 (p = 0.03). CONCLUSIONS: Subannular tube insertion appears to be a safe and effective alternate technique for middle ear ventilation in cases of intractable disease.

Middle ear congenital cholesteatoma: systematic review, meta-analysis and insights on its pathogenesis.

PURPOSE: Congenital cholesteatoma (CC) presents as a white pearl-like lesion behind a normal tympanic membrane (TM), without a history of otorrhea, infection, perforation or previous otologic surgery. Several recent studies provided new data improving this pathology characterization. The aim of this paper is to expand the knowledge about CC and to provide new insights on its pathogenesis. METHODS: The study consisted of two main research parts: (1) systematic review and meta-analysis; (2) medical literature review englobing anatomy, histology, embryology and congenital pathology of the ear. RESULTS: The search strategy identified a total of 636 papers. Seventy retrospective studies were included. A total of 1497 cases were studied and the mean age was 6.58 years, with a male-female ratio of 3:1, 34% were asymptomatic, 26% had hearing loss and 2% had facial dysfunction/paralysis. The overall estimate for antero-superior quadrant involvement was 0.70 [95% confident interval (CI) 0.64-0.76], in the postero-superior quadrant was 0.60 (95% CI 0.52-0.69), in the antero-inferior quadrant was 0.32 (95% CI 0.23-0.41), in the postero-inferior quadrant was 0.38 (95% CI 0.29-0.47), in the attic was 0.53 (95% CI 0.43-0.63) and in the mastoid was 0.33 (95% CI 0.26-0.41). More advanced Potsic stages were present in older patients. The most likely inclusion place seems to be between the pars flaccida and the upper quadrants of the pars tensa. CONCLUSIONS: During the last decades, a substantial improvement in CC diagnosis and management had been achieved. The presented mechanism seems to explain most of middle ear CC.

Körner's septum (petrosquamosal lamina): the anatomical variant or clinical problem?

Körner's septum (KS) or petrosquamosal lamina is a bony lamina beginning at the articular fossa, extending above the middle ear, and running inferiorly and laterally to the facial nerve canal as it proceeds to the mastoid apex. This septum marks the junction of petrous and squamous bones. The paper presents details of the anatomical structure of KS, which is most often present at the level of the head of the malleus and/or the anterior semicircular canal. Attention is paid to embryological aspects of temporal bone development that lead to the formation of KS. Two imaging techniques most frequently used to diagnose KS are described, high resolution computed tomography (HRCT) and cone-beam computed tomography. Also presented is a case report of a 6-year-old patient suffering from chronic otitis media who developed a cholesteatoma due to presence of KS, illustrated with HRCT images and intraoperative capture. The authors describe diagnostic difficulties associated with this anatomical variant in the middle ear. The article also discusses the more frequent occurrence of this clinical problem in ears operated on due to chronic inflammation, retraction pocket or tympanosclerosis in comparison to healthy ears.

[The research progress of keratin's roles in the pathology of middle ear cholesteatoma].

Keratin (K) is the main component of the epithelial cell mesenchymal cytoskeleton, which protects the integrity of epithelial cells and maintains the function of normal epithelial cells. The expression of keratin affects epidermal proliferation and differentiation, and so as to be used as a marker for proliferation, differentiation and migration of keratinocytes. Middle ear cholesteatoma is one of the common ear diseases. In the middle ear cholesteatoma, keratinocytes over-proliferate and keratin debris accumulates. In this paper, we reviewed the recent studies on middle ear cholesteatoma and explained the possible mechanisms of keratin in the pathogenesis of middle ear cholesteatoma from the aspects of "proliferation" and " bone resorption ". At the same time, the existing problems as well as the prospect of the future research were discussed.

Health Related Quality of Life after the Bony Obliteration Tympanoplasty for COM with Cholesteatoma using the COMQ12 - A Disease Specific PROM.

OBJECTIVES: This study aimed to investigate the effect of canal wall up with bony obliteration tympanoplasty (CWU-BOT) on the health-related quality of life (HRQOL) in patients with chronic otitis media with cholesteatoma by using the chronic otitis media questionnaire 12 (COMQ-12). MATERIALS AND METHODS: This study is a retrospective analysis of the COMQ-12 of 26 patients who completed the COMQ-12 before and after a CWU-BOT with eradication of cholesteatoma followed by obliteration of the mastoid and paratympanic space with bone chips and bone pâté and reconstruction of the tympanic membrane and ossicular chain. RESULTS: All patients were operated upon in our institute between 2014 and 2017. The median score of the 12 questions was preoperatively and postoperatively calculated, and then compared. A large effect was observed in the total score and the questions about running ear, discharge, and visits to the general practitioner. A medium positive size effect was observed in the questions about hearing in noisy surroundings, discomfort, dizziness, tinnitus, medication use, and the mental aspect of the patient. In the questions about the hearing at home and quality of life and impact on work, we noted a small positive size effect. In 50% of patients, the HRQOL became normal; the remaining 50% improved to a level very close to normal. CONCLUSION: Canal wall up with bony obliteration tympanoplasty (CWU-BOT) showed a clear decrease in the severity of the symptoms, life and work impact, and health care after surgery.

Blast-Induced Cholesteatomas After Spontaneous Tympanic Membrane Healing.

OBJECTIVES: To characterize blast-induced cholesteatomas (BIC) in terms of symptoms, presentation, and location within the middle ear cleft (MEC). DESIGN: A search for all English language articles in "MEDLINE" via "PubMed" and "Google Scholar" was conducted. RESULTS: A total of 67 ears with BIC were included. Fifty-eight ears in which the traumatic perforation failed to spontaneously close were excluded, leaving seven case reports (eight patients, nine ears) for statistical analysis. Time between blast exposure to spontaneous tympanic membrane (TM) closure was 16 days to 10 months. Time between blast exposure and cholesteatoma diagnosis was 5 months to 4 years. The cholesteatomas were diagnosed due to symptoms in two ears, as asymptomatic finding on physical examination in one ear and as asymptomatic finding in axial imaging in three ears. CONCLUSIONS: BICs can develop behind intact tympanic membrane or along with TM perforation. Based on the current review, when a TM perforation and spontaneous healing were documented, after blast exposure, MRI scan is an integral component of the follow-up. The optimal timing for MRI performance after blast exposure, is yet to be identified.

Chemically Assisted Dissection With Sodium 2-Mercaptoethanesulfonate (MESNA) in the Surgical Management of Pediatric Cholesteatoma.

OBJECTIVE: To evaluate the effectiveness of the chemically assisted dissection with sodium 2-mercaptoethanesulfonate (MESNA), in the reduction of residual and recurrent cholesteatoma after mastoidectomy in children with chronic cholesteatomatous otitis media (CCOM). STUDY DESIGN: Retrospective case-control study. SETTING: Tertiary referral center. POPULATION: One hundred forty mastoidectomies performed in patients under 18 years of age for the treatment of CCOM. INTERVENTIONS: Chemically assisted dissection (CAD) with MESNA compared with surgical dissection without MESNA. MAIN OUTCOME MEASURES: Recidivism of cholesteatoma (recurrence and residual disease), variations in the average of bone conduction threshold after treatment, and complications. RESULTS: Recidivism of cholesteatoma was significantly lower when CAD with MESNA was used (p < 0.0001). No difference was found in the mean variation of the average of bone conduction thresholds between the groups, confirming its safety profile regarding auditory function. Meatoplasty stenosis after surgery was more prevalent within CAD with MESNA group (p: 0.049). CONCLUSION: Recurrent and residual cholesteatoma remains a problem, especially in children and despite surgical techniques such as canal wall down mastoidectomy and endoscopic ear surgery. CAD with MESNA can be safe and effective to reduce recurrence rates. Multicenter and prospective studies with larger number of patients are needed to validate these findings. The higher rate of meatoplasty stenosis after CAD with MESNA merits additional clinical research to confirm these findings, as well as in vitro studies evaluating the effect of the drug on the activity of fibroblasts and other growth factors that may be involved.

Intracranial Complications of Chronic Otitis Media: Why Does It Still Occur?

Intracranial complications secondary to chronic otitis media (COM) include otogenic brain abscess and sinus thrombosis. Intravenous antibiotics and imaging have significantly reduced the incidence of intracranial complications secondary to COM. However, the same does not apply to a developing country like Malaysia, which still experiences persisting otogenic complications. This case series describes 3 patients with COM and intracranial complications. All 3 patients had COM with mastoiditis, with 1 of the 3 having a cholesteatoma. Postulated reasons for the continued occurrence include poor access to health care, poor compliance with medication, and the lack of pneumococcal vaccination during childhood. In conclusion, public awareness and a timely specialty referral can reduce the incidence of intracranial complications of COM.

[Progress in middle ear dysventilation research].

Disfunction of Eustachian tube will cause negative pressure of middle ear, which may result in tympanic membrane retraction pocket. Severe pocket can consequently cause cholesteatoma. In clinical practice it is not uncommon to find a cholesteatoma limited to epitympanum, with an otherwise normal pars tensa and mesotympanum. This review explains the theory of "selective epitympanic dysventilation syndrome" developed by endoscopic technique. In the majority of the patients, the only ventilation pathway to the epitympanum is through the tympanic isthmus. Even if Eustachian tube function has recovered, an isthmus blockage with selective epitympanic dysventilation may lead to common attic cholesteatoma.

Establishment of a Large Animal Model for Eustachian Tube Functional Study in Miniature Pigs.

This study was performed to investigate whether miniature pigs are a suitable animal model for studies of the Eustachian tube (ET). Sixteen Chinese experimental miniature pigs were used in this investigation. Ten animals were used for anatomical and morphometric analyses to obtain qualitative and quantitative information regarding the ET. Three animals were used for histological analysis to determine the fine structure of ET cross-sections. Three animals were used to investigate the feasibility of balloon dilation of the Eustachian tube (BDET). The anatomical study indicated that the pharyngeal orifice and tympanic orifice of the miniature pig ET are located at the posterior end of the nasal lateral wall and anterior wall of the middle ear cavity, respectively. The cartilaginous tube was seen to pass through the whole length of the ET, the length of the cartilaginous part of the ET and the diameter of the isthmus were similar between humans and miniature pigs. The inclination of the ET in miniature pigs was larger than that in humans. The gross histology seemed to be slightly different between miniature pig and human, but the fine structures were essentially the same in both species. BDET experiments verified that the miniature pig model is suitable as a model for clinical operations. The miniature pig ET corresponds very well to that of humans. In addition, the miniature pig ET is suitable as a model for clinical operations. Therefore, the miniature pig is a valid animal model for ET study. Anat Rec, 302:1024-1038, 2019. © 2019 Wiley Periodicals, Inc.

Surgical management of middle ear cholesteatoma in children with Turner syndrome: a multicenter experience.

BACKGROUND AND AIM: As in other syndromes characterized by craniofacial anomalies, middle ear cholesteatoma is known to have a high prevalence in Turner syndrome. The aim of this study was to review a multicenter experience with the surgical management of middle ear cholesteatoma in children with Turner syndrome. METHODS: We retrospectively analyzed sixteen girls with Turner syndrome who underwent otologic surgery for middle ear cholesteatoma between January 2000 and December 2012. Surgery was performed in 3 tertiary care otologic centers. Four patients had bilateral disease, resulting in a total of 20 ears treated. The following data were recorded: age, history of ventilation tube insertion, status of the controlateral ear, cholesteatoma location and extension, and surgical technique involved. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured. RESULTS: Follow-up ranged from 3 to 15 years (mean 7 years). Fourteen ears underwent canal wall down mastoidectomy: no cases of recurrent cholesteatoma were observed in these cases; revision mastoidectomy with cavity obliteration was needed in 2 ears (14.3%) for recurrent otorrhea. In the remaining 6 ears a staged canal wall up mastoidectomy was performed: 1 child showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A postoperative air-bone gap result of 0 to 20 dB was achieved in 6 ears (30%); in 9 ears (45%) postoperative air-bone gap was between 21 and 30 dB, while in 5 (25%) was >30 dB. Bone conduction thresholds remained unaffected in all cases. CONCLUSIONS: Cholesteatoma in children with Turner syndrome is a challenging entity for the otologic surgeon. Although not mandatory, canal wall down mastoidectomy should be regarded as the technique of choice to achieve a safe and dry ear in TS children with middle ear cholesteatoma. Intact canal wall mastoidectomy should be adopted only in appropriately selected patients such as those with limited attic cholesteatoma that can be regularly followed-up.

[Research progress of the cholesteatoma and proliferation pathways].

Cholesteatoma is an abnormal formation of benign cystic lesions from the squamous epithelium of the temporal bone, and the local invasive growth could damage the structures of middle ear. Cholesteatoma has been the research focus in otorhinolaryngology, however the pathogenesis of it is still unclear. From the previous studies we found that the epidermal proliferation was one of the important factors in the pathogenesis of the cholesteatoma, which was also involved in multiple proliferation pathways. In this paper, we briefly reviewed the progress and the mechanisms of the actions of cholesteatoma in these pathways.