Naoki Oishi

Objective: Habitual sniffing resulting from insufficient Eustachian tube closure is an important etiologic factor in the development and postoperative recurrence of pars flaccida cholesteatoma. In this study, we aimed to investigate the prevalence and clinical characteristics of cholesteatoma associated with habitual sniffing (sniff-related cholesteatoma) among patients with surgically indicated pars flaccida cholesteatoma. Study design: Prospective, multicenter, observational study. Setting: Twenty-six tertiary otologic referral centers. Patients: A total of 128 consecutive patients aged 15 years or above with newly diagnosed pars flaccida cholesteatoma (stage Ib or higher according to the Japan Otological Society staging system) were included. Interventions: None. Main outcome measures: The primary outcome was the prevalence of sniff-related cholesteatoma among patients with surgically indicated pars flaccida cholesteatoma. Patients were classified into 3 groups based on the presence of habitual sniffing episodes and the results of the sniffing test: sniff-related cholesteatoma (both positive), non–sniff-related cholesteatoma (both negative), and others. Demographic and clinical characteristics, including age and sex, were compared among the 3 groups. Results: Among the 128 patients, 53 (41.4%) were classified as having sniff-related cholesteatoma. Furthermore, patients in the sniff-related cholesteatoma group were significantly younger than those in the non–sniff-related group. Conclusions: Approximately 40% of patients with surgically indicated pars flaccida cholesteatoma had sniff-related cholesteatoma, suggesting an association with insufficient Eustachian tube closure. The careful assessment of habitual sniffing may be essential for elucidating disease pathophysiology and optimizing treatment strategies, including postoperative management.

Objective: To investigate the factors affecting postoperative quality of life (QOL) and its longitudinal changes in patients undergoing vestibular schwannoma resection through the retrolabyrinthine approach (RLA). Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Fifty-nine patients who underwent RLA for vestibular schwannoma between March 2016 and November 2022 and completed the Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire preoperatively and at least once at ≥6 months postoperatively. Patients were categorized into hearing preservation (n=37), hearing deterioration (n=19), and preoperative deafness (n=3) groups. Intervention: Surgical resection of vestibular schwannoma through RLA with the intent of preserving hearing. Main Outcome Measures: Changes in PANQOL composite and subdomain scores over time. Results: Total PANQOL scores remained stable postoperatively (mean scores: 69.7 preoperative, 72.3 at 6 mo, 76.4 at 1 y, and 75.1 at 2 y). Anxiety scores significantly improved after surgery. At 6 months postoperatively, hearing and balance scores were significantly lower in the hearing deterioration group compared with the hearing preservation group, though differences diminished by 1 and 2 years. Energy scores tended to be higher in the hearing preservation group. Conclusions: RLA contributes to maintaining or improving postoperative QOL. Hearing preservation supports not only auditory function but also emotional and social well-being.

Chronic otitis media (COM) frequently results in persistent hearing loss that is underrecognized and insufficiently addressed, even after successful surgical management. This expert opinion presents statements and treatment recommendations that advocate for the earlier integration of bone conduction device (BCD) solutions into the management of COM-related hearing loss. Expert opinions were developed through a structured, multiphase process integrating interdisciplinary expertise, a literature review, and patient perspectives. A core team drafted preliminary statements based on evidence from COM, hearing loss research, and clinical experience. These statements were discussed during a face-to-face roundtable in Vienna in September 2025 with 12 international experts, using a structured Think–Pair–Share format. Feedback from breakout groups, plenary discussions, and subsequent email reviews informed the final expert statements and treatment recommendations. Three statements were developed, refined, and agreed upon by the participants. Experts emphasized the early and proactive use of nonsurgical and surgical BCDs to support developmental, communicative, and functional outcomes. Optimal hearing interventions should be prioritized in parallel infection management and surgical interventions. Structured monitoring of hearing and quality of life is recommended to guide timely adjustments in the management of COM and related hearing loss. This expert opinion supports a shift toward the earlier integration of BCD solutions into the management of COM, emphasizing a dual focus on infection control and hearing rehabilitation.

Objective: Preservation of the facial nerve is critical in surgery for facial nerve schwannoma. Intra‐operative monitoring systems typically assess facial nerve function by delivering intermittent electrical stimulation through electrodes; however, this approach cannot effectively achieve continuous stimulation and quantitative assessment. This study aimed to assess the efficacy of a quantitative intra‐operative facial nerve continuous monitoring system for preserving post‐operative facial nerve function. Methods: Ten patients who underwent continuous nerve monitoring using this system during surgery for facial nerve schwannoma were included. Clinical data, electrode placement sites, and pre‐ and postoperative facial nerve function assessed using the House–Brackmann grading system were retrospectively analyzed. In patients with conductive hearing loss, postoperative hearing outcomes were also analyzed. Results: Facial nerve function was preserved in all patients, except for one patient who experienced transient postoperative functional deterioration. In several cases, conductive hearing loss improved following schwannoma resection. Conclusion: Continuous and quantitative monitoring of facial nerve function is a reliable intraoperative evaluation that may help prevent unexpected facial nerve injury. This system represents a valuable tool for application in facial nerve schwannoma surgeries. Level of Evidence: Level 4

Aims: Diffuse large B‐cell lymphoma/high‐grade B‐cell lymphoma (DLBCL/HGBCL) with MYC and BCL2 rearrangements (double‐hit lymphoma with BCL2 , DHL‐ BCL2 ) is a mature aggressive B‐cell lymphoma that also includes concurrent triple hit with BCL6 translocation (TH). DHL with MYC and BCL6 (DH‐ BCL6 ) can also occur. The differences among these three DLBCL/HGBCL subtypes have not yet been definitively determined. Methods and Results: This study characterized the clinicopathological features and transcriptomic profiles of a series of 101 cases of DLBCL/HGBCL that were subclassified according to MYC , BCL2 and BCL6 FISH data, including cell‐of‐origin (COO)‐like, molecular high‐grade (MHG)‐like and double‐hit/dark‐zone (DHIT/DZsig)‐like signatures. DLBCL/HGBCL‐DH‐ BCL2 was characterized by higher HGBCL morphology, CD10 positivity, GCB Hans's, GCB COO and MHG molecular subtype. DLBCL/HGBCL‐TH had higher LDH levels and worse overall survival. DLBCL/HGBCL‐DH‐ BCL6 had higher MUM1 expression, non‐GCB Hans', ABC/Unclassified COO, non‐MHG and low DHIT/DZ signatures. Transcriptomic analysis showed that DLBCL/HGBCL‐DH‐ BCL2 and DLBCL/HGBCL‐TH were close but separated from DLBCL/HGBCL‐DH‐ BCL6 . Gene set enrichment analysis (GSEA) revealed different levels of enrichment between the subtypes. Conclusions: DLBCL/HGBCL‐DH‐ BCL6 differs from the DLBCL/HGBCL‐DH‐ BCL2 , and the DLBCL/HGBCL‐TH is associated with the worst survival. Analysis of all three genes of MYC , BCL2 and BCL6 is recommended in the context of DLBCL/HGBCL diagnosis.

Anaplastic large cell lymphomas (ALCLs) are CD30+ T-cell lymphomas that share pathologic features but differ in presentation, outcome, and genetics. Current classification incorporates clinical presentation and anaplastic lymphoma kinase (ALK) status but inadequately addresses molecular heterogeneity and therapeutic vulnerabilities. We studied 689 patients with ALCL in the LLMPP (Lymphoma/Leukemia Molecular Profiling Project) and performed expert consensus review, genetic subtyping (ALK, DUSP22, TP63, and triple negative), and immunohistochemistry for phosphorylated STAT3Tyr705. RNA sequencing with unsupervised gene expression profiling in 393 patients identified 2 main molecular types of ALCL that could be predicted with 91% accuracy based on the presence (type I) or absence (type II) of phosphorylated STAT3Y705 expression. Type I ALCLs included ALK+ ALCL and a subset of triple-negative ALCLs (TN-I); type II ALCLs included tumors with DUSP22 and/or TP63 rearrangements and the remaining triple-negative ALCLs (TN-II). Type I ALCLs were enriched for JAK-STAT3, whereas type II ALCLs were enriched for non–tyrosine kinase pathways, particularly epigenetic regulators such as EZH2. Immunohistochemistry showed overexpression of EZH2 and its trimethylated substrate H3K27. Prognosis in systemic ALCL was favorable for DUSP22-rearranged ALCL (5-year overall survival, 95%) and ALK+ ALCL (88%), intermediate for triple-negative ALCL (TN-I, 52% and TN-II, 37%), and poor for TP63-rearranged ALCL (0%). We introduce an integrated molecular classification that preserves currently diagnosed ALCL entities but identifies 4 molecularly distinct ALK− ALCL subtypes (DUSP22-rearranged, TP63-rearranged, TN-I, and TN-II). This classification can be easily implemented on paraffin tissue in routine practice or clinical trials, and stratifies ALCL into diagnostically, prognostically, biologically, and potentially therapeutically relevant subtypes.