Epidemiology of Congenital Cholesteatoma: Surveys of the Last 17 Years in Japan.

(1) Background: The incidence of congenital cholesteatoma (CC) has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. (2) Methods: The participants were 100 patients (101 ears) who underwent initial surgical treatment at university hospitals in two rural prefectures between 2006 and 2022. A total of 68% of the patients were males and 32% were females, with a median age of 5 years. We reviewed the medical records for the date of birth, date of surgery, stage of disease, and first symptoms of the disease. (3) Results: The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. No significant difference was found between the incidences in the two prefectures. The number of surgeries performed was higher in the second half of the study period. No difference in the stage of progress was observed based on age. (4) Conclusions: The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence.

Canal-wall up cholesteatoma surgery with mastoid obliteration leads to lower rates of disease recurrence without affecting hearing outcomes.

Objectives: The primary objective was to determine whether obliteration of the epitympanic area and mastoid cavity during canal wall up (CWU) cholesteatoma surgery reduces the rate of recurrent and residual cholesteatoma compared to not obliterating the same area. The secondary objective was to compare postoperative hearing outcomes between both techniques. Methods: A retrospective cohort study was conducted in a tertiary referral center. One-hundred-fourty-three ears were included of patients (≥18y) who underwent a CWU tympanomastoidectomy for cholesteatoma with or without bony obliteration between January 2015 and March 2020 in the University Medical Center Utrecht. The median follow-up was respectively 1.4 (IQR 1.1-2.2) vs. 2.0 years (IQR 1.2-3.1) (p = 0.013). Interventions: All patients underwent CWU tympanomastoidectomy for cholesteatoma. For 73 ears bone dust, Bonalive® or a combination was used for obliteration of the mastoid and epitympanic area, the rest of the ears (n = 70) were not obliterated. In accordance with the Dutch protocol, included patients are planned to undergo an MRI scan with diffusion-weighted imaging (DWI) one, three and five years after surgery to detect recurrent or residual cholesteatoma. Main outcome measures: The primary outcome measure was recurrent and residual cholesteatoma as evaluated by MRI-DWI and/or micro-otoscopy and confirmed by micro-otoscopy and/or revision surgery. The secondary outcome measure was the postoperative hearing. Results: In this cohort, the group treated with canal wall up tympanomastoidectomy with subsequent bony obliteration (73 ears, 51.0%) had significantly lower recurrent (4.1%) and residual (6.8%) cholesteatoma rates than the group without obliteration (70 ears, 25.7% and 20.0%, respectively; p < 0.001). There was no significant difference between both groups in postoperative bone conduction thresholds (mean difference 2.7 dB, p = 0.221) as well as the mean air-bone gap closure 6 weeks after surgery (2.3 dB in the non-obliteration and 1.5 dB in the obliteration group, p = 0.903). Conclusions: Based on our results, a canal wall up tympanomastoidectomy with bony obliteration is the treatment of choice, since the recurrent and residual disease rate is lower compared to the group without obliteration. The bony obliteration technique does not seem to affect the perceptive or conductive hearing results, as these are similar between both groups.

Titanium Implant Obscuring Residual Cholesteatoma on Magnetic Resonance Imaging: A Case Report.

Cholesteatomas are benign growths of squamous epithelial tissue in the middle ear resulting in conductive hearing loss and/or erosion of the structures of the middle ear space. These lesions are surgically removed but require postoperative surveillance due to risk of recurrence or residual disease. Second-look surgery remains the gold standard for diagnosis and treatment of residual or recurrent cholesteatoma; however, advanced imaging modalities such as non-echoplanar diffusion weighted magnetic resonance imaging (non-EPI DWI-MRI) offer a less invasive alternative for surveillance. As surgeons become more reliant on advanced imaging, it is important to understand the limitations of the technology. We present a case of a delay in diagnosis of residual cholesteatoma due to a false-negative finding on non-EPI DWI-MRI screening in the presence of a titanium implant.

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.

Occurrence of mucosa-affecting diseases of the upper airways in middle ear cholesteatoma patients: a nationwide case-control study.

PURPOSE: Exploring a possible link between upper airway inflammation and the development of cholesteatoma by studying the association between mucosa-affecting diseases of the upper airways and cholesteatoma surgery. METHODS: This is a nationwide case-control study of 10,618 patients who underwent surgery for cholesteatoma in Sweden between 1987 and 2018. The cases were identified in the National Patient Register and 21,235 controls matched by age, sex and place of residency were included from national population registers. Odds ratios (OR) and corresponding 95% confidence intervals were used to assess the association between six types of mucosa-affecting diseases of the upper airways and cholesteatoma surgery. RESULTS: Chronic rhinitis, chronic sinusitis and nasal polyposis were more common in cholesteatoma patients than in controls (OR 1.5 to 2.5) as were both adenoid and tonsil surgery (OR > 4) where the strongest association was seen for adenoid surgery. No association was seen between allergic rhinitis and cholesteatoma. CONCLUSION: This study supports an association between mucosa-affecting diseases of the upper airways and cholesteatoma. Future studies should aim to investigate the mechanisms connecting mucosa-affecting diseases of the upper airways and cholesteatoma formation regarding genetic, anatomical, inflammatory and mucosa properties.

Hypoxia-inducible factor-1α contributes to the proliferation of cholesteatoma keratinocytes through regulating endothelin converting enzyme 1 expression.

Objective: Cholesteatoma is a hyperproliferative, pseudoneoplastic lesion of the middle ear characterized by aggressive growth and bone destruction. Hypoxia-inducible factor-1α (HIF-1α, also known as HIF1A) is a key transcription factor that enters the nucleus and upregulates many genes involved in cancer progression in the oxygen-free environment. This study is designed to explore the role and mechanism of HIF1A in the progression of cholesteatoma. Methods: HIF1A and endothelin converting enzyme 1 (ECE1) levels were determined using real-time quantitative polymerase chain reaction. The protein levels of HIF1A, Cyclin D1, proliferating cell nuclear antigen, and ECE1 were measured using western blot. Cell viability, proliferation, and cell cycle progression were analyzed using cell counting kit-8, Colony formation, 5-ethynyl-2'-deoxyuridine, and flow cytometry assays. Binding between HIF-1α and ECE1 promoter was predicted by Jaspar and verified using Chromatin immunoprecipitation and dual-luciferase reporter assays. Results: HIF1A and ECE1 were highly expressed in cholesteatoma patients and keratinocytes. Moreover, HIF1A knockdown might suppress the cell viability, proliferation, and cycle progression of cholesteatoma keratinocytes. Furthermore, HIF1A upregulated the transcription of ECE1 through binding to its promoter region. Conclusion: HIF1A might expedite cholesteatoma keratinocyte proliferation partly by increasing ECE1 expression, providing a possible therapeutic target for the cholesteatoma treatment.

Selection of a Surgical Approach for Middle Ear Cholesteatoma Based on the Fusion Images of Non-Echo Planar Diffusion-Weighted MRI and CT.

PURPOSE: This study aimed to explore the ability of fusion images of non-echo planar diffusion-weighted magnetic resonance imaging (non-EPI-DWI MRI) and computed tomography (CT) to accurately locate cholesteatoma and plan the surgical approach. METHODS: In the first part, 41 patients were included. Their CT images and non-EPI DWMRI images were fused. The scope of cholesteatoma in the fusion image was compared with that in the surgical video to evaluate the capability to locate cholesteatoma. A total of 229 patients were included in the second part, and they were divided into 2 groups. We chose the surgical approach for the CT group and the fusion group, and compared the accuracy of surgical approaches in the CT group and the fusion group using the surgical records. RESULTS: The location of cholesteatoma shown in the fusion images was almost identical to that observed during the operation (kappa = .862). The overall specificity and sensitivity of the fusion images in locating cholesteatoma were 94.12% and 93.06%, respectively. The accuracy of surgical approach selection based on the fusion images (99.02%) was higher than that of surgical approach selection based on the CT images (85.83%). CONCLUSION: It is recommended that the fusion images be used to locate the range of the cholesteatoma before operation.

Comparison of Tissue Factors in the Ontogenetic Aspects of Human Cholesteatoma.

BACKGROUND: An acquired cholesteatoma is a benign but locally aggressive lesion in the middle ear. It is characterized by chronic inflammation and the destruction of surrounding bone. Therefore, the aim of this study was to compare defensins HßD-2 and HßD-4; pro- and anti-inflammatory cytokines IL-1α and IL-10; proliferation marker Ki-67; transcription factor NF-κß; angiogenetic factor VEGF; Sonic hedgehog gene protein SHH; and remodeling factors MMP-2, MMP-9, TIMP-2, and TIMP-4 in adult and pediatric cholesteatoma tissue, and to compare these groups with control skin tissue. METHODS: The study included 25 cholesteatoma tissue material samples from children, 25 from adults, and 7 deep external ear canal skin samples from cadavers. The tissues were stained immunohistochemically and evaluated using semi-quantitative methods. Nonparametric tests, such as the Kruskal-Wallis test and Spearman rank correlation, were used. RESULTS: There were no statistically discernible differences between the adult and children groups when comparing the relative numbers of factor-positive cells. CONCLUSIONS: There are no histopathological differences between adult and children cholesteatoma tissues.

Recent advances in Otology: Current landscape and future direction.

Hearing is an essential sensation, and its deterioration leads to a significant decrease in the quality of life. Thus, great efforts have been made by otologists to preserve and recover hearing. Our knowledge regarding the field of otology has progressed with advances in technology, and otologists have sought to develop novel approaches in the field of otologic surgery to achieve higher hearing recovery or preservation rates. This requires knowledge regarding the anatomy of the temporal bone and the physiology of hearing. Basic research in the field of otology has progressed with advances in molecular biology and genetics. This review summarizes the current views and recent advances in the field of otology and otologic surgery, especially from the viewpoint of young Japanese clinician-scientists, and presents the perspectives and future directions for several topics in the field of otology. This review will aid next-generation researchers in understanding the recent advances and future challenges in the field of otology.

Hypertrophic pachymeningitis of the internal auditory canal and cerebellopontine angle from a large recurrent attic cholesteatoma.

OBJECTIVES: To describe a rare complication of cholesteatoma. METHODS: Case report with literature review. RESULTS: We report a case of a 37-year-old male who presented for evaluation of otorrhea, headache, and progressive left sensorineural hearing loss. Clinical and radiologic evaluation demonstrated a large recurrent attic cholesteatoma with erosion into the lateral and superior semicircular canals, and diffuse enhancement of the internal auditory canal and cerebellopontine angle suggestive of hypertrophic pachymeningitis secondary to cholesteatoma. After treatment with a course of antibiotics and canal wall down mastoidectomy surgery for cholesteatoma exteriorization, he experienced improvement of his symptoms and resolution of hypertrophic pachymeningitis. CONCLUSION: Hypertrophic pachymeningitis is a rarely described complication of cholesteatoma. In the context of cholesteatoma, treatment with antibiotics and surgical removal or exteriorization of cholesteatoma are effective treatments for HP.

Augmented Reality-Assisted Transcanal Endoscopic Ear Surgery for Middle Ear Cholesteatoma.

Background: The indications for transcanal endoscopic ear surgery (TEES) for middle ear cholesteatoma have expanded for cases involving mastoid extension. However, TEES is not indicated for all cases with mastoid extension. In addition, predicting the extent of external auditory canal (EAC) removal needed for cholesteatoma resection is not always easy. The purpose of this study was to use augmented reality (AR) to project the lesion onto an intraoperative endoscopic image to predict EAC removal requirements and select an appropriate surgical approach. Methods: In this study, patients showing mastoid extension were operated on using a navigation system with an AR function (Stryker). Results: The results showed that some cases with lesions slightly extending into the antrum required extensive resection of the EAC, while cases with lesions extending throughout the antrum required smaller resection of the EAC, indicating TEES. Conclusions: By predicting the extent of the needed EAC removal, it is possible to determine whether TEES (a retrograde approach) or canal wall-up mastoidectomy, which preserves as much of the EAC as possible, should be performed. We believe that our findings will contribute to the success of middle ear surgeries and the implementation of robotic surgery in the future.

Otogenic brain complications: a systematic review and meta-analysis.

OBJECTIVE: This study aimed to form astute deductions regarding the presentation, treatment and mortality of otogenic brain complications. METHODS: A systematic literature search of four medical databases (PubMed, Embase, Web of Science and Scopus) was conducted. Studies associated with otogenic brain complications were considered eligible. Fixed- and random-effects model meta-analysis was developed to assess the proportion estimate for each outcome individually. RESULTS: Twenty-eight studies, with 1650 patients in total, were included. In 66 per cent of patients there was a known history of chronic otitis media. The most common symptoms were purulent otorrhoea (84 per cent), headache (65 per cent) and otalgia (45 per cent). A brain abscess was observed in 49 per cent of patients, followed by meningitis (34 per cent) and sinus thrombosis (22 per cent). A combination of surgical and conservative therapy was chosen in 84.3 per cent of cases and the mortality rate approached 11.1 per cent. CONCLUSION: Otogenic brain complications are a possibly life-threatening condition. Prompt imaging examination may set the final diagnosis and lead to an effective treatment.

Recurrent Cholesteatoma: Why it occurs?

A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. Choleatomas are always treated surgically. Recurrence of the illness presents another challenge for the patient and the surgeon, though. There have been reports of recurrence rates as high as 30% in adults and as high as 70% in children. Here, we describe a case of persistent recurrent otorrhea following revision surgery, along with acquired recurrent cholesteatoma following canal wall down surgery. A 38-year -male with underlying Diabetes Mellitus and Hypertension presented with left scanty and foul-smelling ear discharge for 2 years and left reduced hearing. He was diagnosed with left chronic active otitis media with cholesteatoma for which he underwent left modified radical mastoidectomy, meatoplasty and tympanoplasty in 2017. Five months post operatively, he presented with left otorrhea. However, he defaulted followed up and presented in April 2018 for similar complaints. Otoscopy examination revealed left tympanic membrane perforation at poster superior quadrant of pars tensa and bluish discoloration behind pars flacida. He was diagnosed as recurrent left cholesteatoma and subsequently he underwent left mastoid exploration under general anesthesia in June 2018. Postsurgery, he developed recurrent ear discharge which was treated with topical antibiotics and ear toileting. We report a case of recurrent Cholesteatoma despite canal wall down procedure requiring a second redo procedure and with persistent recurrent otorrhea after the redo procedure.However, this case demonstrates the need for regular follow ups even after a canal wall down procedure for detecting recurrence of disease. Moreover, this case denotes some of the patient factors and surgeon factors involved in disease recurrence. Furthermore, importance of opting for an imaging study in case of high suspicion of the disease.

Surgical management of secondary acquired cholesteatoma depends on its characteristics.

OBJECTIVE: Cholesteatoma secondary to tympanic perforation, known as "secondary acquired cholesteatoma" may progress slower than a retraction pocket cholesteatoma, with less bone destruction and fewer intracranial complications. However, complete surgical removal remains difficult because the pathological epithelium on the marginal side of the extension is not covered by the subepithelial layer of the cholesteatoma matrix, making the boundary with the middle ear mucosa difficult to identify. Therefore, considering the pathophysiology of secondary acquired cholesteatoma, suitable preoperative evaluation and surgical techniques are required. In this study, we aimed to evaluate (i) the extension of secondary acquired cholesteatoma according to the size and location of tympanic membrane perforation; and (ii) the microscopic surgical outcomes, including the rate of residual cholesteatoma, requirement for specialized surgical management, and changes in hearing. METHODS: This retrospective study included data of cases with secondary acquired cholesteatoma (n = 66; 66 ears of 64 patients, 2 patients had bilateral ear involvement), including those who underwent a staged operation (n = 25). RESULTS: The perforation level of the tympanic membrane was associated with the cholesteatoma extension. When the cholesteatoma extended around the stapes, staged operation was chosen. Six cases of spontaneous resolution of stapes lesions at the time of staged surgery were observed. A significant postoperative improvement in hearing was observed; however, five cases experienced sensorineural hearing loss. CONCLUSION: Surgery, including staged surgery of the stapes lesions, along with careful observation of the perforation, is required in secondary acquired cholesteatoma. For lesions that are visible yet challenging to remove, it is imperative to exercise prudent judgment, taking into account the possibility of spontaneous resolution.

Surgical Option for External Auditory Canal Cholesteatoma: A Case Report.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

The Role of Endoscopic Assistance in Surgery for Pediatric Cholesteatoma in Reducing Residual and Recurrent Disease.

The primary aim of this study was to evaluate long-term recurrent and residual disease after surgery for acquired cholesteatoma in children according to surgical approach. A total of 71 interventions performed on 67 pediatric patients were included in the study. Canal wall-up tympanomastoidectomy (CWUT) was performed in 31 ears (13 with endoscopic assistance), a transcanal esclusive endoscopic approach (TEEA) was used in 22, and canal wall-down tympanomastoidectomy (CWDT) was performed in 18. Overall, the cholesteatoma relapse rate estimated by the Kaplan-Meier method was 47 ± 6% at 12 years; the recurrent cholesteatoma rate was 28 ± 6% and the residual cholesteatoma rate was 26 ± 5%. The relapse rate according to surgical approach was 33 ± 11% for CWDT, 60 ± 9% for CWUT, and 40 ± 11% for TEEA (p = 0.04). The difference for recurrent disease was no recurrent disease for CWDT, 42 ± 9% for CWUT, and 32 ± 11% for TEEA (p = 0.01). The residual disease rate was significantly reduced with endoscopy: 42 ± 8% without endoscopy vs. 9 ± 5% with (p = 0.003). CWDT can still be considered in primary surgery in case of extensive cholesteatomas and small mastoid with poor pneumatization. TEEA can be recommended for small cholesteatoma not extending to the mastoid to reduce morbidity. Endoscopic assistance seems useful to reduce residual disease in CWUT, whereas it does not have a significant impact on preventing recurrent disease.

Endoscopic Assessment of Cholesteatoma Following Microscope Assisted Tympano: Mastoidectomy: Study design: a cross section descriptive study.

Complete surgical removal of cholesteatoma has been traditionally performed by microscopic approaches. In recent years, use of endoscopes in middle ear surgery is gaining increasing importance. The wide field view offered by the endoscopes in comparison to the narrow field of view of the binocular microscope enables a surgeon to see and dissect around corners, thereby exposing 'hidden recesses' of the middle ear which can be useful during cholesteatoma surgery. To identify remnant cholesteatoma in difficult access sites of the middle ear cleft using endoscopes following a microscope assisted mastoidectomy. A cross sectional study was conducted in patients who were clinically diagnosed with cholesteatoma. Patients were subjected to High Resolution Computed Tomography of the temporal bone and pre-operative audiological tests. Mastoid exploration was performed microscopically followed by endoscopic evaluation and clearance of disease in the same setting. Patients were then followed up post-operative and endoscopically evaluated to look for any remnant disease. A total of 45 patients were included, all of which underwent microscopic assisted mastoidectomy. Remnant cholesteatoma was discovered in 15 out of 45 cases (33%) distributed in the hidden areas; in the region of sinus tympani 9(60%), anterior epitympanum 4(27%), hypotympanum 1(7%) and tip Cells 1(7%). It can be concluded that endoscopes have a definite role in evaluation and complete surgical clearance of cholesteatoma particularly from the hidden areas of middle ear cleft which would have otherwise been missed by the straight line view of a microscope thereby grossly reducing the rate of remnant disease. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04263-6.

Subtotal Petrosectomy: Pictorial Review of Clinical Indications and Surgical Approach.

Subtotal petrosectomy (STP) is characterized by obliteration of the middle ear and occlusion of the external auditory canal. The advent of the endoscope has allowed a reduction in morbidity for some conditions such as cholesteatoma and other middle ear disorders, but STP still plays an important role. A retrospective review of medical records and videos of patients who had undergone STP was performed. Perioperative data and images were collected from various clinical cases who had undergone subtotal petrosectomy at our tertiary referral university hospital in Verona. We confronted our experience with a review of the literature to present the main indications for this type of procedure. STP allows a variety of diseases to be managed effectively as it offers the possibility of a definitive healing with radical clearance of temporal bone. Moreover, it can be safely combined with other procedures with a very low complication rate. Although the endoscope represents a revolution in ear surgery, STP, when indicated, is nowadays a surgical option that should be included in the otosurgeon's portfolio.

Congenital cholesteatoma: what radiologists need to know.

Congenital cholesteatoma is a rare, non-neoplastic lesion that causes conductive hearing loss in children. It is underrecognized and often diagnosed only when there is an established hearing deficit. In the pediatric population, hearing deficiency is particularly detrimental because it can impede speech and language development and, in turn, the social and academic well-being of affected children. Delayed diagnosis leads to advanced disease that requires more extensive surgery and a greater chance of recurrence. A need to promote awareness and recognition of this condition has been advocated by clinicians and surgeons, but no comprehensive imaging review dedicated to this entity has been performed. This review aims to discuss the diagnostic utility of high-resolution computed tomography and magnetic resonance imaging in preoperative and postoperative settings in congenital cholesteatoma. Detailed emphasis is placed on the essential preoperative computed tomography findings that facilitate individualized surgical management and prognosis in the pediatric population.

Actinomycosis of the Middle Ear Mimicking Cholesteatoma: A Case Report and Literature Review.

Actinomycosis is a rare infection of the middle ear. Actinomyces is an anaerobic, filamentous bacterium causing granuloma formation and suppurative infection. We present a young male with a nine-month history of unilateral, yellow-coloured otorrhoea and hearing loss. Swabs showed no growth, with the infection not responding to oral or topical antibiotics. Computed tomography of temporal bones was consistent with cholesteatoma and ossicular disruption. Surgical exploration revealed a yellow, cystic mass within the middle ear. Cortical mastoidectomy and washout were performed. Histological diagnosis confirmed Actinomyces clusters with positive gram stain. Actinomycosis of the middle ear typically presents as chronic otitis media. It likely reaches the middle ear via the eustachian tube. It is often misdiagnosed due to culture insensitivity; however, clinical suspicion can aid labs in providing an optimum culture environment. Tympanomastoidectomy allows for histopathological diagnosis. Surgical resection should be followed by a prolonged course of antibiotics.