OBJECTIVE: The purpose of this study was to explore the characteristics of external auditory canal cholesteatoma (EACC) among children and to describe its radiological findings on high-resolution computed tomography (CT) of the temporal bone in order to improve the diagnostic accuracy of primary EACC.
METHODS: The clinical records and CT imaging features of 44 patients who were diagnosed with EACC between January 2017 and May 2022 at Shenzhen Children\'s Hospital were retrospectively reviewed. Clinical features, including external auditory canal wall findings, hearing damage, symptoms and physical examination findings, were analysed against the level of lesion involvement. The correlation between different types of EACC and the incidence of different clinical symptoms was analysed, and the degree of hearing impairment and the rate of bone wall destruction were examined using CT.
RESULTS: The mean age at EACC onset was 9.02 ± 3.15 years, and the mean age at onset for EACC involving the right ear was older than that of EACC involving the left ear (P < 0.05). There were 44 patients (46 ears), including 10 ears with type I EACC, 23 ears with type II EACC, and 13 ears with type III EACC. Conductive hearing loss was the main type of hearing impairment observed among EACC patients. There were differences in types I, II and III EACC in terms of hearing impairment; specifically, there was a significant difference in moderate hearing impairment between type II and type III EACC patients (P < 0.05). The four most common symptoms were otorrhea, otalgia, itching and bleeding. The incidence of itching symptoms was greater in type I EACC than the incidence of otorrhea, and the incidence of otorrhea symptoms in type II and type III EACC was significantly greater than that in type I EACC(P < 0.05). There were no significant differences in the fracture rates of the anterior, posterior, superior or inferior walls of the external auditory canal within or between type II and type III EACC patients (P > 0.05). The failure rate of scute damage was significantly higher in type III EACC patients than in type II EACC patients (P < 0.05).
CONCLUSIONS: The presence of otorrhea and hearing loss as well as the identification of granulation tissue during otoscopy suggest the need for a temporal bone CT scan. This imaging modality can aid in the early detection and accurate classification of EACC, thereby guiding the selection of appropriate surgical interventions and greatly assisting in preventing further progression of hearing impairment.

Pediatric cholesteatomas (PC) have multifactorial aetiology, spread aggressively and there are high chances of residual/ recurrent disease after treatment. The surgical technique to manage this entity has been debatable. This study was done to (i) enumerate the presentation of PC and the surgical techniques adopted (ii) analyse the outcomes viz., residual/ recurrence rates and hearing results. A cross sectional record based study was done on 618 cases of PC operated between 1983 and 2015, at a centre dedicated to otology and lateral skull base surgery. The data which was maintained on the basis of clinical and peri- operative findings was analysed. Otorrhea (59.2%) and hearing loss (54.2%) were the common symptoms. The surgeries done were: canal wall up (CWU) (44.3%), canal wall down (CWD) (41.1%), modified bondy\'s mastoidectomy (5.7%), radical mastoidectomy (4.9%), trans canal excision (1.8%) and subtotal petrosectomy (2.3%). The residual and recurrence rates were 12.6% and 7.9% respectively. A significant difference between the pre and post operative hearing was observed. The mean improvement in air bone gap was 7.7db. Residual/ recurrent disease were higher in CWU as compared to CWD group. The surgery should be individualised so that the patient remains disease free.

UNASSIGNED: Cholesteatoma usually harbors a poly-microbial infection. As the diversity of bacterial pathogens in the Indian COM is unknown, we set out to identify the bacteria associated with cholesteatoma disease in different patients of North India using targeted metagenomic analysis of the 16 S rRNA gene.
UNASSIGNED: We recruited 15 patients of cholesteatomatous chronic otitis media (COM), who underwent surgical disease clearance. We divided these patients into four groups based on the four clinic-radiological stages categorized as per the EAONO/JOS joint consensus statement classification. Representative samples were extracted during the surgery and sent for bacterial culture and sensitivity and 16 S rRNA gene metagenomic analysis.
UNASSIGNED: While 12 (80%) of the patients belonged to clinical Stage I/II; one patient had an extracranial complication (stage III) and two patients had an intracranial complication (stage IV). Our detailed bacterial metagenomics analyses showed that while phylum Proteobacteria was most abundant (reads up to ∼ 95%) in specimens from nine patients, phylum Firmicutes was most abundant (up to ∼ 80%) in specimens from four patients. Gamma (γ) Proteobacteria and Epsilon (ε) Proteobacteria were the most abundant class amongst Proteobacteria. Class Tissierellia stood out as the most abundant Firmicutes (40-60%), followed by Clostridia (20%) and Bacilli (10%). There was negligible difference in the bacterial profiles across all four clinical stages.
UNASSIGNED: Cholesteatoma is primarily associated with Proteobacteria and Firmicutes phyla, even in complicated disease. Further studies with a larger sample size are required to validate our findings.
UNASSIGNED: The online version contains supplementary material available at 10.1007/s12070-024-04678-9.

Sinus tympani is one of the hidden space in the retrotympanic area of middle ear which is commonly invaded by cholesteatoma and known for recidivism. Conventional surgical approaches sometimes fail to access deep sinuses and eradicate the disease. The aim of our study was to measure endoscopic depth of Sinus tympani in Indian Population. Total of 30 patients underwent mastoid exploration and intraoperatively depth of Sinus Tympani was measured using a 0.5 mm calibrated probe with 30 degree rigid Hopkins endoscope. Endoscopic depth in the present study ranges between 2.5 and 5.5 mm. It was higher in right ear in case of females however no significant difference were seen in males and females on left side. It is difficult to clear disease from Sinus Tympani because of its inaccessible location. Sinus Tympani can also show variations in terms of its depth further making difficult to clear disease.

BACKGROUND: Temporal bone computed tomography (CT) helps diagnose chronic otitis media (COM). However, its interpretation requires training and expertise. Artificial intelligence (AI) can help clinicians evaluate COM through CT scans, but existing models lack transparency and may not fully leverage multidimensional diagnostic information.
OBJECTIVE: We aimed to develop an explainable AI system based on 3D convolutional neural networks (CNNs) for automatic CT-based evaluation of COM.
METHODS: Temporal bone CT scans were retrospectively obtained from patients operated for COM between December 2015 and July 2021 at 2 independent institutes. A region of interest encompassing the middle ear was automatically segmented, and 3D CNNs were subsequently trained to identify pathological ears and cholesteatoma. An ablation study was performed to refine model architecture. Benchmark tests were conducted against a baseline 2D model and 7 clinical experts. Model performance was measured through cross-validation and external validation. Heat maps, generated using Gradient-Weighted Class Activation Mapping, were used to highlight critical decision-making regions. Finally, the AI system was assessed with a prospective cohort to aid clinicians in preoperative COM assessment.
RESULTS: Internal and external data sets contained 1661 and 108 patients (3153 and 211 eligible ears), respectively. The 3D model exhibited decent performance with mean areas under the receiver operating characteristic curves of 0.96 (SD 0.01) and 0.93 (SD 0.01), and mean accuracies of 0.878 (SD 0.017) and 0.843 (SD 0.015), respectively, for detecting pathological ears on the 2 data sets. Similar outcomes were observed for cholesteatoma identification (mean area under the receiver operating characteristic curve 0.85, SD 0.03 and 0.83, SD 0.05; mean accuracies 0.783, SD 0.04 and 0.813, SD 0.033, respectively). The proposed 3D model achieved a commendable balance between performance and network size relative to alternative models. It significantly outperformed the 2D approach in detecting COM (P≤.05) and exhibited a substantial gain in identifying cholesteatoma (P<.001). The model also demonstrated superior diagnostic capabilities over resident fellows and the attending otologist (P<.05), rivaling all senior clinicians in both tasks. The generated heat maps properly highlighted the middle ear and mastoid regions, aligning with human knowledge in interpreting temporal bone CT. The resulting AI system achieved an accuracy of 81.8% in generating preoperative diagnoses for 121 patients and contributed to clinical decision-making in 90.1% cases.
CONCLUSIONS: We present a 3D CNN model trained to detect pathological changes and identify cholesteatoma via temporal bone CT scans. In both tasks, this model significantly outperforms the baseline 2D approach, achieving levels comparable with or surpassing those of human experts. The model also exhibits decent generalizability and enhanced comprehensibility. This AI system facilitates automatic COM assessment and shows promising viability in real-world clinical settings. These findings underscore AI\'s potential as a valuable aid for clinicians in COM evaluation.
BACKGROUND: Chinese Clinical Trial Registry ChiCTR2000036300; https://www.chictr.org.cn/showprojEN.html?proj=58685.

OBJECTIVE: The aim of this article was to systematically review the literature on the pediatric population surgically treated for cholesteatoma and describe the applied post-operative follow-up strategies.
METHODS: A systematic review was conducted following the Primary Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement recommendations. After running the selected search string in PubMed, Scopus and Google Scholar, studies in English, reporting on surgically treated pediatric cholesteatoma patients (age younger or equal to 18 year-old) were retrieved. Both primary and revision cholesteatoma surgeries were included. Articles lacking specific data on post-surgical follow-up and case series with less than 10 patients were excluded.
RESULTS: Nineteen papers, published between 2000 and 2023, were included for final analysis. Fourteen studies were retrospective and five prospective, for a total of 1319 patients and 1349 operated ears. Male to female ratio was 1.8:1, with a mean age at surgery of 10.4 years (range 1-18). The mean length of the follow-up after surgery was 4.4 ± 1.7 years (range 1-6.9). Clinical follow-up was detailed in 9 studies (47%) with otomicroscopy being the most common evaluation. In most articles (n = 8, 50%), MRI alone was utilized for radiological follow-up, while in 3 studies (19%), CT scans were employed exclusively. In 5 studies (31%), MRI was combined with CT scans. The timing of radiological investigations varied widely (ranging from 6 months to 3 years). A second-look strategy was reported in 14 studies (74%).
CONCLUSIONS: This systematic review highlights the heterogeneity of the follow-up strategies applied to pediatric patients after cholesteatoma surgery, both in terms of timing and types of investigations.

OBJECTIVE: Chronic otitis media with cholesteatoma is a frequent disease entity in otology, requiring surgery in overwhelming majority of cases. Despite the huge burden there is no established grading system available to assess the severity and extent of disease preoperatively until date. Aim of our study is to assess the applicability of ChOLE staging to preoperative HRCT temporal bone in Chronic otitis media with cholesteatoma.
METHODS: Patients clinically diagnosed as COM with cholesteatoma, who underwent preoperative HRCT temporal bone imaging and mastoid exploration at our tertiary care centre were included. Preoperative radiology was assessed and a radiological ChOLE (r-ChOLE) was given by radiologist. This was then compared with the postop ChOLE.
RESULTS: 21 patients were included in the study. Data was linear and normally distributed (Shapiro wilk test). Pearson\'s product-moment correlation used to see relationship between radiological and postop Total score showed strong statistically significant positive correlation with correlation coefficient (r) of 0.977. Paired t test showed p value was 0.329 (p > 0.05) suggesting no significant difference between radiological and postop Total scores. Cohen kappa test of agreement was applied. It revealed an overall strong agreement (p < 0.001).
CONCLUSIONS: ChOLE staging may be extended to preoperative HRCT of temporal bone in COM with cholesteatoma (rCHOLE). A preoperative radiological staging will help in better prioritizing, planning and execution of tympanomastoid surgeries.

Objectives: Osteomas in the external auditory canal (EAC) can lead to stenosis, and impair epithelium migration and self-cleaning capability, thereby trapping keratinized epithelium and triggering the development of cholesteatoma. Our study aims to identify the risk of cholesteatoma development in patients with osteoma and proposes a stepwise approach to managing patients with EAC osteoma. Methods: The maximum diameter of the osteoma was measured in axial and coronal views on high-resolution computed tomography (HRCT). We calculated the relative obstruction ratio caused by the osteoma in the axial and coronal views. Prior to surgery, otoscopy was employed to identify pedicle formation. The patients were categorized into 2 groups based on the presence of cholesteatoma. Results: We identified 43 patients diagnosed with EAC osteoma. A total of 9 (20.9%) patients with EAC osteomas developed cholesteatoma and the other 34 (79.1%) did not. The maximum diameter of osteomas with and without cholesteatoma was 12.67 ± 4.09 and 7.67 ± 3.27 mm, respectively (P < .001). In the group without cholesteatoma, 21 osteomas had pedicles while the other 13 did not. In the cholesteatoma group, 2 osteomas had pedicles and 7 did not (P = .037). No difference was observed in the relative obstruction ratio between these 2 groups. Conclusions: Our findings indicate that larger osteomas are more likely to develop cholesteatoma, while the formation of a pedicle may reduce the occurrence of cholesteatoma. In symptomatic patients, preoperative evaluation, including HRCT and otoscopy, is vital for assessing the extent of the osteoma and the potential coexistence of cholesteatoma. These factors are critical for preoperative consultations and surgical planning.

OBJECTIVE: Previous studies have also associated cleft palate with increased cholesteatoma risk. Despite this close relation, the incidence of cholesteatoma and associated otologic issues in patients with ectodermal dysplasia types highly associated with cleft palate (EDT-ACPs) has not been formally analyzed. This study provides insight to guide clinicians caring for patients with ED types associated with cleft palate.
METHODS: Individuals with TP63 disorders and Goltz syndrome/Focal Dermal Hypoplasia in the National Foundation for Ectodermal Dysplasia database were contacted for participation in an online REDCap survey from Sept-Dec \'22. Descriptive statistics were generated using SAS JMP Pro 17 statistical software.
RESULTS: 65 individuals participated in the survey (response rate approx. 18 %). The median [IQR] age was 22 [14, 43], 41 (63 %) were female, and Ectrodactyly-Ectodermal Dysplasia-Cleft Lip/Palate Syndrome (EEC) was most common (n = 26, 40 %). We found that, among our respondents with a history of cleft palate, the incidence of cholesteatoma was 39 %. Among respondents without a history of cleft palate, the incidence of cholesteatoma was 13 % CONCLUSIONS: Otologic issues, such as cholesteatoma, can have permanent implications including hearing loss that can be minimized by early identification and treatment. The estimated incidence of cholesteatoma among our participants is far above the estimated incidence of cholesteatoma in the general population with and without a history of cleft palate, suggesting an independent contribution of EDT-ACPs to the risk of cholesteatoma.

BACKGROUND: Mastoiditis frequently occurs in children as they are more susceptible to middle ear infections, but infrequently occurs in adults. A rare complication that results from mastoiditis and an obstructing cholesteatoma is a Bezold\'s abscess, of which there are less than 100 reported cases in literature to date.
METHODS: Here, we present a case of a 72-year-old Caucasian man who has had no history of prior ear infections and was found to have a cholesteatoma and advanced acute coalescent mastoiditis complicated by a Bezold\'s abscess.
CONCLUSIONS: Bezold\'s abscess is a rare entity infrequently encountered in the modern era, likely owing to more prompt treatment of otitis media. Cholesteatoma poses a great risk for both the development of otitis media and further progression to mastoiditis and its associated complications, such as Bezold\'s abscess. Knowledge of said abscess is crucial; without prompt recognition, further spread of infection can occur with vascular or mediastinal involvement.