In daily practice, acute otitis media and otitis media with effusion are usually treated by administration of antibacterial drugs, tympanostomy and insertion of tympanic membrane ventilation tubes. Most inflammatory diseases of the middle and external ear canal are caused by microorganisms such as bacteria and fungi, and identifying the causative microorganisms and selecting appropriate antibacterial and antifungal agents is important. If treatment is unsuccessful despite appropriate therapy, the possibility of other specific non-infectious inflammatory diseases should be considered.

Otitis media with ANCA-associated vasculitis (OMAAV) is one of the intractable types of otitis media that does not respond satisfactorily to conventional treatments. OMAAV is caused by systemic microangiitis and is characterized by positive serology for anti-neutrophil cytoplasmic antibodies. Awareness of this disease in the clinical setting has increased after the diagnostic criteria for OMAAV were published in 2015.

In this review, I shall provide an overview of OMAAV and discuss the diseases that should be differentiated from OMAAV. The causes of intractable otorrhea are not limited to otitis media, but also include external ear diseases, such as otitis externa and fungal infections of the external auditory canal, inflammatory diseases with morphological abnormalities, such as middle ear cholesteatoma, as well as cancer of the external auditory canal and skull-base osteomyelitis, in which inflammation spreads to the bone marrow. On the other hand, the possibility of OMAAV should also be considered in cases of prolonged otitis media with effusion without otorrhea, especially when impaired bone-conduction hearing, progressive hearing loss, and particular tympanic membrane findings are seen. It is important to make the diagnosis based on the presence or absence of findings characteristic of each disease. If the symptoms do not improve or are recurrent, a judgement needs to be made about whether the current diagnosis is consistent or another causative disease needs to be considered.

We report a case of intercapsular resection for a schwannoma arising from the greater auricular nerve.

The patient, a 40-year-old man, presented with a suspicious left cervical mass. The plastic surgeon attempted to remove it under local anesthesia. However, the patient reported numbness and pain around the tumor during the surgery, and the surgery was suspended. MRI was performed, and the findings were suggestive of a schwannoma arising from the greater auricular nerve, and the patient was referred to our center. We explained to the patient that the tumor was small and total resection might be successful, however, the patient wished to be followed up. Two years later, the patient presented with an increased tumor size, and removal of the tumor was scheduled. Since the nerve fibers were compressed by the tumor, we were able to remove the tumor by intercapsular resection.

As far as we could find, there are no reports of interosseous resection of schwannomas arising from the greater auricular nerve. Although the tumor was small, only approximately 10 mm in diameter, it was possible to perform an intercapsular resection while preserving the neurological functions.

Amyloidosis is a disease characterized by the deposition of amyloid in various organs, potentially resulting in organ dysfunction. It has been classified as systemic amyloidosis and localized amyloidosis. Amyloidosis involving the nares is uncommon. We report a rare case of bilateral localized amyloidosis of the external nares.

A 57-year-old woman presented to us with a one-year history of nasal obstruction. Physical examination revealed dense nodules in both nostrils. Histological and immunohistochemical examination confirmed the nodules as representing AL amyloid deposits. Whole-body examination revealed no amyloid deposition in any other organ, so that the patient was diagnosed as having localized nodular amyloidosis of the external nares. We performed two surgeries under general anesthesia, and achieved complete resection of the lesions. Her nasal obstruction improved, and no recurrence was observed until the last follow-up 2 years after the surgery.

Because systemic amyloidosis carries a poor prognosis, a thorough systemic workup is particularly important in patients with localized amyloidosis.

The Onodi cell is a normal variation often seen in the paranasal sinuses and is defined as a posterior ethmoidal cell that develops laterally and/or superiorly to the sphenoid sinus. In this article, we present a case of Onodi cell sinusitis that caused oculomotor and ophthalmic nerve dysfunction. A 73-year-old man visited our clinic with a month’s history of diplopia, left ophthalmalgia, and left eyelid ptosis. There was no visual impairment. CT showed a soft tissue lesion in the Onodi cell on the left side that extended to the outer part of the optic nerve. MRI showed high-intensity areas in the Onodi cell and cavernous sinus. With the diagnosis of Onodi cell sinusitis, we opened the Onodi cell and performed drainage by endoscopic surgery. The diplopia and ptosis improved postoperatively. Cases of visual and oculomotor disturbances due to Onodi cell or sphenoid sinusitis have been reported. In most cases, the oculomotor disturbances are associated with visual impairment. However, the precise mechanism involved in the development of the oculomotor disturbances has not yet been identified, because only a few cases of oculomotor disturbances alone (in the absence of visual disorders) have been reported. The present case suggests that the oculomotor nerve palsy was caused by inflammation that extended beyond the optic canal to the upper part of the cavernous sinus due to the aeration in the anterior clinoid process. As oculomotor nerve palsy tends to improve more easily than optic neuropathy, drainage by endoscopic sinus surgery is recommended even in cases in which a long time has elapsed after the onset of the disease.

Pediatric oropharyngeal trauma is a common injury encountered in daily practice. The pathophysiology varies depending on the nature of the foreign body that causes the injury, the presence or absence of damage of, and the site and extent of the injury; in general, most injuries are minor and do not require medical treatment. On the other hand, however, the possibility of serious complications should be kept in mind. In the case of children, it may be difficult to obtain a detailed history, because the injury might have occurred out of sight of the parents or family members, or it may be difficult to evaluate and treat local findings because of a lack of cooperation for physical examination, so that the initial response should be cautious.

In this article, we report seven cases of pediatric oropharyngeal trauma that required inpatient care at our hospital. Possible complications of oropharyngeal trauma and their treatment are described, and the appropriate methods of induction of and emergence from anesthesia should be considered when a foreign body remains lodged in the oropharynx.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a well-known autoinflammatory disease of childhood. Patients with PFAPA syndrome have been successfully treated by tonsillectomy, while it may be poorly controlled by oral pharmacotherapy. In addition, PFAPA syndrome is a self-limited disease that usually resolves by the age of eight. Therefore, it is mainly a childhood disease, and there are few reports in the literature of adult-onset PFAPA syndrome.

We report a case of adult-onset PFAPA syndrome. A 34-year-old woman underwent tonsillectomy for recurrent tonsillitis. Seven months after the surgery, she presented with periodic fever, pharyngitis, aphthous stomatitis, and cervical lymphadenopathy. She was initiated on treatment for PFAPA syndrome, and the fever resolved within 2 hours after an oral dose of prednisolone. Administration of cimetidine, a leukotriene receptor antagonist, and colchicine resulted in successful control of the PFAPA symptoms.

In the field of otorhinolaryngology, tonsillectomy is often performed for patients with recurrent tonsillitis presenting with recurrent fever and pharyngitis. Some of these cases could actually be cases of PFAPA syndrome. Although PFAPA syndrome in childhood has been shown to be cured by tonsillectomy in 90% of cases, there is no evidence yet to suggest that tonsillectomy might also be effective for adult-onset PFAPA syndrome. Therefore, it is important to consider autoinflammatory diseases, including PFAPA syndrome, even in adult patients presenting with recurrent tonsillitis, especially recurrent fever after tonsillectomy.

Arteriovenous malformation (AVM) is a progressive disease, and the treatment mainstays are intravascular treatment and surgery. However, there are some situations in which surgery cannot be selected due to the state of progression of the lesions in the head and neck region, which are often detected due to bleeding, and the potential functional impairment caused by surgery. Conversely, in regard to endovascular treatment, there are many cases that require multiple embolizations for recanalization and development of collateral circulation.

We encountered a 56-year-old woman who presented to us with bleeding from the oral cavity. Computed tomography showed an AVM at the base of the tongue, and laboratory examination revealed anemia and impaired coagulability of the blood associated with heavy bleeding. Since the lesion was extensive, we elected to perform embolization treatment after performing a tracheostomy. No obvious bleeding was observed after embolization of the lingual arteries of either side. However, due to severe obesity, it was difficult to manage the patient after embolization, and she eventually died of renal failure.

We used n-butyl-2-cyanoacrylate (NBCA) for the embolization in this case. Because NBCA exerts embolic effects regardless of the ability of the blood to coagulate, it is considered as a useful embolic material in cases with impaired coagulability of the blood.

An airway enlargement procedure is performed to close tracheostomies performed for bilateral vocal fold palsy. Vocal fold laterofixation by Ejnell’s method is the most commonly used airway enlargement procedure, but it widens the anterior glottal gap and impaires vocal function. On the other hand, arytenoidectomy widens the posterior glottal gap and can preserve vocal function. We report two cases of bilateral recurrent laryngeal nerve palsy in whom we performed arytenoidectomy.

Case 1: A patient who developed bilateral recurrent laryngeal nerve palsy after undergoing total thyroidectomy for papillary thyroid cancer at another hospital had an MPT of 10 seconds, MFR of 230 ml/s, and breath hoarseness of G1 and B1 on the GRBAS scale. Postoperatively, the patient’s voice function did not deteriorate and we closed the tracheostomy without dyspnea.

Case 2: Similar to Case 1, a patient who had developed bilateral recurrent laryngeal nerve palsy after undergoing total thyroidectomy for papillary thyroid cancer at another hospital was referred to our hospital. The preoperative MPT was 8.6 seconds, MFR was 500 ml/s, and the breath hoarseness was G2 and B2 on the GRBAS scale. Postoperatively, the MPT remained unchanged, the voice function was preserved, and we closed the tracheostomy without dyspnea.

Arytenoidectomy tends to preserve the voice function more easily than Ejnell’s method because it widens only the posterior glottal gap, making arytenoidectomy an effective technique for patients with bilateral vocal fold palsy.

Myxoinflammatory fibroblastic sarcoma (MIFS) is a low-grade malignant soft tissue tumor that typically arises in the distal aspects of the extremities. Although a few cases of the tumors occurring in the nose and eyeball have been reported, MIFS is uncommon in the head and neck region. Herein, we describe a rare case of MIFS arising in the anterior neck. The patient was a 69-year-old woman who presented to our department with a tumor in the anterinr neck measuring 4.4 cm in diameter. Ultrasound-guided fine needle aspiration cytology revealed a poorly differentiated malignant tumor. The tumor was surgically removed, and postoperative histopathology revealed the diagnosis of MIFS. The patient remains recurrence-free one year after adjuvant radiation therapy. The histologic and clinical features of MIFS have been discussed. Because local recurrence is known to occurr in approximately 20% of all reported cases, careful postoperative follow-up is necessary.

We report a case of sialolipoma arising from the submandibular gland. Sialolipoma is a benign salivary gland tumor showing a mixture of mature fat cells and salivary gland parenchyma, and was first described by Nagao et al. in 2001.

The patient was a 49-year-old man who presented with a mass in the left submandibular gland region measuring 5 cm in its greatest dimension. We excised the tumor while preserving the submandibular gland, and postoperative histopathology revealed a sialolipoma measuring 57 × 37 × 23 mm in diameter. The patient was followed up for 11 months, with no evidence of recurrence.

This is the first case report of sialolipoma arising from the submandibular gland in Japan and only seven cases have been reported to date in the English literature.

The differential diagnosis of cystic cervical masses includes benign lesions, such as second branchial cleft cysts, and malignant metastatic tumors. The incidence of human papillomavirus-related oropharyngeal cancer is increasing, and it often presents with a cystic lymph node metastasis. Therefore, in some cases, malignant metastasis is diagnosed after the resection of a cystic cervical cyst diagnosed initially as a benign tumor.

We report a case of human papillomavirus-related oropharyngeal cancer that was diagnosed after the resection of a second branchial cleft cyst. A 40-year-old woman visited our hospital with the chief complaint of a cervical mass. The mass was resected and postoperative histopathology revealed a cystic cervical cyst. Twenty months later, the cystic cervical mass recurred and was treated by resection of the cystic tumor as well as dissection of the lymph nodes around the tumor. The postoperative histopathological diagnosis was a second branchial cleft cyst and there was no evidence of malignancy. After another 30 months, the cystic cervical mass recurred yet again, and about two more months later, it began to grow rapidly in size; MRI revealed a tonsillar tumor. Biopsy confirmed the diagnosis of p16-positive oropharyngeal cancer. A retrospective histologic review of the previously resected cystic lesions demonstrated p16 positivity in both the first and second resection specimens. Therefore, they were also assumed to have been lymph node metastases from the p16-positive oropharyngeal cancer. The patient received concurrent chemoradiotherapy and the tumor disappeared. There was no evidence of recurrence at the one-year follow up. Regardless of the history, the possibility of cystic metastatic lymph node should always be kept in mind in patients presenting with cervical cystic masses, and imaging examinations such as CT and MRI including the pharynx should be performed.

We report a case of sinonasal low-grade, non-intestinal adenocarcinoma (LG non-ITAC). The patient, a 56-year-old woman was referred to our university hospital for a left nasal tumor with nasal obstruction, epistaxis and nasal discharge. Nasal endoscopy revealed a nasal polypoid tumor with a smooth margin occupying the left nasal cavity. The sinonasal tumor was diagnosed as an exophytic papilloma by histopathology. Sinonasal contrast computed tomography (CT) confirmed homogeneous opacification of the left frontal, maxillary, and ethmoidal sinuses. There was no evidence of a potential bone defect. T2-weighted magnetic resonance imaging (MRI) showed a relatively uniform high signal intensity area in the left maxillary and frontal sinuses. An area of high signal intensity was also found in a part of the left ethmoid sinus. Left transnasal endoscopic ethmoidectomy was performed under general anesthesia. The tumor in the nasal cavity was prone to bleeding. We defined the site of tumor attachment as the supra bulla cell. The anterior ethmoidal artery was exposed, and the tumor was carefully dissected and removed. Based on the findings of these radiological and histological examinations, the sinonasal tumor was diagnosed as a LG non-ITAC. Sinonasal non-ITAC is an adenocarcinoma without the histopathological characteristics of salivary or intestinal-type adenocarcinoma. Non-ITAC is additionally classified into the HG and LG types. LG non-ITAC is very uncommon. Thus far, there is only one published report in the literature on the clinical and therapeutic characteristics of LG non-ITAC in Japan. Non-ITAC has a good prognosis, with a low risk of metastasis. Therefore, it is imperative to suspect and distinguish non-ITAC from other sinonasal tumors with similar symptoms preoperatively.