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Patients with benign paroxysmal positional vertigo (BPPV) can have vitamin D deficiency, which is a cause of abnormal bone turnover. Several studies have established a relationship between osteoporosis and BPPV. The World Health Organization Fracture Risk Assessment Tool, widely known as FRAXThe study involved 40 postmenopausal women diagnosed with BPPV between July 2015 and April 2016, and 40 postmenopausal women as controls. The 10-year major osteoporotic and hip fracture risks were calculated using FRAX and were compared between BPPV patients and controls using Welch's t test and a general linear model.The 10-year major osteoporotic fracture risk was 20.4%±12.1% for BPPV patients (aged 72.4±8.6years) and 14.3%±6.5% for controls (aged 71.2±6.3years). The 10-year hip fracture risk was 9.0%±9.8% for BPPV patients and 5.0%±3.9% for controls. The BPPV group had significantly higher 10-year major risks of osteoporotic fracture (p=0.0069) and hip fracture (p=0.0202) compared with controls. Similarly, after adjustment for age, the BPPV group had significantly higher 10-year risks of major osteoporotic fracture (p=0.0007) and hip fracture (p=0.0092) compared with controls.Fracture risk calculated using FRAX was significantly higher in the BPPV group than in controls. Women with BPPV may need early intervention to prevent future fractures.

The purpose of this study was to investigate the existence of endolymphatic hydrops (EH) in affected and unaffected ears in patients with unilateral sensorineural hearing loss (SNHL) using contrast-enhanced magnetic resonance imaging (MRI), and to evaluate the significance of EH in various otological diseases.Retrospective study.University hospital.One hundred eighty-two ears from 91 patients with unilateral SNHL were studied. The endolymphatic space was evaluated using 3-Tesla MRI with gadodiamide hydrate. Imaging data about the degree of EH in the cochlea and vestibule were analyzed and compared between ears with various otological diseases.All affected ears with delayed endolymphatic hydrops had EH. In affected ears with definite Menière's disease, cochlear EH was observed in all ears and vestibular EH in 93% of ears, and these rates were significantly higher in the affected than in the unaffected ears. EH was also observed in the cochlea and vestibule in 66% and 41%, respectively, of the affected ears with idiopathic sudden SNHL; however, these percentages did not differ significantly from those in the unaffected ears (52% and 38%, respectively).MRI showed that a high percentage of ears affected by Menière's disease or delayed endolymphatic hydrops had EH. Further studies should evaluate the implications of EH in ears, especially in those with sudden SNHL, in terms of secondary or pre-existing EH.

To investigate the relationship between endolymphatic hydrops and hearing level, focusing on significant vestibular endolymphatic hydrops adjacent to the stapes footplate and low-frequency air-bone gaps.Retrospective study.The study included 1,548 ears from 775 patients who underwent magnetic resonance imaging examination in our university hospital to investigate possible endolymphatic hydrops between January 2012 and December 2015. Ears were evaluated by magnetic resonance imaging performed 4 hours after intravenous injection of a standard dose of gadodiamide hydrate and/or 24 hours after intratympanic injection of gadopentetate dimeglumine diluted eightfold. Comparison of hearing thresholds on pure-tone audiometry was performed between ears having endolymphatic hydrops adjacent to the stapes footplate and those having nonadjacent endolymphatic hydrops.Forty-one ears (22 men and 19 women, mean age 48.4 years) showed significant cochlear and vestibular endolymphatic hydrops adjacent to the stapes footplate, and 79 ears (30 men and 49 women, mean age 45.0 years) showed significant nonadjacent cochlear and vestibular endolymphatic hydrops. The average air-bone gap at 250 Hz was significantly higher in the group of ears with adjacent hydrops than in those with nonadjacent hydrops.The appearance of low-frequency air-bone gaps suggests deterioration of endolymphatic hydrops, particularly in ears with Ménière's disease, and could be a useful indicator for evaluating and treating patients with endolymphatic hydrops.4. Laryngoscope, 128:1658-1662, 2018.

Objective: We investigated the effect of polyneuropathy, nephropathy, and retinopathy on postural sway in patients with poorly controlled diabetes mellitus (DM) who were hospitalized for DM education. We also investigated the relationship between postural sway and hearing disturbances. Methods: We included 50 subjects who were hospitalized for the purpose of DM education. Mean age was 57.2 years, mean HbA1c was 9.4%, and mean DM duration was 11.1 years. Renal function, retina condition, and nerve conduction were evaluated in relation to DM complications (nephropathy, retinopathy, and neuropathy). Static posturography was performed to assess DM-related postural sway. Nerve conduction studies were used to obtain information on the condition of peripheral nerves. Pure-tone audiometry was measured. Stepwise multiple linear regression was used to assess DM-related factors that affected postural sway. Results: Deterioration of median nerve compound muscle action potential (CMAP) amplitude, tibial nerve CMAP conduction velocity, and peroneal nerve CMAP latency were associated with deterioration of postural sway. Deterioration of hearing in the worse ear was also associated with postural sway. Conclusion: In patients with poorly controlled DM, postural sway was particularly associated with deterioration of motor nerve conduction study parameters represented by median nerve CMAP amplitude, tibial nerve CMAP conduction velocity, and peroneal nerve CMAP latency. A link between diabetic polyneuropathy, diabetes-related postural sway, and diabetes-related hearing loss was recognized.

Objective To examine endolymphatic hydrops (EH) using magnetic resonance imaging (MRI) in patients with definite Meniere’s disease (MD) and those with nonotological diseases. Methods We studied 32 patients with unilateral MD, 10 patients with bilateral MD and 21 patients with control ears who had other benign diseases not associated with hearing or vestibular dysfunction. The mean age of the subjects was 54.0 years (range 27–74) in the MD group and 56.1 years (range 24–79) in the control group. Using MRI, the degree of EH was classified as none, mild and significant in the cochlea and vestibule separately. The ratio of the area of endolymphatic space to the vestibular fluid space was calculated for the vestibule. The duration of MD was defined as the months between the first attack of MD and the MRI study. Results EH was present in the cochlea of 45/52 affected ears of patients with MD (87%) and in 16/42 control ears (38%). Significant cochlear hydrops was present in 37/52 affected ears (71%) and in 4/42 control ears (10%). EH in the vestibule was present in 49/52 affected ears (94%) and in 3/42 control ears (7%). Significant vestibular hydrops was present in 40/52 affected ears (77%) and in none of the 42 control ears. There was no relationship between the degree of EH and its duration. Using a cut off value for the relative size of EH in the vestibule of 41.9%, the test had a sensitivity of 88.5% and a specificity of 100% to diagnose definite MD. Conclusion Cochlear EH was occasionally observed in control ears on MRI, as in normal temporal bone specimens. The presence or absence and degree of vestibular EH were significantly different between ears with MD and control ears. EH in the vestibule might be a specific predictor of definite MD.

OBJECTIVE To evaluate vascular activity in ears with otosclerosis by intraoperative measurement of blood flow using laser Doppler flowmetry and to compare the data with densitometry on computed tomography (CT). DESIGN Retrospective case series. SETTING University hospital. PATIENTS Thirty-nine ears from 33 patients who underwent surgery for otosclerosis. INTERVENTION The subjects were divided into fenestral (29 ears) and retrofenestral (10 ears) groups based on CT findings. Ratios of bone density in the area anterior to the oval window (AOW) or the promontory (PT) near the round window niche to that of the basis labyrinthine otic capsule were calculated on CT images. Measurements of blood flow were performed with a laser Doppler flowmeter at the 2 corresponding areas evaluated by CT. MAIN OUTCOME MEASURES Imaging and blood flow values. RESULTS In both groups, relatively young subjects with high blood flow values in the AOW or PT had rather low ratios of bone density in the corresponding areas. In contrast, old subjects with low ratios of bone density showed low blood flow values in the same areas. In the retrofenestral group, subjects with high blood flow values in the PT showed low blood flow values in the AOW, but their ratios of bone density were similarly low in both areas. CONCLUSION Blood flow varied and did not correlate with CT densitometry. Measurement of blood flow by laser Doppler flowmetry could yield useful information to evaluate the progress of vascular activity.

Folate metabolism is essential for cellular functioning. Despite extensive research on the roles of folate-metabolism-related gene polymorphisms in the pathophysiology of many diseases, such as cardiovascular disease, cancers, and sudden sensorineural hearing loss, little is known about their association with Ménière's disease (MD). The aim of this study was to investigate the effect of methylenetetrahydrofolate reductase (MTHFR) gene polymorphisms (C677T and A1298C) on the risk of MD in a Japanese population. We examined the C677T and A1298C (rs1801133 and rs1801131) polymorphisms in the MTHFR gene and compared them between 1946 adults (986 men and 960 women) participating in the National Institute for Longevity Sciences Longitudinal Study of Aging and 86 cases of MD. A multiple logistic regression was performed to obtain odds ratios (ORs) for the risk of MD regarding the MTHFR polymorphisms before (model 1) and after (model 2) adjustment for age and sex factors. The OR of MTHFR C677T for the risk of MD was 0.669 (95% confidence interval [CI], 0.479-0.934) in model 1 and 0.680 (95% CI, 0.484-0.954) in model 2. In contrast, the OR of MTHFR A1298C for the risk of MD was 1.503 (95% CI, 1.064-2.123) in model 1 and 1.505 (95% CI, 1.045-2.167) in model 2. Our results imply that the MTHFR C677T and A1298C polymorphisms are associated with the risk of MD.

Using magnetic resonance imaging (MRI), endolymphatic hydrops could be visualized on both sides after intratympanic (IT) injection of gadolinium contrast agents (Gd) in one symptomatic ear and subsequent intravenous (IV) Gd injection. The MRI revealed a difference of intracochlear Gd distribution between the IT injection side and the contralateral IV side.Although the IT method allows greater enhancement of the perilymph, many patients feel reluctance in receiving the IT injection in asymptomatic ears. We attempted to evaluate endolymphatic space size on both sides without the IT injection in asymptomatic ears.In 10 patients with Meniere's disease, MRI was performed 24 h after the IT Gd injection in one symptomatic ear and 4 h after the IV Gd injection. The signal intensity of Gd in the basal and apical turns of the cochlea was evaluated.The signal intensity in the scala tympani of the basal turn of the cochlea was 1.70 ± 0.60 on the IT + IV side and 0.42 ± 0.10 on the contralateral (IV) side. Gd was distributed uniformly in the scala tympani in the cochlea on the IV side, whereas it was strongly localized in the basal turn on the IT + IV side.

The presence of endolymphatic hydrops (EH) in the vestibule on magnetic resonance imaging (MRI) might be a high-risk factor for complications in cases that are candidates for stapes surgery.Pre-operative detection of EH could be valuable in cases that are candidates for stapes surgery to prevent unpredictable complications following surgery. Pre-operative MRI findings and post-operative findings following stapes surgery were compared to evaluate the efficacy of such MRI evaluation for the management of cases with otosclerosis.Sixteen cases who underwent pre-operative evaluation by 3T MRI performed 4 h after intravenous injection of gadolinium and stapes surgery for otosclerosis were recruited. Imaging data concerning the degree of EH in the vestibule and cochlea were compared with post-operative clinical findings for all cases.Mild EH in the cochlea or the vestibule was observed in eight ears and one ear, respectively, whereas one ear showed significant EH both in the cochlea and the vestibule. The post-operative course was uneventful in 12 of 14 cases with no EH in the vestibule, and the other two cases had a short period of dizziness, but two cases with EH in the vestibule had a long period of dizziness.

All definite Meniere's disease (MD) had endolymphatic hydrops (EH) at least in the cochlea or the vestibule. Symptoms of MD may appear after formation of EH. It is assumed that the probability of immediate progression to bilateral MD from unilateral MD is very low in patients without EH on the non-affected side.To evaluate the EH on both sides in patients with unilateral and bilateral MD, and to investigate factors with progression to bilateral MD including the degree of EH, hearing level, and the duration of MD.The study included 29 patients with unilateral definite MD and 12 patients with bilateral definite MD. The endolymphatic space size was visualized by using 3-Tesla magnetic resonance imaging.All patients with unilateral and bilateral MD had significant or mild EH at least in the cochlea or the vestibule on the affected side. On the non-affected side, EH was not observed at all in eight patients, but asymptomatic EH was observed in the cochlea in 14 patients and in the vestibule in 16 patients. There was no relationship between the EH on the non-affected side and the duration of MD.

The etiologies of idiopathic sudden sensorineural hearing loss (SSNHL) and Ménière's disease remain unclear. Recently, accumulating evidence has demonstrated that oxidative stress is related to the pathology of inner ear disease. Because genetic factors may contribute partly to the etiologies of SSNHL and Ménière's disease, we investigated the associations between genetic polymorphisms located in oxidative stress response genes and susceptibility to SSNHL and Ménière's disease. We compared 84 patients affected by SSNHL, 82 patients affected by Ménière's disease, and 2107 adults (1056 men and 1051 women; mean age, 59.2 years; range, 40-79 years) who participated in the National Institute for Longevity Sciences, Longitudinal Study of Aging. Multiple logistic regression was used to calculate odds ratios for SSNHL and Ménière's disease in individuals with polymorphisms in the genes glutathione peroxidase 1 (GPX1) (Pro198Leu, rs1050450), paraoxonase 1 (PON1) (Gln192Arg, rs662; and Met55Leu, rs854560), PON2 (Ser311Cys, rs7493), and superoxide dismutase 2 (SOD2) (Val16Ala, rs4880), with adjustment for age and gender. No significant differences in the distribution of the genotypes at these polymorphisms were observed among individuals with SSNHL and Ménière's disease and controls. No significant risk for SSNHL and Ménière's disease was observed in the additive genetic model, regardless of moderating variables. The C allele of SOD2 (rs4880) was more frequent in Ménière's disease cases with a hearing level over 50 dB compared with cases with a hearing level below 50 dB, suggesting that this polymorphism is associated with progression of a hearing loss in Ménière's disease. In conclusion, no significant associations between the polymorphisms of GPX1, PON1, PON2, and SOD2 and risk of SSNHL and Ménière's disease were observed in this Japanese case-control study.

Visualization of endolymphatic hydrops has been performed using magnetic resonance imaging (MRI) after intratympanic or intravenous gadolinium (Gd) injection. Our recent findings indicate that just as the prevalence of asymptomatic glaucoma is greater than that of symptomatic glaucoma, there are also many cases of asymptomatic endolymphatic hydrops. It is assumed that the asymptomatic endolymphatic hydrops that precedes Ménière's disease is found more frequently using MRI than with other techniques. Gd in the inner ear moves into the cerebrospinal fluid (CSF) via the internal auditory meatus. Gd enhancement is also recognized in the ocular fluid after the intravenous Gd administration. In this paper, the relationships between CSF, ocular fluid and inner ear fluid are reviewed. The central nervous system, eye and inner ear contain specialized extracellular fluids that are essential for maintaining their function: CSF, ocular fluid consisting of vitreous humor and aqueous humor, and inner ear fluid consisting of perilymph and endolymph. Abnormal accumulation of or pressure elevation in these fluids is associated with hydrocephalus, glaucoma and Ménière's disease, respectively. The dura mater and the arachnoid membrane of the optic nerve canal and inner ear meatus are very close to the eye and the inner ear, respectively. It has been reported that low CSF pressure is associated with glaucoma and endolymphatic hydrops. In glaucoma and Ménière's disease, nerve damage to ganglion cells rather than damage of the sensory cells is directly associated with progression of the disease. Retinal ganglion cells in glaucoma and spiral ganglion cells in Ménière's disease are targets of the abnormal accumulation of, or increased pressure in, the extracellular fluid, just as neurons are damaged in hydrocephalus. Studies on hydrocephalus, glaucoma and Ménière's disease as a group may deepen our understanding of each disease.

Hereditary hemorrhagic telangiectasia (HHT) is an inherited autosomal dominant vascular dysplasia caused by mutations in mainly the endoglin gene ( ENG ) or activin-like kinase receptor 1 (ALK1) gene ( ACVRL1 ). We investigated the molecular basis of HHT in a Japanese patient, and identified a novel missense mutation in ENG (c.38T>A, p.Leu13Gln) located in the signal peptide's hydrophobic core, but not in ACVRL1 . In experiments in COS-1 cells, the Leu13Gln (L13Q) mutant endoglin appeared to be expressed as a precursor form, probably due to impaired protein processing. Flow cytometry analyses of the COS-1 cells transiently expressing recombinant endoglins revealed that the wild-type endoglin was detected on the cell surface, but the L13Q mutant was not. We also analyzed expression patterns of the recombinant endoglins by immunofluorescent staining, and found that the wild-type co-localized with the endoplasmic reticulum (ER), but the L13Q mutant did not. These results implied that the L13Q mutant endoglin fails to insert into the ER, probably due to destruction of the hydrophobic core structure in the signal peptide to be recognized by signal recognition particles. Thus, the Leu13 in the signal peptide of endoglin might be essential for correct protein processing through the ER and cell-surface expression. Taken together, the novel c.38T>A mutation in ENG would impair co-translational processing of the endoglin, and could be responsible for HHT in this patient.

Clinical findings were assessed in three pediatric cases of dehiscence of the bony labyrinth caused by a high jugular bulb (HJB). Two children had two dehiscent lesions, which included posterior semicircular canal dehiscence (PSCD) and vestibular aqueduct dehiscence (VAD). One child had VAD alone. Two subjects with PSCD, but not with VAD alone, had mixed hearing loss and showed wave motion of the baseline on tympanometry and acoustic reflex testing, and a reduced response on otoacoustic emission. These findings may reflect jugular venous pulsations transmitted through the PSC and represent characteristics of cases with PSCD caused by HJB.

OBJECTIVE The effect of cochlear blood flow (CBF) on speech perception ability in cochlear implant (CI) users has not been reported. We investigated various factors influencing speech perception including CBF in CI users. PATIENTS Eighty-two patients who received CI surgery at an academic hospital. METHODS CBF was measured during CI surgery using laser Doppler flowmetry. The speech perception level was measured after a sufficient interval after CI surgery. Multivariate analysis was used to evaluate the influences of age, duration of deafness, sex, cause of deafness, and CBF on the speech perception level. RESULTS CBF decreased significantly with age but was not related to the speech perception level. In patients with congenital hearing loss, the speech perception level was significantly worse in children who received a CI at 3 years of age than in those who received a CI at 2 years of age or younger. Duration of deafness before CI surgery had deteriorative effects on the speech perception level. CONCLUSION CBF may be associated with progression of hearing loss. However, measuring CBF during CI surgery is not useful for predicting postoperative speech perception.

Objectives/Hypothesis: To compare preoperative computed tomography (CT) and magnetic resonance imaging (MRI) findings to the clinical features in patients with middle ear cholesteatoma with labyrinthine fistulae. Study Design: Retrospective case series. Methods: Twenty-eight patients who underwent surgery for middle ear cholesteatoma with one or more labyrinthine fistulae confirmed by CT were studied. Preoperative imaging analysis was performed using three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) MRI. Fistula size measured by CT and the signal intensity ratio (SIR) between the affected lesion in the inner ear and the cerebellum measured by 3D-FLAIR MRI were evaluated with respect to the clinical features and surgical findings. Results: The SIR was not correlated with fistula size. The hearing threshold determined by preoperative bone conduction correlated with the SIR, especially in patients with acute sensorineural hearing loss, but it did not correlate with fistula size. Patients with fistula symptoms had a significantly higher SIR than those without symptoms, and similar findings were observed in patients with an active infection. Patients with a larger fistula or higher SIR tended to have a deeper fistula and a more adhesive fistula matrix at operation. Conclusions: SIR was more strongly correlated than CT findings to the clinical status of patients with labyrinthine fistulae caused by cholesteatoma. Adhesion of the cholesteatoma matrix to the membranous labyrinth correlated with the SIR; thus, SIR may accurately reflect the extent of inner ear dysfunction with a labyrinthine fistula.

Conclusion. Imaging of the endolymphatic space was possible in most cases after administering a standard dose of gadolinium (Gd) by using heavily T2-weighted 3D fluid-attenuated inversion recovery (hT(2)W-3D-FLAIR) magnetic resonance imaging (MRI). Objectives. The aim of this study was to characterize the contrast enhancement in the inner ear after intravenous injection of Gd and to compare the signal intensity after a double dose or standard dose of Gd. Methods: We injected a standard dose of gadodiamide hydrate (DTPA-BMA) or a double dose of gadoteridol (HP-DO3A) intravenously in patients affected by Meniere's disease and performed hT(2)W-3D-FLAIR 3 T MRI. Contrast enhancement of the basal turn of the cochlea was evaluated semiquantitatively. Results: Endolymphatic hydrops was observed in the cochlea and vestibule of the affected ears of patients with Meniere's disease in both the double-dose group and the standard-dose group.

Clin. Otolaryngol. 2011, 36, 139–146 Objectives: To investigate the pharyngeal morphologic features and its pathogenic role on obstructive sleep apnoea syndrome in the elderly population. Design: Prospective controlled, comparative cohort study. Setting: Territory referral centre. Participants: We enroled 320 consecutive patients with complaints of snoring who visited Nagoya University Hospital from January 2004 to December 2007. We also collected 26 control subjects aged over 60 years from community-dwelling people. Main outcome measures: We underwent a morphological evaluation, measurement of nasal resistance, assessment of daytime sleepiness and nocturnal polysomnography. Results and conclusions: Two hundred and ninety-two patients were analysed. The constitution ratio of men, the body mass index and Epworth sleepiness scale were decreased with ageing. Tonsil size was reduced progressively with ageing. Retroglossal space was wider, and soft palate was lower in ≥60 year group than in

The first visualization of endolymphatic hydrops in patients with Meniere's disease was performed using three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) imaging with a 3 Tesla MRI unit after gadolinium contrast agent (Gd) was injected intratympanically. The 3D-FLAIR MRI could differentiate the endolymphatic space from the perilymphatic space, but not from the surrounding bone. By optimizing the inversion time, the endolymphatic space, perilymphatic space and surrounding bone could be separately visualized on a single image using three-dimensional real inversion recovery (3D-real IR) MRI. Using 3D-FLAIR and 3D-real IR MRI, various degrees of endolymphatic hydrops were observed in the basal and upper turns of the cochlea and in the vestibular apparatus after intratympanic Gd injection. Recently, visualization of endolymphatic hydrops became possible 4 h after intravenous Gd injection in patients with Meniere's disease. We applied a heavily T(2)—weighted 3D-FLAIR technique to detect Gd more sensitively for evaluation of endolymphatic hydrops after an ordinary amount of Gd was administered intravenously. Thus, newly developed MRI techniques have contributed significantly to the evaluation of endolymphatic hydrops. The intravenous administration of an ordinary amount of Gd is routinely done clinically. The relationship between endolymphatic hydrops and clinical symptoms will be investigated widely using new techniques.

Visualization of endolymphatic hydrops became possible after intravenous gadolinium (Gd) injection in patients with Ménière's disease.To visualize endolymphatic hydrops after intravenous Gd injection.Gd (gadoteridol; 0.2 mmol/kg) was injected intravenously in three patients with unilateral Ménière's disease. We performed three-dimensional fluid attenuated inversion recovery (3D-FLAIR) and three-dimensional real inversion recovery (3D-real IR) magnetic resonance imaging (MRI) 4 h after the injection using a 3-Tesla MRI unit. We used a 32-channel array coil to obtain a high signal-to-noise ratio.Endolymphatic hydrops was observed in the ears of patients with Ménière's disease. However, Gd concentration in the perilymph was lower compared with that obtained after intratympanic Gd injection.

Background The role of increased nasal resistance in obstructive sleep apnea syndrome (OSAS) remains controversial. The aim of this study was to examine the pathogenetic role of nasal obstruction in obese patients with OSAS. Methods Patients with OSAS (n = 125) at a university hospital were divided into three groups according to body mass index (BMI): nonobese (BMI < 25 kg/m2), mildly obese (25 kg/m2 ≤ BMI < 30 kg/m2), and obese (BMI ≥ 30 kg/m2). The subjects underwent nasopharyngoscopy, measurement of nasal resistance, and polysomnography. Results We studied 42 nonobese, 47 mildly obese, and 36 obese patients with OSAS. Among the obese, but not the nonobese and mildly obese patients, we found significant correlations between the oxygen desaturation index (ODI) and bilateral nasal resistance (BNR; r = 0.412; p = 0.013), between the ODI and unilateral higher nasal resistance (UHNR; r = 0.413; p = 0.012), and between the apnea index and UHNR (r = 0.334; p = 0.046). Multiple regression analysis incorporating all patients showed that BMI (p < 0.001) and BNR (p = 0.033) were independently related to the ODI. Conclusions In obese patients with OSAS, increased nasal resistance could play an important pathogenetic role in hypoxemic apnea.

The purpose of this study was to evaluate the relationship between the endolymphatic space image obtained using magnetic resonance imaging (MRI) and the results of electrocochleography. Electrocochleography recordings were obtained from 25 ears of 24 patients, who underwent MRI 1 day after the intratympanic injection of gadolinium diethylenetriamine pentaacetic acid bismethylamide. The average summating potential to action potential (SP/AP) ratio in patients with significant endolymphatic hydrops in the cochlea was 54 ± 17%. However, in some patients who had significant endolymphatic hydrops in the cochlea, the SP/AP ratio was not enlarged. This may imply that elevation of the SP/AP ratio is related to not only the degree of endolymphatic hydrops but also to the persistence of hydrops.

Objective: Many recent studies have reported on intratympanic gentamicin therapy for the treatment of intractable Meniere's disease. Intratympanic administration of steroids has also been used to treat sudden sensorineural hearing loss. These intratympanic drug therapies are based on the assumption that the drug administered intratympanically enters the inner ear through the round window membrane. We used magnetic resonance imaging (MRI) to evaluate whether and how intratympanically administered gadolinium (Gd) enters the inner ear. Methods: GD hydrate was injected intratympanically through the tympanic membrane using a 23-G needle into 61 ears of 55 patients with inner ear diseases. The injected Gd was diluted 8-fold in saline for injection into 58 ears and 16-fold for 3 ears. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) imaging was performed using a 3-Tesla MRI unit 1 day after the intratympanic injection. Results: In 53 of 61 ears, the Gd-containing inner ear was detected well as a high signal on 3D-FLAIR imaging. However, Gd was not visible in 2 ears with Meniere's disease and in 1 ear with profound deafness. The concentration of Gd in the perilymph was lower in 4 ears with Meniere's disease and 1 ear with delayed endolymphatic hydrops than after intratympanic administration of the 16-fold Gd dilution. Conclusion: Round window permeability was absent in 5% of ears, and 13% of ears had poor round window permeability. These results should be considered when planning intratympanic drug administration therapy to treat inner ear diseases.

The objective of the present study was to investigate endolymphatic space images in patients with fluctuating hearing loss without vertigo, and to elucidate its underlying pathophysiology. Eight patients with fluctuating hearing loss without vertigo were included in this study. 3T MRI was taken, 24 h after intratympanic injection of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA). Electrocochleography and VEMP tests were performed to evaluate cochlear and vestibular functions. Endolymphatic hydrops were observed both in the cochlea and in the vestibule of all eight patients. Three patients out of six whose summating potential/action potential (SP/AP) ratio was recordable showed an elevation of SP/AP ratio. In the two patients with remarkable endolymphatic hydrops in the vestibule, VEMP was absent from the affected ear. In conclusion, 3T MRI after intratympanic injection of Gd-DTPA revealed endolymphatic hydrops both in the cochlea and in the vestibule in the patients with fluctuating hearing loss without vertigo.

Objectives/Hypothesis: To estimate the effectiveness of nasal surgery on the occurrence of sleep apnea, and to analyze the pharyngeal morphology of apnea patients whose sleep-disordered breathing was ameliorated postoperatively. Study Design: Prospective study. Methods: Thirty-five consecutive patients with apnea and nasal obstruction underwent polysomnography and a morphological examination of the upper airway before and after nasal surgery, which included septoplasty, inferior turbinectomy, and/or functional endoscopic sinus surgery. Results: Sleep apnea was significantly ameliorated in only eight patients. The postoperative reduction in the apnea-hypopnea index tended to be lower in those with a low-positioned soft palate, reflected in an elevated modified Mallampati score, and a narrow retroglossal space. Neither swollen tonsils nor narrow fauces affected the surgical outcome. Regression analysis showed that the modified Mallampati score (P < .05) and the retroglossal space (P < .05) were significant predictors of postoperative improvement in the apnea-hypopnea index. Conclusions: Among sleep apnea patients suffering from nasal obstruction, nasal surgery is effective in those with a high-positioned soft palate and/or a wide retroglossal space. Laryngoscope, 2009

Grading of endolymphatic hydrops in the vestibule and the cochlea using magnetic resonance imaging (MRI) is proposed (2008 Nagoya scale).To standardize the evaluation of endolymphatic hydrops in both the vestibule and the cochlea using MRI.The endolymphatic space was evaluated after intratympanic gadolinium injection using three-dimensional fluid attenuated (3D-FLAIR) MRI and three-dimensional real inversion recovery (3D-real IR) MRI.A simple three-stage grading system was acceptable for hydrops in both the vestibule and the cochlea: none, mild, and significant. In the vestibule, the grading was determined by the ratio of the area of endolymphatic space to the vestibular fluid space (sum of the endolymphatic and perilymphatic spaces). Patients with no hydrops have a ratio of one-third or less, those with mild hydrops have between one-third and a half, and those with significant hydrops have a ratio of more than 50%. In the cochlea, patients classified as having no hydrops show no displacement of Reissner's membrane; those with mild hydrops show displacement of Reissner's membrane but the area of the endolymphatic space does not exceed the area of the scala vestibuli; and in those with significant hydrops the area of the endolymphatic space exceeds the area of the scala vestibuli.

A 67-year-old man presented with a rare case of cavernous sinus thrombophlebitis (CST) caused by Porphyromonas gingivalis with abscess formation extending to the orbital cavity. Neuroimaging demonstrated a cystic lesion in the right cavernous sinus that was hyperintense on diffusion-weighted imaging. The patient was successfully treated with surgical drainage and antibiotic administration. CST is rare and often has a fulminant progression with high rates of morbidity and mortality. The differential diagnosis of cavernous sinus lesions should include CST. Early recognition and differentiation from other diseases with aggressive medical and possible surgical intervention are necessary to reduce mortality and long-term sequelae. Diffusion-weighted imaging is useful for the early recognition and differentiation of CST from other diseases.

Using three-dimensional real inversion recovery (3D-real IR) and three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI), various degrees of endolymphatic hydrops were observed in the basal and upper turns of the cochlea and in the vestibular apparatus after intratympanic gadolinium (Gd) injection. MRI may contribute to our understanding of inner ear diseases and may be a useful addition to intratympanic drug therapy in the management of inner ear diseases.To evaluate 3D-real IR MRI and 3D-FLAIR MRI with clinical symptoms and signs in patients with inner ear disease.Gd was diluted in saline and injected intratympanically in 73 patients with inner ear disease. The endolymphatic space was evaluated with 3-Tesla MRI at 1 day after the intratympanic Gd injection.3D-real IR MRI was generally better than 3D-FLAIR MRI in discriminating between the perilymphatic space and endolymphatic space in the cochlear turns and in the vestibular apparatus. However, when Gd concentration was insufficient in the perilymph, it was more difficult to visualize the Gd with 3D-real IR MRI than with 3D-FLAIR MRI. Endolymphatic hydrops was observed using MRI in patients with 'probable' Meniere's disease based on the criteria.

The 3D models of endolymphatic and perilymphatic spaces enabled us to obtain normal and pathological volumes of each space and helped us to understand the 3D structure of various parts of the inner ear and of endolymphatic hydrops.To make a 3D model of the inner ear using sections of temporal bone with and without hydrops.Every 10th 20 microm thick section of temporal bone was collected from two ears with endolymphatic hydrops and five ears without hydrops. Using ZedView, 3D Doctor, FreeForm as analytical software, a 3D model of the inner ear was obtained by reconstruction of these sections. The volumes of the endolymphatic (EV) and perilymphatic spaces (PV) were calculated in each part of the cochlea and vestibular apparatus including the semicircular canals, but the endolymphatic duct and sac were not included.In normal ears (controls), the average cochlear EV was 5.1 microl and the PV was 41.9 microl, and the average vestibular EV was 24.0 microl and the PV 75.7 microl. In one hydropic ear, the cochlear EV was 17.5 microl, cochlear PV 30.7 microl, vestibular EV 42.5 microl, and vestibular PV 33.4 microl. In the other hydropic ear, cochlear EV was 31.2 microl, cochlear PV 30.1 microl, vestibular EV 25.6 microl, and vestibular PV 71.8 microl.

Topical application of dexamethasone may support autoregulation of cochlear blood flow (CBF), although it had no direct effect on CBF.Although intratympanic steroid therapy for patients with inner ear disorders is common, the mechanism by which steroids exert their effect is unclear. We investigated the response of CBF to topical application of dexamethasone onto the round window.Two concentrations of dexamethasone (3.3 mg/ml and 33 mg/ml dexamethasone in 0.5 microl saline) were applied to the round windows of rats, and CBF responses were measured using a laser Doppler flowmeter. The effects on CBF of a 2 h occlusion of the anterior inferior cerebellar artery (AICA) and subsequent release of the clamp with or without previous dexamethasone application were investigated.No significant change in CBF was observed after topical application of dexamethasone, and it did not affect the decrease in CBF caused by AICA occlusion. However, recovery of CBF after release of the AICA clamp was better in animals treated with dexamethasone than in those that did not receive dexamethasone.

Lymphangioma localized to the bones of the skull base is rare. The authors report herein the case of a 5-year-old boy who presented with lymphangioma of the bone, localized to the skull base and leading to cerebrospinal fluid (CSF) rhinorrhea with meningitis. Neuroimaging demonstrated lytic destruction with a cyst in the right middle skull base. The patient was successfully treated with resection of the tumor and prevention of CSF leakage. Histopathological examination revealed a lymphangioma. An enlarging lymphangioma can lead to bone destruction. A differential diagnosis of a lytic lesion for a cyst at the skull base is important for proper case management.

Background The purpose of this study was to evaluate the effects of nasal surgery on nasal resistance, sleep apnea, and sleep quality in adult male patients with obstructive sleep apnea syndrome (OSAS). A prospective study was performed in OSAS patients who underwent isolated nasal surgery in a tertiary referral center. Methods During the 3-year study period, 49 OSAS patients suffering from symptomatic nasal obstruction/impaired nasal breathing underwent the standard polysomnography before and after surgery. Polysomnography along with measures of nasal resistance and daytime sleepiness (the Epworth sleepiness scale [ESS] scores) were reviewed also. Results Surgery decreased the nasal resistance (0.55 ± 0.37 Pa/cm3 per second versus 0.17 ± 0.19 Pa/cm3 per second; p < 0.001) and ESS scores (11.7 ± 4.1 versus 3.3 ± 1.3; p < 0.001), without changes in the apnea-hypopnea index (AHI; 44.6 ± 22.5 versus 42.5 ± 22.0). Surgery increased nadir oxygen saturation (76.2 ± 10.9% versus 78.8 ± 8.1%; p < 0.01), shortened apnea–hypopnea duration (averaged/maximum; 33.5 ± 7.3/61.1 ± 46.0 versus 28.8 ± 7.4/47.3 ± 36.1 second; p < 0.05/p < 0.01), and improved sleep quality. Conclusion The results suggest that nasal surgery is useful for lowering nasal resistance, ameliorating sleep-disordered breathing, and improving sleep quality and daytime sleepiness in OSAS.

Objective:To evaluate the vestibular aqueduct in patients with sudden sensorineural hearing loss.Methods:We evaluated 19 patients (12 men and seven women; age range, 22−79 years) with unilateral sudden sensorineural hearing loss, using computed tomography and magnetic resonance imaging. All these patients had unilateral sudden sensorineural hearing loss. We also evaluated 47 control subjects (22 men and 25 women; age range, 22–79 years).Results:In sensorineural hearing loss affected ears, the width of the vestibular aqueduct at the midpoint and at the operculum was significantly greater than that in contralateral ears or in control ears. The width of the vestibular aqueduct at the midpoint and the operculum did not correlate with the audiometric threshold or the audiogram configuration. Contrast enhancement of the ipsilateral endolymphatic sac was observed in 17 of 19 patients with sudden sensorineural hearing loss (89 per cent). Eleven of these 17 patients also showed enhancement on the contralateral side, but no patient showed enhancement only on the contralateral side. In sensorineural hearing loss affected ears, the width of the vestibular aqueduct did not differ significantly between those patients with and without enhancement.Conclusions:The vestibular aqueducts of sudden sensorineural hearing loss affected ears are wider than those of controls. Precise imaging and evaluation of the inner ear is essential when investigating the pathological conditions responsible for sudden sensorineural hearing loss.

Background The aim of this study was to investigate the effects of simple tonsillectomy on nasal resistance in patients with obstructive sleep apnea syndrome (OSAS). Methods Conventional tonsillectomy was performed in 20 patients who were refractory to treatment with continuous positive airway pressure. The subjects consisted of 17 men and 3 women (mean age, 32.9 ± 6.3 years). The effects of tonsillectomy were evaluated with preoperative and postoperative polysomnography and nasal resistance. Results After tonsillectomy, nasal resistance decreased significantly from 0.39 ± 0.30 Pa/cm3 per second to 0.27 ± 0.16 Pa/cm3 per second (p < 0.05). Simultaneously, the apnea–hypopnea index decreased significantly from 55.7 ± 22.5 to 21.2 ± 14.2 (p < 0.05). There was no significant correlation between tonsillar weight and percentage of change in bilateral nasal resistance (p > 0.05). Conclusion The reduction in nasal resistance induced by simple tonsillectomy could play an important role in improving OSAS, as does nasal surgery or adenotomy.

Conclusion. This study demonstrated that precise analysis shows that the inner ear shape in sudden deafness (SD) is different from that in controls in that the fluid-filled area of SD labyrinths is significantly larger than that of controls. Reduced cochlear modiolus area and inner area of the lateral semicircular canal (LSCC) may be associated with insufficient maturation of the inner ear. Objective. The aim of this study was to quantify the morphologies of the cochlea and LSCC using magnetic resonance imaging (MRI) and to evaluate their relationships with clinical symptoms in SD. Subjects and methods. Twenty-six unilateral SD patients with vertigo, 26 unilateral SD patients without vertigo and a matched control group without hearing loss were studied. The areas of cochlear modioli and LSCCs were traced on the MRI console and compared between SD patients with or without vertigo and control subjects. The ratio of the LSCC fluid-filled area to the total LSCC area was used to index the degree of dysplasia. Results. The cochlear modiolus area was significantly less in SD ears (4.190.2 mm 2 ) than in controls (4.390.4 mm 2 ). The LSCC inner area was significantly less in SD ears (6.99 1.7 mm 2 ) than in controls (9.191.8 mm 2 ). These results suggest that the fluid-filled area of SD labyrinths is significantly larger than controls. Morphology did not differ between affected and contralateral sides or between ears with or without vertigo in SD patients.

突発性難聴症例12例に対し Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) MRIによる検討を行い, 造影前3D-FLAIRでは12例中9例 (75%) で患側内耳に高信号を認めた。その9例中2例は造影後3D-FLAIRで内耳に造影所見を認めた。この2例と造影後3D-FLAIRで患側内耳に造影所見を認めた感音難聴悪化の1例につき報告した。これらの症例では血液迷路関門破綻による内耳障害が疑われた。血液迷路関門の破綻と突発性難聴の予後との関連は今後の検討課題である。内耳性難聴における病態診断についてMRI3D-FLAIRは多大な貢献をすることが期待される。

The estimated annual number of patients with sudden deafness treated in Japan has increased during the last 30 years, especially in the elderly population. Hypertension and diabetes mellitus could contribute to the etiology of this disorder.To investigate the epidemiological trends of sudden deafness in Japan over the past 30 years by analyzing data from national surveys.Nationwide epidemiological surveys on sudden deafness were conducted four times by the Research Committee of the Ministry of Health and Welfare in Japan. The first, second, third, and fourth surveys were performed in 1972, 1987, 1993, and 2001, respectively.The estimated annual numbers of patients with sudden deafness treated in Japan were 4000 in 1972; 16,700 in 1987; 24,000 in 1993, and 35,000 in 2001. The average ages of patients at onset were 39.1, 45.4, 49.3, and 51.3 years, respectively. Thus, both the number of patients and the average age of patients with sudden deafness have increased during the past 30 years. Patients with sudden deafness in the fourth survey exhibited hypertension and diabetes mellitus more frequently than did patients in the first survey; this trend was particularly noticeable among elderly patients.

Pathologic third window lesions, such as superior semicircular canal dehiscence syndrome (SCDS) or large vestibular aqueduct syndrome (LVAS), cause several auditory and vestibular symptoms, which might affect perilymphatic pressure and induce endolymphatic hydrops (EH). In this study, the existence of EH in subjects with SCDS or LVAS was investigated using contrast-enhanced magnetic resonance imaging (MRI).Case series at university hospital.Seventeen ears from nine subjects who were diagnosed as having SCDS (five ears from three cases) or LVAS (12 ears from six cases) were studied. Ears were evaluated by 3-T MRI performed 4 hours after intravenous injection of gadodiamide hydrate. Imaging data concerning the degree of EH in the cochlea and the vestibule were compared with clinical symptoms and hearing levels for all ears.All ears showed air-bone gaps at low frequencies on pure tone audiometry. None of the subjects with SCDS had episodes of acute sensorineural hearing loss (SNHL) or vestibular symptoms, except for one patient who complained of head vibration induced by loud noise. Conversely, five of six subjects with LVAS had episodes of acute SNHL or vestibular symptoms. Four of five ears with SCDS showed severe EH in the cochlea, and two ears showed mild EH in the vestibule. All ears with LVAS showed mild to severe EH in both the cochlea and vestibule.The present study demonstrated the existence of EH in ears with pathologic third window lesions, which might affect patients' auditory or vestibular symptoms.4 Laryngoscope, 126:1446-1450, 2016.

Hearing loss is a risk factor for the progression of dementia. Cognitive improvement is occasionally found after removal of cerumen impaction. Because patients with dementia do not usually complain about cerumen impaction, detection methods are important. The present study aimed to investigate whether cerumen impaction is observable using brain magnetic resonance imaging.Participants were six patients whose hearing level improved 15 dB or more unilaterally or bilaterally after the removal of cerumen impaction. A radiologist who was blind to the impaction side and whether magnetic resonance imaging scans were taken before or after impaction removal classified cerumen impaction as positive, negative or unclear.Three ears classified as impaction positive and five ears classified as impaction negative corresponded accurately to the presence or absence of cerumen impaction. Among four ears classified as unclear, two did and two did not have cerumen impaction.Careful examination of the external ear canal on brain magnetic resonance imaging can be used to detect cerumen impaction.

To evaluate the inner ear in sudden sensorineural hearing loss (SNHL) using three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) at 3 Tesla (T).3D-FLAIR MRI has recently been developed to detect high concentrations of protein or hemorrhage. Application of this method to sudden SNHL has not been described.We used 3D-FLAIR at 3 T with and without gadolinium enhancement to evaluate eight patients with sudden SNHL.In four of eight ears with idiopathic sudden SNHL, high precontrast signals were observed within the inner ear on 3D-FLAIR. The high-signal areas observed on 3D-FLAIR were not detected by T1- or T2-weighted MRI in any of these patients. In one of these four ears, significant gadolinium enhancement was observed on 3D-FLAIR.Half the patients with sudden SNHL examined had high signals in the SNHL-affected ear on 3D-FLAIR at 3 T. High precontrast signals in the inner-ear fluid space may reflect minor hemorrhage, or an increased concentration of protein that had passed through blood vessels with increased permeability, or had originated from disrupted cells in the inner ear. Gadolinium enhancement in one ear suggested the breakdown of the blood-labyrinth barrier. 3D-FLAIR MRI should contribute markedly to the elucidation of pathologic conditions in the inner ears of patients with idiopathic sudden SNHL.

The distribution of pendrin, which is encoded by the Pendred syndrome gene, has been investigated immunohistochemically in the inner ear. In the cochlea, pendrin has been found in the spiral prominence, external sulcus cells, Hensen's cells and Claudius cells, but its expression in the organ of Corti remains unclear. We examined whether pendrin localizes in the organ of Corti by postembedding immunogold analysis. In the organ of Corti, gold particles were clearly observed in outer and inner hair cells, including the stereocilia. The density of the particles was especially high in the cuticular plates of the hair cells. Gold particles were also detected in the external sulcus, in part of the spiral ligament adjacent to the external sulcus, in supporting cells, and in the spiral ganglion of the cochlea. Our study revealed that pendrin occurs in the organ of Corti. The role of pendrin in the organ of Corti and its association with the Cl- or pH regulation of neurotransmission require further study.

Frequency analysis of the natural phenomena more or less exhibits a time fluctuation. Precise analysis of this fluctuation leads to the famous "1/f fluctuation" characteristic that gives mental as well as psychological relaxation effects to human.In the present paper, we focus on this "1/f fluctuation" characteristic observed in natural phenomena, in particular irreversible energy exchanging processes. As a result, it is found that a phase transition phenomenon such as a state changing to liquid from a solid accompanying irreversible energy exchanging process may exhibit "1/f fluctuation" characteristic.Thus it is revealed that any of the nonlinear phenomena accompanying irreversible energy exchanging processes may exhibit the 1/f fluctuation characteristic.

2001年に発症した突発性難聴症例を対象に全国調査が行われた。二次調査により2815例の調査票が得られた。このうち一側性の突発性難聴確実例で発症日, 初診時および固定時オージオグラムがすべて記載されていた1285人 (男性600人, 女性685人) を対象に, 聴力の予後に及ぼす因子について検討した。1285人の5周波数の初診時平均聴力レベルは, 57.4±24.9dBで固定時平均聴力レベルは33.3±23.6dBであった。多変量解析を用いて検討を行い, 初診時聴力レベル, 来院までの日数, 年齢, めまいの有無, オージオグラムの傾きが独立的に予後に関係していることが示された。耳鳴, 耳閉塞感の有無と予後との間には関係は認められなかった。治療法として複数の方法が組み合わされていた例が多かったが, ビタミンは91.8%, ATPは87.2%, ステロイドは85.3%, プロスタグランディンは32.6%, 高気圧酸素療法は11.1%, 星状神経節ブロックは7.9%の症例に用いられていた。

Leflunomide (LFM) is an inhibitor of mitochondrial enzyme dihydroorotate dehydrogenase (DHODH) that catalyzes the conversion of dihydroorotate to orotate coupled with the generation of reactive oxygen species (ROS) from mitochondria. We demonstrate here that LFM causes an unrestrained proliferation of mitochondria both in human osteosarcoma cell line 143B cells and rat liver derived RL-34 cells. Increases in the total mass of mitochondria per cell in LFM-treated cells were evidenced by the application of Green FM or 10- n -nonyl acridine orange to flow cytometry, an enhanced replication of mtDNA and electron microscopy. Externally added uridine improved the disturbance in cell cycle progression in LFM-treated cells, but failed to suppress such unrestrained mitochondrial proliferation. On the contrary, lapacol and 5-fluoroorotate, inhibitors of DHODH besides LFM, suppressed the biogenesis of mitochondria during the cell cycle progression. LFM, but not lapacol or 5-fluoroorotate, caused increases of the intracellular level of acetylated α-tubulin. These data suggest that the inhibition of DHODH may not be at least primarily related to the LFM-induced abnormal proliferation of mitochondria, and support our recent published observation that changes in the physicochemical properties of microtubules may be in someway concerned with the biogenesis of mitochondria.

Although there have been many studies on the round window membrane (RWM), little information has been reported about changes in the membrane associated with aging. We have undertaken morphological studies of RWMs using young (7-8 weeks old) and aged (27-29 months old) C57BL/6 mice. The RWM was thinner in mice from the aged group compared with that of the young group. The cell density in the epithelial and inner layers was also reduced in the aged group. In the middle layer of the RWM in aged mice, transmission electron microscopy revealed many degenerated short and thick elastic fibers. Confocal laser microscopy using fluorescein isothiocyanate(FITC)-wheat germ agglutinin (WGA) staining was used to identify WGA-positive fibers in the middle layer of the RWM, which changed in a similar manner to the fibers in aging skin.

1993年の突発性難聴全国疫学調査で突発性難聴として報告された一側例2,790症例のうち急性低音障害型感音難聴がどれくらい含まれているか検討した。 2,790症例中252症例が急性低音障害型感音難聴であった。急性低音障害型感音難聴252症例について性別, 年齢分布, 聴力の変化, 発症から初診までの期間, 予後を検討した。 男女比は1:2, 平均年齢は34.8歳であった。 発症日から初診日までの日数が短いほど予後は良く, 予後の男女差は認めなかった。 急性低音障害型感音難聴は平成12年度急性高度難聴調査研究班の診断基準 (試案) を用いたところ突発性難聴全国疫学調査の登録患者の9%を占めており, 突発性難聴との差が明瞭ではなかったことを示唆していた。