Recent advances in magnetic resonance imaging (MRI) techniques have enabled clinical application of this imaging modality for investigating inner ear disorder. The fluid-attenuated inversion recovery (FLAIR) MRI sequence has been applied for the detection of inflammatory lesions of the inner ear. FLAIR MRI enables detection of breakdown of the blood-labyrinth barrier in the inner ear in cases of otitis media associated with anti-neutrophil cytoplasmic antibody-associated vasculitis and cases of cholesteatoma with labyrinthine fistulae. Another advance is the possibility of visualizing endolymphatic hydrops (EH) on gadolinium-enhanced MRI. MRI studies in this field have demonstrated the existence of EH in various otological disorders, including Meniere’s disease. In this paper, the utility of MRI for the evaluation of inner ear disorder is reviewed, with a focus on representative middle and inner ear diseases.

We report a case of acute sensorineural hearing loss caused by multiple cavernous hemangiomas. The patient was a 38-year-old woman who presented to us with a history of sudden loss of hearing on the left side and left-sided tinnitus. After two weeks, both the symptoms improved, but a pure-tone audiometry revealed sensorineural hearing loss in the zone of scale-out on the left side. Initially, we suspected functional deafness, however, the patient was finally diagnosed as having retrocochlear deafness based on evaluation of the auditory brainstem response (ABR) following that of the distortion product optoacoustic emission (DPOAE). Furthermore, MRI revealed multiple cavernous hemangiomas, one of which was at the left cerebellopontine angle.

We conclude that objective auditory testing is very useful for evaluation of the hearing ability in patients with sensorineural hearing loss, but it is necessary to understand the characteristics of each test for an accurate interpretation of the results.

Herein, we report two cases of cerebrospinal fluid leakage in children that we encountered at our hospital. Both presented with hearing loss.

Case 1 also suffered from blurred vision and photophobia. He exhibited a series of problem behaviors at school and violent behavior at home. These behaviors had become pronounced after a traffic accident. Ophthalmological testing using an autorefractor revealed the presence of hypertonia of the ciliary muscle and unstable refraction in both eyes. After 4 sessions of treatment with an epidural autologous blood patch (BP), the symptoms improved. The findings in this case suggest that hypertonia of the ciliary muscle and unstable refraction causing visual impairment can occur in cases of cerebrospinal fluid leakage. His abnormal behavior was the result of the abnormalities of the eyes and ears.

Case 2 had chronic dizziness in addition to the hearing loss. The dizziness and abnormalities in the stabilometer test improved a long time after BP treatment. However, the hearing impairment never complately improved.

The interval from the traffic accident to the correct diagnosis was 3 years 9 months in Case 1, and from the initial examination to the correct diagnosis was 3 years 10 months in Case 2. Patients often consult the ENT clinic due to such symptoms as headache, dizziness, hearing loss, tinnitus, and/or visual impairment. We should always bear in mind the possibility of cerebrospinal fluid leakage in such cases. Especially, children with this condition may not be able to precisely express their mental and physical distress, and may exhibit problem behaviors or strange behaviors. Therefore, it is important to obtain a careful history.

Hypohidrotic ectodermal dysplasia is a hereditary syndrome characterized mainly by the triad of less hair, less sweat and less teeth. It is associated with hypoplasia of the ectoderm caused by a genetic abnormality, and treatment remains mainly symptomatic. I encountered a case in a boy who presented with rhinostenosis at one month after birth. There were no anatomic abnormalities of the nasal cavity, however, the entire nasal cavity was filled with crust associated with atrophic nasal inflammation. Nose breathing was disturbed because of the pervasive crust and the child developed recurrent episodes of upper respiratory tract infection with a bad odor of the nasal mucus. Provision of care to prevent nasal crust formation for 4 years 6 months at the outpatient department led to improvement of the nose breathing and the bad odor of the nasal mucus. Herein, I report the method and the outcome.

Inverted papilloma (IP) of the sinonasal cavity occurs most commonly in the fifth to seventh decades of life, and accounts for 0.5–4% of all primary nasal tumors.

We report the case of a 60-year-old man who was diagnosed as having benign IP with intracranial extension. The patient was treated by sinonasal IP resection several times, however, each time, the tumor recurred. Eventually, as the tumor extended to the nasal cavity, right maxillary sinus and bilateral ethmoidal sinuses, we performed endoscopic sinus surgery and skull base resection. However, the tumor recurred again and extended into the skull. Finally, we performed skull base operation with craniotomy and administered postoperative radiation therapy to achieve complete control of the tumor. Until now, 12 months since completion of the last treatment, there has been no evidence of recurrence, and no evidence of brain dysfunction.

IP with intracranial extension without malignant transformation is uncommon. However, according to some reports, once it occurs, the mortality rate is 30–40%. Aggressive treatments, such as skull base operation with craniotomy and/or radiotherapy, are needed to achieve complete recovery.

Chondromyxoid fibroma is a rare cartilaginous tumor, accounting for only 1% of all bone tumors. In most cases, these tumors arise from the tubular bones, and cases of the tumor arising from the skull base are extremely rare.

In this paper, we report a case of chondromyxoid fibroma of the sphenoidal sinus. The patient was a 65-year-old woman who presented to us with a 1-year history of double vision. At first, based on the imaging findings, we suspected sinusitis with fungal infection of the sinus. Biopsy revealed chondromyxoid fibroma, and the patient was treated by radiotherapy. However, at present, 7 months after the treatment, the tumor remains unchanged, indicating the need for careful follow-up of the patient.

Stevens-Johnson syndrome (SJS) is a hypersensitivity reaction of the skin and mucosa to medications or infection. Herein, we report the case of a 15-year-old boy with SJS who presented with mucous membrane lesions, but without skin lesions. He was admitted with fever, extensively ulcerated and swollen lips, sore throat, and conjunctivitis. He had a one-week history of high fever prior to admission, and had been prescribed an antibiotic with an antipyretic-analgesic by his family doctor. His fever resolved, however, he presented soon thereafter with a 2-day history of mucosal erosions and conjunctivitis. He complained of a sore throat and was unable to swallow. His mouth and pharynx were covered with pseudomembranous lesions. There were no remarkable lesions on the skin. Although skin lesions were absent, the mucosal erosions and conjunctivitis were suggestive of SJS. He was begun on steroid therapy, which led to prompt resolution of the mucosal erosions and conjunctivitis.

Atypical SJS with an absence of skin lesions, but with strongly evident mucosal erosions can occur. The mucosal erosions in the oral cavity rarely are caused by SJS. Therefore, in cases with mucosal erosions, it is always necessary to take into consideration the possibility of SJS.

Salivary gland carcinoma was originally classified as acinic cell carcinoma because of its histological similarity to secretory breast carcinoma. However, in 2010, Skalove et al. characterized this tumor as mammary-analogue secretory carcinoma, because like secretory breast carcinoma, it often expresses the ETV6-NTRK3 fusion oncogene. We encountered a case of mammary-analogue secretory carcinoma arising from the parotid gland. This specific diagnosis was made because (1) a follicular variant was present, (2) physaliphorous cells were observed, (3) no zymogen granules were observed, (4) the mucoid discharge in the follicular lumen was D-PAS-positive, and (5) the tumor showed immunostaining characteristics consistent with those of mammary-analogue secretory carcinoma (e.g., positive for S-100 and adipophilin). No additional treatment was administered to the patient after the resection, as the tumor is known to show a low malignancy potential and the surgical margins were negative. Tumor recurrence or metastasis has not been observed until date.

A previous retrospective study reported that the sympathetic hypersensitivity caused by chronic stress might induce orthostatic blood pressure elevation (OBPE) and dizziness. This prospective study was planned to endorse that suggestion. Tofisopam, which is effective for normalizing sympathetic hypersensitivity, was administered to 13 dizzy patients with OBPE with no other abnormal findings. The patients were asked about whether they were aware of their exposure to stress or not. After the start of administration of tofisopam, a significant decrease of the OBPE and improvement of dizziness were observed in 12 (92.3%) of the 13 patients. Eleven (84.6%) of the 13 patients were considered to be exposed to chronic stress from their living environment, however, only 8 (61.5%) were aware of their exposure to chronic stress. These results validate the findings of the previous aforementioned study. OBPE may be a promising biomarker in the objective evaluation of chronic stress, because it was observed even in those patients who did not recognize their exposure to chronic stress.