Search Results (4)

Background: Acquired cholesteatoma is a chronic inflammatory middle ear disease characterized by keratinizing squamous epithelium overgrowth and bone erosion. While the upregulation of pattern-recognition receptor (PRR) signaling has been consistently observed, it remains unclear whether this reflects a secondary response to microbial infection or a primary dysfunction driven by genetic predisposition. Methods: Using the UK Biobank, we analyzed 678 individuals with cholesteatoma (ICD-10: H71) among 502,164 participants. Candidate genes implicated in cholesteatoma-related inflammatory pathways (n = 17) were selected, and 147 polymorphisms were studied. Gene-specific genetic risk scores (GRSs) were calculated for cholesteatoma patients (GRSchol) and the general UK Biobank population (GRSpop). The difference (ΔGRSchol-GRSpop) was used to assess the relative contribution of each gene. Results: Genes with the highest ΔGRS were IL6, TREM1, IL1R1, IL1A, HIF1A, ID1, RAGE, and TNFA. These genes represent key downstream mediators and amplifiers of PRR signaling rather than the receptors themselves. Variants in cytokine genes (IL6, IL1R1, IL1A, and TNFA) may enhance inflammatory signaling and bone resorption; Trem1 amplifies TLR responses; RAGE sustains sterile DAMP-driven inflammation, while HIF1A and ID1 implicate hypoxia, tissue remodeling, and keratinocyte proliferation in disease persistence. Conclusions: Our findings suggest that cholesteatoma pathogenesis may not be driven solely by microbial activation of PRRs but rather by genetic variants that amplify and sustain downstream inflammatory responses. This supports a model of cholesteatoma as a disease of self-perpetuating inflammation triggered by diverse stressors, including microbial and non-microbial insults. These insights may inform preventive strategies targeting environmental stressors, as well as therapeutic approaches using biologics to interrupt chronic inflammatory amplification in cholesteatoma. Full article

Background: Cholesteatoma is a lesion capable of destroying surrounding tissues, which may result in significant complications. Surgical resection is the only effective treatment; however, the presence of cholesteatoma recidivism is common. This study evaluated the effectiveness of the Attic Exposure-Antrum Exclusion (AE-AE) surgical technique in treating cholesteatomas and identifying factors associated with recidivism. Additionally, the study aimed to assess the utility of non-echo-planar diffusion MRI (non-EPI-DWI MRI) in detecting cholesteatoma recidivism in patients undergoing AE-AE surgery. Methods: The study involved 63 patients who underwent AE-AE surgery for primary acquired cholesteatoma and were followed up clinically and radiologically for at least five years. The radiological follow-up included a non-EPI-DWI MRI. Results: Results showed that the AE-AE technique successfully treated cholesteatomas, with a recidivism rate of 5.2%. The study also found that non-EPI-DWI MRI was a useful diagnostic tool for detecting cholesteatoma recidivism, although false positives could occur due to the technique’s high sensitivity. As Preoperative Pure-tone average (PTA) increases, there is a higher probability of cholesteatoma recidivism in imaging tests (p = 0.003). Conclusions: Overall, the study highlights the importance of the AE-AE surgical technique and non-EPI-DWI MRI in managing cholesteatoma recidivism in patients, providing valuable insights into associated risk factors and how to manage recidivism. Non-EPI-DWI MRI can assist in patient selection for revision surgery, reducing unnecessary interventions and associated risks while improving treatment outcomes and patient care. Full article

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. Full article

Objective: To evaluate factors that influence the rate of cholesteatoma recurrence (growth of new retraction cholesteatoma) in children. Methods: Review of children with primary acquired or congenital cholesteatoma. Severity was classified by extent and EAONO-JOS stage, and surgery by SAMEO-ATO. Primary outcome measure was 5-year recurrence rate using Kaplan–Meier or Cox regression analysis. Results: Median age was 10.7 years for 408 cholesteatomas from which 64 recurred. Median follow up was 4.6 years (0–13.5 years) with 5-year recurrence rate of 16% and 10-year of 29%. Congenital cholesteatoma (n = 51) had 15% 5-year recurrence. Of 216 pars tensa cholesteatomas, 5-year recurrence was similar at 14%, whereas recurrence from 100 pars flaccida cholesteatomas was more common at 23% (log-rank, p = 0.001). Sub-division of EAONO-JOS Stage 2 showed more recurrence in those with than without mastoid cholesteatoma (22.1% versus 10%), with more in Stage 3 (31.9%; p = 0.0003). Surgery without mastoidectomy, including totally endoscopic ear surgery, had 11% 5-year recurrence. Canal wall-up tympanomastoidectomy (CWU) and canal wall-down/mastoid obliteration both had 23% 5-year recurrence. Multivariate analysis showed increased recurrence for EAONO-JOS Stage 3 (HR 5.1; CI: 1.4–18.5) at risk syndromes (HR 2.88; 1.1–7.5) and age < 7 years (HR 1.9; 1.1–3.3), but not for surgical category or other factors. Conclusion: Young age and more extensive cholesteatoma increase the risk of recurrent cholesteatoma in children. When controlling for these factors, surgical approach does not have a significant effect on this outcome. Other objectives, such as lower post-operative morbidity and better hearing outcome, may prove to be more appropriate parameters for selecting optimal surgical approach in children. Full article