Osteopontin-driven partial epithelial-mesenchymal transition governs the development of middle ear cholesteatoma.

Cholesteatoma is a common disease of the middle ear. Currently, surgical removal is the only treatment option and patients face a high risk of relapse. The molecular basis of cholesteatoma remains largely unknown. Here, we show that Osteopontin (OPN), a predominantly secreted protein, plays a crucial role in the development of middle ear cholesteatoma. Global transcriptome analysis revealed the loss of epithelial features and an enhanced immune response in human cholesteatoma tissues. Quantitative RT-PCR and immunohistochemical staining of middle ear cholesteatoma validated the reduced expression of epithelial markers, as well as the elevated expression of mesenchymal markers including Vimentin and Fibronectin, but not N-Cadherin, α-smooth muscle actin (α-SMA) or ferroptosis suppressor protein 1 (FSP1), indicating a partial epithelial-mesenchymal transition (EMT) state. Besides, the expression of OPN was significantly elevated in human cholesteatoma tissues. Treatment with OPN promoted cell proliferation, survival and migration and led to a partial EMT in immortalized human keratinocyte cells. Importantly, blockade of OPN signaling could remarkably improve the cholesteatoma-like symptoms in SD rats. Our mechanistic study demonstrated that the AKT-zinc finger E-box binding homeobox 2 (ZEB2) axis mediated the effects of OPN. Overall, these findings suggest that targeting the OPN signaling represents a promising strategy for the treatment of middle ear cholesteatoma.

Cholesterol Granuloma From a Developmental Odontogenic Cyst: A Report of a Rare Case and a Literature Review.

Dentigerous cysts are the second most common developmental odontogenic cysts that develop around the crown of unerrupted teeth with the maxillary canine region being one of the common sites of occurrence. The cystic lining of this lesion has been shown to develop into ameloblastoma, Muco epidermoid carcinoma, and squamous cell carcinomas. However, the development of cholesterol granuloma (CG) in the cystic lining of a dentigerous cyst is extremely rare. CG is a histological observation distinguished by the presence of a conglomeration of connective tissue and granulation tissue. The condition is predominantly seen in the field of otolaryngology, with very few cases reported in the maxillofacial region, most of which are associated with the maxillary sinus. This article presents the findings of a CG in a 39-year-old male patient that developed within the dentigerous cyst and discusses the possible etiopathogenesis, surgical management, and histological presentation.

Fusion computed tomography-magnetic resonance imaging scans for pre-operative staging of congenital middle-ear cholesteatoma.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Middle Ear Cholesteatoma and Vestibular Schwannoma Resection Followed by Cochlear Implant: Surgical Challenges and Audiological Outcomes.

(1) Background: The occurrence of vestibular schwannoma (VS) associated with cholesteatoma is rare. A hearing impairment is one of the most significant issues in such cases. Moreover, the presence of middle and inner ear pathologies combined may represent a surgical challenge. No studies have described a combined surgical approach for these coexisting conditions (VS and cholesteatoma), nor the hearing rehabilitation outcomes of using cochlear implants for these patients. (2) Case Report: This paper is on a female patient who underwent simultaneous surgical treatments for VS and middle ear cholesteatoma in the right ear followed by a cochlear implant, describing the technique and the audiological results. (3) Conclusions: The surgical approach was successful and enabled the resection of lesions with the auditory nerve and cochlea preservation. Cochlear implantation in the right ear showed positive postoperative results, with an improvement in the results with the CI in silent and noisy environments.

High-Resolution Computed Tomography and Intraoperative Correlation in Cholesteatoma: Enhancing Preoperative Evaluation and Surgical Management.

INTRODUCTION: Cholesteatoma, a hazardous non-neoplastic lesion of the temporal bone, is prevalent in socio-economically disadvantaged groups in developing nations like India. Timely detection and surgical intervention are essential for effective management. High-resolution computed tomography (HRCT) has revolutionized the assessment of temporal bone pathology, though its role in preoperative evaluation remains debated. This study aimed to validate HRCT's utility in diagnosing cholesteatoma, compare its findings with intraoperative observations, and assess sensitivity and specificity. METHODS: This diagnostic accuracy study was conducted at a tertiary care center in Western India, from March 2021 to November 2022. HRCT findings of 54 cholesteatoma patients were evaluated and compared with intraoperative findings. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), accuracy, and Cohen's kappa coefficient were calculated. RESULTS: HRCT demonstrated a sensitivity exceeding 90% in identifying scutum erosion, mastoid sclerosis, and abnormalities in the tympanic membrane, along with a specificity surpassing 90% in detecting various conditions, including facial canal erosion, sinus plate erosion, lateral semicircular canal erosion, erosion of the posterior wall of the external auditory canal, and abnormalities in the tympanic membrane. Furthermore, HRCT exhibited an accuracy of over 90% in detecting most pathologies. There was a perfect or near-perfect agreement observed for abnormal tympanic membrane, sinus plate erosion, mastoid sclerosis, and erosion of the posterior wall of the external auditory canal (with kappa values > 0.8). Moderate to fair agreement was noted for other pathologies. CONCLUSION: HRCT offered precise detection of the majority of pathologies, thereby facilitating surgical planning. However, the presence of limitations in distinguishing specific abnormalities highlights the significance of utilizing HRCT in tandem with other diagnostic modalities to ensure meticulous diagnosis and effective treatment planning.

Clinical analysis, diagnosis, and treatment strategies for otogenic brain abscesses.

Objective: To explore the clinical features, diagnosis, and treatment strategies for otogenic brain abscesses (OBA). Methods: Clinical data from 10 patients with OBA were analyzed retrospectively. Clinical characteristics, diagnosis, and treatment experiences were summarized. Results: Two were first diagnosed in the Department of Otorhinolaryngology, 5 in Neurosurgery, and 3 in other departments. There were 6 cases of temporal lobe abscess and 4 cases of cerebellar abscesses. Five cases were accompanied by 1 or more intracranial complications. Headache is a common presentation in all cases. The main pathogenic bacteria were anaerobic bacteria. All patients had no previous ear or brain surgery history, and no history of traumatic brain injury, 7 received surgical treatment in the neurosurgery and/or otolaryngology department. Two patients died, the other 8 fully recovered and so were discharged. Conclusions: Diagnosis and treatment of OBA must involve multiple departments. Multidisciplinary consultation (MDT) is crucial to the success of the first OBA diagnosis. The diagnosis and treatment team develops personalized treatment plans by integrating MDT treatment opinions and combining the actual condition of patients, thereby making the diagnosis and treatment of OBA accurate and timely.

Analysis of mRNA m6A modification and mRNA expression profiles in middle ear cholesteatoma.

Introduction: Middle ear cholesteatoma is characterized by the hyperproliferation of keratinocytes. In recent decades, N6-methyladenosine (m6A) modification has been shown to play an essential role in the pathogenesis of many proliferative diseases. However, neither the m6A modification profile nor its potential role in the pathogenesis of middle ear cholesteatoma has currently been investigated. Therefore, this study aimed to explore m6A modification patterns in middle ear cholesteatoma. Materials and methods: An m6A mRNA epitranscriptomic microarray analysis was performed to analyze m6A modification patterns in middle ear cholesteatoma tissue (n = 5) and normal post-auricular skin samples (n = 5). Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analyses were performed to predict the potential biological functions and signaling pathways underlying the pathogenesis of middle ear cholesteatoma. Subsequently, m6A modification levels were verified by methylated RNA immunoprecipitation-qPCR (MeRIP-qPCR) in middle ear cholesteatoma tissue and normal skin samples, respectively. Results: A total of 6,865 distinctive m6A-modified mRNAs were identified, including 4,620 hypermethylated and 2,245 hypomethylated mRNAs, as well as 9,162 differentially expressed mRNAs, including 4,891 upregulated and 4,271 downregulated mRNAs, in the middle ear cholesteatoma group relative to the normal skin group. An association analysis between methylation and gene expression demonstrated that expression of 1,926 hypermethylated mRNAs was upregulated, while expression of 2,187 hypomethylated mRNAs and 38 hypermethylated mRNAs was downregulated. Moreover, GO analysis suggested that differentially methylated mRNAs might influence cellular processes and biological behaviors, such as cell differentiation, biosynthetic processes, regulation of molecular functions, and keratinization. KEGG pathway analysis demonstrated that the hypermethylated transcripts were involved in 26 pathways, including the Hippo signaling pathway, the p53 signaling pathway, and the inflammatory mediator regulation of transient receptor potential (TRP) channels, while the hypomethylated transcripts were involved in 13 pathways, including bacterial invasion of epithelial cells, steroid biosynthesis, and the Hippo signaling pathway. Conclusion: Our study presents m6A modification patterns in middle ear cholesteatoma, which may exert regulatory roles in middle ear cholesteatoma. The present study provides directions for mRNA m6A modification-based research on the epigenetic etiology and pathogenesis of middle ear cholesteatoma.

[A case report of middle ear cholesteatoma complicated with labyrinthine fistulaand delayed endolymphatic hydrops].

Delayed endolymphatic hydrops （DEH） is a rare disease that causes vertigo and is often misdiagnosed as other vertigo diseases. This article reports on a patient with vertigo who was easily misdiagnosed. The patient was a middle ear cholesteatoma complicated by labyrinthine fistula （LF）; however, his vertigo was episodic vertigo, which could not be explained solely by LF causing labyrinthitis. The possibility of endolymphatic hydrops was suspected, which was confirmed by inner ear magnetic resonance gadolinium imaging. This is the first reported case of middle ear cholesteatoma complicated by LF and DEH. The patient underwent surgical resection of the cholesteatoma and three semicircular canal obstructions at the same time. During two years postoperative follow-up, the patient did not experience a recurrence of vertigo. When diagnosing vertigo diseases, a careful history of vertigo is of utmost importance.

Colesteatoma congénito: revisión de la literatura / Congenital cholesteatoma: literature review

El colesteatoma congénito es una entidad clínica única y desafiante, que se caracteriza por acumulación anormal de queratina en el oído medio, medial a la membrana timpánica. Se presenta, mayoritariamente, en el género masculino, con una incidencia estimada de 0.12 por 100.000 habitantes, representando el 4% a 24% de los colesteatomas en población pediátrica y un 2% a 5% del total de colesteatomas. Su origen aún es controversial, siendo la teoría más aceptada, la del arresto epitelial. Su diagnóstico es clínico, variando la sintomatología según severidad del compromiso, presentándose desde hallazgo incidental, hipoacusia de conducción, hasta presentar otalgia y perforación timpánica. Las imágenes se consideran un apoyo complementario preoperatorio. El tratamiento es quirúrgico, con diferentes técnicas disponibles, las cuales se deben definir de manera individual en el caso de cada paciente. Es fundamental su diagnóstico y manejo precoz, para lograr un tratamiento oportuno con menor tasa de complicaciones y compromiso a largo plazo. A continuación, se presenta una revisión de la literatura respecto de esta patología, para difusión en nuestro medio.

Congenital cholesteatoma (CC) is a unique and challenging clinical entity characterized by abnormal accumulation of keratin in the middle ear, medial to the tympanic membrane, being more frequent in the male gender, with an estimated incidence of 0.12 per 100,000 inhabitants. It represents 4% to 24% of cholesteatomas in the pediatric population and 2% to 5% of all cholesteatomas. Its cause is still controversial, the most accepted theory being epithelial arrest. The diagnosis is clinical, varying the symptoms according to the severity of the compromise, from incidental finding, through conduction hearing loss, to presenting otalgia and tympanic perforation. Images are considered additional preoperative support. Treatment is predominantly surgical, with different techniques available, which must be defined individually. Its early discovery and management are essential to achieve proper treatment with a lower rate of complications and long-term commitment. We present a review of the literature regarding CC to provide information relevant to our area of expertise.

Bacterial diversity of middle ear cholesteatoma by 16S rRNA gene sequencing in China.

In this study, the bacterial diversity of acquired middle ear cholesteatoma (MEC) was evaluated to reveal its pathogenesis and provides a guide for the use of antibiotics. Twenty-nine cases of acquired MEC and eight cases of healthy middle ears undergoing cochlear implantation (CI) were evaluated. Full-length 16S rRNA gene sequencing was performed to profile the bacterial communities in lesions and healthy tissues of the middle ear. ACE (P = 0.043) and Chao1 (P = 0.039) indices showed significant differences in alpha diversity (P < 0.05). Analysis of PERMANOVA/Anosim using the Bray-Curtis distance matrix results suggested that the between-group differences were greater than the within-group differences (R = 0.238, P < 0.05, R2 = 0.066, P < 0.05). Bacterial community analysis revealed that Alphaproteobacteria at the class level and Caulobacterales and Sphingomonadales at the order level were significantly different (P < 0.05). In the LefSe (Linear discriminant analysis effect size) analysis, Porphyromonas bennonis was elevated, and Bryum argenteum and unclassified Cyanobacteriales were reduced at the species level in MEC (P < 0.05). Fifteen metabolic pathways were found to be significantly different between the two groups by analysing the abundance of metabolic pathways in level 2 of the Kyoto Encyclopaedia of Genes and Genomes (KEGG). Seven and eight metabolic pathways were significantly elevated in the MEC and control groups, respectively (P < 0.05). The role of bacteria in the pathogenesis of acquired MEC was further refined through analysis of metabolic pathways. These findings indicate that the acquired MEC and healthy middle ear contain more diverse microbial communities than previously thought.

[Etiology analysis and diagnosis of noninflammatory conductive hearing loss in children].

Objective:To investigate the etiology, diagnosis and treatment of noninflammatory conductive hearing loss in children. Methods:The clinical data of children patients admitted to the Eye & ENT Hospital of Fudan University from January 2019 to November 2022 were retrospectively analyzed. Results:A total of 179 cases（189 ears） were analyzed. The main symptoms from high to low were: ear tightness, hearing loss, earache, and facial paralysis. The degree of hearing loss was mild in 34 ears（19.5%）, moderate in 70 ears（40.2%）, moderate-severe in 52 ears（29.9%）, severe in 18 ears（10.3%）. The mean hearing threshold of otosclerosis was the highest（63.5±7.8） dB HL, and the mean air-bone gap of ossicular chain malformation was the largest（35.4±9.8） dB HL. The mean hearing threshold of the affected ear was（50.4±14.5） dB HL, and the mean air bone gap was（30.3±10.4） dB HL. After operation, the results were（36.1± 14.5） dB HL and（20.0±8.6） dB HL, respectively. Distribution of surgical methods for ossicular chain reconstruction: 88 ears（46.6%） of TORP, 49 ears（25.9%） of PORP, 8（4.2%） ears of Piston, 9 ears（4.8%） of autogenous ossicular reconstruction, and 35 ears（18.5%） of ossicular chain relaxation. CT diagnostic rate showed more sensitivity to malleus and incus abnormalities, the diagnosis rate of congenital middle ear cholesteatoma was the highest. The mean duration time of diagnosis was（2.2±2.9） years, while the diagnosis of ossicular chain malformation（[5.2±4.2]years）, otosclerosis（[4.4±4.1]years）, tympanosclerosis（[5.4±0.9]years） took longer. Conclusion:In the diagnosis and treatment of noninflammatory conductive hearing loss in children, the combination of detailed medical history, specialized examination and imaging examination can maximize the accuracy of diagnosis and achieve the purpose of personalized comprehensive treatment. Surgical intervention with appropriate timing is important to remove lesions and improve hearing.

An automatic diagnosis model of otitis media with high accuracy rate using transfer learning.

Introduction: Chronic Suppurative Otitis Media (CSOM) and Middle Ear Cholesteatoma are two common chronic otitis media diseases that often cause confusion among physicians due to their similar location and shape in clinical CT images of the internal auditory canal. In this study, we utilized the transfer learning method combined with CT scans of the internal auditory canal to achieve accurate lesion segmentation and automatic diagnosis for patients with CSOM and middle ear cholesteatoma. Methods: We collected 1019 CT scan images and utilized the nnUnet skeleton model along with coarse grained focal segmentation labeling to pre-train on the above CT images for focal segmentation. We then fine-tuned the pre-training model for the downstream three-classification diagnosis task. Results: Our proposed algorithm model achieved a classification accuracy of 92.33% for CSOM and middle ear cholesteatoma, which is approximately 5% higher than the benchmark model. Moreover, our upstream segmentation task training resulted in a mean Intersection of Union (mIoU) of 0.569. Discussion: Our results demonstrate that using coarse-grained contour boundary labeling can significantly enhance the accuracy of downstream classification tasks. The combination of deep learning and automatic diagnosis of CSOM and internal auditory canal CT images of middle ear cholesteatoma exhibits high sensitivity and specificity.

Change in chorda tympani nerve function after two-stage tympanoplasty for cholesteatoma.

Objectives: Chorda tympani nerve (CTN) function may be damaged more by two-stage than by one-stage surgery for middle ear cholesteatoma. However, few studies have reported the relationship between two-stage cholesteatoma surgery and CTN function. This study aimed to investigate CTN function after two-stage surgery for cholesteatoma. Methods: In this prospective study, 35 patients underwent two-stage canal wall up tympanoplasty (CWUT). Perioperative CTN function was assessed using questionnaires and electrogustometry (EGM). Participants were categorized into minor, major, and section groups, based on the degree of CTN manipulation during surgery. Results: In the first-stage surgery, posterior tympanotomy with an intact canal wall reduced the degree of CTN manipulation. The incidence of taste disorder after the first-stage surgery was 71.4%. Postoperative taste disorder and the EGM threshold improved early in the minor manipulation group. In the second-stage surgery, no new CTN damage occurred, even if this surgery involved removal of residual cholesteatoma. The incidence of taste disorder after second-stage surgery was less than that after first-stage surgery, independent of CTN preservation. However, the recovery rate of the EGM threshold after second-stage surgery was significantly lower in the section group than in those with CTN preservation. Conclusion: CTN function, including symptoms and EGM threshold, can be preserved during two-stage cholesteatoma surgery if care is taken to preserve the CTN in both the first- and second-stage surgeries. A two-stage CWUT, ensuring an intact bony annulus, may be effective to facilitate CTN preservation. Level of Evidence: 2b.

Analysis of postoperative effects of different semicircular canal surgical technique in patients with labyrinthine fistulas.

Objective: Different semicircular canal surgery techniques have been used to treat patients with labyrinthine fistulas caused by middle ear cholesteatoma. This study evaluated postoperative hearing and vestibular function after various semicircular canal surgeries. Materials and methods: In group 1, from January 2008 to December 2014, 29 patients with middle ear cholesteatoma complicated by labyrinthine fistulas were treated with surgery involving covering the fistulas with simple fascia. In group 2, from January 2015 to October 2021, 36 patients with middle ear cholesteatoma complicated by labyrinthine fistulas were included. Cholesteatomas on the surface of type I labyrinthine fistulas were cleaned using the "under water technique" and capped with a "sandwich" composed of fascia, bone meal, and fascia. Cholesteatomas on the surface of type II and III fistulas were cleaned using the "under water technique," and the labyrinthine fistula was plugged with a "pie" composed of fascia, bone meal, and fascia, and then covered with bone wax. Results: Some patients with labyrinthine fistulas in group 1 exhibited symptoms of vertigo after surgery. In group 2 Patients with type II labyrinthine fistulas experienced short-term vertigo after semicircular canal occlusion, but no cases of vertigo were reported during long-term follow-up. "sandwich." In patients with type II labyrinthine fistulas, the semicircular canal occlusion influenced postoperative hearing improvement. However, postoperative patient hearing was still superior to preoperative hearing. Conclusion: The surface of type I labyrinthine fistulas should be capped by a "sandwich" composed of fascia, bone meal, and fascia. Type II and III labyrinthine fistulas should be plugged with a "pie" composed of fascia, bone meal, and fascia, covered with bone wax.

Potential otogenic complications caused by cholesteatoma of the contralateral ear in patients with otogenic abscess secondary to middle ear cholesteatoma of one ear: A case report.

BACKGROUND: Otogenic brain abscess caused by middle ear cholesteatoma is a potentially serious and life-threatening complication in the ear, nose, and throat clinic. The mortality rate associated with otogenic brain abscesses is 8%-26.3%. Recently, in China, the incidence of brain abscess secondary to middle ear cholesteatoma has started to increase due to antibiotic resistance. CASE SUMMARY: A 55-year-old male presented hearing loss in the right ear and headache for 1 mo in 2018. Computed tomography (CT) showed an area of low density in the right middle ear and mastoid and auditory ossicle defects and a small amount of soft tissue density in the left middle ear. The parietal wall of the right tympanic cavity and the posterior wall of the mastoid sinus were thin and less continuous. Cranial magnetic resonance imaging revealed an area of low intensity encapsulated by an area of high intensity in the right temporal lobe. We diagnosed him with a brain abscess secondary to middle ear cholesteatoma. He received surgery to drain the abscess followed by a modified radical mastoidectomy. The patient visited our department 3 years later because of intermittent otorrhea in the left ear. CT revealed that the area of the soft tissue density in the left middle ear and mastoid was significantly increased. The posterior wall of the mastoid sinus was destroyed, leaving the left middle ear connecting with the brain. The patient underwent a modified radical mastoidectomy in the left ear. CONCLUSION: Regular follow-up and timely treatment of contralateral ear disease are vital for the prevention of otogenic complications in patients with otogenic abscesses secondary to middle ear cholesteatoma in the unilateral ear.

Application of high resolution computed tomography image assisted classification model of middle ear diseases based on 3D-convolutional neural network. / 基于3Då·ç§¯ç¥žç»ç½‘ç»œçš„ä¸­è€³ç–¾ç— é«˜åˆ†è¾¨çŽ‡CTå›¾åƒè¾ åŠ©åˆ†ç±»è¯Šæ–­æ¨¡åž‹çš„åº”ç”¨.

OBJECTIVES: Chronic suppurative otitis media (CSOM) and middle ear cholesteatoma (MEC) are the 2 most common chronic middle ear diseases. In the process of diagnosis and treatment, the 2 diseases are prone to misdiagnosis and missed diagnosis due to their similar clinical manifestations. High resolution computed tomography (HRCT) can clearly display the fine anatomical structure of the temporal bone, accurately reflect the middle ear lesions and the extent of the lesions, and has advantages in the differential diagnosis of chronic middle ear diseases. This study aims to develop a deep learning model for automatic information extraction and classification diagnosis of chronic middle ear diseases based on temporal bone HRCT image data to improve the classification and diagnosis efficiency of chronic middle ear diseases in clinical practice and reduce the occurrence of missed diagnosis and misdiagnosis. METHODS: The clinical records and temporal bone HRCT imaging data for patients with chronic middle ear diseases hospitalized in the Department of Otorhinolaryngology, Xiangya Hospital from January 2018 to October 2020 were retrospectively collected. The patient's medical records were independently reviewed by 2 experienced otorhinolaryngologist and the final diagnosis was reached a consensus. A total of 499 patients (998 ears) were enrolled in this study. The 998 ears were divided into 3 groups: an MEC group (108 ears), a CSOM group (622 ears), and a normal group (268 ears). The Gaussian noise with different variances was used to amplify the samples of the dataset to offset the imbalance in the number of samples between groups. The sample size of the amplified experimental dataset was 1 806 ears. In the study, 75% (1 355) samples were randomly selected for training, 10% (180) samples for validation, and the remaining 15% (271) samples for testing and evaluating the model performance. The overall design for the model was a serial structure, and the deep learning model with 3 different functions was set up. The first model was the regional recommendation network algorithm, which searched the middle ear image from the whole HRCT image, and then cut and saved the image. The second model was image contrast convolutional neural network (CNN) based on twin network structure, which searched the images matching the key layers of HRCT images from the cut images, and constructed 3D data blocks. The third model was based on 3D-CNN operation, which was used for the final classification and diagnosis of the 3D data block construction, and gave the final prediction probability. RESULTS: The special level search network based on twin network structure showed an average AUC of 0.939 on 10 special levels. The overall accuracy of the classification network based on 3D-CNN was 96.5%, the overall recall rate was 96.4%, and the average AUC under the 3 classifications was 0.983. The recall rates of CSOM cases and MEC cases were 93.7% and 97.4%, respectively. In the subsequent comparison experiments, the average accuracy of some classical CNN was 79.3%, and the average recall rate was 87.6%. The precision rate and the recall rate of the deep learning network constructed in this study were about 17.2% and 8.8% higher than those of the common CNN. CONCLUSIONS: The deep learning network model proposed in this study can automatically extract 3D data blocks containing middle ear features from the HRCT image data of patients' temporal bone, which can reduce the overall size of the data while preserve the relationship between corresponding images, and further use 3D-CNN for classification and diagnosis of CSOM and MEC. The design of this model is well fitting to the continuous characteristics of HRCT data, and the experimental results show high precision and adaptability, which is better than the current common CNN methods.

Immunohistochemical localization of d-ß-aspartic acid in congenital and acquired middle ear cholesteatoma.

Objective/Hypothesis: Middle ear cholesteatoma is characterized by abnormal growth of the keratinizing squamous epithelium of the temporal bone. d-ß-aspartic acid is the major isomer of d-aspartic acid found in elderly tissue. We assessed the immunoreactivity to k-ß-aspartic acid of congenital and acquired middle ear cholesteatomas. Study Design: Case-control studies. Material and Methods: Tissue samples were collected from 21 patients comprising 21 ears with congenital middle ear cholesteatoma and 26 patients comprising 29 ears with acquired type. Their clinical and histopathological features were investigated. We divided the middle ear cholesteatoma samples into three layers: the perimatrix, matrix, and cystic contents. The patterns of immunoreactivity to d-ß-aspartic acid expression were then assessed immunohistochemically. Results: Two patterns of immunoreactivity to d-ß-aspartic acid were detected in middle ear cholesteatoma: infiltrative and diffuse. In congenital middle ear cholesteatoma, d-ß-aspartic acid expression was observed throughout all the layers (perimatrix, matrix, and cystic contents), and immunoreactivity to d-ß-aspartic acid was dramatically strong in all layers. The expression levels of d-ß-aspartic acid to the cystic content and perimatrix were significantly higher in congenital middle ear cholesteatoma than in the acquired type. Conclusions: This study showed the expression levels of d-ß-aspartic acid in middle ear cholesteatoma to differ significantly between congenital and acquired middle ear cholesteatoma. Our results indicate that overexpression of d-ß-aspartic acid is likely to be involved in the pathogenesis of cholesteatoma, and we speculate that d-ß-aspartic acid could be a novel biomarker for, and a therapeutic target in, congenital and acquired middle ear cholesteatoma. Level of Evidence: 4.

Middle ear cholesteatoma prevalence in over 3,600 children with Turner Syndrome.

OBJECTIVE: To investigate the prevalence of middle ear cholesteatoma in children with Turner syndrome (TS) as compared to the general population using a large database. METHODS: The TriNetx Analytics Network, a federated health research network that aggregates the de-identified electronic health record data of over 78 million patients across the United States, was queried for patients 18 years old or younger with TS. Patients in this group with any occurrence of a diagnosed middle ear cholesteatoma were recorded and reported. RESULTS: Out of 3,682 children 18 years old or younger with diagnosed TS, 1.47% (95% CI: 1.10%-1.91%) had a history of middle ear cholesteatoma. Out of 12,836,624 children 18 years or younger without TS, 0.09% (95% CI: 0.09%-0.09%) had a history of diagnosed middle ear cholesteatoma. The relative risk for middle ear cholesteatoma in children with TS was 16.74 (95% CI: 12.84-21.83). The relative risk for diagnosed cleft palate among children with TS was 8.56 (95% CI: 6.67-10.98) which, because of the Eustachian tube dysfunction in this population, may contribute to the cholesteatoma risk. CONCLUSION: The rate of diagnosed middle ear cholesteatoma was found to be 16 times higher in patients with Turner syndrome versus children without Turner syndrome. Of studies examining middle ear cholesteatoma in children with TS, the present study has the largest sample size thus providing reliable evidence for prevalence in this population. Clinical monitoring for cholesteatoma should be especially rigorous and frequent in this population.

Management of the association of otosclerosis and cholesteatoma: Which pathology to treat first?

Hearing loss can result from a wide range of pathologies affecting patients of all ages. It may be due to abnormalities of the outer, middle or inner ear. In this article, we present a case of a 50 years old female patient presenting to the ENT and Head & Neck surgery department of 20 August hospital, complaining of bilateral hearing loss, in whom clinical examination found right attical retraction pocket with scales and left normal tympanic membrane. Complementary investigations revealed the association of two distinct conditions, rarely described in literature: otosclerosis, and chronic otitis media with cholesteatoma in right ear and left otosclerosis. The challenge in this case was to determine the therapeutic strategy: start with left otosclerosis? the right cholesteatoma? simultaneously treat otosclerosis and cholesteatoma right?

Comparison of the EAONO/JOS, STAMCO and ChOLE cholesteatoma staging systems in the prognostic evaluation of acquired middle ear cholesteatoma in children.

OBJECTIVE: To compare the performance of the EAONO/JOS, STAMCO, and ChOLE Cholesteatoma Staging Systems in prognostic evaluation of children acquired middle ear cholesteatoma after primary surgery and identify the other factors that could predict cholesteatoma recidivism. And the correlation between the staging and the recidivism of cholesteatoma after grouping according to operation was evaluated. METHODS: A total of 123 ears of 118 patients that underwent surgery for primary cholesteatoma from November 2008 to May 2020 were included in this retrospective study, and then classified and staged according to the EAONO/JOS, STAMCO, and ChOLE cholesteatoma staging system, respectively. Each indicator involved in the system above was analyzed separately to evaluate its prognostic value for cholesteatoma recidivism. RESULTS: The type of surgical procedure performed (P = 0.020) was shown to be associated with cholesteatoma recidivism. Cholesteatoma location the supratubal recess (S1) (P = 0.026, HR = 3.614, 95% CI 1.137, 7.945), and the sinus tympani (S2) (P = 0.004, HR = 4.208, 95% CI 1.574, 11.250) were shown to be significantly associated with disease recidivism. When focusing on the CWU operation group, ossicular chain status in STAMCO stage (P = 0.043) and in the ChOLE stage (P = 0.018) were significantly associated with cholesteatoma recidivism. The results had shown no association between the three stages and cholesteatoma recidivism in the CWD and endoscopic surgery groups. CONCLUSIONS: Based on our study, the EAONO/JOS, STAMCO, and ChOLE Classifications have limited value in predicting cholesteatoma recidivism, in acquired middle ear cholesteatoma in children. Adding the pathological status of the ossicular chain may be useful for predicting the recidivism of cholesteatoma. Additional validation studies are entailed to definitively assess the clinical utility of these classifications.