Congenital Cholesteatoma.

Congenital cholesteatoma is a cyst of keratinizing squamous cell epithelium in the setting of an intact tympanic membrane, in a patient without a history of otorrhea, tympanic membrane perforation, or otologic surgery. The most common presentation of a congenital cholesteatoma is that of an asymptomatic pearly white mass in the anterosuperior quadrant of the tympanic cavity. The etiology of congenital cholesteatoma has been debated at length, with the leading theory being the epithelial rest theory. Treatment for congenital cholesteatoma is surgical, with advances in endoscopic ear surgery allowing for improved intraoperative visualization and postoperative lowered recidivism rates.

Management of Cholesteatoma: Extension Beyond Middle Ear/Mastoid.

Petrous bone cholesteatoma, or cholesteatoma that extends beyond the middle ear and mastoid, represents a rare but destructive pathology. Diagnosis can be difficult before substantial morbidity is incurred, and patients can present with life-threatening complications. Determination of disease extent and the functional status of the facial nerve and cochleovestibular system are critical in surgical planning. Typically, surgery involves ablative procedures with the goal of complete disease resection given the low likelihood of preserved inner ear function. In experienced hands, disease control and facial nerve outcomes are favorable; however, disease recidivism is not uncommon and, thus, these patients require lifelong surveillance.

Recidivism and Recurrence.

Recidivistic cholesteatoma encompasses residual as well as recurrent disease, and can occur in up to 61% of cases. Pediatric disease may have a higher propensity for recidivism. Serial physical examination and MRI including non-EPI DWI sequences are useful in surveillance. Canal wall down approaches with mastoid obliteration may be an approach to reduce recidivism while minimizing the need for mastoid cavity maintenance. Modern techniques of Eustachian tube dilation and endoscopic ear surgery may yet prove particularly helpful in reducing re-retraction and residual disease in the retrotympanum, respectively; however, they require further study.

Evaluation of Cholesteatoma.

Evaluation of cholesteatoma depends on clinical history and examination, with microscope and/or endoscope. A history of hearing loss with a chronic draining ear, refractory to ototopical medication, raises suspicion for cholesteatoma. Symptoms of Eustachian tube dysfunction or prior ear surgery including ear tubes should be elicited. Inflammation can be severe and should be suppressed if possible. Once cholesteatoma is diagnosed or strongly suspected, further workup includes audiometry prior to surgical excision. Imaging may supplement the workup and is especially helpful if there are concerning features including vertigo, third window symptoms, asymmetric bone line, facial nerve weakness, or for anticipatory guidance.

Techniques in Management of Cholesteatoma: Modified Radical and Retrograde Mastoidectomy.

When surgically managing cholesteatoma, the Bondy modified radical mastoidectomy (MRM) and retrograde mastoidectomy can enhance visualization while minimizing the extent of surgery required for disease removal. The Bondy MRM can be used for disease limited to the atticoantral space, when the ossicles and middle ear space are otherwise healthy, and for canal cholesteatomas. The retrograde mastoidectomy offers a safe alternative for patients with unfavorable tegmen and sigmoid sinus positions and poorly pneumatized mastoids. Both approaches achieve favorable recidivism rates and postoperative hearing outcomes. This article serves as a reference regarding indications, techniques, and complications for these approaches.

MRI-DWI detection of residual cholesteatoma: moving toward an optimum follow-up scheme.

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

Comparison of Endoscopic and Microscopic Surgery for the Treatment of Acquired Cholesteatoma by EAONO/JOS Staging.

Cholesteatoma is a benign tumor of the middle ear. Tympanoplasty is performed to remove cholesteatoma, prevent recurrence, and avoid complications. Previously, microscopy was used for tympanoplasty; however, endoscopy has become increasingly popular for this procedure. The effectiveness of endoscopy and the appropriate candidacy for endoscopic ear surgery remain controversial. In this retrospective chart review study, we enrolled 107 patients with cholesteatoma who underwent tympanoplasty and compared the microscopic approach (71 patients) and the endoscopic approach (36 patients) at different stages to clarify the benefits of using endoscopy and to determine candidacy for endoscopic ear surgery. Approach selection, complication rate, recurrence rate, and postoperative hearing threshold were compared between microscopic and endoscopic approaches in stages I, II, and III. Endoscopy was used more often than microscopy for early-stage (stage I) cholesteatoma (p = 0.005) and less frequently for advanced-stage (stage II) cholesteatoma (p = 0.02). Endoscopy surgery resulted in chorda tympani injury less often than microscopic surgery (p = 0.049); however, there were no significant differences between these two groups in terms of recurrence rate or postoperative hearing outcome. Endoscopy is particularly useful for early-stage cholesteatoma, and both approaches show no difference in hearing outcome in stage I and II; nevertheless, further research is required to determine an appropriate approach for more advanced stages (stage III).

Anaerobic bacteria dominate the cholesteatoma tissue of chronic suppurative otitis media patients.

The aim of this study was to investigate both the microbial composition and absolute abundance of clinically relevant bacteria in tissue specimens from patients with chronic suppurative otitis media with cholesteatoma (CSOM with cholesteatoma). Mastoid mucosa and cholesteatoma tissue from eleven subjects with CSOM with cholesteatoma, and mastoid mucosa from ten controls were examined using standard hospital culture swabs, Gram staining, bacterial 16S rRNA gene sequencing, Droplet Digital PCR (ddPCR), and multiplex PCR. Positive results from culture swabs were reported in half the CSOM with cholesteatoma samples and 1 control sample. In contrast, ddPCR detected bacterial genes copies in all 11 mucosa and cholesteatoma of CSOM subjects and 3 control samples. The average bacterial gene copies in tissue samples with CSOM with cholesteaotoma (1.6 ± 0.7 log10) was significantly higher compared to healthy controls (0.3 ± 1.6). These results were corroborated with Gram-staining that identified the large presence of Gram-positive cocci cells in the cholesteatoma tissue of CSOM subjects which were not seen in the mucosa of controls. The most abundant genus detected by sequencing in the mucosa and cholesteatoma of CSOM samples was Anaerococcus (93.5% of all reads), and genus Meiothermus (0.9%) in the control sample. The 3 samples with the highest sequencing reads (>300) were further analysed using multiplex PCR to identify the dominant Anaerococcus species. Anaerococcus hydrogenalis was the dominant species identified in these samples. In contract, commonly named ear pathogens, genera Staphylococcus and Pseudomonas, were detected in low numbers (<0.001% of all sequencing reads) and low prevalence (2/16 samples) in the tissue samples of this study. The results show that culture severely underestimated the bacterial diversity in CSOM samples and investigating tissue rather than standard culture swabs might be advantageous to understanding the disease process. The high abundance of bacteria and the large presence of Gram-positive cells detected in the cholesteatoma tissue of CSOM compared to mucosa of CSOM or controls could be members from the genus Anaerococcus. Anaerococcus may well be a pathogen in CSOM with cholesteatoma, but their role in this condition requires further investigation.

Principles of Cholesteatoma Management.

Surgery for cholesteatoma should be tailored to individual patients, considering demographic and disease factors, to obtain a dry, safe, and functional ear. The EAONO/JOS classification and staging system provide a valuable framework for data collection and outcome assessment. Canal wall-up and canal wall-down surgical approaches each have their advantages and disadvantages, though it is not definitive that one approach is clearly more advantageous than the other. Mastoid obliteration techniques show promise in reducing recidivistic disease rates but require further research and standardization. Endoscopic ear surgery further augments our surgical capabilities to visualize and eradicate cholesteatoma.

Complications of Surgery: Recognition and Management.

The middle ear and mastoid spaces contain delicate and functionally important structures. Safe cholesteatoma surgery requires mastery of otologic anatomy as well as thorough preoperative investigation. Cholesteatoma can damage otologic structures on its own; however, there is also an opportunity for an iatrogenic complication given the distortion of anatomy by the disease process. This article explores complications that can occur during cholesteatoma surgery, guidance on how to avoid intraoperative complications, and treatment of such complications.

Techniques in Management of Cholesteatoma: Radical and Canal Wall down Mastoidectomy.

Canal wall down mastoidectomy is performed when demanded by the extent of disease: presence of a large labyrinthine fistula, extensive erosion of posterior auditory canal wall, prior failed intact canal wall surgery, a contracted, sclerotic mastoid with extensive disease, or need for exteriorization due to inadequate exposure or inability to remove disease safely with a canal wall up approach.

Management of Cholesteatoma: Surgical Options for Disease Limited to Middle Ear/Mastoid.

Surgery remains the mainstay of cholesteatoma management. Through advancement in technique and technology, the available surgical approaches have expanded to include not only the traditional procedures, but also endoscopic procedures, canal wall reconstruction procedures, mastoid obliteration, and retrograde mastoidotomy. Selection of management technique will depend on disease characteristics, patient factors, and surgeon preference.

Beyond the otoscope: an imaging review of congenital cholesteatoma.

Congenital cholesteatoma (CC) is a non-neoplastic lesion of keratin debris lined by epithelium found in the temporal bone. It is the lesser-known sibling of the acquired cholesteatoma and may be classified as congenital middle ear cholesteatoma and congenital petrous bone cholesteatoma. The incidence is rising, probably owing to increased recognition and advances in imaging modalities. Cone beam CT provides detailed anatomical information, highlighting quadrant location, ossicular involvement, and mastoid extension. MRI aids in lesion characterization and detection of complications. The classification systems for congenital middle ear and petrous bone cholesteatoma are helpful in the preoperative workup and have a role in predicting postoperative recurrence rates. Management almost invariably involves surgical intervention aimed at preserving middle and inner ear function. Follow-up of CC is mainly based on MRI together with otoscopic examination. Non-echo planar diffusion-weighted imaging, especially, has proven essential for detecting residual disease. This review article emphasizes the significance of imaging in the timely diagnosis and management of CCs. CLINICAL RELEVANCE STATEMENT: This article underscores the crucial role of imaging for prompt detection, preoperative assessment, and postoperative follow-up of CCs, a condition with rising incidence associated with potentially severe complications. KEY POINTS: Timely diagnosis of CCs is imperative for avoiding complications. Imaging is key in detection, preoperative evaluation, and postoperative management. Cone Beam CT and non-echo planar DWI represent state-of-the-art imaging techniques.

Surreptitious Area of Middle Ear - the Sinus Tympani.

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.

Metagenome Analysis of Cholesteatoma-associated Bacteria: A Pilot Study.

Introduction: 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. Methods: 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. Results: 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. Conclusion: 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. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-024-04678-9.

Pediatric Cholesteatoma: An Overview of Presentation, Surgical Strategy and Outcomes of an Individualized Approach.

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.

External auditory canal cholesteatoma in children: clinical manifestations.

PURPOSE: 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). CONCLUSION: 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.

ESR Essentials: imaging of middle ear cholesteatoma-practice recommendations by the European Society of Head and Neck Radiology.

Although non-malignant, middle ear cholesteatoma can result in significant complications due to local bone erosion and infection. The treatment of cholesteatoma is surgical, but residual disease is common and may be clinically occult, particularly when the canal wall is preserved or reconstructive techniques are employed. Imaging plays a pivotal role in the management of patients with middle ear cholesteatoma-aiding clinical diagnosis, identifying complications, planning surgery, and detecting residual disease at follow-up. Computed tomography is the primary imaging tool in the preoperative setting since it can provide both a surgical roadmap and detect erosive complications of cholesteatoma. The ability of magnetic resonance imaging with non-echoplanar diffusion-weighted sequences to accurately detect residual disease has led to a shift in the diagnostic paradigm for post-surgical follow-up of cholesteatoma, such that routine "second-look" surgery is no longer required. The following practice recommendations are aimed at helping the radiologist choose appropriate imaging approaches and understand the key diagnostic considerations for the evaluation of pre- and post-surgical middle ear cholesteatoma. KEY POINTS: In the preoperative setting, CT is the first-line imaging modality and MRI is reserved for rare clinical scenarios (low evidence). Non-echoplanar imaging (EPI) DWI is the optimal MRI sequence for the detection of residual cholesteatoma (moderate evidence). Non-EPI DWI plays an important role in the postoperative surveillance of cholesteatoma (moderate evidence).

PTHrP participates in the bone destruction of middle ear cholesteatoma via promoting macrophage differentiation into osteoclasts induced by RANKL. / PTHrP促进RANKLè¯±å¯¼å·¨å™¬ç»†èƒžåˆ†åŒ–ä¸ºç ´éª¨ç»†èƒžå‚ä¸Žä¸­è€³èƒ†è„‚ç˜¤éª¨ç ´å.

OBJECTIVES: Progressive bone resorption and destruction is one of the most critical clinical features of middle ear cholesteatoma, potentially leading to various intracranial and extracranial complications. However, the mechanisms underlying bone destruction in middle ear cholesteatoma remain unclear. This study aims to explore the role of parathyroid hormone-related protein (PTHrP) in bone destruction associated with middle ear cholesteatoma. METHODS: A total of 25 cholesteatoma specimens and 13 normal external auditory canal skin specimens were collected from patients with acquired middle ear cholesteatoma. Immunohistochemical staining was used to detect the expressions of PTHrP, receptor activator for nuclear factor-kappa B ligand (RANKL), and osteoprotegerin (OPG) in cholesteatoma and normal tissues. Tartrate-resistant acid phosphatase (TRAP) staining was used to detect the presence of TRAP positive multi-nucleated macrophages in cholesteatoma and normal tissues. Mono-nuclear macrophage RAW264.7 cells were subjected to interventions, divided into a RANKL intervention group and a PTHrP+ RANKL co-intervention group. TRAP staining was used to detect osteoclast formation in the 2 groups. The mRNA expression levels of osteoclast-related genes, including TRAP, cathepsin K (CTSK), and nuclear factor of activated T cell cytoplasmic 1 (NFATc1), were measured using real-time polymerase chain reaction (real-time PCR) after the interventions. Bone resorption function of osteoclasts was assessed using a bone resorption pit analysis. RESULTS: Immunohistochemical staining showed significantly increased expression of PTHrP and RANKL and decreased expression of OPG in cholesteatoma tissues (all P<0.05). PTHrP expression was significantly positively correlated with RANKL, the RANKL/OPG ratio, and negatively correlated with OPG expression (r=0.385, r=0.417, r=-0.316, all P<0.05). Additionally, the expression levels of PTHrP and RANKL were significantly positively correlated with the degree of bone destruction in cholesteatoma (r=0.413, r=0.505, both P<0.05). TRAP staining revealed a large number of TRAP-positive cells, including multi-nucleated osteoclasts with three or more nuclei, in the stroma surrounding the cholesteatoma epithelium. After 5 days of RANKL or PTHrP+RANKL co-intervention, the number of osteoclasts was significantly greater in the PTHrP+RANKL co-intervention group than that in the RANKL group (P<0.05), with increased mRNA expression levels of TRAP, CTSK, and NFATc1 (all P<0.05). Scanning electron microscopy of bone resorption pits showed that the number (P<0.05) and size of bone resorption pits on bone slices were significantly greater in the PTHrP+RANKL co-intervention group compared with the RANKL group. CONCLUSIONS: PTHrP may promote the differentiation of macrophages in the surrounding stroma of cholesteatoma into osteoclasts through RANKL induction, contributing to bone destruction in middle ear cholesteatoma.