Clinical relevance of the new classification for mastoid progression in the staging and classification criteria for middle ear cholesteatoma proposed by the Japan Otological Society.

Recurrent congenital cholesteatoma in autism spectrum disorder: A rare and complex case report and literature review

Ossicular Chain Status Guides Surgical Selection in Pediatric Congenital Cholesteatoma: Outcomes from 48 Cases

Chronic suppurative otitis media (CSOM) of the atticoantral type is a distinct otologic pathology driven by cholesteatoma, characterized by enzymatic bone erosion and a high risk of intracranial complications. While typically acquired through retraction pockets, a subset of these cases represents congenital cholesteatomas (CC) that have silently expanded and ruptured the tympanic membrane. This presentation mimics acquired disease, creating a diagnostic blind spot. We report the case of a 4-year-old male presenting with a one-year history of persistent, foul-smelling otorrhea and otalgia in the right ear. Despite a history of recurrent cold, there was no prior trauma or otologic surgery. Physical examination revealed an attic perforation with purulent discharge. Computed tomography scan (CT-scan) identified a soft tissue mass filling the epitympanum and mesotympanum with significant erosion of the scutum and ossicles. The patient underwent a modified radical mastoidectomy with type II tympanoplasty. Intraoperative findings revealed an extensive cholesteatoma sac consistent with a congenital origin that had secondarily ruptured. In conclusion, congenital cholesteatoma must be considered in the differential diagnosis of pediatric CSOM, even in the presence of a perforation. This ruptured presentation highlights the necessity of early HRCT imaging over conventional radiography. The canal wall down approach remains a critical strategy for eradicating extensive pediatric disease to prevent recidivism.

The aim of this study was to evaluate the recurrence rate of congenital cholesteatoma using Kaplan-Meier survival analysis. Retrospective study. Tertiary referral center. Eighty-one patients with middle ear congenital cholesteatoma underwent surgery or had surgeries supervised by the same surgeon from 1991 to 2021. The patients underwent tympanoplasty. The cumulative recurrence rate of congenital cholesteatoma was calculated using Kaplan-Meier survival analysis. The 7-year recurrence rates of residual and retraction cholesteatoma were 11.0% and 14.9%, respectively. The overall 5-year and 7-year recurrence rates were 17.1% and 25.4%, respectively. No recurrence occurred beyond 7 years after surgery. The 7-year recurrence rates of retraction cholesteatoma were 24.2% and 0.0% in patients who underwent surgery from 1991 to 2010 and from 2011 to 2021, respectively (log-rank test: P =0.014). The overall recurrence rates were 5.3% for stages I and II and 31.1% for stages III and IV (log-rank test: P =0.082). Residual cholesteatoma occurred in 9 patients, whereas retraction cholesteatoma was observed in seven. The median time to retraction cholesteatoma was significantly longer than that to residual cholesteatoma (Mann-Whitney U test: P =0.029). Kaplan-Meier survival analysis revealed that recurrence rates increased with longer observation periods. Although no recurrence occurred beyond 7 years, long-term follow-up is recommended. Kaplan-Meier survival analysis should be used to account for censored data when evaluating recurrence rates of congenital cholesteatoma.

Objective:To explore the application value of high-resolution temporal bone CT and DW-MRI fusion technology in achieving precise diagnosis and anatomical localization of middle ear cholesteatoma during endoscopic surgery. Methods:Eighteen patients initially diagnosed with middle ear cholesteatoma in the Department of Otolaryngology Head and Neck Surgery, Shaanxi Provincial People's Hospital, from January to June 2024 were enrolled.Preoperative high-resolution temporal bone CT and DW-MRI were performed, and rtStation software was used for image fusion to construct CT-MRI fused images. The involvement of cholesteatoma in six anatomical subregions of the temporal bone was evaluated. Using surgical pathology as the gold standard, and combining surgical videos and anatomical records, the sensitivity, specificity, and accuracy of pure CT, pure DW-MRI, and CT-MRI fused images in evaluating middle ear cholesteatoma lesions were compared. Results:A total of 18 patients were included, and 17 cases were pathologically confirmed as middle ear cholesteatoma postoperatively. The sensitivity of the preoperative of preoperative CT was 100%, but the specificity was only 44.44%, with an overall accuracy of 72.22%; the sensitivity and specificity of DW-MRI evaluation were 81.46% and 85.19%, the accuracy was 83.33%, respectively. In contrast, the sensitivity and specificity of CT-MRI fusion image to the spatial localization of cholesteatoma were higher than that of DW-MRI alone（92.59% vs 81.46%; 98.15% vs 85.19%）, and the diagnostic accuracy was also significantly improved（95.37% vs 83.33%）. The Kappa values for the agreement between HRCT, DW-MRI, and CT-MRI segmentation localization and pathological results were 0.444, 0.667, and 0.907 respectively. The chi-square paired t-test confirmed statistically significant diagnostic differences between groups（P<0.001）. Results demonstrated that CT-MRI significantly outperformed HRCT and DW-MRI in diagnostic efficacy for segmental localization of primary posterior congenital middle ear cholesteatoma. Conclusion:High-resolution temporal bone CT combined with DW-MRI fusion technology demonstrates higher sensitivity, specificity, and accuracy in the diagnosis and spatial localization of middle ear cholesteatoma than single imaging modalities. It can provide more precise evaluation of lesion scope for endoscopic surgery, showing important clinical application value.

BACKGROUND Congenital cholesteatoma is a relatively rare condition accounting for about 2% to 5% of all cholesteatomas. Congenital cholesteatomas pose a significant challenge to treat. Failure to detect cholesteatoma early can lead to progressive and severe damage to middle ear structures, resulting in hearing loss. If conventional hearing aids are not a viable option, a bone conduction implant may be considered. One of the most successful is the Bonebridge implant, designed for adults and children with conductive or mixed hearing loss. The aim of this study was to analyze the surgical implications, efficacy, and safety of the Bonebridge implant in our patient. CASE REPORT We report the case of a 10-year-old girl with a diagnosis of congenital cholesteatoma and progressive hearing loss. She had undergone multiple surgical procedures, with no lasting results. To improve hearing, she underwent a comprehensive diagnostic evaluation for an implantable bone conduction device, and she was found to be eligible for a Bonebridge implant. To verify the improvement in hearing, pure-tone audiometry, speech audiometry, and the Polish Matrix Sentence Test were performed before implantation, during processor activation, and at the follow-up visit 3 months later. Follow-up was planned at 3 and 6 months to monitor for recurrence of cholesteatoma, with cavity checks every 4 weeks. CONCLUSIONS After Bonebridge implantation, the patient demonstrated significant improvement in hearing thresholds and speech recognition. Implantation was uncomplicated and regular follow-ups were scheduled to monitor for recurrent cholesteatoma.

Congenital Middle Ear Cholesteatoma in Children: A 12-Case Series

To present surgical and audiological outcomes of the pure endoscopic transcanal approach in the treatment of congenital cholesteatoma (CC). The present retrospective multicentre study included 34 patients who underwent exclusive transcanal endoscopic ear surgery (ETEES) for CC treatment by senior surgeons with expertise in endoscopic ear surgery. The extent of the CC was classified according to the Potsic stage. A comprehensive investigation was conducted, encompassing a multitude of parameters, including patients' demographics, CC characteristics, ossicular chain status, ossiculoplasty techniques, operative time, complications, residual or recurrent cholesteatoma, and audiological outcomes. Of the 34 CC patients enrolled in the study, 19 (55.9%) were male and 15 (44.1%) were female; their mean age was 15.2 ± 6.1 years, and their average follow-up period was 36.4 ± 10.2 months. There were 11 cases (32.4%) of Potsic stage I CC, 7 (20.6%) stage II, 10 (29.4%) stage III, and 6 (17.6%) stage IV. The average operating time was 86.2 ± 16.9min (ranging 40-180min). Overall, 9 (26.5%) patients underwent a second-look and revision surgery via ETEES approach, due to conductive hearing loss and recurrence, in 4 (11.8%) cases with Potsic stage III and in 2 (5.8%) cases with Potsic stage IV iatrogenic and residual CC were observed. The preoperative air-bone gap (ABG) values demonstrated a notable postoperative improvement, declining from a mean of 31.4 ± 8.1 dB preoperatively to 13.5 ± 3.6 dB (p < 0.001). ETEES, with the advantage of being able to visualize concealed areas of the middle ear with minimal bone work, is a feasible, safe, and effective surgical technique in the treatment of CC, even when CC extends into the mastoid, offering comparable audiological outcomes with lower recurrence ratios relative to conventional surgical techniques present in the current literature.

Relationship between timing of tympanostomy tube insertion and mastoid air cell development in children with otitis media.

ABSTRACTIntratympanic membrane cholesteatoma (ITMC) is a rare type of cholesteatoma located within the tympanic membrane. Surgery is a treatment option if the ITMCs do not spontaneously disappear. Transcanal endoscopic ear surgery is anticipated to become the standard approach in the future.

To analyze the clinical characteristics of bilateral cholesteatoma in children and summarize the clinical treatment pathway. In this retrospective cohort study, we analyzed the records of children with bilateral middle ear cholesteatoma who underwent surgery in our department between 2016 and 2023. The clinical characteristics, treatment and prognosis were analyzed. The records of 12-children (24-ears, 4girls and 8-boys) were reviewed. The mean age was 6.5 ± 2.6-years. Four children had Congenital Cholesteatoma (CC) in one ear and Acquired Cholesteatoma (AC) in the other ear and 8-children had AC in both ears. The onset time and lesion degree of the bilateral ears are different. Nine children had maxillary developmental deformities (75%). One child underwent simultaneous binaural surgery, and 11 underwent staged surgery, with a median interval of 3-months between surgeries. The follow-up period was 4.28 ± 2.58-years. No postoperative complications. Six children (7-ears) underwent revision surgery due to recurrence. There was no significant difference in hearing results between pure-tone average of preoperative (41.23 ± 16.50 dB) and postoperative (37.79 ± 14.42 dB) (p = 0.291, t = 1.082). There was no significant difference in postoperative hearing between CC and AC (p = 0.355, F = 4.268). There were significant differences in postoperation hearing among different surgical methods (p = 0.006, H = 12.630). There were significant differences in postoperation hearing among different ossiculoplasty methods (p = 0.001, H = 17.590). In our cohort, the average age at diagnosis of bilateral cholesteatoma in children was notably young, with a high proportion presenting concurrent cleft palate deformities. Staged surgical intervention prioritizing the more severely affected ear was adopted for bilateral cases. Furthermore, long-term follow-up is necessary due to the observed high recurrence rate in this population. A management flowchart has been proposed based on our therapeutic experience. Level 4.

Pediatric Cholesteatoma: A Case Report.

Congenital stenosis and atresia of the external auditory canal are the most common developmental anomalies of the external ear. Cases of combination of these malformations with cholesteatoma of the temporal bone have been repeatedly described in the world literature. This combined pathology is more typical of congenital stenosis of the external auditory canal. Total atresia of the external auditory canal is rarely associated with cholesteatoma. Cholesteatoma found behind the atretic plate is classified as congenital, excluding other possible mechanisms of it's formation. The article presents two clinical cases of a combination of congenital cholesteatoma and complete atresia of the external auditory canal: in an 8-year old girl and a 19-year old boy without the development of intratemporal and extratemporal complications.

The main objective of this study was to compare the residual and recurrence rates in canal wall up, canal wall down, and canal wall down with reconstruction tympanoplasty for the treatment of cholesteatoma, considering the different filling materials used: no mastoid obliteration versus obliteration using Bone Pate versus obliteration using G45S5 (Glassbone®). This was a retrospective cohort study conducted at a single tertiary medical center. Patient who underwent canal wall up, canal wall down or canal wall down with reconstruction tympanoplasty for a cholesteatoma (primary, secondary, recurrent or residual), with or without mastoid obliteration, with Bone Pate or G45S5 between January 2007 and March 2023 were included. Patients with congenital cholesteatoma and with a follow-up less than 6 months or with any other type of surgery techniques were excluded. A hundred and eight (46%) surgeries were performed without any mastoid obliteration, 66 (28%) with Bone Pate obliteration and 62 (26%) with G45S5 obliteration with a total of 236 surgeries. Survival analysis revealed significantly better outcomes for the Bone Pate cohort compared to without obliteration cohort on the recurrence and recidivism rate (HR 0.26; p = 0.03 and HR 0.39; p = 0.01 respectively). However, none of the statistical analyses showed significant differences between the non-obliteration cohort and the G45S5 cohort. Our study highlights the benefits of Bone Pate obliteration in cholesteatoma surgery. However, it does not provide conclusive evidence on the effectiveness of G45S5 obliteration in preventing residual and recurrent cholesteatoma.

Congenital middle ear cholesteatoma: A case report and literature review

Routine health check-ups and medical consultations are essential methods for the early identification of congenital cholesteatoma (CC). This study aims to examine the differences in disease staging, surgical approaches, duration of surgery, length of hospital stay, and hospitalization costs between cases of CC detected during routine health check-ups and those identified through medical consultations. A retrospective review was performed on 28 pediatric patients diagnosed with CC at the Children's Hospital of Chongqing Medical University between January 2018 and March 2024. The patients were categorized based on the method of CC detection-either through routine health check-ups or medical consultations. Subsequently, the study assessed variables including patient age, Potsic stage, surgical methods, duration of surgery, length of hospital stay, and hospitalization costs for each category. A cohort of 28 patients were included in this study, with an average age of 6.02±1.99 years (range: 3.25-10.33y). The mean hospitalization duration was 9.00 days (interquartile range: 6.25-11.00d), and the average operation time was 123±64 minutes (range: 30-320min). Significant differences were observed between cases of CC identified through different detection methods in terms of Potsic stage ( P =0.011), surgical approach ( P =0.02), surgical duration ( P =0.028), length of hospital stay ( Z =3.84, P =0.000), and hospitalization costs ( Z =3.99, P =0.000). In addition, a significant positive correlation was found between Potsic stage and age ( r =0.434, P =0.021), detection method ( r =0.572, P =0.001), and surgical approach ( r =0.954, P =0.000). The early detection of congenital cholesteatomas through physical examinations is significant for effective treatment, enhanced prognosis, and enhanced quality of life for patients.

The main objective of this report is to describe a unique clinical case of a congenital cholesteatoma with complete lateral displacement of the cochlea and aggressive erosion of the internal auditory canal and lateral semicircular canals in a very young child. This report involves a 10-month-old female who initially presented with left intermittently bloody otorrhea at 2.5 months of age and audiology testing in the clinic demonstrated sensorineural hearing loss in the left ear. Initial computerized tomography (CT) revealed a destructive left temporal bone lesion displacing the left otic capsule and vestibular aqueduct with erosion and involvement of the inner ear structures including erosion into the cochlea and semicircular canals, and posterior fossa dura. A left transtemporal approach for cholesteatoma removal and ear canal closure was done. 4 months postoperatively, the patient required revision of the left transtemporal approach to the posterior fossa for recurrent cholesteatoma in the hypotympanum adjacent to the carotid artery and eustachian tube. This case is unique in the complete lateral displacement of the cochlea and aggressive erosion of the internal auditory canal and lateral semicircular canals in a very young child. It serves to remind otolaryngologists of the silent yet infiltrative nature of congenital cholesteatoma. The very young age of the patient and the presence of otorrhea at 1 month of age raise the question of whether this lesion could have been developing in utero. It lends support to the theory of developmental epithelial rests in congenital cholesteatoma.

Objective:To retrospectively analyze the clinical features and surgical efficacy of congenital cholesteatoma （CC） and acquired cholesteatoma （AC） in children. Methods:Clinical data of 169 children with middle ear cholesteatoma were reviewed in the Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from January 2010 to July 2020. The clinical characteristics, stages, surgical methods, and postoperative recurrence rates were analyzed and summarized. Results:The age distribution of enrolled children ranged from 2 to 14 years. The mean age of the CC group was （5.60±2.48） years compared with （6.45±2.48） years in the AC group, and the difference was statistically significant （P<0.05）. Preoperative hearing in the CC group was （40.06±13.52） dB HL, which was better than in the AC group at （48.40±13.84） dB HL （P<0.05）. The proportion of stage Ⅰ in the CC group was lower than that in the AC group according to EAONO/JOS staging （P<0.05）. The recurrence rate after primary surgery was 19.23% （10/52） in the CC group compared with 36.29% （45/124） in the AC group （P<0.05）. The mastoid retention rates after all operations were 28.85% （15/52） in the CC group and 5.65% （7/124） in the AC group （P<0.05）. Conclusion:Compared with congenital cholesteatoma, acquired cholesteatoma in children is more aggressive and has more complications, higher postoperative recurrence rate, and less possibility of mastoid retention. Early clinical detection and treatment are required, and canal wall-down tympanoplasty should be considered in surgery.

Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal clonal proliferation of Langerhans cells, which are antigen-presenting cells derived from the bone marrow, along with inflammatory cell infiltration. The disease can present as a solitary lesion or multisystem lesions, reflecting a diverse clinical spectrum. LCH lesions in the head and neck region are relatively common, accounting for approximately 70% of all the cases, and these patients are often evaluated by otolaryngologists.