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To evaluate if fusion computed tomography-diffusion-weighted magnetic resonance imaging may have a role in the pre-operative assessment of congenital middle-ear cholesteatoma. A retrospective chart review of surgically treated congenital middle-ear cholesteatoma patients over a 2-year timespan was conducted. Pre-operative staging was performed on computed tomography and fusion computed tomography-diffusion-weighted magnetic resonance imaging based on extension of the disease according to the ChOLE classification system and the Potsic classification system. Intra-operative staging was compared to imaging findings to evaluate accuracy of the two imaging modalities in predicting congenital middle-ear cholesteatoma extent. Computed tomography was able to correctly predict congenital middle-ear cholesteatoma extent in three out of six cases according to the ChOLE classification system, all of which were staged as Ch1a and Ch1b on pre-operative computed tomography. Cases in which computed tomography was not able correctly to determine congenital middle-ear cholesteatoma extent were staged as Ch3 on pre-operative computed tomography. Fusion scans correctly determined congenital middle-ear cholesteatoma extent in all cases according to the ChOLE classification. Fusion computed tomography-diffusion-weighted magnetic resonance imaging may be helpful in cases of congenital middle-ear cholesteatoma where pre-operative computed tomography shows mastoid and antrum opacification, in which computed tomography alone may overestimate cholesteatoma extension beyond the level of the lateral semi-circular canal.

The existence of acquired cholesteatoma has been recognized for more than three centuries; however, the nature of the disorder has yet to be determined. Without timely detection and intervention, cholesteatomas can become dangerously large and invade intratemporal structures, resulting in numerous intra- and extracranial complications. Due to its aggressive growth, invasive nature, and the potentially fatal consequences of intracranial complications, acquired cholesteatoma remains a cause of morbidity and death for those who lack access to advanced medical care. Currently, no viable nonsurgical therapies are available. Developing an effective management strategy for this disorder will require a comprehensive understanding of past progress and recent advances. This paper presents a brief review of background issues related to acquired middle ear cholesteatoma and deals with practical considerations regarding the history and etymology of the disorder. We also consider issues related to the classification, epidemiology, histopathology, clinical presentation, and complications of acquired cholesteatoma and examine current diagnosis and management strategies in detail.

To investigate the differences in computed tomography (CT) features between closed-type congenital cholesteatoma (CCC) and open-type congenital cholesteatoma (OCC) of the middle ear and to evaluate the usefulness of preoperative CT examination for staging workup of congenital cholesteatoma (CC) in correlation with the surgical findings. We retrospectively reviewed the preoperative CT scans of the temporal bone obtained from 80 patients with surgically confirmed CC of the middle ear. All patients had a solitary lesion, except for one patient with two lesions, resulting in 81 CCs, which formed the basis of this study. We compared the CT features between CCCs and OCCs, focusing on their morphological characteristics, such as size, shape, location, and bone change. Based on the Potsic classification, the stage of CCs was determined at CT and surgery, and the results were compared between CCCs and OCCs. Of the 81 CCs, surgery revealed 43 CCCs and 38 OCCs. On CT scans, CCC was frequently seen as a small (median: 3.15 mm), round to oval (65.1%) mass, most commonly located in the anterosuperior quadrant (74.4%) of the middle ear with less frequent ossicular erosion (14.0%). In contrast, OCC was frequently seen as a large (median: 6.70 mm), irregular (94.7%) mass, most commonly located in the posterosuperior quadrant (68.4%) of the middle ear with frequent ossicular erosion (55.3%). The size, shape, location, and presence of ossicular erosion were significantly different between the two types. Overall, the CT and surgical stages of CCs demonstrated good agreement (kappa value: 0.77) and the CT and surgical stages of OCCs were statistically significantly higher than those of CCCs ( < 0.001 in both). CT is useful for preoperative determination of the types and staging of CC of the middle ear. Preoperative differentiation between CCC and OCC is important to avoid reoperation and prevent an extensive surgery. By providing valuable information on the morphology and extent of the lesions, CT is useful for not only the accurate preoperative determination of the type of CCs but also the accurate prediction of staging of the lesion, which should be important to preparing optimal treatment plans.

Purpose To investigate the rate of residual disease in the Potsic staging system for congenital cholesteatomas. Methods A protocol registration was published on PROSPERO (CRD42022383932), describing residual disease as a primary outcome and hearing improvement as secondary. A systematic search was performed in four databases (PubMed, Embase, Cochrane Library, Web of Science) on December 14, 2022. Articles were included if cholesteatomas were staged according to the Potsic system and follow-up duration was documented. Risk of bias was evaluated using the Quality In Prognosis Studies (QUIPS) tool. In the statistical synthesis a random effects model was used. Between-study heterogeneity was assessed using I 2 . Results Thirteen articles were found to be eligible for systematic review and seven were included in the meta-analysis section. All records were retrospective cohort studies with high risk of bias. Regarding the proportions of residual disease, analysis using the χ 2 test showed no statistically significant difference between Potsic stages after a follow-up of minimum one year (stage I 0.06 (confidence interval (CI) 0.01–0.33); stage II 0.20 (CI 0.09–0.38); stage III 0.06 (CI 0.00–0.61); stage IV: 0.17 (CI 0.01–0.81)). Postoperative and preoperative hearing outcomes could not be analyzed due to varied reporting. Results on cholesteatoma location and mean age at staging were consistent with those previously published. Conclusion No statistically significant difference was found in the proportions of residual disease between Potsic stages, thus the staging system’s applicability for outcome prediction could not be proven based on the available data. Targeted studies are needed for a higher level of evidence.

Purpose This study aimed to compare the outcomes of patients with advanced congenital cholesteatoma who underwent microscopic or endoscope-combined Canal Wall Up Tympanomastoidectomy (CWUT) in our clinic and to determine the contribution of endoscope use in reducing recurrence/residual rates. Methods In this retrospective study, the data of individuals who underwent microscopic or combined endoscopic surgery between 2008 and 2022 in our clinic were scanned from the database. Demographic data, preoperative computed tomography (CT) findings, preoperative and postoperative hearing results, operation and intraoperative status of the ossicles, duration of surgery, postoperative follow-up period, recurrence and residual disease status during follow-up were investigated. Results The data of 37 pediatric cases operated in our clinic were included in the study. All of the included cases were Potsic Stage 4 patients who underwent CWUT. The mean age of the operated individuals was 8.7 years (5–12 years) and the mean follow-up period was 47.3 months (12–112 months). 19 cases were performed microscopically only, 2 recurrences and 5 residuals were detected. 18 cases were performed combined and 1 recurrence and 1 residual was found. Conclusion In this study, it was determined that using an endoscope together with a microscope in congenital cholesteatoma cases, decreased the rate of recurrence and residual disease by protecting the external auditory canal in patients with advanced mastoid invasion.

The European Academy of Otology and Neurotology in collaboration with the Japanese Otological Society (EAONO/JOS) recently produced a joint consensus document outlining the definitions, classification and staging of middle ear cholesteatoma. The goals were to provide terminologies in the description of cholesteatoma, classify cholesteatoma into distinct categories to facilitate the comparison of surgical outcomes and to provide a staging system that reflects the severity, difficulty of complete removal and restoration of normal function. Cholesteatoma is considered a benign, expanding and destructive epithelial lesion of the temporal bone that is the result of a multifactorial process. If undetected and left treated, cholesteatoma may lead to significant complications including hearing loss, temporal bone destruction and cranial invasion. Recent advances in imaging modalities have allowed for high sensitivity and specificity in identifying the presence of cholesteatoma. Despite these advances, deficiencies exist around the world with access to health care facilities meaning cholesteatoma remains a serious and challenging entity to manage whether found within the pediatric or adult population. Proper diagnosis and management of each form of cholesteatoma is achieved by a thorough understanding of the etiology, classification, clinical presentation and histology, thereby facilitating prevention, early detection and appropriate treatment.

Bilateral congenital cholesteatoma (BCC) is rare among congenital cholesteatoma (CC) cases, and bilateral surgery poses a significant psycho-physical burden on pediatric patients. Here, we present 2 pediatric cases of BCC that were successfully managed using simultaneous bilateral transcanal endoscopic ear surgery (TEES). The surgical approach provided clear visualization of the middle ear structures, enabling successful cholesteatoma removal with minimal invasiveness and a short operative time. In both cases, follow-up computed tomography showed no recurrence, and pure-tone audiometry results demonstrated good outcomes. Simultaneous bilateral TEES can be a viable option for pediatric BCC, as it reduces patient burden due to its less invasive procedure and short operative time.

Purpose To analyse diagnostic accuracy of MRI-DWI in detecting residual disease after cholesteatoma surgery and propose an optimum follow-up (FU) scheme. Method A retrospective chart review of patients who had cholesteatoma surgery in a tertiary referral centre. 3.0 T non-echo planar diffusion weighted imaging was performed as part of routine FU or indicated on the basis of clinical suspicion of disease. Imaging outcome was verified per-operatively during a second-look procedure or ossicular chain reconstruction. Diagnostic parameters were calculated and stratified by FU length. Results For the FU of 664 cholesteatoma surgeries, 1208 MRI-DWI were obtained and 235 second-look procedures were performed. Most MRI-DWI were obtained within 1.5 yrs of surgery. In this period, significantly less true positive MRI-DWI and significantly more false negative MRI-DWI for residual disease were found compared to other FU periods. Scanning after approximately 3 yrs yielded a significantly higher rate of true positive MRI-DWI, while sensitivity surpassed 80%. Younger patients had a higher risk of developing residual disease. Patients undergoing canal wall up surgery, as well as patients < 12 yrs, were at risk for false negative MRI-DWI. Obliteration reduces the risk of residual disease, while leading to less false negative MRI-DWI. Conclusion A novel radiologic FU scheme for detecting residual disease is suggested for stable ears after cholesteatoma surgery: standard MRI-DWI approximately 3 and 5 yrs after primary surgery, as well as MRI-DWI after approximately 9 yrs for patients with specific risk factors (i.e., patients < 12 yrs or patients undergoing canal wall up surgery without obliteration).