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Background Strong eosinophil infiltration in chronic rhinosinusitis with nasal polyp (CRSwNP) is highly associated with recalcitrance and higher nasal polyp recurrence rate after surgery. The prevalence of eosinophilic CRSwNP (ECRS) is increasing in Asian countries including Japan. Benralizumab is a humanized anti-IL-5R alpha monoclonal antibody that depletes eosinophils by antibody-dependent cell-mediated cytotoxicity. Objective To assess the efficacy and safety of benralizumab in patients with ECRS. Methods This phase II, randomized, double-blind, placebo-controlled study was conducted in Japan. Patients were randomized 1:2:2 to placebo, a single administration of benralizumab 30 mg, or benralizumab 30 mg every 4 weeks (q4w) for a total of three doses. The primary endpoint was the change in nasal polyp score from baseline at Week 12. Results Overall, 56 patients were enrolled (placebo, n = 11; benralizumab single dose, n = 22; benralizumab q4w, n = 23). Although the mean total nasal polyp score began to decrease after the initiation of benralizumab treatment, there were no statistically significant differences in change in nasal polyp score from baseline at Week 12 between benralizumab and placebo (placebo, −0.5 ± 0.8; benralizumab single, −0.3 ± 0.8; benralizumab q4w, −0.5 ± 1.5). Post-hoc analysis showed that the administration of benralizumab decreased nasal polyp scores ≥2 points in 42.2% of ECRS patients and that patients with high blood eosinophil levels had a greater tendency to respond to benralizumab treatment. The safety profile was similar to that in previous studies and no unexpected adverse events were noted. Conclusion Although benralizumab did not meet the primary efficacy endpoint, reductions of nasal polyp scores were seen in the benralizumab group compared with the placebo group over the whole study period, especially in patients with high levels of blood eosinophils.

OBJECTIVES/HYPOTHESIS The aim of this study was to evaluate the utility of 18 F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) for accurately diagnosing immunoglobulin G4-related sclerosing sialadenitis (IgG4-SS). STUDY DESIGN Retrospective cohort study. METHODS We reviewed the records of 64 patients with IgG4-SS (35 male and 29 female patients) and 10 patients with clinically suspected IgG4-SS. Pathological diagnoses of patients clinically suspected with IgG4-SS included four cases of malignant lymphoma, one case of multicentric Castleman disease, one case of Sjogren's syndrome, and four cases of sialadenitis. All patients underwent submandibular gland (SMG) biopsies and baseline FDG-PET/CT evaluation. Clinical, serological, pathological, and PET/CT findings were analyzed. We also investigated maximum standardized uptake values (SUVmax) in the salivary glands of 15 patients with malignant disease of the salivary glands during the same period. RESULTS Increased FDG uptake in the SMG and parotid gland was found in 63 (98%) and 23 (35%) patients with IgG4-SS, respectively. FDG uptake of the bilateral SMG and unilateral SMG was recorded in 57 patients (89%) and six patients (9%), respectively. Mean SUVmax in patients with malignant disease of the salivary glands was significantly higher than that in patients with IgG4-SS (P = .035). We defined a positive test for IgG4-SS diagnosis as high SMG FDG uptake and serum IgG4 level ≥135 mg/dL, resulting in a sensitivity, specificity, and accuracy of 96.9%, 90.0%, and 86.4%, respectively. CONCLUSIONS FDG-PET/CT findings in combination with serological and clinical findings may have the capacity to diagnose IgG4-SS and lead to less-invasive biopsy procedures. LEVEL OF EVIDENCE 4. Laryngoscope, 128:1120-1125, 2018.

We present two cases of an HIV-associated parotid gland cyst. One case was a 36-year-old HIV infected woman. She was diagnosed with HIV infection and presented with slowly enlarged parotid gland cysts together with elevation of HIV viral RNA copies/mL in her serum. She was performed parotid gland biopsy under the general anesthesia. The histopathologic analysis revealed negative HIV p24-antigen in her parotid gland tissue. The other case was a 43-year-old man found his parotid gland swelling shortly after highly active antiretroviral therapy (HAART). He was diagnosed with HIV infection 2 years previously. He had started HAART several days before. He showed exceeding elevation of IgE in his serum. We treated him with medication using anti-histamic drugs for his cyst. A computed tomography scan revealed a complete response of his parotid gland cyst 4 weeks after the medication. His serum IgE level was decreased to half of the level before the medication. These findings suggested that the parotid gland swelling associated with HIV was due to various factors including immune reconstitution inflammatory syndrome (IRIS). In case such a parotid gland swelling, we could avoid invasive treatments.

An aberrant internal carotid artery (ICA) is a rare abnormality associated with life-threatening otorrhagia if inadvertently injured during middle-ear surgery including myringotomy. We present a case where a 3-year-old girl experienced massive otorrhagia following myringotomy, and computed tomographic scan showed the aberrant ICA. Bleeding was controlled by ear canal packing, but rebleeding occurred. Investigations by carotid angiography demonstrated a pseudoaneurysm of the aberrant ICA in the middle ear. We attempted surgical repair using a high-flow bypass technique; however, the bypass graft was occluded by embolic complications, and eventually, ligation of the ICA was performed, which led to the paralysis of the patient's left limbs. In this report, management of iatrogenic aberrant ICA injuries and pseudoaneurysms in the middle ear are discussed based on the case that we experienced.

OBJECTIVE To investigate the relationship between the severity of ear anomaly and mandibular dysplasia in congenital microtia. STUDY DESIGN Retrospective case review. SETTING Sapporo Medical University Hospital. PATIENTS Congenital microtia: 44 patients over a period of 4 years. INTERVENTIONS The height of the condylar process of the mandible was assessed by three-dimensional computed tomography (CT), and the patients were divided into three groups based on the ratio of the condylar process height on the affected side to that on the unaffected side: Group A, ≥1.00; Group B, 0.99 to 0.85; Group C

Objective Adrenergic receptors (ARs) include four general types (α1, α2, β1 and β2), which are found in different target tissues. α-AR agonists are commonly used for decongestant therapy of upper airway diseases. In order to clarify the roles of AR subtypes in the upper airways, we investigated the localization of these receptors by immunohistochemistry. Methods Human turbinates were obtained after turbinectomy from 12 patients with nasal obstruction refractory to medical therapy. The specific cells expressing α- and β-AR proteins were identified by immunostaining using an anti-human AR subtype-specific antibodies (α1A-, α1D-, α2C- and β2-ARs) antibody. Results Immunohistochemical analysis revealed that immunoreactivities for α1D- and β2-ARs were densely distributed in submucosal glands. In contrast, immunoreactivities for α1A- and 2C-ARs were densely distributed in vascular smooth muscle. Conclusion Our results suggested that adrenergic receptor (AR) subtypes had different roles in upper airway diseases, such as allergic rhinitis and nonallergic rhinitis.

Conclusion The diagnosis of immunoglobulin G4-related disease (IgG4-RD) should be based on the morphology of tissue biopsy, and this study recommends a submandibular gland (SMG) biopsy for accurate diagnosis and to exclude malignant disease. Objective To clarify which type of biopsy specimen (SMG or labial salivary gland [LSG]) should be taken from patients with IgG4-RD. Methods This study included 33 patients with IgG4-RD (21 women; 12 men) who were subjected to both SMG and LSG biopsies at Sapporo Medical University between 2011-2015. Tissues obtained from the SMG and LSG specimens were evaluated. Results All SMG specimens satisfied the diagnostic criteria for IgG4-RD, whereas 19 (57.6%) LSG specimens satisfied the diagnostic criteria for IgG4-RD. Histological evaluation showed fibrosis in all the SMG specimens and in eight LSG specimens (24.2%). Obliterative phlebitis was seen in nine SMG specimens (27.3%), but it was absent in all the LSG specimens.

Tricellular tight junctions (tTJs) are specialized structures that occur where the corners of three cells meet to seal adjacent intercellular space. The molecular components of tTJs include tricellulin (TRIC) and lipolysis-stimulated lipoprotein receptor (LSR) which recruits TRIC, are required for normal hearing. Although loss of TRIC causes hearing loss with degeneration of cochlear cells, the detailed mechanisms remains unclear. In the present study, by using temperature-sensitive mouse cochlear cells, US/VOT-E36 cell line, we investigated the changes of TRIC and LSR during cochlear cell differentiation and the effects of histone deacetylase (HDAC) inhibitors against cell degeneration induced by loss of TRIC and LSR. During cell differentiation induced by the temperature change, expression of TRIC and LSR were clearly induced. Treatment with metformin enhanced expression TRIC and LSR via AMPK during cell differentiation. Loss of TRIC and LSR by the siRNAs induced cell death in differentiated cells. Treatment with HDAC inhibitors trichostatin A and HDAC6 inhibitor prevented the cell death induced by loss of TRIC and LSR. Collectively, these findings suggest that both tTJ proteins TRIC and LSR have crucial roles for the differentiated cochlear cell survival, and that HDAC inhibitors may be potential therapeutic agents to prevent hearing loss.

Objective Tracheo-innominate artery fistula (TIF) is a rare but life-threatening complication following tracheostomy or tracheoesophageal diversion (TED). Although successful surgical intervention for TIF has been reported, few studies have been performed in patients with severe motor and intellectual disability (SMID). Therefore, we aimed to analyze TIF in patients with SMID to clarify the clinical variables predicting the occurrence and adequate management for lifesaving of TIF. Methods We retrospectively reviewed the records of patients with SMID undergoing surgical tracheostomy and TED between 2006 and 2012 and identified those with TIF. When TIF occurred, we obtained the clinical status and emergency management. Results Of 70 patients who underwent tracheostomy or TED during the study period, three patients had TIFs; in one case, TIF was avoided by ligation of the innominate artery before TED. The incidence of TIF in those undergoing tracheostomy and TED was 2.3% and 7.4%, respectively. The interval between tracheostomy and TIF was 14–50 months. Conclusions Patients with SMID may have an increased risk of TIF. Prompt diagnosis and surgical intervention to control the bleeding is the only effective management at present.

The current clinical study was performed on 311 cases of pars flaccida and 89 cases of pars tensa cholesteatoma which were treated with canal wall reconstructed tympanoplasty between 1991 and 2012. The average follow-up time of these patients was 5.3 years. Since follow-up periods were different in each case and some censored patients were involved, we used survival analysis on this study to discuss the cumulative rates of disease-free successful cases and the rates of recurrent cholesteatoma throughout the postoperative course. The disease-free successful cases were defined as those cases in which patients were both out of re-operation with recurrent and residual cholesteatoma and out of revision operation with another problem, furthermore, maintained good hearing outcome. Based on the criteria set by the Japan Otological Society (2010), the cases that satisfied the following were evaluated as good hearing results; (a) a successful case in which preoperative bone conduction was used, and (b) a case in which the postoperative air-bone gap was within 20dB after tympanoplasty for chronic otitis media. The analysis results were shown for each of (a) and (b). 1. In pars flaccida cholesteatoma, the 5-year survival rate of successful case was (a) 76.1% and (b) 83.9%, the 10-year survival rate was (a) 58.9% and (b) 73.0%. In pars tensa cholesteatoma, the 5-year survival rate of successful cases was (a) 57.7% and (b) 63.5%, the 10-year rate was (a) 42.1% and (b) 56.9%. A significant difference was seen between pars flaccida and pars tensa cholesteatoma (p < 0.001). 2. In pars flaccida cholesteatoma, the 5-year recurrence rate was 7.6% and the 10-year rate was 15.3%, and the recurrence rate increased gradually throughout the follow-up period. On the other hand, in pars tensa cholesteatoma, the increase in the recurrence rate reached a peak 15.8% at 5.5 years after the surgery. A long-term follow-up is necessary when evaluating the clinical results after tympanoplasty.

A nerve reconstruction was performed in 20 patients whose spinal accessory nerve was resected during total neck dissection. Re-anastomosis or a cable graft was performed between both cut ends of the accessory nerve in 14 patients (accessory nerve reconstruction group), and between the peripheral cut end of the accessory nerve and the central cut end of the cervical nerve (C2 or C3) in 6 patients (cervical/accessory nerve reconstruction group). There was no difference in the postoperative shoulder functions between the reconstruction groups, and both groups were significantly better than the group without reconstruction (n = 13), although they tended to be poorer than the nerve preservation group (n = 41).

Occurrence of airborne pollen in Sapporo has been studied for 19 years during the period between 1995 and 2013. There are wide year-to-year variations in the quantities of birch pollens. A simple linear regression with the least squares method was used for studying correlations between the annual quantities of birch pollens and the meteorological factors. A significant positive correlation was found between the hours of sunlight in June of the preceding year and the annual birch pollen concentrations with the correlation coefficient, R = 0.667. Also, we found the significant positive correlation between the hours of sunlight in March and the annual birch pollen concentrations with the correlation coefficient, R = 0.684. These results suggest that the atmospheric birch pollen counts can be predicted from meteorological factors.

CONCLUSION This is the first report to investigate the correlation between ear anomalies related to the development of specific ear structures and chorda tympani dysfunction (CTD) in congenital microtia. CTD is not always consistent with the severity of the ear anomaly or the presence of facial nerve paralysis (FNP). OBJECTIVES To investigate the relationship between the severity of ear anomalies and CTD as well as FNP in congenital microtia. METHODS A retrospective assessment was performed for all patients with microtia over the period 2010-2016. All ears were graded based on the severity of ear deformity using the Jahrsdoerfer system, based on findings on computed tomography of the temporal bone. Electrogustometry (EGM) was performed to evaluate CTD. RESULTS The group included 110 male and 62 female patients. The right ear was the most commonly affected (right 106, left 47). Eighteen patients (10.5%) had abnormal EGM thresholds. The mean (± SD) Jahrsdoerfer scores in the without CTD and positive for CTD groups were 6.53 ± 0.32 and 7.06 ± 0.37, respectively. In terms of sub-total points, there was no significant correlation between anatomic structure and CTD. There was no significant correlation between CTD and the presence of FNP.

IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder, characterized by elevated serum IgG4 levels as well as abundant infiltration of IgG4-positive plasmacytes and fibrosis in various organs, including the head and neck region. In particular, the salivary glands, orbit, and thyroid are common sites of disease involvement. IgG4-RD is diagnosed based on various clinical, serological, and histopathological findings, none of which are pathognomonic. Hence, various differential diagnoses, which exhibit elevated serum IgG4 levels and infiltration of IgG4-postive cells into tissues, need to be excluded, especially malignant diseases and mimicking disorders. Systemic corticosteroids are generally effective in inducing IgG4-RD remission; however, recurrent or refractory cases are common. In addition, although the pathogenic mechanisms of IgG4-RD remain unclear, an antigen-driven inflammatory condition is believed to be involved. Recent studies have indicated the important pathogenic role of B cell/T cell collaboration and innate immunity in this disease. Nevertheless, additional research and discussions are needed to resolve many remaining questions. In this review, we provide an overview of the recent insights on the history, clinical features, diagnosis, and treatment of IgG4-RD in the head and neck region. Furthermore, we have also addressed the pathogenesis of this disease.

Objective In northern Japan, birch pollen is the major allergen in pollinosis, while oral allergy syndrome (OAS) is caused primarily by apple and peach, and is almost exclusively related to birch pollinosis. To clarify the clinical benefit of allergen-based component-resolved diagnosis (CRD) in Japanese birch-allergic patients with OAS, we present an analysis of IgE profiles in response to crude extracts and recombinant component-resolved allergen to birch pollen and Rosaceae fruits allergens. Methods The sera of 30 patients with birch pollen-related OAS to apple or peach were analyzed for specific IgE reactivity to pathogenesis-related class 10 (PR-10) family (birch: rBet v 1, apple: rMal d 1, and peach: rPru p 1), profilin (birch: rBet v 2 and peach: rPru p 4), and lipid transfer protein (LTP) (apple: rMal d 3 and peach: rPru p 3) allergens, as well as to conventional crude, unfractionated extracts (birch: T3, apple: f49, and peach: f95) using the ImmunoCAP System™. Allergen-specific IgE values Results Of the 30 sera CAP-positive for natural birch pollen extract, 28 (93.3%) exhibited specific IgE against Bet v1, and two (6.7%) contained specific IgE against Bet v2. Of the 26 sera of OAS to apple patients, only 17 were positive for specific IgE against f49 extract (65.4%); however, 24 were positive for specific IgE against rMal d 1 (92.3%). Similarly, only 17 of the 23 sera of OAS to peach patients contained specific IgE against the f95 extract (73.9%); however, 22 were positive for specific IgE against rPru p 1 (95.7%). Conclusion Our data suggest that CRD constitutes a reliable tool for the diagnosis of birch pollen-related OAS.

The 3-year progression-free survival rate of non-invasive salivary duct carcinoma (SDC) or adenocarcinoma not otherwise specified (NOS) was significantly better than that of invasive SDC or adenocarcinoma NOS in Carcinoma ex pleomorphic adenoma (CXPA). The presence of invasion is an important prognostic factor for SDC and adenocarcinoma NOS in CXPA.CXPA is a rare parotid gland malignant tumor for which therapy is not yet standardized. The purpose of this study was to review the characteristics of CXPA patients and to analyze their outcomes in the Northern Japan Head and Neck Cancer Society.The medical records of 33 patients who had been provided initial treatment in 12 institutes of northern Japan from 2002-2011 were reviewed as a multi-institutional retrospective study.The 3-year overall and progression-free survival rate of all patients was 79.9% and 76.8%, respectively. Both the 3-year overall and progression-free survival rates were 87.5% for patients with non-invasive SDC or adenocarcinoma NOS. The 3-year overall and progression-free survival rates for patients with invasive SDC or adenocarcinoma NOS were 60.4% and 30.5%, respectively. The progression-free survival rates for patients with invasive SDC or adenocarcinoma NOS was significantly poor (p 0.05).

Chronic sclerosing sialadenitis, which clinically resembles a salivary gland tumor, is considered a salivary gland lesion of the IgG4-related inflammatory disease. Corticosteroids are effective for the treatment of chronic sclerosing sialadenitis, but the natural clinical course of this disease is not fully understood. We report a 67-year-old man with chronic sclerosing sialadenitis who showed lung lesions, which spontaneously regressed without steroid therapy after excision of the submandibular gland. He presented with several weeks’ history of continuous swelling in the right submandibular region. Physical examination showed a hard swelling of the right submandibular gland; the cytological finding was Papanicolaou class III. A chest X-ray demonstrated bilateral infiltration of the right middle and left lower lobes. The right submandibular gland was excised under general anesthesia for definite diagnosis, and histopathological examination revealed an IgG4-positive plasmacytic infiltration accompanied by periductal fibrosis and interlobular sclerosis, consistent with chronic sclerosing sialadenitis. Five months after operation, the lung lesions regressed spontaneously.

Nasal dermoplasty is currently the treatment of choice in severe epistaxis in those with hereditary hemorrhagic telangiectasia. The Japanese literature dose not, to our knowledge, report the combination of inferior tubinectomy and nasal dermoplasty in such treatment. A 69-year-old woman with hereditary hemorrhagic telangiectasia and recurrent epistaxis treated combining tubinectomy and dermoplasty and who had required 174 units of blood 1 year before dermoplasty enjoyed a decrease to 18 units 1 year after dermoplasty.

Altered expression of claudin-1, claudin-7, and tricellulin in early tonsillar squamous cell carcinoma (SCC) independent of human papilloma virus (HPV) infection may lead to tumor progression.Integral tight junction proteins, the claudins and tricellulin, play a crucial role in all tissues. HPV is significantly associated with tonsillar SCC. We sought to determine the expression of claudin-1, claudin-7, and tricellulin in HPV-infected and HPV-free tonsillar SCC.Twenty-eight tonsillar SCCs were studied by immunohistochemical analysis and real-time reverse transcription polymerase chain reaction with in situ hybridization analysis.Compared with normal tissues, claudin-1 was strongly expressed, whereas claudin-7 and tricellulin were weakly expressed or were absent in primary SCC and metastatic lymph nodes. Claudin-7 and tricellulin were markedly reduced at all stages of tumor development. In situ hybridization analysis showed no correlation between HPV infection and altered expression of the tight junction proteins.

Although the most common soft-tissue sarcoma in children, rhabdomyosarcoma (RMS) is rare in adults. We report 2 cases of adult RMS. Case 1: A 59-year-old man seen for a large right-neck mass was found in computed tomography (CT) and magnetic resonance imaging (MRI) to have an enhanced mass in the right ethmoid sinus with bone destruction and multiple bilateral cervical lymph nodes, but was no evident distant metastasis. Tumor biopsy showed alveolar rhabdosarcoma. The man was treated with chemoradiotherapy, but died of multiple metastases 5 months after treatment. Case 2: A 75-year-old woman was found in cranial MRI to have a right ethmoid sinus mass confirmed in CT to involve bone destruction, but no evident metastasis. The pathological diagnosis was embryonal rhabdosarcoma. After a poor result with chemoradiotherapy, the woman underwent surgery, and remains alive without local reccurence or distant metastasis in the 26 months since surgery.

Ranulas are conventionally treated by total resection, marsupialization or intracystic OK-432 injection. We clinically studied 16 such cases from 2000 to 2008—15 sublingual and 1 mixed. The ranula was excised in 7 cases with recurrence in 42.9%, marsupialized in 3 with 100% recurrence, the sublingual gland totally resected in 4 with no recurrence, and OK-432 injected intracystically in 2 with 50% recurrence. Our results thus suggest that total sublingual gland resection is an effective method for the treatment of ranula.

Objective: To determine the frequency of hearing impairment in children with congenital anomalies of the central nervous system (CNS) by using detailed audiological evaluation methods. Methods: The patients were 78 children with congenital anomalies of the CNS with a mean age of 29.5 months. They had been observed for a mean period of 38.5 months. Hearing levels were evaluated behavioral observation audiometry (BOA), visual reinforcement audiometry (VRA) and distortion product otoacoustic emissions (DPOAEs) were performed. Auditory brainstem responses (ABRs) and computed tomography (CT) scans of the temporal bone were performed in the cases in which the minimum response levels (MRLs) were above 30 dBHL. All cases were assessed in terms of developmental age. Results: A total of 14.1% (11/78) of the children with congenital anomalies of the CNS were initially diagnosed with bilateral sensorineural hearing loss (SNHL). However, the hearing levels of nine of them improved by the time of the last diagnosis. Therefore, the patients with bilateral SNHL were only 2.6% (2/ 78) of the total patients with congenital anomalies of the CNS at last diagnoses. As shown by our results, many children with bilateral SNHL at initial diagnosis showed improved ABR thresholds and behavioral hearing thresholds with age. In this series, the use of hearing aids was arranged for six patients. However, four patients stopped using hearing aids when their hearing threshold levels improved. In two cases, there were no changes in hearing levels and the children continued using hearing aids. Conclusion: Our results suggest that hearing level recovery can occur in some children with CNS anomalies. Confirmation of hearing loss in children with congenital anomalies of the CNS takes a long time. There are improvements in hearing loss during the observation period. Therefore periodic assessment of hearing is important.

Kawasaki disease was first reported by Tomisaku Kawasaki in 1967 and there have been 200,000 cases reported in Japan. It is predominantly a disease of young children, and 80% of the patients are younger than 5 years of age. We report a case of Kawasaki disease that was difficult to diagnose. A 12-year-old boy was referred to our hospital with a fever of 40 degrees Celsius and swelling of the right neck lymph nodes, despite a course of antibiotics. A CT scan of the neck showed multiple swollen lymph nodes and laryngeal-edema. He was hospitalized on the same day. After 10 days of fever, point-like eruptions appeared on his chest and he developed conjuctival congestion. Therefore, he was diagnosed as having Kawasaki disease and was treated with γ-globulin (2 g/kg) and aspirin (40 mg/kg/day). On the day after beginning this treatment, his body temperature fell to 37 degrees Celsius, and the pointed eruptions and conjuctival congestion improved gradually. He was discharged from the hospital after 23 days after on set of fever.

Summary Hamartoma, defined as tumour-like overgrowths of tissue, may occur in any organ in the body and is commonly seen in liver, spleen, kidney and lung, however, lingual hamartoma is a rare entity. Terminal deletion of chromosome 2q is a chromosomal abnormality causing various craniofacial malformations, and no reports can be obtained about patients who have chromosomal disorder presenting with lingual hamartoma. We describe the first case of lingual hamartoma in a patient with terminal deletion of chromosome 2, and summarize the clinical management and the histogenesis of lingual hamartoma from the literature. A 9-month-old girl who had chromosome 2q terminal deletion consulted Sapporo Medical University hospital for evaluation of upper airway obstruction. She had retractive breathing soon after birth and required treatment with continuous positive airway pressure (CPAP). Physical examination showed craniofacial abnormalities, such as round face, high palate, aural atresia, anotia and so on. Fiberoptic examination and CT scan revealed a mass on the right side of the tongue base. She underwent surgical resection and the tumour was pathologically diagnosed as hamartoma. After surgery, her respiratory condition improved to the extent where CPAP was withdrawn. There has been no tumour recurrence during the 1-year follow-up period.

Fungal paranasal sinusitis is included in the differential diagnosis of unilateral paranasl lesion. Recently the incidence of fungal paranasal sinusitis has been increasing. We reviewed 24 patients (9 males and 15 females) with fungal paranasal sinusitis treated at Muroran City Hospital between January 2001 and May 2006, and clinical presentation and CT findings with those of 56 patients (36 males and 20 females) with chronic unilateral sinusitis. Fungal sinusitis patients ranged in age from 45 to 87, and the average age was 65.9 years old. In contrast, the age of chronic sinusitis patients ranged from 24 to 83, and the average age was 54.4 years old. The chief complaint of both fungal sinusitis and chronic sinusitis included rhinorrhea, nasal obstruction and post nasal discharge.CT exam was performed in all patients. In 23 cases of paranasal fungal sinusitis and 54 cases of chronic sinusitis the findings involved the maxillary sinus. The most common observation (69.6%) was bone density within the affected sinus in fungal sinusitis. However, only 2 cases of chronic sinusitis (3.9%) showed calcification.All cases of fungal sinusitis were diagnosed by pathological examinations. Most cases were proved to be aspergillus, while only one case was mucor. We treated all cases surgically, 18 cases underwent Caldwell-Luc's procedure and 5 cases underwent endoscopic sinus surgery under local anesthesia.

We reported three cases of Mikuhcz's disease. Mikulicz's disease was diagnosed by the following criteria: 1) symmetrical enlargement of the lacrimal and salivary glands lasting at least 3 months', 2) the lacrimal and salivary glands were microscopically involved in the infiltration of inflammatory cells, 3) sarcoidosis and other lymphoproliferative diseases were excluded. Three patients with Mikulicz's disease were all characterized by elevated IgG4 concentration in the serum and prominent infiltration of IgG4-positive plasmacytes in the minor salivary glands. On the contrary, there were no findings indicating sicca syndrome or anti-SS-A and SS-B antibodies, which characterize Sjogren syndrome. It is suggested that Mikulicz's disease is an IgG4-related plasmacytec disease, with a pathogenesis differing from that of Sjogren syndrome.

Angioleiomyoma is a benign tumor composed of smooth muscle cells and vascular endothelial cells, which is most commonly found in the uterus, skin, and alimentary canal as a painful mass. In the head and neck region, the incidence of this tumor is rare and pain is usually absent. We report a rare case of angioleiomyoma occurring in the left subcutaneous extrernal nose. A 41-year-old man visited our hospital complaining of a swelling on the left side of the nose. Physical examination revealed a soft mass in the left subcutaneous extrernal nose. CT scan and MRI with gadolinium enhancement showed well-enhanced mass in the left subcutaneous extrernal nose without bone destruction or apparent invasion into the left nasal cavity. Aspiration biopsy cytology revealed blood only. The tumor was fully excised without major intraoperative bleeding, and the postoperative course was successful. Immunohistochemical study revealed postive staining for vimentin and α-smooth muscle actin in tumor cells, and the tumor was diagnosed as angioleiomyoma. For over a year following surgery, there has been no evidence of recurrence.

The buccal space is a potential anatomical space bounded by the buccinator muscle medially and the parotid space posteriorly. This space chiefly consists of adipose tissue named the Buccal fat pad. Other contents are the parotid duct, facial nerve, accessory parotid gland, buccinator lymph nodes, facial artery and vein. Here we report a case of malignant lymphoma arising in the Buccal space.A 78-year-old woman presented with a painless tumor in her left cheek. The tumor had been present for approximately nine months and was slowly enlarging. Magnetic resonance imaging (MRI) and computed tomography (CT) of the head and neck demonstrated a 32mm×30mm×27mm tumor in the left buccal space. We surgically removed this tumor through the transoral approach. The pathologic diagnosis was follicular lymphoma grade 2 and there was no lesion in other organizations, therefore, she was not treated with radiotherapy and chemotherapy. No sign of recurrence or metastatic lesion has so far been found postoperatively.

Purpose: To review 13 cases of pyriform sinus fistula (PSF) and to summarize the clinical and histologic features. Materials and methods: A retrospective review and immunohistochemical analysis of 13 cases of PSF treated in Sapporo Medical University Hospital from 1985 to 2005. This study includes 13 patients with PSF diagnosed by the existence of fistulous tract radiologically and/or pathologically. Fistulectomy was performed in all cases. Surgically obtained specimens were processed for immunohistochemistry with commercial anticalcitonin antibody. Results: The patients consisted of 4 males and 9 females, and the ages ranged from 4 to 55 years. The fistula was detected by barium study in 12 cases (92.3 %), and all cases were on the left side. Pathologic detection of the fistula was achieved in only 8 cases (69.2 %). Immunohistochemistry showed abnormal distribution of calcitonin-positive cells in 12 cases (92.3 %). No recurrence has occurred in any of the patients. Conclusions: Surgical removal of the inflammatory tissue is worthy of consideration in suspected PSF cases as in patients who exhibit similar manifestations without preoperative detection of the tract.

Background Persons allergic to birch pollen often report oral and pharyngeal hypersensitivity to fruit and vegetables, due to immunological cross-reactivity between pollen and foods. This phenomenon is referred to as the oral allergy syndrome (GAS). Such cross-reactive antigen reactions mainly involve Bet v 1, which is the major birch-pollen allergen, and partially involve birch-pollen profilin Bet v 2. Soybean contains Bet v 1-related antigen (Gly m 4), and soy milk often causes the OAS with severe symptoms such as precordial and abdominal burning sensation because soy milk undergoes little denaturation, and this water-soluble liquid is consumed by most people rather quickly. We evaluated the frequency of the oAS after ingestion of soymilk and examined IgE antibodies to various allergens. Methods A total of 167 patients [122 women, 45 men; age range, 4-72 years (mean age, 32 years)], who had experienced GAS episodes and had IgE birch--pollen antibodies, were interviewed. Using the CAP system, we examined IgE antibodies to birch pollen and other allergens. Of 167 patients, 161 were examined for IgE antibodies to Bet v 1, Bet v 2, Gly m 4, and soybean. We evaluated the frequency of the GAS after soy milk ingestion based on reports by GAS patients with birch pollen allergy, and evaluated the positive rates of some of the IgE antibodies. Results Among the 167 patients with birch-pollen allergy and GAS on ingestion of any of the foods, there were 16 cases (10%) with OAS following soy milk ingestion. In addition, the foods that caused OAS most often were apples (123 cases, 74%), peaches (67%), and cherries (55%), followed by pears (37%) and kiwi (37%). A higher CAP class for birch pollen, Bet v 1, Gly m 4, and soybean was associated with a higher prevalence of OAS to soy milk. Of 15 patients who had GAS on ingestion of soy milk and had birch-pollen allergy, 47% (7cases) were CAP class 1 for soybean and only 7% (case) was CAP class c2, whereas 93% (14cases) were CAP class 1 for Gly m 4, and 87% (3cases) were CAP class ≥ 2 for Gly m 4. Conclusion Among the birch-pollen allergic OAS patients, 10% had the OAS on ingestion of soy milk, and among these with birch-pollen allergy and the OAS on ingestion of soy milk, the positive rate for soy milk CAP was low, whereas that for Gly m 4 CAP was high.

Tracheoesophageal diversion (TED) and laryngotracheal separation (LTS) can prevent aspiration pneumonia and improve the morbidity of patients with severe motor and intellectual disability (SMID). By improving hospitalization rates and care needs, the quality-of-life can be improved for the patients and their parents.This study evaluated the clinical outcomes of TED and LTS in patients with intractable aspiration and SMID.This study retrospectively reviewed patients with SMID and intractable aspiration pneumonia who underwent TED or LTS at the institution between January 2008 and January 2015. It assessed the frequency of sputum suctioning, the number of pre-operative and post-operative hospitalizations, the operative time, and complications.Forty patients were identified during the study period. After surgery, there were significant reductions in the frequency of secretion suctioning (from 165.0 times/day to 33.0 times/day) and the number of hospitalizations because of aspiration pneumonia (from 5.4 times/year to 0.2 times/year). A tracheocutaneous fistula occurred in one (2.5%) patient, and two (5.4%) patients developed tracheoinnominate artery fistulas. In the latter group, the innominate arteries were successfully ligated and endovascular embolization was performed.

Objectives: Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease entity characterized by elevated serum IgG4 and extensive IgG4-positive plasma cell infiltration of various organs. Patients with IgG4-RD show nasal manifestations with chronic rhinosinusitis. The objective of this study was to evaluate the clinical characteristics of sinonasal lesions in patients with IgG4-RD. Methods: We evaluated radiological findings of sinonasal lesions in 79 patients with IgG4-RD who were divided into 3 groups according to severity. We also compared serological findings, including serum IgG4 and IgE levels, and eosinophil counts. Results: Rhinosinusitis was found in 41 patients (51.9%). Although there were no significant differences in the serum IgG4 and IgE levels of the groups, there was a significant increase in eosinophil counts (445 ± 311.9/mm3) in Group C. Furthermore, 14 of the 41 patients with rhinosinusitis (34.1%) showed improvement after prednisolone administration. Patients with IgG4-RD and serum eosinophilia tend to also have sinonasal lesions. Conclusions: Rhinosinusitis is common in patients with IgG4-RD, and its pathogenesis can be similar to eosinophilic chronic rhinosinusitis.

Objective Patients with multiple sensory deficits, including hearing loss and visual impairment, present a unique problem. We evaluated the clinical outcomes of cochlear implantation in patients with severe to profound sensorineural hearing loss and visual impairment. Methods We retrospectively reviewed eight patients with severe sensorineural hearing loss and visual impairment who underwent cochlear implantation at our institution between 1993 and 2014. The follow-up period was between 2 and 20 years. We evaluated the case histories, etiologies of hearing loss and visual impairment, pre- and postoperative pure-tone thresholds, speech perception rates after CI using the Japanese CD speech discrimination scoring system (CI-2004 test) for words and sentences, and pre- and postoperative communication means. Postoperative speech discrimination scores were compared between patients with and without visual impairment who underwent cochlear implantation. Results The outcomes of cochlear implantation were good in all patients, with seven showing the ability to hold a conversation with others. The average proportion of correct answers for words and sentences in the CI-2004 test was 72.3 ± 19.1% and 86.0 ± 16.1%, respectively, for the patients with visual impairment and 62.1 ± 21.7% and 78.5 ± 20.9%, respectively, for those without visual impairment (based on auditory senses only). There were no significant differences in results between the patients with and without visual impairment. Conclusions Cochlear implantation is important for the rehabilitation of patients with severe auditory loss and visual impairment. Medical staff members require additional skills to perform auditory evaluations and rehabilitate patients with multiple sensory deficits.

Ossifying fibroma is an uncommon benign osteogenic neoplasm arising from cells of the periodontal ligament, typically with a slowly progressive enlargement of the affected bone. The neoplasm sometimes presents with hyperparathyroidism, most of which cases are due to familial parathyroid tumours. We report a rare case of ossifying fibroma of the mandible which showed very rapid growth and presented with primary hyperparathyroidism due to non-familial parathyroid adenoma. Despite improvement of parathyroid dysfunction after removal of the parathyroid adenoma, the tumour continued to grow very aggressively. The case required partial mandibular resection for complete resection of the tumour, and fixation of the remaining mandible with a titanium plate.

Summary Objective Colonization of middle ear pathogens is facilitated in the nasopharynx of otitis-prone children, and is associated with the development of otitis media. Recently, a new species of bacterium, Alloiococcus otitidis , is considered as one of the major middle ear pathogens. However, as far as we know, no study has been reported concerning the prevalence of A. otitidis in the nasopharynx of otitis-prone children. And, no study has been conducted on the association of A. otitidis in the nasopharynx with otitis media. Methods The frequency of A. otitidis in 83 middle ear effusions (MEE) and 56 nasopharyngeal swab (NPS) specimens from 56 children with otitis media was investigated by culture and polymerase chain reaction (PCR). Results A. otitidis was detected in 24 (28.9%) of MEE and in 6 (10.7%) of NPS specimens. When the frequency was investigated in relation to proneness to otitis media, A. otitidis was detected in 16 (64%) of 25 MEE and in 5 (29.4%) of 17 NPS specimens from otitis-prone children, whereas it was detected in 8 (13.8%) of 58 MEE and in 1 (2.6%) of 39 NPS specimens from non-otitis-prone children. The frequency of A. otitidis in both NPS and MEE specimens was significantly higher in otitis-prone than in non-otitis-prone children. Conclusions Our results suggest that colonization of A. otitidis is facilitated in the nasopharynx of otitis-prone children. And, nasopharyngeal colonization of A. otitidis may be associated with the frequency of otitis media.

The high density of [3H]-pranlukast binding sites on the local leukocytes in human nasal mucosa suggests that CysLT1 receptor antagonists may directly modulate cellular function of the local leukocytes through binding to CysLT1 receptor on allergic nasal mucosa.The cysteinyl leukotrienes (CysLTs) are lipid mediators that have been implicated in the pathogenesis of allergic diseases. Pharmacological studies using CysLTs indicate that two classes of receptors named CysLT1 and CysLT2 receptor exist. The former is sensitive to the CysLT1 receptor antagonist currently used to treat asthma and allergic rhinitis. To confirm the binding sites of CysLT1 receptor antagonist in human nasal mucosa, the autoradiographic distribution of CysLT1 receptor was studied in human nasal inferior turbinates.Cryostat sections were incubated with [3H]-pranlukast for autoradiography. Nonspecific binding was determined by adding unlabelled pranlukast.Autoradiograms indicated [3H]-pranlukast densely labeled on the interstitial cells. Blood vessels were sparsely labeled. There was no specific labeling in the submucosal glands or epithelium. These results support our previous report from in situ hybridization and immunohistochemistry of CysLT1 receptor.

Small cell carcinoma is relatively common in the lung, but very rare in the head and neck region. We describe our experience with two extremely rare cases of small cell carcinoma arising in the nasal cavity and paranasal sinus.(Case 1) The patient was a 54-year-old man who had complained of left eyelid swelling and diplopia. He had a mass in the left maxillary sinus. He was treated with irradiation and chemotherapy. However, during the treatment, distant metastasis appeared in the skin and he died 4 months after the initial treatment.(Case 2) The patient was a 47-year-old woman who had complained of left nasal obstruction. She had a mass in the left nasal cavity. Surgical resection and postoperative radiotherapy were performed. However, after the initial treatment, a metastatic swelling of the left cervical lymph node appeared. Left neck dissection was performed, and she underwent two courses of adjuvant chemotherapy. Following the initial treatment, no evidence of recurrence has been observed for 29 months.

Hemangioma occurs in every systemic region, and in the head and neck region, it is often found in the tongue, the lip, and the buccal mucosa. However, intramuscular hemangioma in the head and neck region is relatively rare.We treated two cases of hemangioma in the masseter muscle. Both the tumors contained various sizes of phlebolith. Combination of CT and MRI analysis was useful for identifying the tumor localization. Both tumors were excised by external incision, which enabled a secure enough field of operation and a certain extraction. Histologically, they were diagnosed as hemangioma.Here, we report the diagnosis and surgical treatment of hemangioma in the masseter muscle with reference to the literature.

Hemangiopericytoma is a relatively rare mesenchymal vascular tumor, which originates from pericytes that are small, oval cells around capillaries. This tumor can be found throughout the body, but most commonly hemangiopericytoma arises from the lower extremity, pelvis and retroperitoneum. The patient described in this report presented symptoms of snoring and sleep apnea. Magnetic resonance imaging (MRI) and computed tomography (CT) demonstrated a soft tissue mass in the right parapharyngeal space. This tumor was completely removed and the histological appearance of the specimen indicated a diagnosis of hemangiopericytoma.