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Past studies have found an association between cochlear hydrops or early Meniere's disease and acute low-frequency hearing loss (ALHL) without vertigo. However, its mechanism remains unclear in some ALHL cases. This report presents a case of ALHL associated with iron-deficiency anemia (IDA). The patient was a 49-year-old female who had previously been treated with betamethasone for sudden hearing loss in the right ear. Eight months later, the symptoms recurred and cochlear hydrops was diagnosed. Isosorbide and betamethasone were administered orally, and intravenous hydrocortisone tapering was added 1 week later, but these treatments were ineffective. At the same time and subsequently, iron sucrose was intravenously administered for IDA, and the patient's hearing loss gradually resolved within 2 months. In view of the increase in serum hemoglobin levels after iron therapy, this might have been the most effective treatment in this case. The hearing loss could therefore be associated with the patient's IDA.

Objective Irritability is an emotional stress symptom that causes or exacerbates dizziness. Antidepressants may be helpful for some conditions that are accompanied by irritability; however, they do not completely inhibit irritability. Yokukansan (YKS), a traditional Japanese herbal medicine, has been used for neurosis, insomnia, and children's irritability and night crying. The study investigated the efficacy of YKS in nystagmus in patients with chronic dizziness and irritability. Methods Twenty-two cases with chronic dizziness and irritability were reviewed retrospectively. The patients were divided into two groups: control patients (0–7 days of treatment) and YKS-treated patients (YKS cases; >7 days of treatment). Dizziness before and during (after, in the controls) YKS treatment was evaluated by scoring the nystagmus intensity on a 5-point scale. The average scores were calculated within a maximum of 6 months before and during or after treatment. The normalized scores were also calculated. The optimal treatment regimen was calculated via receiver operating characteristic (ROC) curve analysis. Results There were six control cases (1 male, 5 females; mean age: 59.5 years). There were 16 YKS cases (3 males, 13 females; mean age: 61.8 years). While the group mean nystagmus intensity scores significantly decreased from 1.18 to 0.73 in the YKS cases, it did not change in the control cases. The group mean of the normalized nystagmus intensity scores during treatment was 0.73 in the YKS cases. The results of the ROC curve analysis indicated the optimal cut-off period of the YKS treatment was 10 days. Conclusion The oral administration of YKS for more than 10 days was optimal. The treatments with YKS could be a good option for the treatments of vertigo.

Objective To investigate the relationship between end-tidal CO2 (EtCO2) and seasickness (motion sickness at sea) during an Antarctic voyage. Methods In this study, we measured EtCO2 and severity of seasickness using the subjective symptoms of motion sickness (SSMS). We sampled EtCO2 and SSMS every 3–4 h for 3 days from the date of sail in 16 healthy subjects. This experiment was performed on an icebreaker (standard displacement: 12,650 t). Results Since 2 subjects dropped out because of severe motion sickness, available data were collected from 14 subjects. On analysis of all data of all subjects grouped together, there seemed to be a significant negative correlation between EtCO2 and SSMS (R = −0.27, P = 0.0005). However, in individual subjects, this correlation was not obvious. During the voyage, EtCO2 level in the seasickness susceptible group was lower than that in the non-susceptible group (P = 0.018). Both EtCO2 increasing in the non-susceptible group and decreasing in the susceptible group contribute to the difference in EtCO2 levels. We suggest that the cause of this increase in EtCO2 level in the non-susceptible group was unwitting slow and deep breathing to resist seasickness. Conclusion We revealed that for seasickness during an Antarctic voyage, EtCO2 level relates to susceptibility, but not occurrence or severity. Measurement of EtCO2 levels may be useful to identify seasickness-susceptible persons and to efficiently prevent seasickness.

OBJECTIVES Urinary disorders are generally well understood, but there are few reports on the urinary status of people living in unusual climates such as the polar regions. We studied the impact of living conditions on the urinary status of members of the Japanese Antarctic Research Expedition. METHODS This prospective study enrolled 12 consenting members of the wintering party stationed at the Syowa Station in Antarctica between November 2012 and March 2014. The subjects completed questionnaires (the International Prostate Symptom Score [IPSS], Overactive Bladder Syndrome Score [OABSS] and Pittsburgh Sleep Quality Index [PSQI]) and kept daily voiding dairies for 3 days consecutively every 2 months. RESULTS Compared with baseline values, the mean scores on all the questionnaires decreased during the stay in Antarctica, from 3.42 to 2.31 for the IPSS, 1.25 to 0.986 for the OABSS, and 4.58 to 3.78 on the PSQI. A significant difference was noted in the scores of seven subjects with a 3 point or more decrease in IPSS score and five members with a decrease less than 3 points (-2.22 vs 0.448, P = 0.0416). CONCLUSION This is the first survey of the urinary status of people living in a polar environment. The findings obtained by questionnaires and voiding dairies indicated that urination and sleep status did not worsen during the stay in Antarctica.

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a novel ataxic disorder consisting of the triad of cerebellar impairment, bilateral vestibular hypofunction, and a somatosensory deficit. We report the first Japanese case of CANVAS. The patient is a 68-year-old Japanese male. He was referred to our university for further evaluation of progressive gait disturbance and ataxia. He exhibited horizontal gaze-evoked nystagmus and sensory deficit. Nerve conduction studies showed sensory neuronopathy. Magnetic resonance imaging showed the atrophy of vermis but not of the brainstem. The caloric stimulation and video head impulse test (vHIT) showed bilateral vestibulopathy. The visually enhanced vestibulo-ocular reflex (VVOR) was also impaired. In addition to neurological and electrophysiological examinations, simple neuro-otological examinations (i.e., caloric stimulation, vHIT, and VVOR) may reveal more non-Caucasian cases.

We report two cases of spontaneous intracranial hypotension (SIH) that presented with orthostatic hearing impairment. Pure tone audiometry in case 1 and distortion-produced otoacoustic emissions (DPOAEs) in case 2 clearly revealed orthostatic low-frequency hearing impairment. The symptoms resolved with conservative therapy in case 1, but they did not resolve completely after three treatments with epidural blood patch in case 2. The orthostatic changes at low-frequency that were present in both examinations indicated a relative endolymphatic hydrops due to SIH. Both otological examinations were useful to infer the pathophysiology of hearing impairment associated with SIH.

The superior semicircular canal dehiscence (SSCD) syndrome was first reported by Minor et al. in 1998. They reported that patients with SSCD typically exhibit symptoms of oscillopsia and pressure-/sound-induced vertigo due to the absence of the bony layer covering the superior semicircular canal. Although many cases of SSCD have been reported from America and Europe, only fourteen cases have been reported in Japan since 2003. We report a case of SSCD. The patient was a 30-year-old man who presented with the complaint of unsteadiness. He had been administered streptomycin for the treatment of pulmonary tuberculosis when he was 12 years old. Pure-tone audiometry showed a sloping pattern of sensorineural hearing loss bilaterally, with detection of 40 to 55 dB at high frequencies. Rightward-beating nystagmus was observed when the patient lay down, but not when he was sitting. Caloric responses were normal, ruling out the effect of streptomycin. Coronal computed tomography with 1.0-mm collimation revealed dehiscence of the left superior semicircular canal. Fistula symptoms were observed when positive pressure was applied to the external auditory canal. Tullio's phenomenon was also observed with sound stimulation (100 dB, 5Hz, click sound) to the left ear. These findings were compatible with the features of SSCD. The body-position dependency of the rightward-beating nystagmus could be attributable to changes in the intracranial pressure or intralabyrinthine pressure with the body posture, or to the nystagmus being of the pulse-synchronous type. A sloping pattern of the sensorineural hearing loss may be one of the characteristics of SSCD.

We treated two cases of tumors arising from accessory parotid gland and Stensen's duct. The first case was a 30-year-old female with a 10-year history of right cheek tumor. Sialo-CT findings suggested that the tumor detached from the parotid gland and enhancement around the tumor was not remarkable. Fine needle aspiration cytology demonstrated normal salivary gland cells. Therefore, this case was diagnosed as accessory parotid gland tumor and extirpation surgery was performed. The other case was an 86-year-old female with a 4 year history of right cheek tumor. Sialography demonstrated obstruction of Stensen's duct. Based on the surgical specimen, this case was diagnosed as tumor arising from Stensen's duct. We discuss the management of the facial nerve during resection of these tumors.

Anterior inferior cerebellar artery (AICA) syndrome involves sudden hearing loss, vertigo, and several neurological symptoms and is being reported with increasing frequency due to diagnostic adavances. Herein, we describe 3 cases of anterior inferior cerebellar artery syndrome. Case 1 was a 67-year-old woman with sudden onset of vertigo. Initially, we treated her for sudden deafness with vertigo. Hearing loss improved almost completely by steroid and prostaglandin E1 administration. However, MRI demonstrated cerebellopontine infarction. Case 2 was a 53-year-old woman with hypertension and hyperlipidemia. She had dizziness and headache. CT and MRI demonstrated infarction of the cerebellum and brain stem. Hearing loss and dizziness were improved by steroid administration, control of blood pressure, and antithrombus therapy. She did not have cerebellar symptoms. Case 3 was a 58 year-old man with hypertension, who developed vertigo and vomiting. He demonstrated also hearing loss and ataxia. MRI demonstrated infarction of the cerebellum and brain stem, and otoneurological examination supported these findings. Vertigo and ataxia improved considerably. However, hearing loss was not completely improved. Steroid and prostaglandin E1 administration may improve hearing loss of anterior inferior cerebellar artery syndrome.

Salivary duct carcinoma is an uncommon tumor, and mainly originates in the parotid gland. We treated two patients with salivary duct carcinoma in the submandibular gland. One patient was a 60-year-old male who complained of right submandibular swelling. Submandibular sialoadenectomy, right neck dissection and postoperative radiation were performed. Four months later, multiple bone metastase were found. The other patient was a 40-year-old male who complained of right submandibular and bilateral cervical swelling. Submandibular sialoadenectomy, bilateral neck dissection, and postoperative radiation and chemotherapy were performed. Six months later, skin metastasis was found. Histologically, both were diagnosed as salivary duct carcinoma based on the presence of cribriform pattern and comedo necrosis. Preoperative diagnosis of salivary duct carcinoma is difficult. This carcinoma resists combined therapy and has a poor prognosis.

A 65-year-old female was referred to Kyoto Pref ectural University of Medicine with a tumorous lesion revealed by nasopharyngeal fiberscopy on June 7th, 1999. Angiography of the carotid artery revealed a large aneurysm in parapharyngeal space that resulted in right otitis media with effusion. Ligation and embolization with GDcoil of the aneurysm was performed by the Neurosurgery Department of Kyoto Pref ectural University of Medicine.Interventional radiology was useful for treating a case of aneurysm in the Parapharyngeal space.

Summary We report here the case of a 7-month-old boy with congenital cholesteatomas in the tympanic membrane. A large mass was found occluding most of the inferior half of the tympanic membrane, with a small mass just posterior to the malleus handle. The cholesteatomas were removed via an endaural approach without violating the integrity of the tympanic membrane. Our patient is probably the youngest reported with congenital cholesteatomas in the tympanic membrane. The importance of early diagnosis of congenital cholesteatoma of the tympanic membrane is suggested, and the treatment and etiology of congenital cholesteatoma of the tympanic membrane are discussed.

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is characterized by spastic paraparesis in the lower extremities, and urinary disturbance. HAM/TSP has also been less frequently associated with cerebellar syndromes and nystagmus. We report a case of HAM/TSP presenting with cerebellar ataxia and nystagmus. The patient was a 73-year-old woman who was born in southern Japan. At age 41, she developed pain and spasticity in the bilateral lower limbs and gradually progressive gait disturbance. At age 57, she was diagnosed with HAM/TSP based on spastic paraparesis in the lower limbs, urinary disturbance and positive anti HTLV-I antibody in serum and cerebrospinal fluid. In June 2008, she was referred to our university and hospitalized for rehabilitation. Twenty days later, she experienced rotatory vertigo sensation. Magnetic resonance imaging revealed pontocerebellar atrophy. The patient presented with cerebellar signs in the upper limbs, gaze-evoked nystagmus in the sitting position and right-beating horizontal nystagmus in the supine and head-hanging positions. Electronystagmography (ENG) showed horizontal saccadic overshoot dysmetria and horizontal saccadic pursuit. Nystagmus is rare among the literature on HAM/TSP. ENG is helpful to evaluate and confirm the cerebellar syndromes of HAM/TSP.

Objective To study the difference in the findings between the causes of angioedema and the characteristics of angioedema induced by angiotensin receptor II blockers (ARBs), and to investigate whether laboratory examinations for acute phase inflammatory markers can aid in diagnosis and predict airway risk. Methods We retrospectively reviewed fourteen cases of patients with angioedema that were treated from 2000 to 2006. Data were collected regarding age, sex, location of the edema, cause, time course of resolution and laboratory examinations (leukocyte counts, serum C-reactive protein (CRP) level, complement function and the activity of C1 esterase inhibitor). Results The causes of angioedema were ACEIs in six patients (42.9%), candesartan (ARB) in three (21.4%), HAE (types 1 and 2) in two, and unknown in three. Of these patients, 71.4% exhibited edema in the floor of the mouth, irrespective of the cause. Two patients with edema induced by candesartan exhibited both lingual and laryngeal edemas. The remaining one with candesartan-induced edema exhibited edema in the neck and mediastinum and pleural effusion. The average time to resolution was 4.1 days, ranging from one to twelve days. The edema in eleven patients resolved with conservative therapy, while three patients underwent tracheotomy. In two patients with candesartan-induced edema, although the edemas resolved completely after cessation of candesartan administration, the edemas reappeared in the same locations, two and thirty days after the cessation of candesartan for each patient. None of the patients with angioedema induced by ACEIs exhibited elevation of serum CRP levels. No significant differences were found for leukocyte counts and serum CRP levels between patients with angioedemas induced by ACEIs, ARB and those of unknown cause. No significant differences were observed in the above findings between the patients who underwent tracheotomy and those who did not. Two patients exhibited low C4 levels, and one of the two exhibited no activity of C1 esterase inhibitor. Conclusion Consistent with previous reports, angioedema in the floor of the mouth extending to the tongue should be considered as a possible risk factor for airway compromise. Laboratory examinations for acute phase inflammatory markers are not useful for diagnosis and are not predictive for airway intubation and tracheotomy. Angioedema induced by candesartan can present in anomalous sites and reappear following drug cessation even if the edema has resolved completely.

We usually move both our eyes and our head when pursuing a high-speed moving object. However, the vestibulo-ocular reflex (VOR), evoked by head motion, seems to disturb smooth pursuit eye movement because the VOR stabilizes the gaze against head motion. To determine whether head motion is advantageous for pursuing a high-speed moving object, we examined dynamic visual acuity (DVA) for a high-speed (80 degrees /s) rightward moving object with and without active linear rightward head motion (HM) at a maximum of 50 cm/s in nine healthy subjects. Furthermore, we analyzed eye and head movements to investigate the contribution of linear VOR (LVOR) and smooth eye movement under these conditions. In most subjects, active linear head motion improved DVA for a high-speed moving object. Subjects with higher DVA scores under HM had robust rightward gaze (eye + head) velocities (60 cm/s), i.e., rightward smooth eye movements (10 degrees /s). With the head stationary (HS), faster smooth eye movements (40 degrees /s) were generated when the subjects pursued a high-speed moving object. They also showed anticipatory smooth eye movements under conditions HM and HS. However, the level of suppression of their LVOR abilities was equal to that of the others. These results suggest that the ability to generate anticipatory smooth pursuit eye movements for following a high-speed moving object against the LVOR is a determining factor for improvement of DVA under HM.