Airway diseases can be life-threatening and lower the quality of life of the patients. Airway reconstruction after resection of malignancies or stenotic lesions remains a difficult surgical challenge. The etiologies of airway diseases, including congenital, traumatic, inflammatory and tumorous lesions, are described in this article. At present, congenital subglottic stenosis/tracheal stenosis are officially considered as intractable diseases in Japan. Conventionally, reconstruction for large airway defects is performed using several kinds of cartilage grafts. In most cases, repeated surgeries at multiple sites are necessary, making it considerably invasive for the patients. Moreover, even with such invasive treatment, the tracheal stoma may have to remain in several patients because of unstable grafts or airway re-stenosis. As a new perspective, in situ tissue regeneration-inducing artificial trachea may significantly contribute to effective airway reconstruction.

The diagnosis procedure combination（DPC）database was utilized for estimating the number of patients who undergo tracheoplasty and related procedures. In the database, thyroid cancer with tracheal invasion accounts for the highest proportion of patients undergoing these operative procedures.

The Rare Disease Data Registry of Japan (RADDAR-J) has constructed a system to establish a sustainable framework that provides an environment where participation is significantly beneficial for all individuals concerned with and involved in the treatment of intractable diseases, such as researchers, physicians, and patients.

A pharyngeal, laryngeal and tracheal stenosis patient registry is under construction, based on RADDAR-J. This will serve as a reference source for the various evidences of rare diseases.

Persistent postural-perceptual dizziness (PPPD) is new conception published by the Barany Society in 2017. There are five diagnostic criteria for PPPD. The main symptom is continuous dizziness for more than three months, that is not explained by other diseases. The symptom is exacerbated by an upright position, active or passive movement, and moving or complex visual stimuli. The appearance of a balance disorder (e.g., vertigo) precedes PPPD. It is possible that PPPD patients can be handicapped in their activities of daily living (ADL).

We report herein on two cases of Ménière’s disease associated with PPPD. The affected patients were in the interval stage of Ménière’s disease, and had no vestibular dysfunction. No abnormality was seen on head and cerebellopontine angle MRI. However, the patients had been experiencing both a floating and a rotating sensation over a prolonged period. Their symptom was exacerbated by an upright position, active or passive movement, and moving or complex visual stimuli. The floating sensation made them inactive. We distinguished PPPD from Ménière’s disease, and correctly diagnosed PPPD. The reported treatments of PPPD are medication, rehabilitation, and cognitive behavior therapy, and cognitive behavior therapy was effective for both patients.

These two Ménière’s disease cases indicated the possibility that the Ménière’s disease may sift to PPPD during the interval stages, and it requires a different strategy of treatment compared with Ménière’s disease-­related symptoms.

Wernicke encephalopathy is a clinically-diagnosed disease characterized by disturbed consciousness, disordered eye movements, and ataxia. Given its high specificity for detecting Wernicke encephalopathy, brain MRI has an important role in the diagnosis of this disease. On the other hand, the sensitivity of MRI is relatively low. Therefore, normal brain MRI findings often do not show non-Wernicke encephalopathy.

A 76-year-old man presented to our hospital with a ten-day history of dizziness. Five days earlier, he underwent brain MRI at another hospital. The scans appeared normal, and he was referred to an ear, nose, and throat hospital in order to rule out potential inner ear involvement.

He did not exhibit quadriplegia, spontaneous nystagmus, or positional nystagmus but appeared unsteady when standing during our medical examination. Additionally, his eyes showed leftward and downward gaze-evoked nystagmus, slight rightward and upward gaze-nystagmus, and conjugate gaze palsy toward the right side. We suspected a central nervous system disorder because of the disordered eye movements and carried out a second brain MRI at our hospital. Although the results did not show any cerebral infarction or cerebral hemorrhage, fluid-attenuated inversion recovery (FLAIR) imaging showed an area of slight high intensity in the medial thalami and periaqueductal region. Such findings are characteristic of Wernicke encephalopathy. We administered intravenous doses of vitamin B1 to the patient for one week, during which time his dizziness improved day by day. The disordered eye movements also improved and were resolved completely nine days after beginning the vitamin treatment. A repeat brain MRI was completed the same day, and the high-intensity areas that were noted previously were not visualized on FLAIR. He started rehabilitation three days after beginning treatment and left the hospital 24 days later.

In patients with Wernicke encephalopathy, brain MRI scans may appear normal. If Wernicke encephalopathy is diagnosed clinically based on physical examination findings, then vitamin B1 should be promptly administered.

Medium-term hearing outcomes after 116 ossiculoplasties using hydroxyapatite (HA) and cartilage were reviewed in this paper. The successful rate of hearing outcomes was 83.0% in the HA group and 63.8% in the cartilage group (p<0.05). A multivariate analysis showed that HA was a significantly better prognostic factor than cartilage (odds ratio 2.77). Concerning postoperative complication, extrusion of HA was found in one case in the HA group. On the other hand, there was no complication in the cartilage group.

A subperiosteal abscess (SPA) of the orbit is a serious complication of sinusitis in children. Here we present a case of a 6-year-old boy with SPA treated with endoscopic surgical intervention. Computed tomography (CT) revealed acute sinusitis and a left medial SPA. He was treated with meropenem intravenously for 2 days. However, the cavity of the SPA appeared enlarged in a follow-up CT. Transnasal endoscopic sinus surgery was performed and the SPA was drained on the same day. An SPA is a serious stage of orbital infections which can lead to blindness and cavernous sinus thrombosis. Although the timing of the surgical intervention is controversial, we safely drained the SPA with transnasal endoscopic surgery, without any subsequent complications.

In the treatment of orbital subperiosteal abscesses, conservative treatment is given first. Generally, conservative treatment in children is said to be more effective than in adults. However, in cases with a poor response to conservative treatment, it is necessary to carry out surgical treatment promptly. If treatment is delayed, irreversible visual damage may occur or intracranial complications may lead to death, so prompt and appropriate measures are essential.

Children often fall down while holding an object in the oral cavity which often results in puncture laceration injuries of the oral cavity or oropharynx. We report herein on a case of oropharyngeal trauma with mediastinal emphysema caused by a metal spoon.

A 3-year-old girl fell down while holding a metal spoon in her mouth, and sustained an injury to her oral cavity. She bled from the mouth and visited an emergency room on the day of the injury. At the time of examination, bleeding from oropharyngeal trauma was stopped. CT revealed emphysema in the neck, otherwise the physical exam was normal, so she was prescribed a preventive oral antibiotic and her mother was instructed regarding home treatment. The girl developed a fever the next day, and CT showed enlargement of the emphysema to the upper mediastinum. She was admitted to a referral hospital, stopped oral intake, and received antibiotic infusion.

She was referred to our hospital on the third day of the injury because the fever continued. She was maintained on the conservative treatment, and was discharged without any serious complication on the seventh post-injury day.

In similar accident, some cases have been reported with complications such as respiratory failure due to mediastinal emphysema, abscess formation, and internal carotid artery obstruction. Appropriate diagnosis and watchful observation are important.

Neuro-Sweet disease is characterized by benign, recurrent encephalitis and meningitis with painful elevated erythema. Viral and bacterial infections are known to predispose to the development of neuro-Sweet disease. We report a possible case of neuro-Sweet disease with skin lesions, disturbance of consciousness, and various neurologic symptoms that developed while the patient was receiving medical treatment for acute upper respiratory tract inflammation. The patient was a 26-year-old male who presented with persistent sore throat, fever, and facial skin lesions.

We observed multiple aphthous ulcers on the soft palate, and erythema nodosum in the left buccal region. The patient was initiated on antibiotic treatment for acute upper respiratory inflammation caused by viral, bacterial or mixed infection. On day 2 after the initiation of treatment, the patient showed acute onset of several neurologic symptoms, including consciousness disturbance and eye movement disorders. Brain MRI revealed multiple high signal intensities in the frontal lobe and thalamus. Neurologists at our institution began the patient on pulse therapy with methylprednisolone. After 2 courses of steroid pulse therapy, the patient’s level of consciousness, neurologic symptoms, and facial skin lesions improved dramatically. HLA typing revealed that the patient was positive for B54 and Cw1. Based on these findings, the patient was diagnosed as a possible case of neuro-Sweet disease.

Although acute upper respiratory inflammation is encountered frequently by otolaryngologists, care should be taken to watch for the development of hidden complications in patients with acute tonsillitis presenting with atypical findings, such as neurologic symptoms or skin lesions.

Granular cell tumors are an uncommon soft tissue neoplasm of neurogenic origin. Although they originate from a Schwann cell, the neurogenic origin is rarely confirmed during the surgery. We report herein on the case of a 43-year-old woman with a granular cell tumor originating from the ansa cervicalis. The mass was located in the right neck. The tumor was firm, oval in shape, and about 3 cm in diameter. Ultrasonography showed a 30 mm oval formation localized in the right neck, anterior to the sternocleidomastoid muscle. On computed tomography (CT) scan, the lesion showed up as non-enhanced on contrast medium injection, and magnetic resonance imaging (MRI) showed a hyperintense mass on T1 and T2-weighted sequences, enhancement by contrast medium injection. There was no invasion of the surrounding tissues. Based on the results of fine-needle aspiration cytology, there was no malignancy. The tumor was surgically removed, and the histopathological diagnosis was a granular cell tumor. The patient has had an uneventful course after hospital discharge without recurrence for more than 6 months. There are few reports on granular cell tumors associated with the nerves in the head and neck region. This is the first report of granular cell tumor originating from the ansa cervicalis.

Subcutaneous emphysema occasionally occurs during head and neck surgery. While it resolves spontaneously in many cases, in some, the condition progress to mediastinal emphysema, and preventive measures against infections, such as mediastinitis, are needed. Case 1 was a 60-year-old man who had undergone emergency tracheotomy. After the surgery, he had persistent dyspnea and complained of anterior chest pain. A chest CT revealed subcutaneous and mediastinal emphysema. Furthermore, the patient also developed bilateral pneumothorax. Case 2 was a 70-year-old woman who had undergone transoral videolaryngoscopic surgery (TOVS) for cancer of the pyriform sinus of the left side. After the surgery, she also developed persistent anterior chest discomfort and continuous coughing. A chest CT obtained on the following morning revealed emphysema of the subcutaneous, mediastinal and retroperitoneal spaces. Fortunately, the condition resolved spontaneously in both the patients, without any severe infections. Based on our observations, we consider that emergency tracheotomy and resection of the anterolateral part of the pyriform sinus during TOVS are risk factors for the development of severe subcutaneous emphysema.

With the objective of identifying the factors affecting the nasal resistance in patients undergoing sinus surgery, we evaluated the data of 95 patients with chronic sinusitis who underwent endoscopic sinus surgery (ESS) between 2015 and 2018 at our hospital. We determined the nasal resistance by rhinomanometry and also patients’ sensation of nasal obstruction before and 3 months after the ESS. In total, 49 bilateral and 46 unilateral ESS procedures were performed, including ESS alone, ESS+septoplasty, and ESS+septoplasty+conchotomy. In addition, the surgical cases were classified into single sinus or poly sinus surgery, and groups with or without polyps. We compared the nasal resistance and degree of change in the nasal resistance among the groups. The same comparisons were also conducted separately in cases with nasal resistance levels of >0.25 Pa/cm³/s.

Nasal resistance improved following the ESS in both the bilateral and unilateral cases. In almost all the subgroups, the nasal resistance improved following ESS, with the exception of the ESS with septoplasty group with nasal resistance levels of >0.25 Pa/cm³/s. The improvement in the nasal resistance was not significantly different between the ESS alone, ESS+septoplasty, and ESS+septoplasty+conchotomy groups. Similarly, the improvement in the nasal resistance was also not significantly different between the single sinus and poly sinus surgery groups, or between patients with and without polyps. Furthermore, there were no differences among the above groups of patients with nasal resistance levels of >0.25 Pa/cm³/s either.

The patients’ sensation of nasal obstruction improved following the ESS. The nasal resistance measured by rhinomanometry was weakly correlated with the sensation of nasal obstruction prior to the ESS; however, the nasal resistance following the ESS and the degree of change of the nasal resistance following the ESS were not correlated with the sensation of nasal obstruction or the degree of change in the sensation of nasal obstruction.

Future studies are needed for a precise clarification of the factors that affect nasal resistance.