Intranasal administration of ceramide liposome suppresses allergic rhinitis by targeting CD300f in murine models.

Allergic rhinitis (AR) is caused by type I hypersensitivity reaction in the nasal tissues. The interaction between CD300f and its ligand ceramide suppresses immunoglobulin E (IgE)-mediated mast cell activation. However, whether CD300f inhibits the development of allergic rhinitis (AR) remains elusive. We aimed to investigate the roles of CD300f in the development of AR and the effectiveness of intranasal administration of ceramide liposomes on AR in murine models. We used ragweed pollen-induced AR models in mice. Notably, CD300f deficiency did not significantly influence the ragweed-specific IgE production, but increased the frequency of mast cell-dependent sneezing as well as the numbers of degranulated mast cells and eosinophils in the nasal tissues in our models. Similar results were also obtained for MCPT5-exprssing mast cell-specific loss of CD300f. Importantly, intranasal administration of ceramide liposomes reduced the frequency of sneezing as well as the numbers of degranulated mast cells and eosinophils in the nasal tissues in AR models. Thus, CD300f-ceramide interaction, predominantly in mast cells, alleviates the symptoms and progression of AR. Therefore, intranasal administration of ceramide liposomes may be a promising therapeutic approach against AR by targeting CD300f.

Association between habitual snoring and vigilant attention in elementary school children.

OBJECTIVES: Vigilant attention (VA) is a fundamental neurocognitive function. However, the association between habitual snoring (HS) and VA in community-based children remains unclear. Therefore, this study aimed to elucidate the association. METHODS: The study included 2014 children from grades 1-6 across six elementary schools. Snoring frequency was evaluated using a questionnaire administered to parents. VA was assessed using a brief 3-min psychomotor vigilance test (PVT-B). Generalized linear models and multivariate logistic regression analysis were utilized to examine the association between snoring frequency and PVT-B performance. Impaired PVT-B performance was defined as the worst quartile of PVT-B metrics. RESULTS: The PVT-B performance significantly improved with advancing school grade level (p trend < 0.0001). A significant negative correlation was observed between snoring frequency and PVT-B performance. Particularly, in grade 1, HS was associated with a higher risk of impaired PVT-B performance, including response speed (mean reciprocal reaction time) (adjusted odds ratio [aOR] 2.56, 95% confidence interval [CI]: 1.20-5.50), more slowest 10% RT (aOR 3.28, 95% CI: 1.51-6.88), and more lapse500 (number of lapse of reaction time ≥ 500 ms) (aOR 3.18, 95% CI: 1.45-6.80) compared to children without snoring. CONCLUSIONS: Our findings show that VA rapidly improves throughout elementary school. Additionally, younger children with HS are at risk of VA deficits, emphasizing the importance of early intervention for HS.

Intravascular papillary endothelial hyperplasia of the maxillary sinus extending into the contralateral nasal cavity.

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH) predominantly occurs in the subcutaneous and dermal regions and rarely originates from the sinonasal mucosa. CASE PRESENTATION: We report on the case of a 58-year-old male patient who presented with progressive bilateral nasal obstruction, left-sided epiphora, and intermittent epistaxis. Computed tomography revealed a soft tissue opacity in the left maxillary sinus with intersinusoidal nasal wall demineralization, extending into the surrounding ethmoid cells and the right nasal cavity through a contralateral deviation of the nasal septum. Contrast-enhanced T1-weighted magnetic resonance imaging further confirmed these findings. The IPEH originating from the maxillary sinus extended into the contralateral nasal cavity, and it was successfully removed using an endoscopic endonasal approach, avoiding overly aggressive treatment. CONCLUSION: This case report highlights the diagnostic challenges of IPEH in the sinonasal region and the importance of considering IPEH as a differential diagnosis in patients presenting with nasal obstruction, epiphora, and intermittent epistaxis.

A Case Report of Superficial Solitary Fibrous Tumor in the Right Parotideomasseteric Region.

Solitary fibrous tumors (SFTs) are rare mesenchymal tumors that occur in various soft tissues and organs throughout the body. Superficial SFTs in the head and neck region are uncommon and reports of such cases are limited. An elderly Japanese woman in her 80s presented to our clinic with a smooth mass in the right parotideomasseteric region. Ultrasonography and magnetic resonance imaging revealed a well-defined subcutaneous mass measuring 20 mm. For a definitive diagnosis and treatment, surgical resection was performed under local anesthesia. Intraoperatively, a brown-colored tumor was identified beneath the skin, without surrounding tissue adhesion, and was completely excised. Immunohistochemistry, revealing the expression of STAT6, confirmed the diagnosis of SFT. Based on the SFT risk classification, this case was classified into the low-risk group, and no recurrence occurred during the 4 year postoperative period. According to the 2020 World Health Organization Classification of Soft Tissue Tumors, SFTs belong to the intermediate category with recurrence potential. In previous studies, cases of positive margins and recurrence have been reported in superficial SFTs, and SFTs may show benign features on clinical presentation but require precise surgical manipulation. Therefore, it is crucial for otolaryngologists to gain a comprehensive understanding of this tumor for appropriate treatment.

A Patient With Lobular Capillary Hemangioma Originating From the Inferior Turbinate Following Cauterization With Silver Nitrate.

Lobular capillary hemangiomas (LCH), which usually originate in the skin and mucous membranes of the oral cavity, are uncommon from the posterior portion of the inferior turbinate. Although the exact cause of LCH in the nasal cavity has not been elucidated, trauma, caused by factors such as intranasal packing and habitual nose-picking, has been reported as one of the causes. In addition, 2 cases of LCH caused by submucosal resection with powered instrumentation to the inferior turbinate have been reported, suggesting that various types of traumas to the nasal mucosa can cause LCH. The authors report the first case of LCH formation in the posterior portion of the inferior turbinate after cauterization with silver nitrate.

A Rare Case of Sinonasal Seromucinous Hamartoma Developing from the Nasal Septum.

A 45-year-old man presented with a history of chronic left nasal congestion. Nasal endoscopy revealed a pedunculated polypoid mass with glandular epithelium surface on the posterior nasal septum. Computed tomography revealed a 25-mm mass-like growth in the left posterior nasal cavity attached to the nasal septum with a stalk. The patient underwent transnasal endoscopic surgery, and the tumor was removed under a block with safety margin. The final pathological diagnosis was sinonasal seromucinous hamartoma (SH). Sinonasal SH is a rare tumor with only 31 reported cases. Transnasal endoscopic surgery is currently the first-line treatment for sinonasal SH. Differential diagnoses of this lesion include inflammatory polyps, respiratory epithelial adenomatoid hamartoma, and adenocarcinoma. Although SH is a benign tumor, its progression to adenocarcinoma has been reported. Therefore, unilateral posterior nasal tumors must be diagnosed precisely.

Clinical outcomes of head and neck mucosal melanoma treated with surgery: a single-center study in Japan.

BACKGROUND: Head and neck mucosal melanomas are rare malignancies. Although the prognosis is poor owing to the high incidence of distant metastases, locoregional control remains important. It is difficult to obtain results in a large cohort because of its rarity. This study aimed to elucidate the survival outcomes of patients with head and neck mucosal melanoma treated with surgery in Japan. METHODS: Patients with head and neck mucosal melanoma who were surgically treated between 2007 and 2021 at the National Cancer Center Hospital were retrospectively analyzed. RESULTS: A total of 47 patients were included in this study. The 5-year overall survival, disease-specific survival, locoregional control and relapse-free survival rates were 42%, 50%, 79% and 13%, respectively. The disease-specific survival of the oral mucosal melanoma group was significantly better than that of the sinonasal mucosal melanoma group (5-year disease-specific survival rate: 70% versus 37%, respectively; P = 0.04). Multivariate analyses revealed that sinonasal mucosal melanoma were independently significant adverse prognostic factor, for overall survival and disease-specific survival. Patients with oral mucosal melanoma patients had a higher incidence of lymph node metastasis than those with sinonasal mucosal melanoma patients (P < 0.0001). CONCLUSION: This study demonstrated the survival outcomes of the largest cohort of patients with head and neck mucosal melanomas treated surgically at a single institution within the past 20 years in Japan. We found that survival outcomes and incidence of nodal metastases varied by site.

A Case of Woakes' Syndrome With A Bilateral, Large Nasal Polyp.

Woakes' syndrome, first reported by Edward Woakes in 1885, is an extremely rare, recurrent sinonasal polyposis leading to bone erosion of the sinus walls with consequent nasal pyramid deformity and facial disfigurement. We report a 66-year-old man who presented with severe nasal obstruction. His external nose was deformed and distended with complete obstruction of the bilateral nasal cavities by nasal polyps. The normal structure of the nose was disrupted. Thus, super-selective embolization was performed before surgery to minimize bleeding. The day after the embolization, polypectomy was performed with the navigation system. The progression was uneventful, and the patient was discharged on postoperative day 7. Pathological examination revealed inflammatory polyps with no eosinophil infiltration. Thus, we diagnosed it as Woakes' syndrome. Although there have been few reports of Woakes' syndrome in the past, these are the largest polyps reported to the best of our knowledge.

Drainage Tube Placement for the Management of Rebleeding After Vascular Embolization in a Patient With Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF-1), a hereditary disease caused by a mutation of a gene on chromosome 17q11.2, is associated with manifestations in several organs. Although infrequent, vascular abnormalities are a complication of NF-1, and they are the second most common cause of death in patients with NF-1. Repairing the nutrient artery and achieving hemostasis are difficult once the artery has failed, thereby resulting in poor treatment outcomes. Herein, we report a case of a patient with NF-1 who presented with an enormous cervical hematoma caused by bleeding from a branch of the external carotid artery. Vascular embolization was performed initially; however, rebleeding from the embolized site occurred. Following the removal of the hematoma, drainage tube placement was effective in preventing micro-bleeding. Thus, drainage tube placement may be an effective treatment option in patients with rebleeding.

Generation and characterization of a human iPSC line (JUFMDOi007-A) from a patient with Usher syndrome due to mutation in USH2A.

Usher syndrome type 2A (USH2A) gene mutations have been identified as the most frequent genetic causes of hereditary deafness in Usher syndrome, and an effective treatment has yet to be established. The encoded protein, Usherin, is essential for the ankle link associated with extracellular connections between the stereocilia of inner ear hair cells. We report the generation of a patient-derived USH2A iPSC line with compound mutations c.1907_1912ATGTTT > TCACAG (p.D636V + V637T + C638G) and c.8328_8329delAA (p.L2276fs*12). The iPSC showed the expression of pluripotency markers, the ability to differentiate into three germ layers in vitro, and USH2A mutations with normal karyotype.

Impact of antiallergy agents on CPAP therapy and sleep quality with spring pollinosis in Japanese.

PURPOSE: Allergic rhinitis (AR) is associated with obstructive sleep apnea (OSA) and nasal obstruction causes decreased adherence to continuous positive airway pressure (CPAP). The purpose is to evaluate the effects of antiallergic agents on CPAP adherence and sleep quality. METHODS: A longitudinal study was made of patients who use CPAP for OSA and treated with antiallergy agents for spring pollinosis. We compared the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), nasal symptoms scores (NSS), and data from CPAP before and after treatment. Then, we classified the subjects into two groups based on the baseline PSQI score: one group without a decreased sleep quality (PSQI < 6) and the other group with decreased sleep quality (PSQI ≥ 6). RESULTS: Of 28 subjects enrolled, 13 had good sleep quality and 15 had poor sleep quality. PSQI showed significant improvements after medication (p = 0.046). ESS showed no significant differences after AR medication (p = 0.565). Significant improvement was observed after the prescription of antiallergy agents in all items of NSS (sneezing, p < 0.05; rhinorrhea, p < 0.01; nasal obstruction, p < 0.01; QOL, p < 0.01). The percentage of days with CPAP use more than 4 h increased significantly after the administration of rhinitis medication (p = 0.022). In the intragroup comparisons of PSQI ≥ 6 group, PSQI decreased significantly (p < 0.05). For the NSS in intragroup comparisons of PSQI ≥ 6 group, all parameters showed significant improvement (sneezing, p = 0.016; rhinorrhea, p = 0.005; nasal obstruction, p < 0.005; QOL, p < 0.005). CONCLUSION: The use of antiallergy agents can improve CPAP adherence and sleep quality in patients with OSA on CPAP.

A Rare Case of Scalp Metastasis from Encapsulated Angioinvasive Follicular Thyroid Carcinoma.

Cutaneous metastases from thyroid carcinomas are extremely rare; however, the scalp is a common site for cutaneous metastases from follicular thyroid carcinomas (FTCs). We report the case of a 77-year-old male patient with a blood-rich scalp lesion. Histopathological tests of punch biopsy specimens revealed subcutaneous well-formed follicular structures that were similar to those found in the thyroid gland. Immunohistochemistry using thyroid transcription factor-1 (TTF-1) and paired-box gene family 8 (PAX8) revealed an FTC metastasis. We performed total thyroidectomy and resection of the scalp lesion at the same time and administered postoperative radioactive iodine treatment. The primary thyroid lesion was diagnosed as an FTC based on extracapsular extension and vessel invasion. The patient has not experienced disease recurrence since the treatment. When scalp metastasis of thyroid carcinoma is suspected, we recommend total extirpation, including the primary tumor and scalp metastasis, for an improved prognosis.

Creutzfeldt-Jakob disease with dizziness initially presenting to the otolaryngology department.

Creutzfeldt-Jakob disease is a fatal transmissible prion disease of the central nervous system. Dizziness as an initial manifestation of Creutzfeldt-Jakob disease is rare. However, patients with Creutzfeldt-Jakob disease and dizziness may initially visit the otolaryngology department, but this is uncommon. We report the case of a 56-year-old woman with Creutzfeldt-Jakob disease who initially presented with dizziness as an emergency patient to the otolaryngology department. Primary position upbeat nystagmus was identified using a charge-coupled device camera with infrared illumination. Electronystagmography revealed impaired smooth pursuit and diminished optokinetic nystagmus. Based on these findings, we immediately suspected an intracranial cause of dizziness and reached a presumptive diagnosis of sporadic Creutzfeldt-Jakob disease, thus preventing severe transmission. This case emphasizes that Creutzfeldt-Jakob disease should be included as a differential diagnosis for patients with dizziness and abnormal eye movements, such as primary position upbeat nystagmus, which might be caused by intracranial disease.

Congenital Aural Fistula Developing Between the Third and Fourth Hillocks of the Embryonal Helix and Presenting a Large Temporal Mass: A Case Report.

This report aimed to introduce a very rare presentation of congenital aural fistula and its treatment. A 13-year-old girl presented with a mass on the right temporal region with protrusion of the helix. She noticed a mass a month previously, and the mass gradually swelled with pain. Pus discharged from the pit behind the helix. Mastoiditis was suspected; however, the tympanic membrane was normal. Magnetic resonance imaging revealed a cystic mass in the temporal region. The surgical removal of the mass was performed using a postauricular incision. The mass was cystic and had a stem connected to the pit. Insertion of a probe into the pit showed a connection to the mass. The mass was totally removed with the skin around the pit. Histologically, the cyst connected to the fistula and its lumen was covered with squamous cells. A diagnosis of a congenital aural fistula developed posterior to the helix was made. Considering its location, the fistula had been formed between the third and fourth hillocks of the embryonal helix. Aural fistula developed posteriorly is very rare, and it mimicked a temporal tumor or mastoiditis with a protruding auricle. Careful observation of the skin and consideration from developmental aspects are needed for an accurate diagnosis.

Cholesteatoma Surgery With a Dehiscent High Jugular Bulb Treated With Surgery Assisted With Underwater Endoscopy: A Case Report.

A dehiscent high jugular bulb would be a pitfall in middle ear surgery especially for cholesteatoma. We report a case of cholesteatoma attached to a dehiscent high jugular bulb successfully treated with surgery assisted with underwater endoscopy. To the best of our knowledge, no previous study has reported a case of cholesteatoma with dehiscent high jugular bulb treated with surgery assisted with underwater endoscopy. Owing to the risk of jugular bulb injury, underwater endoscopy is a good indication for middle ear cases with a dehiscent high jugular bulb to obtain a clear operative field and avoid an unexpected air embolism.

Inflammatory Myofibroblastic Tumor (IMT) of the Trachea Excised by Transtracheal Surgery: Case Report.

This report describes an extremely rare case of a primary inflammatory myofibroblastic tumor of the trachea. The patient underwent surgical resection by a transtracheal approach and reconstruction with esophageal tracheoplasty. This case report highlights the rarity of such tumors and a minimally invasive and safe surgical technique for tumors around the central neck structures.

Head and neck small-cell carcinoma: A multicenter study of 39 cases from 10 institutions.

Objective: Basal information of head and neck small-cell carcinoma (HNSmCC) including epidemiology, primary site, treatment, and prognosis remains sparse due to its rarity. We report here a multicenter retrospective study on the diagnosis, treatment, and outcomes of patients with HNSmCC. Materials and methods: This study involved 47 patients with HNSmCC from 10 participating institutions. Eight patients were excluded for whom no pathological specimens were available (n = 2) and for discrepant central pathological judgements (n = 6). The remaining 39 patients were processed for data analysis. Results: As pretreatment examinations, computed tomography (CT) was performed for the brain (n = 8), neck (n = 39), and chest (n = 32), magnetic resonance imaging (MRI) for the brain (n = 4) and neck (n = 23), positron emission tomography-CT (PET-CT) in 23 patients, bone scintigraphy in 4, neck ultrasonography in 9, and tumor markers in 25. Primary sites were oral cavity (n = 1), nasal cavity/paranasal sinuses (n = 16), nasopharynx (n = 2), oropharynx (n = 4), hypopharynx (n = 2), larynx (n = 6), salivary gland (n = 3), thyroid (n = 2), and others (n = 3). Stages were II/III/IV-A/IV-B/IV-C/Not determined = 3/5/16/6/5/4; stage IV comprised 69%. No patient had brain metastases. First-line treatments were divided into 3 groups: the chemoradiotherapy (CRT) group (n = 27), non-CRT group (n = 8), and best supportive care group (n = 4). The CRT group included concurrent CRT (CCRT) (n = 17), chemotherapy (Chemo) followed by radiotherapy (RT) (n = 5), and surgery (Surg) followed by CCRT (n = 5). The non-CRT group included Surg followed by RT (n = 2), Surg followed by Chemo (n = 1), RT alone (n = 2), and Chemo alone (n = 3). The 1-year/2-year overall survival (OS) of all 39 patients was 65.3/53.3%. The 1-year OS of the CRT group (77.6%) was significantly better compared with the non-CRT group (31.3%). There were no significant differences in adverse events between the CCRT group (n = 22) and the Chemo without concurrent RT group (n = 9). Conclusion: Neck and chest CT, neck MRI, and PET-CT would be necessary and sufficient examinations in the diagnostic set up for HNSmCC. CCRT may be recommended as the first-line treatment. The 1-year/2-year OS was 65.3%/53.3%. This study would provide basal data for a proposing the diagnostic and treatment algorithms for HNSmCC.

Upper and Lower Respiratory Mucous Membrane Plasmacytosis with a Cobblestone Appearance: a Case Report.

A 53-year-old woman with a recurrent sore throat, xerostomia, nasal obstruction, and nasal discharge came to our hospital. At the first visit, she did not have a fever, but a blood test showed an intense inflammatory response. She had oral mucosal erosion. She developed dyspnea 2 months later. Nasendoscopy, laryngoscopy, and bronchoscopy showed upper and lower respiratory mucosa cobblestone appearance. Microscopy of the nasal and pharyngeal mucosa biopsy found regular mononuclear inflammatory cell dense submucosal infiltrate, mainly plasma cells. A mucous membrane plasmacytosis diagnosis was made. Prednisolone 1 mg/kg/day rapidly improved bronchial symptoms and cobblestone appearance.

Comparison of different methods for evaluating the relationship between facial nerve and benign parotid tumors.

Objectives: The goals of benign parotid gland tumor resection are complete resection of the lesion and preservation of the facial nerve function. As the facial nerve cannot be directly visualized via imaging modalities, several methods, including the facial nerve line, Utrecht line, retromandibular vein, Stenon duct, and minimum fascia-tumor distance techniques, have been developed to estimate its location. However, there are no reports on their accuracy in determining tumor location. In the present study, we aimed to assess the diagnostic accuracy of these methods based on tumor location. Methods: This retrospective study analyzed medical records and histological reports of 359 patients with various types of benign parotid gland tumors who underwent a parotidectomy between April 2014 and March 2020. The tumor location was subdivided into the following sections: anterior, superior, inferior, and middle. The tumor location was estimated using five methods: facial nerve line, Utrecht line, retromandibular vein, Stenon duct, and minimum fascia-tumor distance. The final diagnosis of superficial or deep lobe tumor was made based on surgical findings. Results: Each method showed a higher accuracy for superficial tumors (was more than 90%) than for deep lobe tumors. In contrast, for deep lobe tumors, the accuracy of diagnosis with the facial nerve line, Utrecht line, and retromandibular vein methods was low, in the 30% range. Among all methods, the Stenon duct method had the highest accuracy in the diagnosis of deep lobe tumors. The SD method was most useful in cases where both the duct and tumors were detected. The minimum fascia-tumor distance method had the second highest diagnostic accuracy (63%); however, for anterior tumors, it tended to provide false negatives. Conclusions: All tested methods were useful in diagnosing superficial lobe tumors; however, they were not helpful in diagnosing deep lobe tumors, especially anterior tumors.

Olfactory Preservation in Craniofacial Resection of Tumor Invading Hemianterior Skull Base: Operative Video.

In traditional craniofacial resection of tumors invading the anterior skull base, the bilateral olfactory apparatus is resected. Recently, transnasal endoscopy has been used for olfactory preservation in resections of unilateral low-grade malignancies. However, for tumors that invade the orbita or for high-grade malignancies, the transnasal endoscopic skull base surgery has been controversial. This video demonstrates the surgical techniques of olfactory preservation during craniofacial resection of a high-grade malignancy invading the hemianterior skull base and orbita. We present the case of a 32-year-old woman with osteosarcoma in the right ethmoid sinus. The tumor invaded the ipsilateral cribriform plate, dura menta, and orbital periosteum; however, the nasal septum and crista galli were intact ( Fig. 1A, B ). Because the tumor was a high-grade malignancy and the orbita had been invaded, we performed craniofacial resection instead of endoscopic resection ( Fig. C2A ). We drilled into the right side of the crista galli, midline of the cribriform plate, and perpendicular plate of the ethmoid bone via craniotomy. As a result, we accessed the nasal cavity directly ( Fig. 2B ). To preserve the nasal septum, we detached the remaining right septal mucosa through the transfacial approach ( Fig. 2C ). Because of the high risk of cerebrospinal fluid leakage as a result of previous irradiation, we performed vascularized free flap reconstruction of the skull base instead of pericranial flap. Postoperative computed tomography revealed no evidence of tumor ( Fig. 1C, D ). The patient's sense of smell returned after 1 postoperative day, and she was discharged on the postoperative day 14. The link to the video can be found at: https://youtu.be/XzPABYwzkjs .