(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.

Werner syndrome (WS) is an autosomal recessive disease characterized by premature aging. Malignant tumors such as thyroid carcinoma and malignant melanoma occur frequently in WS patients. We describe 2 siblings with WS who suffered from sinonasal malignant melanoma (MM). Both patients initially experienced nasal obstruction and recurrent nasal bleeding and died within 2 years of the diagnosis of MM. Otolaryngologists should recognize that WS patients have a high risk for head and neck malignant disease, particularly sinonasal MM, even if they are aged below the expected age range and undergo periodic examinations. Furthermore, it is important that WS patients are aware that a prompt nasal examination is indicated if they experience continuous nasal obstruction or recurrent nasal bleeding.

Background/Objectives: Acute otitis media is a common pediatric infection caused primarily by nontypeable Haemophilus influenzae. With rising antibiotic resistance, vaccines are essential for combating this public health issue. Although the PD-1/PD-L1 pathway has been extensively studied for its role in tumor immunity, its impact on mucosal immunity, particularly in vaccine responses, is unclear. Methods: BALB/c mice were intranasally immunized with nontypeable H. influenzae outer membrane protein and treated with anti-PD-L1 antibodies. Immune responses were evaluated in middle ear mucosa (MEM), the cervical lymph node, and the spleen using an enzyme-linked immunosorbent assay, an enzyme-linked immunospot assay, and flow cytometry. The effects on CD4⁺ T cells, T follicular helper (Tfh) cells, and B-cell differentiation were analyzed. Results: Anti-PD-L1 antibody treatment increased CD3⁺CD4⁺CD185⁺ (CXCR5⁺) Tfh cells in MEM, which play a crucial role in supporting B-cell activation and antibody production. This correlated with a significant increase in IgA- and IgG-producing cells in MEM, which enhanced local bacterial clearance. Although B-cell activation and differentiation into plasmablasts were observed in MEM, no significant changes were noted in the cervical lymph node and spleen, suggesting a localized enhancement of mucosal immunity. Conclusions: Anti-PD-L1 antibodies promoted Tfh cell expansion and B-cell differentiation in MEM, leading to enhanced antibody production and improved bacterial clearance. These findings suggest that PD-L1 blockade can potentiate mucosal vaccine-induced immunity by strengthening local humoral responses. This supports its potential application in developing intranasal vaccines for acute otitis media.

In our case, tumor tissues were collected thrice during the course of treatment for immunohistological evaluation. The tumor was consistently positive for VEGF, suggesting that pazopanib was effective in the treatment of solitary fibrous tumor.

Objectives In preparation for endoscopic sinus surgery (ESS), the most important computed tomography (CT) image for otolaryngologists is the coronal plane image because it has a viewpoint similar to that of the surgical visual field. Contrastingly, otologic surgeons refer to axial and coronal plane images before ear surgery and must imagine the anatomical structure of the temporal bone by reconstructing three-dimensional (3D) images in their minds. We propose a “surgical position view (SPV) image,” a novel viewpoint of CT images that enables otologic surgeons to see a perspective similar to the surgical visual field. Method Sagittal plane CT images of the temporal bone were created from axial plane images with multi-planar reconstruction (MPR). Then, the SPV image was obtained by rotating it 90° to the supine position. The entire process can be performed anywhere in the hospital within 1 minute using the electronic medical record computer's image viewer. Results SPV images show anatomical structures of the temporal bone, external ear canal, mastoid cavity, sigmoid vein, facial nerve, ossicles, and cochlea, in a similar view to the actual ear surgery. Soft tissue such as cholesteatoma is depicted in the same concentration as the normal CT image. Conclusion The SPV image enables an otologic surgeon to see the temporal bone CT image from the actual ear surgery viewpoint simply and quickly. It helps to visualize the 3D anatomical structure of the temporal bone and can be useful for ear surgery planning. Level of evidence 5

Acute otitis media (AOM) is a common pediatric condition, with facial nerve paralysis (FNP) as a rare complication. It occurs in approximately 0.004%–0.005% of cases. This report presents a unique case of a female infant with recurrent FNP related to AOM who experienced four episodes within the first 3 months of life. The patient was referred to our department at the 34th day of life with facial paralysis linked to left AOM. Treatment with intravenous antibiotics and systemic steroids initially improved FNP. Tympanotomy was performed during the second episode, and a tympanostomy tube was inserted during the third episode. However, left facial paralysis persisted after the fourth episode. Imaging studies showed effusion in the middle ear bilaterally, and a horizontal bone defect and peripheral stenosis of the left facial nerve canal. FNP management in AOM remains a subject of debate, with differing recommendations, especially regarding systemic steroid administration. This case highlights the criticality of considering anatomical factors in pediatric patients presenting with FNP and AOM, underscoring the requirement for further research on effective treatment strategies and understanding the pathophysiology of this rare condition.