Vertical nystagmus is an important sign of central nervous system disorder. Herein, we report a case of upbeat nystagmus, in a case of as the initial symptom of clinically isolated syndrome. The patient was a 45-year-old male who developed dizziness on November 7 and visited our hospital on November 12. We observed upbeat nystagmus in the gaze of all direction and primary and all positions and adduction failure of the right eye. The patient was hospitalized because MRI revealed a high-intensity area on the posterior aspect of the pons and medulla oblongata on T2-weighted and FLAIR MRI imaging. The patient was started on anticoagulant therapy as infarction was suspected, but no improvement of the symptoms was noted. A review of the second MRI revealed another high-intensity area in the right superior cerebellar peduncle, and we started steroid pulse treatment for the patient under the suspicion of multiple sclerosis. The patient's symptoms completely resolved with this treatment, and he was discharged from the hospital on November 26. We suspected that the upbeat nystagmus in this case was mainly caused by disorder of the central vestibuloocular reflex pathway in the pitch plane and area of the nucleus prepositus hypoglossi as the neural integrator. We think that the upbeat nystagmus was a very important symptom for early diagnosis and treatment in this case.

IgG4-related disease was recently proposed under the classification of systemic chronic inflammatory disease. In the field of otolaryngology, organ-specific diagnostic criteria have been established for the occurrence of this condition in the salivary glands, but not in the sinonasal cavity. Here we report a case involving a 70-year-old man with IgG4-related disease in the sinonasal cavity. The patient, with the chief complaint of nasal bleeding, first visited a physician. However, the patient experienced recurrent bleeding with intranasal structure loss and was subsequently referred to our hospital. His IgG4 level was elevated, and histopathological examination of a tissue sample obtained from the edematous sphenoid sinus showed increased IgG4-positive plasma cells and storiform fibrosclerosis. A definitive diagnosis of IgG4-related rhinosinusitis was made on the basis of comprehensive criteria for IgG4-related disease. The disease showed a progressively destructive course that was clearly different from that of chronic sinusitis and represented a very rare case of IgG4-related rhinosinusitis. IgG4-related disease originating in the sinonasal cavity is rare, and, to the best of our knowledge, this is the first primary case of IgG4-related disease that originated in one side of the sinonasal cavity and showed progressive destruction.

We encountered a case of conductive hearing loss due to stapes fixation, accompanied by broad thumbs, broad toes, and hyperopia. The patient was a 26-year-old man who had been using hearing aids for bilateral hearing loss since childhood. A detailed examination performed at our ENT department revealed conductive hearing loss and absence of the stapedial reflex, and otosclerosis was suspected as the cause of the conductive hearing loss. Physical findings included fusion of the second and third toes, broad thumbs, and proximal symphalangism in the left fifth finger and hyperopia, which led us to suspect the diagnosis of Teunissen-Cremers Syndrome, a genetic disorder first reported by Teunissen and Cremers in 1990. Teunissen-Cremers Syndrome is an autosomal-dominant inherited disorder presumably caused by mutations in the human noggin (NOG) gene. Our patient did not have a positive family history for the syndrome, although isolated cases have also been reported in the past. However, none of these cases were subjected to detailed genealogical research; therefore, the possibility of familial occurrence cannot be excluded.There are approximately 50 genes believed to be associated with conductive hearing loss. If we could perform genetic testing with the patient’s consent and clarify the correspondence between the NOG mutations and the phenotypic patterns, we might be able to develop a new classification of conductive hearing loss.

Meningeal carcinomatosis is characterized by multifocal spread of tumor cells in the leptomeninges from distant solid tumors. It is the clinical condition that is relatively rare in intracranial metastasis. It is easy to suspect meningeal carcinomatosis when a variety of neurological symptoms appear in patient with a known malignancy. However, we may appear as presenting symptoms of a latent malignant tumor. The prognosis is very poor and often results in death after the onset for neurologic symptoms in 1–2 months.We experienced a case of meningeal carcinomatosis associated with preceding symptoms of sensorineural hearing loss. Further examinations revealed gastric cancer and metastasis of the tumor cell.We consider our experience was important to differentiate rare case of meningeal carcinomatosis for sudden sensorineural hearing loss and recurrent facial palsy.

Sarcoidosis is a systemic granulomatous disease of unidentified cause, most commonly affecting the lungs, heart, eyes and skin; neurologic involvement is seen in approximately 5% of cases. Neurologic involvement in sarcoidosis could affect the brain parenchyma, meninges, or indeed any other site of the central nervous system; however, cranial nerve involvement is the most common site of neurologic involvement. The most frequently affected cranial nerve in neurologic sarcoidosis is the facial nerve, with 40–60% of cases manifesting facial paralysis.We encountered a case of sarcoidosis in which facial paralysis was the presenting symptom. It is important to rule out intracranial lesions or neuromuscular disorders as the cause in patients presenting with facial paralysis, and evaluation by chest X-ray, electrocardiography, and brain MRI are important to identify relevant findings consistently, such as the shifted strain of the forehead.

In 1791, Mondini wrote the Latin article entitled “Anatomica Surdi nati Sectio.” It became the first report of the dissection of the temporal bone in an eight-year-old boy with congenital deafness and reported the findings of the inner ear anomaly. Mondini’s original paper is a historical one for otological researchers, but it has been difficult, indeed rather impossible to read for a long time. We fortunately had the chance to review the original copy of the paper.The summary of Mondini’s report described several conditions: there was a large opening of the vestibular aqueduct; labyrinth fluid was escaping from the enlarged vestibular aqueduct; and the cochlea consisted of only one and a half turns, among others. According to his paper, the common cavity is not an inner ear anomaly of the Mondini type. Currently, recent progress in three-dimensional imaging can detect and visualize the fine and detailed structure of the inner ear. Therefore, imaging diagnosis is able to discriminate Mondini anomalies from other types.

In ear surgery, chronic otitis media or choleateatoma are very important training operations for ear nose and throat (ENT) residents. They are common ear disease and so young doctors can get a standard skill of ear surgery. Stapes surgery is relatively uncommon. It is also important training program for them. Because they can learn normal anatomy of the middle ear and treat ossicular conduction.We have performed questionnaire survey to explore training condition of ear surgery for young ENT doctors. List of questions were sent to the committee members of the Japan Otological Society. Questions were the number of annual stapes surgery and operators, when to start ear surgery training, necessary or disadvantage items for their training, what ear surgery is appropriate for young doctors and so on. More than ninety percent leading doctors answered our survey. This seemed to be the result how important they were thinking ear surgery training was for residents.Total number of ENT doctors has been decreasing and hospital available for ear surgery has been also limited recently. Even in this adverse circumstance, training for residents to learn ear surgery is very important and they should experience hearing improvement of the ear diseased patient after ear surgery. Therefore, anatomically normal operation like stapes surgery can be very useful training course for premature ENT residents.

For treatment of a sinonasal inverted papilloma (IP), it is essential to have a definite diagnosis, to identify its origin by computed tomography (CT) and magnetic resonance imaging (MRI), and to select the appropriate surgical approach based on the staging system proposed by Krouse. Recently, a new surgical approach named endoscopic modified medial maxillectomy (EMMM) was proposed. This approach can preserve the inferior turbinate and nasolacrimal duct. We successfully treated sinonasal IP with EMMM in a 71-year-old female patient. In this patient, the sinonasal IP originated from the entire circumference of the maxillary sinus. EMMM is not a difficult procedure and provides good visibility of the operative field. Lacrimation and empty nose syndrome do not occur postoperatively as the nasolacrimal duct and inferior turbinate are preserved. EMMM is considered to be a very favorable approach for treatment of sinonasal IP.

The clinical pathway is being used increasingly as the standard hospital treatment policy for major disorders in Japan. Tonsillectomy accounts for most operations per year at many ear, nose, and throat facilities. Tonsillectomies accounted for 14.8% of 155 clinical pathway cases in the last 3 years at our hospital. The percentage of tonsil operations is second after ear surgery, at 18.4%, for the same number of nasal operations.Medical intern doctors usually perform tonsillectomies under the direction of senior doctors, and guidelines are needed to standardize hospital treatment and ensure safety against risks such as postoperative hemorrhage.It would also help to standardize team treatment to prevent preoperative infection in cooperation with health-care providers.

The ear surgery of chronic otitis media or choleateatoma needs very important operations training for ear nose and throat (ENT) residents. They are common ear diseases and so young doctors should get a standard skill of ear surgery. However, stapes surgery is relatively uncommon. It is also important training program for them. They need to learn normal anatomy of the middle ear and treat ossicular conduction. We performed questionnaire survey to explore training conditions of ear surgery for young ENT doctors. List of questions were sent to the committee members of the Japan Otological Society. Questions consisted of the number of annual stapes surgery and operators, when to start ear surgery training, necessary or disadvantage items for their training, what ear surgery is appropriate for young doctors and so on. More than ninety percent leading doctors answered our survey. This seemed to show the result how important they were thinking ear surgery training. In Japan, total number of ENT doctors has been decreasing and hospitals available for ear surgery have been also limited recently. Even in this adverse circumstance, training for residents to learn ear surgery is very important and they should experience hearing improvement of patients after ear surgery. Therefore, in normal middle ear anatomy operation like stapes surgery can be very useful training course for young ENT residents.