Laryngotracheal separation (LTS) is the most effective surgical procedure when other conservative treatments, such as medications and posture control are ineffective for the prevention of recurrent aspiration pneumonia. One of the major complications of LTS is innominate artery fistula. In adults, tracheostomy management without a tracheostomy tube is recommended as the safest and most effective method, although this would be challenging in children with progressive respiratory distress due to progressive scoliosis. Children with neurological disorders often have thoracic deformities with severe scoliosis. As a result, the innominate artery may be compressed against the trachea, which gradually increases the risk of TIF bleeding. Despite the lower risk of aspiration pneumonia after LTS in neurologically impaired children, obstructive respiratory distress requires a tracheostomy tube as a stent. Respiratory management should be considered in cases of thoracic deformity with progressive scoliosis in children with neurological disorders. We examined 28 cases of LTS performed for short-term and long-term management without a tracheostomy tube. The results showed that management without a tracheal tube was possible in the short term, but was associated with an increase in the number of cases requiring reinsertion of a tracheostomy tube or ventilator postoperatively in the long term. A sternal-vertebral distance of <20 mm and tracheal aspect ratio of <0.6 should be considered as high risk factors for respiratory problems. As a resuts, respiratory management should be considered in cases of thoracic deformity with progressive scoliosis in children with neurological disorders.

Relapsing polychondritis (RP), which is a multisystem, inflammatory, autoimmune disease affecting the cartilage, is relatively rare. RP is characterized by swelling of the auricles, audiovestibular disorders, and airway chondritis. Symptoms commonly appear in the ears, neck, and throat. Here in, we describe the case of a patient with RP who developed complete, bilateral hearing loss over the course of one to two months.

A 69-year-old, woman presented with a history of left auricular swelling, vertigo, and bilateral hearing impairment. She had previously been diagnosed as having incomplete Ramsay Hunt syndrome and treated with steroids, but her hearing had failed to improve. Over the course of time, the bilateral hearing impairment progressed to complete deafness. She was referred to our hospital for further management of the deafness and subglottic swelling and was admitted for treatment.

An auricular cartilage biopsy revealed no lymphocytic infiltration, but showed cartilage degeneration. Based on these findings, the patient was diagnosed as having auricular chondritis.

RP was later diagnosed based on the presence of bilateral auricular swelling, airway chondritis, episcleritis, and audiovestibular disorders, which fulfilled the diagnostic criteria for the disease. Pulse methylprednisolone therapy improved the subglottic swelling, auricular swelling, and episcleritis. Although the patient also received intravenous cyclophosphamide therapy (IVCY), the bilateral hearing loss failed to improve.

Inner ear disorders in RP are often resistant to steroid therapy and have a poor, long-term prognosis. The patient received steroid treatment from an early stage, but her hearing failed to improve. Based on a previous study demonstrating the efficacy of cochlear implants in cases of RP-related hearing loss, cochlear implants were recommended to the patient, but she declined the surgery.

Histopathological diagnosis of RP is often made during steroid and/or immunosuppressant therapy administration, as in this case, or afterwards, so that the typical features of the disease may not be evident. Therefore, when making a histopathological diagnosis, it is necessary to consider the possibility that the findings may have been affected by previous treatments.

In this study, we reviewed the data of 115 patients with pars flaccida cholesteatoma who underwent initial surgery at the Yamanashi University Hospital between January 2010 and December 2017. For cases of pars flaccida cholesteatoma that had progressed to the mastoid antrum, we chose to perform canal wall down tympanoplasty with soft wall reconstruction. On the other hand, for cases in which the disease had not progressed to the mastoid antrum, we chose to perform transcanal atticotomy. We analyzed the rates of recurrence, favorable hearing improvement rates, and the postoperative ear canal morphology after the tympanoplasties. According to the degree of progression of the disease, the patients were classified as stage I (16 cases, 13.9%), stage II (91 cases, 79.1%), stage III (7 cases, 6.1%), or stage IV (1 case, 0.9%). Transcanal atticotomy was performed in 11 cases (9.6%), and canal wall down tympanoplasty in 104 cases (90.4%). The overall recurrence rate was 8.7% (10/115), and all the recurrences were cases of residual disease. In 8 cases, the recurrence was found during the planned second-look operation. After the operation, more than half of the cases (62.8%) developed retraction of the canal wall, but cavity problem was observed only in 2 cases. The overall hearing improvement rate was 77.0% (67/87). The hearing improvement rate in type I tympanoplasty cases was 92.9% (13/14), while the rate in type III tympanoplasty cases was 75.4% (52/69), and that in type IV tympanoplasty cases was 50.0% (2/4). From the above, we conclude that both transcanal atticotomy and canal wall down tympanoplasty with soft wall reconstruction are effective for preventing recurrence and postoperative cavity problems in cases of pars flaccida cholesteatoma. The hearing improvement rates were not inferior to previously reported rates, but we would like to investigate whether further improvement can be expected with the use of artificial ossicles in type IV tympanoplasty.

We report a case of a large cholesterol cyst of the temporal bone. A 44-year-old man was referred to our clinic with a mass in his right temple that he had first noticed more than 10 years earlier. He had congenital deafness of the right ear due to inner ear malformation. His right external auditory canal was completely occluded by the swelling. CT revealed a large cystic mass in the temporal bone which caused a bony defect in the tegmen of the middle cranial fossa and squamous part of the temporal bone. The cyst compressed the temporal lobe. MRI showed a smooth mass which visualized as an iso-intensity signal on T1-weighted images and as a hyperintensity on T2-weighted images; the lesion was showed scarce enhancement with gadolinium. Surgery was performed, and the large cyst, which extended from the middle cranial fossa to the mastoid portion and epitympanum, was completely resected without cerebrospinal fluid leakage. The cavity was obliterated with abdominal fat and the external ear canal was closed. Histopathological examination revealed that the cyst wall lined by thin squamous epithelium was made of fibrous connective tissue containing a cholesterol granuloma. No recurrence was noted at the last follow-up performed two years after surgery. The cyst was thought to arise as a result of inflammation of the epitympanum and increased with blood supply from the cerebral dura mater.

Streptococcal toxic shock syndrome (STSS) is a group A streptococcal sepsis of acute onset. Patients with this condition rapidly develop shock and multiple organ failure, often resulting in death. The sites of primary infection in cases of STSS are most often the extremities, with few reports in the head and neck region.

We report the case of a 39-year-old woman with right ear pain, bleeding in the ear canal, headache, nausea, and chills. On examination, redness, swelling, and bloody exudate were noted from the posterior wall of the right ear canal to the tympanic membrane. Blood tests revealed a white blood cell count of 9610/μL with 62.7% neutrophils and a serum C-reactive protein level of 20.29 mg/dL. Contrast-enhanced computed tomography revealed edema of the right external auditory canal wall with a contrast effect, prominent edema of the right parotid gland, masseter muscle, medial pterygoid muscle, and thrombus formation in the right internal jugular vein. The patient was transferred to the emergency room because of rapid respiratory and circulatory deterioration while she waited at the outpatient clinic. She was intubated and placed on a ventilator. Fluids and noradrenaline were administered; however, she went into cardiac arrest. Percutaneous cardiopulmonary support was started, but her condition deteriorated, and she died of septic shock 10 hours after arrival. Streptococcus pyogenes was detected on blood culture, and we made the diagnosis of STSS.

The progression of STSS is rapid, with septic shock developing within a few hours. In this case, the rapid course of the disease resulted in death. Early diagnosis and treatment are essential to save lives. At our department, the general condition of patients presenting with severe soft tissue infections is checked frequently, bearing in mind the possibility of STSS.

We conducted a retrospective review of the data of 16 cases of minor salivary gland tumors arising from the palate that were managed at our institute over a period of 13 years. The data reviewed included the age, gender, tumor subsite, chief complaint, pathological diagnosis, treatment modality employed, and survival outcomes. The age range of the patients was 34–84 years (median, 62.5 years); 6 patients were male and the remaining 10, female. The tumor subsite was the soft palate in 8 cases, hard palate in 7 cases, and retromolar triangle in 1 case.

The chief complaint was an oral mass in 12 cases, and interestingly, 2 cases presented with a cervical mass.

The diagnosis was benign pleomorphic adenoma in 8 cases. The remaining cases had malignant tumors, including low-grade mucoepidermoid carcinomas (4 cases), adenoid cystic carcinoma (1 case), adenocarcinoma-not otherwise specified (1 case), acinic cell carcinoma (1 case), and secretary carcinoma (1 case). Fourteen of the patients were treated by transoral surgery, 1 case by surgery via a mandibular swing approach, and 1 case by partial maxillectomy. Additional surgery was performed in one case in whom the histopathological report described an insufficient resection margin, and adjuvant radiotherapy was undertaken in 2 cases that were reported as being margin-positive. The 5-year disease-specific survival rate was 100% and the recurrence-free survival rate was 86%.

We concluded that minor salivary gland tumors of the palate show a higher rate of malignancy than major salivary gland tumors, and warrant special attention, because in some cases of our series, the diagnosis was made at an advanced disease stage, in the absence of any symptoms of the primary lesion.

Internal carotid artery (ICA) pseudoaneurysm caused by skull base osteomyelitis is a rare complication of radiotherapy for nasopharyngeal carcinoma (NPC). Rupture of the pseudoaneurysm can occur with massive bleeding, which could be fatal. A 74-year-old man with NPC who had received chemoradiotherapy was detected as having an ICA pseudoaneurysm. He presented with a massive nosebleed of acute onset. Coil embolization of the ICA was performed, and he was saved without serious complications. It is important to keep in mind the possible development of an ICA pseudoaneurysm after radiotherapy for NPC.

Carcinoma showing thymus-like differentiation (CASTLE) is a rare malignant tumor, with its histological features resembling those of squamous cell carcinoma of the thyroid. Because of its diverse histology, however, it is often difficult to diagnose this tumor preoperatively. Thus, it is important to have an index of suspicion for CASTLE and differentiate it from other aggressive and advanced thyroid carcinomas based on the clinical findings, site of origin, and histological features. Herein, we report a case of CASTLE that was difficult to diagnose preoperatively. The patient was a 57-year-old man who was referred to our department for the management of an incidental thyroid tumor; the findings of fine needle aspiration cytology suggested poorly differentiated carcinoma. Total thyroidectomy with right paratracheal lymph node dissection was performed. During surgery, the recurrent laryngeal nerve was found to be strongly adherent to the tumor, and therefore required to be cut and reconstructed using the ansa cervicalis. The postoperative histopathological diagnosis was CASTLE, confirmed by the positive immunohistochemistry for CD5, a marker of carcinoma of thymic origin. The margin of the tumor was reported as positive, so that the patient underwent additional postoperative radiotherapy. He is currently under regular follow-up and is symptom-free, without any recurrence. As compared to squamous cell carcinoma of the thyroid, CASTLE is commonly more slow-growing, and with appropriate treatment, has a more favorable prognosis. Therefore, it is imperative to suspect and distinguish CASTLE from other thyroid neoplasms with similar symptoms preoperatively.

We report the case of a female patient with a first branchial cleft fistula. A 1-year girl child was brought to us with the complaint of otorrhea from the right external ear canal. There was no pain or facial swelling.

We planned a two-stage operation for resection of the fistula. The first operation was performed according to the method used for parotid gland tumor surgery. The fistula was resected from the external ear to the cheek, without the development of facial palsy. The second surgery for correction for the tympanic membrane anomaly was performed one year later.

The type of anomaly found in this case lends support to Anson’s theory rather than Reichert’s theory about developmental genetics of the tympanic membrane.

In this paper, we report the case of a patient who presented with atlantoaxial rotatory fixation after upper respiratory infection, which is extremely rare in the field of otorhinolaryngology.

A 7-year-old boy presented to us complaining of right neck pain; he had had a common cold for 3 days prior to the visit. He had visited a local otorhinolaryngologist, who suspected cervical lymphadenopathy based on the medical history and symptoms, and referred him to our hospital. His neck was fixed in a tilted position to the left and turned to the right, in the so-called cock-robin position. Since there were no other pathological findings in the neck and a cervical spinal condition was suspected, the patient was examined by an orthopedic surgeon. Cervical X-rays and CT showed misalignment of the dens in the axis with respect to those of the atlas. Based on the findings, the patient was diagnosed as having atlantoaxial rotatory fixation. After one week of local rest with a cervical collar, both the symptoms and the findings improved.

Although, most cases of atlantoaxial rotatory fixation recover with local rest with a cervical collar and anti-inflammatory medication, refractory cases require surgical therapy. Early diagnosis is necessary to avoid intractable cases. In our case reported herein, the symptoms improved promptly due to the early diagnosis. Since the main symptom of the disease is torticollis and neck pain, these patient often initially visit the otorhinolaryngologist rather than the orthopedic surgeon. Therefore, otorhinolaryngologists need to be aware of the condition of atlantoaxial rotatory fixation.

Glossopharyngeal neuralgia is a relatively rare disorder, and the symptoms often led to an initial consultation with an otorhinolaryngologist. Glossopharyngeal neuralgia is believed to be caused by compression of the nerve by blood vessels in most of the cases. The initial treatment is drug therapy, but if the effect is inadequate, microvascular decompression (MVD) by craniotomy can be performed, with good results.

The patient was a 51-year-old man who presented to us with a one-month history of left mandibular pain, which had become severe in the previous two weeks. He gave a history of having received carbamazepine treatment for left mandibular pain 10 years earlier and the symptom had resolved. This time, carbamazepine had no effect, and the patient was unable to eat or speak because of the pain. We diagnosed glossopharyngeal neuralgia based on the location and type of pain, and consulted the Department of Neurosurgery to investigate the cause. A thin-slice MRI showed that the left anterior inferior cerebellar artery was compressing the left glossopharyngeal nerve. MVD was performed and his symptom improved significantly immediately after the surgery. He is currently under outpatient observation, without any recurrence.

MVD is an effective treatment for glossopharyngeal neuralgia in patients who fail to respond to initial drug therapy and who have evidence of vascular compression of the nerve.

Acupuncture is widely used for the treatment of stiff shoulders and back pain, but complications can occur if the needles break off and remain in the body. A 77-year-old woman was referred to our hospital for sphenoid sinus mycosis. CT revealed a needle-like artifact in the posterior cranial fossa, in addition to the findings of right sphenoid sinus mycosis. We thoroughly reviewed her medical history and found out that she had undergone acupuncture treatment 33 years earlier for neck pain. During the treatment, a needle had got stuck and she had consulted a neurosurgeon. At the time, imaging had revealed the tip of the needle in the posterior cranial fossa. Surgical removal was performed under general anesthesia; however, the entire needle could not be removed in one piece and the patient was kept under observation. At the present admission, endonasal endoscopic sinus surgery was performed under general anesthesia. We used a video laryngoscope for safer intubation and avoidance of excessive neck extension. The sphenoid sinus was sufficiently opened and the fungal mass removed. The postoperative course was favorable and the histopathological findings revealed the lesion as an aspergillus fungus ball. Fortunately, the acupuncture needle had not damaged any blood vessels, nerves or the brain. The needle had apparently not caused any infection or allergy, and the patient remained asymptomatic for over 30 years. If the patient had not remembered the presence of the broken needle in her head due to cognitive impairment, etc., she could well have undergone MRI, etc., and suffered a fatal outcome. Confirming the detailed medical history and preoperative imaging findings, as well as sharing of information with the family and medical institutions is important for safe medicine.

Eosinophilic esophagitis (EoE) is one of the significant adverse reactions of sublingual immunotherapy (SLIT). Although there are some case reports of EoE and asymptomatic esophageal eosinophilia developing during SLIT with pure cedar pollen extract, to the best of our knowledge, no cases have been reported in the field of otorhinolaryngology.

A 64-year-old woman was initiated on SLIT for cedar pollen allergy. Two years after the start of SLIT, she developed mild retrosternal discomfort and dysphagia. Based on histopathological examination, she was diagnosed as having EoE. SLIT was discontinued and the symptoms improved within one month.

As the number of reports of EoE and asymptomatic esophageal eosinophilia developing during SLIT is on the rise, a consensus needs to be reached on discontinuation of SLIT in patients diagnosed as having EoE or asymptomatic esophageal eosinophilia.