Superior canal dehiscence syndrome: A review.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages.

Dizziness after an earthquake tilted the house.

Major earthquakes occasionally cause vertiginous attacks or persistent dizziness, which may be related to the effects of autonomic stress on equilibrium function and/or psychological factors. We present the case of a 73-year-old woman who suffered from persistent dizziness for 1 year, during which she lived in a house tilted by the great 2011 Tohoku earthquake. An electronystagmogram demonstrated leftward horizontal and downbeat nystagmus in the sitting and supine positions with eyes closed. Caloric testing and cervical vestibular-evoked myogenic potentials showed normal responses in both ears. Posturography indicated no involvement of psychological factors or autonomic stress. After moving into a new apartment, both the dizziness and nystagmus completely disappeared within 10 weeks. We speculate that the gravity-related linear acceleration with vertical and horizontal components in the tilted house may have chronically stimulated the otoliths, inducing the symptoms and nystagmus via the velocity storage mechanism.

Two cases of Tullio phenomenon in which oval and round window reinforcement surgery was effective.

AIM: To report the effect of oval and round window reinforcement surgery performed in two patients with the Tullio phenomenon. Case 1: A male with bilateral superior canal dehiscence syndrome. Downbeat nystagmus with leftward horizontal eye movement was recorded in an electronystagmogram using a pure-tone sound of 110dB at 2000 or 4000Hz in the right ear. Case 2: A female who had undergone stapes surgery. Computed tomography revealed an ossicular prosthesis in the vestibule. An audiogram indicated mild to moderate hearing impairment in the right ear. Leftward horizontal nystagmus was recorded in an electronystagmogram using a pure-tone sound of 110dB at 500 or 1000Hz in the right ear. Surgical findings indicated that the prosthesis was inserted deep into the oval window, which was closed with thin connective tissue. OUTCOMES: After oval and round window reinforcement surgery was performed in the right ear, and loud, pure-tone sounds elicited neither nystagmus nor dizziness in either patient.

Dominant foot could affect the postural control in vestibular neuritis perceived by dynamic body balance.

During attacks of vestibular neuritis (VN), patients typically lose postural balance, with resultant postural inclination, gait deviation toward the lesion side, and tendency to fall. In this study, we examined and analyzed static and dynamic postural control during attacks of VN to characterize differences in postural control between right and left VN. Subjects were patients diagnosed with VN at the Department of Otolaryngology, Toho University Sakura Medical Center, and underwent in-patient treatment. Twenty-five patients who had spontaneous nystagmus were assessed within 3days after the onset; all were right-foot dominant. Right VN was detected in nine patients (men: 4, women: 5; mean age: 57.6±17.08years [range: 23-82]) and left VN in 16 patients (men: 10, women: 6; mean age: 58.4±14.08years [range: 23-85 years]); the percentages of canal paresis of right and left VN were 86.88±18.1% and 86.02±15.0%, respectively. Statistical comparisons were conducted using the independent t-test. In stabilometry, with eyes opened, no significant differences were found between patients with right and left VN. However, with eyes closed, the center of horizontal movement significantly shifted ipsilateral (p<0.01). The differences in the lateral and anteroposterior body tracking test (BTT) were statistically significant (p=0.0039 and p=0.0376, respectively), with greater changes in cases with right VN. Thus, the dominant foot might contribute to the postural control mechanism.

Pterygopalatine fossa metastasis with severe trigeminal neuralgia from neuroendocrine carcinoma of the breast.

A 57-year-old woman presented to our department with a 2-month history of pain and paresthesia on the left side of the face 12 years after having undergone surgery for breast cancer. We performed an endoscopic biopsy and diagnosed metastatic breast cancer to the pterygopalatine fossa. There has been no recurrence for two years since the metastatic tumor was treated by radiation therapy. A literature search shows only one case of metastatic breast cancer with severe trigeminal neuralgia located in the V2 division of the trigeminal nerve area. Metastatic disease should be considered part of the differential diagnosis in patients presenting with trigeminal neuropathy.

Clinical Efficiencies of Stabilometry and Visual Feedback Test for Differentiating the Patients with Psychogenic Vertigo from Healthy Subjects.

The psychogenic vertigo has been diagnosed based on subjective dizzy symptom without abnormal findings of oculomotor tests and vestibular tests. We investigated the characteristics of the postural control system in patients with psychogenic vertigo using stabilometry and Body Tracking Tests with a visual feedback test to assess the dynamic body balance. This study consisted of 14 patients with psychogenic vertigo and 92 aged-match healthy subjects. They were instructed to keep the center of pressure constantly in the target circle displayed on the screen in front of the subjects. The dynamic body balance was evaluated by the proportion of the center of pressure (COP) including in the target circle during the test. The psychogenic vertigo group showed a larger area and a smaller locus length per unit area in comparison with the healthy subject group (p<0.01). In spectral analysis with the maximum entropy method (MEM), the power of the medio-lateral and antero-posterior positional power spectrum under eyes open condition were significantly largest at around 0.125 Hz in the psychogenic vertigo group. No significant difference in the result of Body Tracking Tests with a visual feedback test was found between both groups. Our results suggest that the patients with psychogenic vertigo maintain body balance with extremely slowly and large movements for quiet stance during eyes open condition. The results of Body Tracking Tests with a visual feedback test may indicate that the spontaneous postural control itself in patients with psychogenic vertigo does not differ from that in healthy individuals. We believe that this test could be useful as one of the significant diagnostic tests for psychogenic vertigo.

Measuring local immunoglobulin E in the inferior turbinate nasal mucosa in patients with allergic rhinitis.

BACKGROUND: Studies show that immunoglobulin E (IgE) is produced in the local nasal mucosa in allergic rhinitis patients. However, no study involved the measurement of IgE levels in the local nasal mucosal tissue in allergic rhinitis patients. This study aimed to measure the local IgE levels in the nasal mucosal tissue and to compare the levels of total IgE and specific IgEs in the serum and the inferior turbinate nasal mucosa in allergic rhinitis patients using the AlaSTAT 3gAllergy assay (Siemens Healthcare Diagnostics AG, Erlangen, Germany). METHODS: Total IgE antibodies and allergen-specific IgE antibodies in each sample of nasal mucosal tissue from 11 allergic rhinitis patients were measured with the AlaSTAT 3gAllergy assay. We compared the levels of total IgE and IgEs specific for house dust (HD), mites, and cedar pollen in the serum and the inferior turbinate. RESULTS: The total IgE levels and the cedar pollen-specific IgE levels in the inferior turbinate mucosal tissue correlated significantly with their respective levels in serum. The HD- and mite-specific IgE levels in the inferior turbinate mucosal tissue did not correlate significantly with their respective levels in the serum. CONCLUSIONS: Our results evaluating the correlations between nasal mucosal and serum levels of antigen-specific IgE indicate that IgE produced in the nasal mucosa affects the IgE levels in the serum, especially the cedar pollen-specific IgE.

Age-related morphological changes in the basement membrane in the stria vascularis of C57BL/6 mice.

Basement membrane anionic sites (BMAS) are involved in the selective transport of electrically charged macromolecules in cochlear capillaries. Using cationic polyethyleneimine (PEI), we examined age-related changes in BMAS in the cochleae of C57BL/6 mice. The mice were grouped according to age as follows: 3 days, 4 weeks, 8 weeks, 6 months, and 12 months. In the right bony labyrinths, widths of the stria vascularis were measured in paraffin-embedded sections using light microscopy. The left bony labyrinths were immersed in a 0.5 % cationic PEI solution and embedded in epoxy resin. Ultrathin sections of the left cochlea were examined using transmission electron microscopy. A significant difference in stria vascularis width was observed between the 4-week-old and 12-month-old mice. The PEI distribution in the capillary and epithelial basement membranes (BMs) of the cochlea was observed. In all animals, PEI particles were evenly distributed in the capillary BM of the spiral ligament and in the subepithelial BM of Reissner's membrane. In the stria vascularis, PEI particles were evenly distributed in the capillary BM in 3-day-old mice. In 4- and 8-week-old mice, PEI particle sizes were markedly lower than those observed in 3-day-old mice. In 6- and 12-month-old mice, PEI particles were hardly detected in the strial capillary BM. In the strial capillary BM in these mice, the laminae rarae externa and interna disappeared, but the lamina densa became larger. We speculated that age-related changes of strial capillary BMAS may affect electrically charged macromolecule transport systems in the stria vascularis of C57BL/6 mice.

Discrimination of Japanese monosyllables in patients with high-frequency hearing loss.

OBJECTIVE: To analyze the difficulty of discriminating Japanese nonsense monosyllables in each of several grades of high-frequency hearing loss and to evaluate the limitations of amplification. METHODS: We collected retrospective data on the discrimination of Japanese nonsense monosyllables by patients with three grades of high-frequency hearing loss who fulfilled or nearly fulfilled the Japanese criteria for EAS. Discrimination of the twenty monosyllables included in the 67-S speech audiometric test, which is approved by the Japan Audiological Society, was evaluated under quiet conditions. RESULTS: One hundred and five ears of ninety-one adults with high-frequency hearing loss were tested. We classified the ears according to hearing threshold at 1000 Hz; Group 1: <45 dB; Group 2: ≥45 dB and <70 dB; Group 3: ≥70 dB. Under the best conditions, the best speech discrimination scores were 72.3 ± 18.6% (mean ± SD, N=11), 56.9 ± 19.9% (N=57) and 38.1 ± 22.6% (N=37) in Group 1, Group 2 and Group 3, respectively. For most of the monosyllables, discrimination score declined gradually as high-frequency hearing loss became more severe. The high incidence in the Japanese language of [k], an easy consonant to distinguish, may be an advantage for patients with high-frequency hearing loss who use hearing aids. By employing a new confusion matrix that displays consonants and the following vowels separately, we were able to reveal the interactions of those two components. We observed that discrimination of preceding nasal consonants and that of the following vowels were not independent in patients with high-frequency hearing loss. CONCLUSION: Our classification based on threshold at 1000 Hz was useful to predict the effectiveness and limitations of amplification in high-frequency hearing loss. Threshold at 1000 Hz can be an index enabling us to refine the indications of EAS for native Japanese speakers to maximize its effectiveness against high-frequency hearing loss.

Subfraction analysis of circulating lipoproteins in a patient with Tangier disease due to a novel ABCA1 mutation.

Tangier disease, characterized by low or absent high-density lipoprotein (HDL), is a rare hereditary lipid storage disorder associated with frequent, but not obligatory, severe premature atherosclerosis due to disturbed reverse cholesterol transport from tissues. The reasons for the heterogeneity in atherogenicity in certain dyslipidemias have not been fully elucidated. Here, using high-performance liquid chromatography with a gel filtration column (HPLC-GFC), we have studied the lipoprotein profile of a 17-year old male patient with Tangier disease who to date has not developed manifest coronary atherosclerosis. The patient was shown to be homozygous for a novel mutation (Leu1097Pro) in the central cytoplasmic region of ATP-binding cassette transporter A1 (ABCA1). Serum total and HDL-cholesterol levels were 59mg/dl and 2mg/dl, respectively. Lipoprotein electrophoretic analyses on agarose and polyacrylamide gels showed the presence of massively abnormal lipoproteins. Further analysis by HPLC-GFC identified significant amounts of lipoproteins in low-density lipoprotein (LDL) subfractions. The lipoprotein particles found in the peak subfraction were smaller than normal LDL, were rich in triglycerides, but poor in cholesterol and phospholipids. These findings in an adolescent Tangier patient suggest that patients in whom these triglyceride-rich, cholesterol- and phospholipid-poor LDL-type particles accumulate over time, would experience an increased propensity for developing atherosclerosis.

Eosinophilic granulomatosis with polyangiitis demonstrating IgE-immune complexes and the possible involvement of IgE autoantibodies.

We herein report a case of female eosinophilic granulomatosis with polyangiitis (EGPA) in which polyethylene glycol (PEG) precipitation was used to evaluate the patient's levels IgE-immune complexes (IC). Her serum IgE (7,110 IU/mL) and IgE-IC (1,880 IU/mL) levels were observed with an IgE PEG precipitated index of 26.4%. We speculate that the circulating IgE-IC were formed by anti-neutrophil IgE autoantibodies. Therefore, the large amount of IgE autoantibodies in the patient's serum appears to have induced a constant allergic pathology. This pathology may have resulted in a marked infiltration of eosinophils into the tissues, as well as intensified the EGPA pathology.

Prognostic factors for long-term hearing preservation after canal-tympanoplasty for congenital aural atresia.

The present study aimed to determine favorable prognostic factors for long-term postoperative hearing outcome after canal-tympanoplasty for congenital aural atresia (CAA). We retrospectively reviewed pre and postoperative hearing results and image findings of 51 ears with CAA performed by canal-tympanoplasty for primary repair. Averages of the postoperative air and bone-conduction thresholds, and the air-bone gap (ABG) were calculated from the last pure-tone audiometry. Follow-up duration ranged from 16 to 139 months. A successful hearing result was defined as a postoperative ABG of ≤15 dB, or a postoperative pure-tone average of ≤30 dB. The influence of the following factors on the success of surgery was assessed by multivariate logistic regression analysis: total Jahrsdoerfer grading system score, age at surgery, and dimensions of middle ear including incudostapedial joint angulation, mesotympanic height, mesotympanic width, mesotympanic depth, mesotympanic area, mesotympanic volume, reconstructable external auditory canal (EAC) diameter, and reconstructable EAC height. Successful hearing outcomes were achieved in 24 of 51 ears (47.1%). A multivariate logistic regression analysis showed that an EAC area >72.3 mm(2) was the most significant favorable predictive factor (P = 0.006), followed by mesotympanic depth >5.5 mm (P = 0.013), mesotympanic height >4.6 mm (P = 0.016), and EAC diameter >9.5 mm (P = 0.029). In conclusion, the size of the reconstructable EAC and mesotympanum is important for predicting long-term favorable hearing outcome following canal-tympanoplasty for CAA.

The dominant foot affects the postural control mechanism: examination by body tracking test.

CONCLUSION: The antero-posterior (AP) body tracking test (BTT) showed that the dominant foot could affect the tilt angle of the sway movement, delineated by primary component analysis. Differences associated with the dominant foot could represent the difference in space perception of each person. OBJECTIVES: To examine whether the dominant foot could affect the postural control mechanism using the BTT. METHODS: Ninety-seven healthy participants enrolled in the study were classified into right-foot and left-foot dominance groups, and their performances were compared. For the BTT, each participant stood on a stabilometer and caught the movement of a visual target moving vertically (anterior-posterior) or horizontally by the center of pressure movement, displayed on a 14-inch screen monitor at 100 cm in front of the subject. The mean displacement angle of the obtained stabilogram was evaluated by principal component analysis. RESULTS: The AP BTT in the right-foot dominance group showed a clockwise tilt with a mean displacement angle of 3.022 ± 3.761°, whereas the group with left-foot dominance had a modest counter-clockwise tilt with a mean displacement angle of -0.694 ± 4.497°. This difference was found to be significant by the independent t test (p < 0.0001). In the lateral BTT, the mean displacement angles were not significant.

Favorable prognostic factors for long-term postoperative hearing results after canal tympanoplasty for congenital aural stenosis.

OBJECTIVE: We aimed to determine favorable prognostic factors for long-term postoperative hearing results after canal tympanoplasty for congenital aural stenosis (CAS). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Canal tympanoplasty for CAS was performed in 25 ears. INTERVENTION: Primary repair of CAS. MAIN OUTCOME MEASURES: The influences of the following factors on the success of surgery were assessed by univariate and multivariate logistic regression analyses: modified Jahrsdoerfer grading system total score; age at surgery; patterns of presentation (whether sporadic or syndromic); presence of external auditory canal (EAC) cholesteatoma; presence of ossicular fixation, including the malleus bar; presence of a partial atretic plate; exposure of the facial nerve at the tympanic portion; type of tympanoplasty; and each component of the modified Jahrsdoerfer grading system. RESULTS: The univariate analysis revealed that the absence of EAC cholesteatoma (p = 0.029) and the presence of a partial atretic plate (p = 0.040) were significant predictive factors for favorable hearing prognosis, whereas the multivariate logistic regression analysis showed that an absence of EAC cholesteatoma was the most significant favorable predictive factor (p = 0.011), followed by anterolateral position of the malleus/incus complex with respect to the stapes as the second-most favorable factor (p = 0.021). CONCLUSION: The absence of EAC cholesteatoma and anterolateral position of the malleus/incus complex with respect to the stapes are considered useful in predicting long-term favorable hearing results after canal tympanoplasty for CAS.

The relationship between vasomotor symptoms and menopause-associated dizziness.

CONCLUSION: Vasomotor symptoms (VMS) and especially hot flushes are negatively associated with recovery from menopause-related dizziness. A further study is justified to determine whether hormone replacement therapy (HRT) can shorten this recovery time. OBJECTIVES: This study aimed to compare the rate of recovery from dizziness between patients who had VMS and those who did not. We also evaluated the effect of HRT on recovery from dizziness. METHODS: The medical records of 85 women aged between 40 and 59 years were analyzed retrospectively. The patients were asked detailed questions about their menopausal symptoms as well as any episodes of dizziness, which were diagnosed on the basis of neuro-otological examinations and were treated appropriately. All patients were followed up until dizziness improved. RESULTS: Over a 2-month period, the rate of recovery from vertigo was significantly higher in patients without VMS (no-VMS group, n = 38) than in those with VMS (VMS group, n = 29). In the VMS group, patients treated with HRT (n = 7) recovered from dizziness more quickly than those who were not treated with HRT (n = 22), although this difference was not statistically significant.