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Hirayama disease is characterized by juvenile onset of unilateral muscular atrophy of a distal upper extremity. The pathogenic mechanism of Hirayama disease is cervical cord compression by the posterior dura with forward displacement in the neck flexion position. A few cases of 'proximal-type Hirayama disease' have been described as showing muscular weakness and atrophy of the proximal upper extremities caused by the pathogenic mechanism similar to that of Hirayama disease. We report herein the case of a 16-year-old boy with proximal-type Hirayama disease, who developed symptoms after he began kyudo (Japanese traditional archery). Neurological examination revealed bilateral weakness of the muscles innervated by C5 and C6 segments (the deltoid, biceps brachii, brachioradialis), bilateral mild sensory disturbance in the radial side of the forearm, absent tendon reflexes of the biceps brachii and brachioradialis with preserved triceps reflex, pyramidal signs of the bilateral lower extremities (pathologically brisk reflexes of lower extremities, Babinski's signs). MR images in the neck flexion position showing expansion of the posterior extradural space and forward displacement of the spinal cord at the C3/4, C4/5, C5/6 and C6/7 disk levels. CT myelogram revealed spinal cord compression not only in neck flexion but also in neck left axial rotation. His symptoms improved after the restriction of neck flexion and axial rotation. Weakness of the upper extremities improved after 2 months. Pyramidal signs of the lower extremities disappeared after 18 months. The pathogenic mechanism in this case may be associated with not only neck flexion but also neck axial rotation.

Background and objective It is well known that recombinant human fibroblast growth factor-2 (rhFGF-2) signaling plays an important role in tissue repair and regeneration. rhFGF-2 strongly binds to acidic gelatin via ionic linkages and is gradually released upon gelatin decomposition. On the other hand, the linkage between rhFGF-2 and basic gelatin is so weak that most rhFGF-2 is rapidly released from basic gelatin by simple desorption. Gelatin/β-tricalcium phosphate (β-TCP) sponges, which comprise 50 wt% gelatin and 50 wt% β-TCP in a cross-linked structure, can release rhFGF-2 gradually owing to their electrical features. In a previous study, we reported that new bone height in the test group using rhFGF-2 with acidic gelatin/β-TCP sponges was significantly greater than that in the control group using acidic gelatin/β-TCP sponges alone in a ridge augmentation model in dogs. However, whether these results depend on controlled release by the gelatin/β-TCP sponges remains controversial. In this study, we evaluated the effects of controlled release by comparing acidic and basic gelatin/β-TCP sponges with different isoelectric points (IEP) on ridge augmentation in dogs. Materials and methods Twelve weeks after extraction of the maxillary second and third incisors of six dogs, critically sized saddle-type defects (8 mm length × 4 mm depth) were surgically created bilaterally 2 mm from the mesial side of the canine. Acidic gelatin/β-TCP sponges (IEP 5.0) soaked with 0.3% rhFGF-2 were applied to the defect in the acidic group, whereas basic gelatin/β-TCP sponges (IEP 9.0) soaked with 0.3% rhFGF-2 were applied to the defect in the basic group. Twelve weeks after surgery, biopsy specimens were obtained and subjected to microcomputed tomography (micro-CT) and histological analyses. Results New bone area detected by micro-CT analysis was significantly smaller in the basic group than in the acidic group. New bone height calculated by histologic sections was significantly lower in the basic group than in the acidic group. The total tissue height was lower in the basic group than in the acidic group. However, the differences between both sites were not significant. Conclusions These findings suggest that in ridge augmentation of saddle-type defects, controlled release of rhFGF-2 induces notably more alveolar bone formation than does short-term application of rhFGF-2.

Intramedullary spinal cord hemorrhage (hematomyelia) is rare and usually related to trauma. Spinal vascular malformations such as intramedullary cavernomas and intradural arteriovenous malformations are the most common cause of atraumatic hematomyelia. Other considerations include warfarin or heparin anticoagulation, bleeding disorders, spinal cord tumors, and delayed complication of spinal radiation. We report the case of 48-year-old man receiving warfarin and aspirin therapy, who showed upper limb pain and dysesthesia from left axilla to left femur. Paraplegia, sensory disturbance, bladder and rectal disturbance developed gradually over two weeks, accompanied by severe back and neck pain. MRI showed hematomyelia extending from the C1 to T11 segments of the spinal cord. The hemorrhage was located mainly in the left side of the posterior column. Few cases of hematomyelia extending over 18 segments of the spinal cord have been reported. Past literature reports of hematomyelia tend to extend longitudinally above and below the area of initial hemorrhage. We thought that the shape of this hematomyelia extending longitudinally over several segments was formed by a similar pathogenesis to pencil-shaped softening of the spinal cord.

Primitive arteries are persistent fetal anastomoses between the carotid and vertebral-basilar circulation. Although rare, persistent primitive arteries can remain at birth. A 73-year-old woman presented with cerebral infarction to the posterior circulation caused by symptomatic common carotid artery stenosis with an unnamed and extremely rare persistent primitive artery. This anomalous vessel branched from the extracranial internal carotid artery and passed through the ipsilateral jugular foramen into the posterior cranial fossa and merged into the basilar artery. To our knowledge, this is the first case of a persistent primitive artery passing through the jugular foramen with symptomatic common carotid artery stenosis.

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

A 53-year-old male with fluctuating low frequency sensorineural hearing loss and tinnitus, but without vertigo, was evaluated by MRI obtained by intratympanic injection of a gadolinium-based contrast agent (GBCA) before and after the administration of isosorbide. The endolymphatic hydrops was semi-quantitatively evaluated by a 3.0-T MR scanner. For quantification, the affected side/contralateral side ratios were calculated. A gadodiamide (a kind of GBCA)-enhanced space surrounding the endolymph in the affected side with a 0.50 ratio (which may have represented endolymphatic hydrops) improved after isosorbide therapy to a 0.98 ratio. Thus, endolymphatic hydrops was demonstrated in a patient with 'atypical' Meniere's disease (MD), suggesting that at least some atypical MD may share similar etiology with, and therefore be a continuum of, MD. Also, therapeutic effects could be visualized by using MRI. Therefore, MRI-based diagnosis of MD-related disease will be a powerful tool not only because of its precision but also its usefulness for therapeutic evaluation.

A 48-years-old man presented with slowly progressive bradykinesia, personality change and rapidly progressive left hemiparesis. On admission, he presented dementia, poor judgment, left hemiparesis. MRI revealed a widespread high intensity area in right hemisphere and MRA was almost normal. Serological tests of serum and CSF demonstrated high titers of antibodies to Treponema pallidum. He was treated for syphilis with daily penicillin injections without improvement. He died of sepsis eight months after admission. At autopsy, the brain weighed 1,100 g and the right cerebral hemisphere was atrophic, especially in frontal base, temporal, parietal, angular, and posterior regions covered by thickened, fibrotic leptomeninges. Microscopically, chronic meningoencephalitis was observed. Severe neuronal loss with gliosis was seen in the right cerebral cortices. Scattered rod-shaped microglia and inflammatory cell infiltration were visible in the cerebral parenchyma. The dorsal column of the spinal cord was not involved and meningovascular syphilis was unclear. The distribution of the encephalitic lesions was well correlated with the clinical and neuroradiological findings. This was a rare autopsy case presenting Lissauer's general paresis, clinically manifesting as rapidly progressive stroke-like episode.

Bilateral intratympanic administration of a gadolinium-based contrast agent (GBCA) in MRI was successfully performed and proved to be beneficial in the semi-quantitative evaluation of endolymphatic hydrops. Such image-based diagnosis will lead to re-revaluation and reclassification of the diagnostic criteria for Meniere's disease (MD).To visualize endolymphatic hydrops semi-quantitatively in patients with MD, by using bilateral intratympanic GBCA administration with MRI.A total of 13 patients were evaluated, including 12 with MD and one with acute low-tone sensorineural hearing loss. Diluted gadodiamide (a kind of GBCA) was administered to the bilateral tympanic cavity by injection through the tympanic membrane. After 24 h, the endolymphatic hydrops was evaluated with a 3.0 T MR scanner. The areas enhanced by gadodiamide were measured semi-quantitatively.Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) MRI showed that the gadodiamide successfully penetrated the round window membrane, entering the perilymphatic space and delineating the gadodiamide-enhanced perilymphatic and gadodiamide-negative endolymphatic spaces of the inner ear. All the patients with MD showed a reduced gadodiamide-enhanced area representing the perilymphatic space, and the quantitative ratio was 0.15 to 0.85. Furthermore, endolymphatic hydrops was also demonstrated in the patient with atypical MD who had fluctuating low frequency sensorineural hearing loss without vertigo.

An enlarged endolymphatic duct and sac (EDS) that makes contact with the cerebrospinal fluid–dural interface plays an important role in the pathway of bone conduction and enhances bone conduction at lower frequencies. Objectives. We investigated whether the bone conduction threshold was improved when the EDS was enlarged. Subjects and methods. Twenty-three patients (46 ears) with large vestibular aqueducts underwent standard pure tone audiometry (PTA) and magnetic resonance imaging (MRI) to investigate the relation between the diameter of the endolymphatic duct (ED) and the air or bone conduction threshold. We also investigated the relation between the volume of the EDS and the air or bone conduction threshold. Results. All ears had a mixed type hearing loss. The air–bone gaps were significantly larger at 250 and 500 Hz than at higher frequencies. The bone conduction thresholds were significantly lower at 250 Hz and 1000 Hz when the diameter of the ED was large, whereas there was no relation between the diameter of the ED and the air conduction threshold. In addition, there was no correlation between the volume of the EDS and air or bone conduction thresholds.

Objective To investigate the cause of inner ear disturbances in cases of middle ear cholesteatoma with labyrinthine fistula. Setting University hospital. Study design Prospective case study. Patients Eight patients who were scheduled to undergo surgery for middle ear cholesteatoma with labyrinthine fistula were included in this study. Intervention Imaging analysis was performed using a 3-dimensional fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging sequence. Main outcome measures Three-dimensional FLAIR findings were compared with clinical symptoms associated with inner ear disturbance and surgical observations of the fistula. Results Three-dimensional FLAIR in 6 patients revealed areas of high signal intensity in the inner ears on the affected sides and areas with increased signal after the administration of gadolinium, especially in cases accompanied by acute sensorineural hearing loss. These images were considered to be indicative of breakdown of the blood-labyrinth barrier due to middle ear cholesteatoma. This finding was also present in a patient with no clinical symptoms of inner ear disturbances. Conclusion Three-dimensional FLAIR images of the inner ear are valuable in evaluating labyrinthine fistula in patients with cholesteatoma. Future studies are needed to better understand the role of 3-dimensional FLAIR in predicting the severity of inner ear disturbance.

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.

The Bing test is based on the principle that occlusion of the external auditory meatus improves the perception of bone-conducted sounds unless there is a conductive hearing impairment. An air-bone gap has been reported in patients with large vestibular aqueduct (LVA) syndrome without apparent middle ear dysfunction. We therefore performed the Bing test on nine patients with this syndrome to evaluate whether it is associated with an air-bone gap or middle ear dysfunction. Bone conduction thresholds did not change significantly during the Bing test in any patient. Because an air-bone gap is observed in patients with abnormal communication between the inner ear and cerebrospinal fluid through the LVA, dehiscent superior canal, or dilated inner ear meatus; we propose that a 'three windows' model (in which the abnormal communication provided by the enlarged endolymphatic duct and sac in LVA acts as the 'third window' for sound conductance) might explain the air-bone gap in such patients.

The authors report a rare case of primary mucinous carcinoma of the skin initially diagnosed as a metastatic adenocarcinoma. The tumor occurred in the right axilla in a 75-year-old man. Initial pathological diagnosis was metastatic adenocarcinoma. However, no primary focus was found in the body. The revised diagnosis by the authors was primary cutaneous mucinous carcinoma. The tumor (1.5 cm) was characterized by proliferation of atypical epithelial cells arranged in cell nests with many pseudolumens resembling adenoid cystic carcinoma. It was also characterized by much mucinous stroma or pool around tumor cells. No apparent eccrine or apocrine differentiation was noted histologically and immunohistochemically. The present case suggests that primary cutaneous mucinous carcinoma may be misdiagnosed as metastatic adenocarcinoma, and that it may resemble adenoid cystic carcinoma.

Pendred syndrome (PDS) is characterized by profound deafness in childhood, positive perchlorate challenge, and goiter. PDS is often associated with enlarged endolymphatic duct and sac (EEDS), and recently, PDS gene mutations have been reported even in those patients with EEDS without classic Pendred syndrome. In a previous report, the number of mutant alleles was correlated with the degree of subclinical thyroid abnormality, but not with hearing loss, in patients with missense mutation H723R. It also has been reported that the hearing loss in EEDS was not correlated with the EEDS volume, cochlear modiolar area, or signal intensity of the endolymphatic sac. We evaluated the correlations between the number of mutant alleles and these parameters in patients with EEDS to investigate the mechanisms underlying this condition. The study group was comprised of 16 Japanese patients with EEDS diagnosed by MR imaging. The H723R mutation was homozygous in six patients and heterozygous in six patients, with no mutation found in four patients. The modiolar area, EEDS volume, and signal intensity ratio (sac signal/cerebrospinal fluid signal) were not significantly correlated with the number of mutant alleles. PDS gene mutations may not be the only cause of EEDS, and the mechanisms underlying EEDS remain unclear.

We report a rare case of pigmented squamous cell carcinoma (SCC) of the cheek skin probably arising from solar keratosis. An 80-year-old man was referred to our clinic because of a black skin nodule in the right cheek. The nodular lesion was 1 cm in diameter, dome-shaped, hard, sharply demarcated, partially erosive and telangiectatic at the border. The lesion was completely excised under the clinical diagnosis of probable seborrheic keratosis. Microscopically, cutaneous horn and mildly atypical squamous epithelia suggestive of previous solar keratosis were present in the surface of the lesion. The lesion consisted of atypical squamous cells with keratinization and intercellular bridges, and it was regarded as SCC. The SCC cells were seen to invade lightly into the upper dermis, where lymphocytic infiltrations and melanophages were noted. Characteristically, heavy deposition of melanin pigment was recognized in the SCC cells as well as in proliferated dendritic and pigment blockade melanocytes that were scattered or colonized within the SCC cell nests. Masson-Fontana stain revealed numerous melanin granules in the SCC cells, as well as in dendritic and pigment blockade melanocytes. Immunohistochemically, the SCC cells were positive for cytokeratins and epithelial membrane antigen, and negative for S-100 protein and HMB45 antigen. Dendritic and pigment blockade melanocytes were negative for cytokeratins, epithelial membrane antigen, and HMB45 antigen, but positive for S-100 protein. The present case suggests that SCC cells of the skin may induce proliferation of melanocytes. The differential diagnosis and the histogenesis of pigmented SCC of the skin are discussed.

High concentration of acetylcholine (ACh) caused a rapid and long lasting relaxation response in age-matched controls, whereas this response was significantly weaker in streptozotocin (STZ)-diabetic and cholesterol fed mice. The levels of basal and ACh-stimulated cyclic GMP in the aorta was also significantly smaller in STZ-diabetic and cholesterol-fed mice. The attenuated relaxation responses to ACh in both STZ-diabetic and cholesterol-fed mice were ameliorated by the chronic administration of cholestyramine. A prior incubation of aortic strips with superoxide dismutase (SOD, 60 U/ml) improved the recovery phase of the relaxation of diabetic aorta after single administration of ACh, whereas SOD had no effects on ACh-induced relaxation of aortic strips from cholesterol fed mice. These results suggest that superoxide anion may be responsible for an impairment of endothelium-dependent relaxation of aorta from STZ induced diabetic mice. It is further suggested that impairment of endothelium-dependent relaxation in STZ-diabetic and cholesterol fed mice may be caused by different mechanisms.

We examined the effects of taurine on levels of low-density lipoprotein (LDL) cholesterol and glucose, and an endothelium-dependent relaxation in response to acetylcholine in cholesterol-fed or streptozotocin-induced diabetic mice. The acetylcholine-induced concentration-dependent relaxation was significantly attenuated in aortic rings from cholesterol-fed and streptozotocin-induced diabetic mice. The attenuated vasodilation in both cholesterol-fed and streptozotocin-induced diabetic mice was normalized by the chronic administration of taurine. The endothelium-independent relaxation of aortic rings induced by sodium nitroprusside was not significantly different between control, cholesterol-fed and streptozotocin-induced diabetic mice. The increased serum levels of LDL cholesterol in cholesterol-fed and diabetic mice were returned to normal by the chronic administration of taurine. The chronic administration of taurine had no effects on serum glucose levels. These results suggest that the impaired endothelium-dependent vasodilation seen in both cholesterol-fed and streptozotocin-diabetic mice can be normalized by the chronic administration of taurine and this effect may be, at least in part, due to lowering of serum LDL levels.

Sensory neuropathy with prominent ataxia reflecting kinesthetic sensory impairment is a well recognised form of neuropathy associated with Sjogren’s syndrome.1–4 Pathologically, T cell invasion of dorsal root ganglia, loss of large sensory neurons, and secondary large fibre degeneration is seen in this neuropathy.4 However, a form of neuropathy associated with Sjogren’s syndrome, presenting with pain and superficial sensory involvement without sensory ataxia has been described anecdotally5 and in a case report.6 Clinicopathological details of the second form of neuropathy have not been elucidated. In this report we describe seven patients with Sjogren’s syndrome showing painful sensory neuropathy without sensory ataxia. Patients studied were referred for painful neuropathy to Nagoya University Hospital and its affiliated institutions. All seven patients fulfilled diagnostic criteria for Sjogren’s syndrome by the American-European Consensus Group7 and showed painful peripheral neuropathy (table 1). Patients included six women and one man, ranging from 25 to 72 years old. In all patients initial symptom of neuropathy was paraesthesia or painful dysaesthesia in the most distal portions of the extremities, later extending proximally to involve the entire legs and arms. The trunk became involved in three patients, and the trigeminal nerve was impaired in three patients. Asymmetry in sensory impairment was present in four patients. None of the patients showed sensory ataxia in the initial phase. Most patients retained essentially normal muscle strength, but patient 1 showed slight weakness in distal limb muscles. …

Magnetic resonance imaging (MRI) after intratympanic gadolinium injection can reveal endolymphatic hydrops (ELH) in patients with delayed ELH (DELH). Patients with contralateral DELH may have bilateral ELH.DELH has previously been diagnosed based on clinical history, hearing and vestibular examinations. DELH is classified into three types: ipsilateral, contralateral and bilateral indicate the side with the longstanding hearing loss. Ipsilateral DELH occurs in the ear with a profound hearing loss, contralateral DELH in the better hearing ear and bilateral DELH in both ears. Imaging diagnosis of the endolymphatic space may add a new dimension to the diagnosis and treatment of DELH.Gadodiamide hydrate was diluted eightfold with saline. The diluted gadodiamide hydrate was injected intratympanically through the tympanic membrane in two patients with ipsilateral DELH and five patients with contralateral DELH. One day after the injection, 3 Tesla MRI was performed to evaluate the endolymphatic space.ELH was observed in all patients. In three patients who underwent bilateral intratympanic injection of gadolinium and were diagnosed with contralateral DELH, ELH was observed bilaterally. In one of these three patients, ELH was observed in the cochlea on the left and in the vestibule on the right.

We report a 62-year-old woman presenting with nocardia brain abscess that mimics metastatic brain tumor. Six months before admission to our hospital, she presented with persistent hemoptysis, and a friable endobronchial mass was detected at another hospital. However, a definite diagnosis had not been made, and then she presented with hemianopia. Her laboratory analysis results showed that she had no immunological abnormalities. T1-weighted magnetic resonance images of the brain with Gd-DTPA revealed hyperintensive multiple masses with surrounding edema. We suspected this case as metastatic brain tumor of unknown origin, and the patient underwent biopsy of the supraclavicular lymph node. Histopathology results indicated abscess, and gram staining of material obtained from the abscess showed Gram-variable rods, which were determined upon culture as acid-fast Gram-positive branching rods and identified as Nocardia asteroides. Accordingly, we diagnosed the patient as having nocardia brain abscess, and she was treated with an oral antibiotic (TMP-SMX). Four months later, the abscesses disappeared. The patient recovered after prolonged TMP-SMX therapy with no recurrence in over 9 months.

Objectives/Hypothesis: Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) has recently been developed to detect high concentrations of protein or hemorrhage. We have previously reported that 50% of patients with sudden sensorineural hearing loss (SNHL) show high signals in the affected inner ear on 3D-FLAIR MRI. However, the relationship between 3D-FLAIR findings and hearing prognosis is unclear. Our objective was to evaluate the relationship between the results of 3D-FLAIR MRI at 3 Tesla and prognosis in sudden SNHL. Study Design and Methods: We used 3D-FLAIR at 3 Tesla with and without gadolinium enhancement to evaluate the pathologic conditions in the inner ears of 48 patients with sudden SNHL. Results: Thirty-one of 48 patients with sudden SNHL showed high signals in the affected inner ear on precontrast 3D-FLAIR. Hearing improvement in patients with high signals in the affected inner ear on precontrast 3D-FLAIR (25 ± 19 dB) was significantly worse than that in patients with no signal (45 ± 27 dB; P < .05). Our analysis suggests that high signals in the affected inner ear on precontrast 3D-FLAIR MRI is a new prognostic factor for sudden SNHL. Conclusions: 3D-FLAIR findings show that high signals in the cochlea on precontrast 3D-FLAIR are related to a poor hearing prognosis. These signals may reflect minor hemorrhage or an increased concentration of protein in the inner ear, which has passed through blood vessels with increased permeability or has originated in disrupted cells in the inner ear.

Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging (MRI) has recently been developed to detect hemorrhage or high concentrations of protein. We present a patient with Ramsay Hunt syndrome, in whom high signals in the cochlear and vestibular apparatus were identified with 3D-FLAIR. The high signal areas in 3D-FLAIR were not detected by T1- and T2-weighted MRI in this case. This is the first report of high concentrations of protein in the inner ear in Ramsay Hunt syndrome using 3D-FLAIR, and suggests that high concentrations of protein in the inner ear are associated with hearing deterioration in some patients with Ramsay Hunt syndrome. 3D-FLAIR could be a useful diagnostic tool in the early stages of Ramsay Hunt syndrome.

Objective: Recently, there have been many reports of 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. We attempted to visualize how the intratympanically administered drug enters the inner ear. Methods: Gadolinium hydrate diluted eightfold with saline was injected intratympanically through the tympanic membrane using a 23 G needle in nine patients with inner ear diseases. With a 3 Tesla magnetic resonance imaging (MRI) unit, three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) imaging was performed. Results: 3D-FLAIR MRI clearly revealed that the gadolinium entered the perilymphatic space and delineated the perilymphatic and endolymphatic spaces of the inner ear. In patients with endolymphatic hydrops, the perilymphatic space surrounding the endolymph was small or had disappeared. Gadolinium appeared first in the scala tympani of the basal turn of the cochlea and the perilymphatic space of the vestibule. One day after the intratympanic injection of gadolinium, the gadolinium was observed in almost all parts of the perilymph. Six days after the intratympanic injection, the gadolinium had almost disappeared from the inner ear. Conclusion: We reported the first visualization of endolymphatic hydrops in patients with Meniere's disease. The relationship between the image of the endolymphatic space and functional tests, such as electrocochleography and vestibular-evoked myogenic potential, must be examined in the near future. It is important for the development of intratympanic drug therapies for inner-ear diseases to investigate how the drugs enter and leave the inner ear.

Objective To evaluate the hypothesis that there are differences in the morphology of the lateral semicircular canal (LSCC) between patients with large vestibular aqueduct syndrome (LVAS) and control subjects and to investigate the clinical implications of these differences. Study design Retrospective case review. Setting Tertiary referral center. Patients Nine patients (two male patients and seven female patients; age range, 8-54 yr) with LVAS (one patient had unilateral LVAS, and eight patients had bilateral LVAS). Five patients had vertigo, and four patients, including the one with unilateral LVAS, did not have vertigo. Main outcome measures The area of the LSCC was traced on the magnetic resonance imaging console and compared between LVAS patients and 12 control subjects who did not have sensorineural hearing loss. The LSCC fluid-containing area was divided by the sum of the LSCC inner area and the LSCC fluid-containing area for evaluation of the degree of the LSCC dysplasia. Results The LSCC fluid-containing ratio was significantly larger in LVAS patients than in control subjects. Moreover, the LSCC fluid-containing ratio was significantly larger in the eight ears with vertigo than in the nine ears without vertigo. There was no relationship between hearing level and the LSCC fluid-containing ratio. Conclusion Patients with LVAS may have disturbed morphogenesis of both membranous and bony labyrinths. Our results reveal that the morphology of semicircular canals is clinically associated with vertigo.

Objective: To evaluate the inner ear of a patient with an enlarged vestibular aqueduct and acute hearing deterioration, using three-dimensional fluid-attenuated inversion recovery (3D FLAIR) magnetic resonance imaging (MRI).Background: Three-dimensional fluid-attenuated inversion recovery MRI has been recently developed to detect haemorrhage and high concentrations of protein. Application of this method to inner-ear diseases has not been fully described.Method: We used 3D FLAIR MRI to evaluate a patient with an enlarged vestibular aqueduct and acute hearing deterioration.Results: On 3D FLAIR MRI, this patient had high signals in the endolymphatic duct and sac, the vestibule and the cochlea and was diagnosed as having a high protein concentration in the inner ear. These high signal areas were not detected by T1- and T2-weighted MRI.Conclusion: This is the first published case in which a high protein concentration in the cochlea of a patient with an enlarged vestibular aqueduct was detected by 3D FLAIR MRI. These findings strongly supported the theory of the pathophysiology of hearing deterioration in patients with an enlarged vestibular aqueduct, which states that hyperosmolar fluid in the enlarged endolymphatic sac refluxes into the cochlea, resulting in damage to the hair cells. Thus, these finding may help clarify the cause of hearing deterioration in the presence of a large endolymphatic duct and sac.

A 33-year-old man had systemic lupus erythematosus (SLE) with anticardiolipin antibody. At the age of 40 years, he experienced bilateral hearing deterioration. The three-dimensional fluid-attenuated inversion recovery of magnetic resonance imaging showed high signals bilaterally in the cochlea and vestibule. He was diagnosed with bilateral inner ear hemorrhage on the basis of the presence of anticardiolipin antibody. This is the first published case of inner ear hemorrhage in a patient with SLE. Our findings suggest that inner ear hemorrhage is associated with hearing deterioration in some patients with SLE.

Objective: To investigate Schwartze sign with measurements of blood flow to the promontory in patients with cochlear otosclerosis. Design: Prospective clinical study. Setting: Tertiary referral centre. Participants: Five patients with cochlear otosclerosis and five control subjects. Significant decalcification around the cochlea was observed by computed tomography (CT) in patients with cochlear otosclerosis. However, no recognizable lesion was observed at the oval window in two patients. One patient had mixed hearing loss and four patients had sensorineural hearing loss without an air–bone gap. Main outcome measures: The relationship between CT findings and the presence or absence of Schwartze sign was investigated. Blood flow to the promontory was measured through the tympanic membrane using laser speckle flowgraphy and laser Doppler flowmetry. Results: The Schwartze sign correlated significantly with otosclerotic lesions invading the promontory. Patients with otosclerosis exhibited elevated and pulsating blood flow to the promontory with the Schwartze sign. Conclusions: Computed tomography demonstrated that cochlear otosclerosis can exist without the oval window lesion. Schwartze sign can be used as a sign of the otosclerotic invasion to the promontory. The reddening of the Schwartze sign is likely due to increased blood flow.

A 6-year-old boy suffered acute profound right side deafness after his classmates had mumps. Although his salivary glands were not swollen, he had high levels of anti-mumps IgM and IgG antibodies. The three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) procedure applied to magnetic resonance imaging (MRI) showed high signals in the right cochlea and vestibule. This indicated hemorrhage or a high concentration of protein in the right inner ear. This is the first case demonstrating a high 3D-FLAIR MRI signal of the inner ear in a patient with mumps deafness. Our findings suggest that 3D-FLAIR MRI may help to identify and define labyrinthitis in mumps deafness.

This study was conducted to evaluate the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss and in control subjects.Retrospective case review.A tertiary referral center.We evaluated 366 patients (165 men and 201 women; age range, 3-91 yr) with sudden sensorineural hearing loss and 228 control subjects without sensorineural hearing loss using magnetic resonance imaging. Three hundred fifty-six patients had unilateral and 10 patients had bilateral sudden sensorineural hearing loss.Eleven (2.9%) of 376 ears with sudden sensorineural hearing loss had inner ear anomaly. Nine patients (2.5%) had inner ear anomaly associated with sudden sensorineural hearing loss, but none of the 228 control subjects had the anomaly. The current study demonstrated that the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss was significantly higher than in control subjects.Our study reveals that inner ear anomaly may be associated with sudden sensorineural hearing loss in 2.5% of patients.

Objective To compare clinicopathologic findings in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis-associated neuropathy (MPAN). Methods Patients clinicopathologically confirmed to have NSVN ( n = 23) or MPAN ( n = 40) were compared with respect to clinical, electrophysiologic, and histopathologic features. Results Clinical features of neuropathy such as initial symptoms, progression, and distribution of sensory and motor deficits were similar in both groups, while functional compromise was greater in MPAN than NSVN. Abnormalities of laboratory data including those reflecting severity and extent of inflammation such as C-reactive protein were more conspicuous in MPAN than NSVN. Perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were positive in two-thirds of patients with MPAN but negative in all NSVN. Electrophysiologic and histopathologic findings indicated axonal neuropathy in both groups, whereas the reduction of compound muscle action potentials in the tibial nerve and sensory nerve action potentials in the median nerve was significantly more profound in MPAN than NSVN. As for the epineurial perivascular infiltration, frequencies of cell-specific markers for T lymphocytes, macrophages, and B lymphocytes among cells infiltrating the vasculitic lesions were essentially similar between groups. Conclusions Clinicopathologic profiles and vascular pathology were similar between NSVN and MPAN but the age at onset, severity, and presence of p-ANCA were clearly different. Further study is needed to clarify the pathogenesis of NSVN and its place in the vasculitic spectrum of diseases.

Fourteen patients with a Pendred syndrome gene (Pds) mutation and three patients without the mutation were studied to evaluate long-term vestibular and auditory manifestations among patients with bilateral enlarged vestibular aqueducts (EVA). Charts were reviewed retrospectively for age, gender, otological history, presence or absence of vertigo, results of magnetic resonance imaging, relevant gene mutations and perchlorate discharge test. A missense mutation, His 723 Arg (H723R), was identified in the homozygous state in six patients and in the heterozygous state in seven patients. Another missense mutation, Tyr 410 Met (T410 M), was identified in the heterozygous state in one patient. Patients with vertigo tended to have hearing fluctuation, compared with the patients without vertigo. Patients homozygous for H723R tended to have more episodes of vertigo than the heterozygous individuals. Only one patient who was homozygous for H723R had goiter. The long-term outcome of hearing level in patients with the H723R mutation was significantly better compared with published results for patients with a Pds mutation, but not for those with the H723R mutation. Whether environmental factors or a subtype of the Pds mutation H723R are related to the long-term outcome for these patients must be clarified.

The etiology of acute low-tone sensorineural hearing loss (ALHL) may be associated with deteriorated function of the endolymphatic sac. Using magnetic resonance (MR) imaging on a 1.5-tesla MR system, we evaluated the frequency of visualization and contrast enhancement of the endolymphatic sac in 25 patients (9 men and 16 women; age range 11–64 years) with ALHL and 21 control subjects. The present study demonstrated that the frequency of visualization and enhancement of the endolymphatic sac in patients with ALHL was not significantly higher than in control subjects.

A 7-year-old boy with a history of purulent meningitis and watery rhinorrhea was studied using computed tomography (CT) and magnetic resonance imaging (MRI). He had a common cavity in the left inner ear. With high-resolution heavily T2-weighted MRI, leakage of the inner ear fluid into the middle ear at the oval window area through a congenital perilymphatic fistula could be visualized. Surgery to close the fistula showed a perforation in the stapes footplate.

A 41-year-old Japanese-Brazilian man, living in Japan since 1991, visited our hospital in August 1998 complaining of a scaly annular erythema which had been present on his right forearm since 1996. Granulomatous inflammation was revealed in the dermis upon skin biopsy. Sclerotic cells were present within the granulomatous lesions. Fonsecaea pedrosoi was isolated from tissue cultures. The restriction fragment length polymorphism pattern of mitochondrial DNA of the causative fungus was compatible with F. pedrosoi type 1, the commonest type in Japan. Since type 4 is usually found in South America, the patient was assumed to have become infected in Japan. An oral dose of terbinafine at 125 mg/day was ineffective. 125 mg/day single dose of terbinafine reaches an average plasma concentration of only 0.69 g/ml, where as 250 mg/day reaches 0.96 micro g/ml. Because the minimum inhibitory concentration (MIC) of terbinafine against the isolated fungus was found to be 0.76 micro g/ml, the terbinafine dose was increased from 125 mg/day to 250 mg/day, which slowly led to remission. Chromomycosis is caused by several species of dematiaceous fungi, and terbinafine efficacy may vary depending on the causative fungus. MIC values may differ even within the same species. It is important to isolate the pathogenic fungus in each case and measure the MIC value to determine the optimal dose of terbinafine.

The relaxation of aortic rings in response to acetylcholine (ACh) was significantly decreased in cholesterol-fed mice. The attenuated relaxation in cholesterol-fed mice was preserved by the chronic administration of prazosin (20 mg/kg/day) or pravastatin (12.5 mg/kg/day). Serum low-density lipoprotein (LDL) levels were significantly increased in mice given cholesterol. The increased serum LDL levels in cholesterol-fed mice were returned to normal by the chronic administration of prazosin and pravastatin. A prior incubation of aortic rings with lysophosphatidylcholine (LPC) significantly attenuated ACh- and A23187-induced endothelium-dependent relaxation. The inhibitory effects of LPC on endothelium-dependent relaxation were not affected by indomethacin or superoxide dismutase. The sodium nitroprusside-induced relaxation of aortic rings was not changed by LPC. The inhibitory effects on ACh-induced relaxation by NG-monomethyl- L -arginine were restored by a prior exposure to L-arginine, whereas the inhibition of endothelium-dependent relaxation by LPC was not affected by L -arginine. These results suggest that cholesterol-fed mice are useful animal models of hypercholesterolemia, and chronic administration of prazosin or pravastatin can preserve endothelium-dependent relaxation by lowering serum LDL in these animals. It is further suggested that LPC derived from oxidized LDL may be involved in the reduced endothelium-dependent relaxation in hyperlipidemia.

The duct-like structures present in 7 cases of benign and malignant eccrine poroma were examined by immunohistochemical staining for carcinoembryonic antigen (CEA), involucrin, and S-100 protein. The demonstration of CEA and involucrin was helpful in the recognition of these structures. The overt immunopositivity precedes morphological evidence for duct formation. On the basis of the CEA immunostaining, the duct-like structures were divided into 4 types: 1) mature acrosyringeal structure, 2) cystic luminal structure lined by elongated cells, 3) immature acrosyringeal structure, and 4) vacuole- or dot-like potential lumen in a single cell. Involucrine was observed in the lining cells of 1) and 2). None of the 4 types showed positive reactivity for S-100 protein, suggesting the irrelevance of these structures to the secretory element of sweat gland. The polymorphism of the ductal formation tended to be more remarkably observed in malignant eccrine poromas than in the cases of benign eccrine poroma and poroepithelioma tested.

The authors obtained the following results in an experiment of 30 lateral head impacts applied to 10 monkeys. 1. Lateral impact tolerance which is an index of concussion was estimated to be about twice as high as sagittal impact tolerance, but more experimental cases in the future will be required to decide the proper tolerance level. 2. Concussions had three types of effects on the cardiopulmonary system, and significant mutual correlations and correlation with concussion severity were shown. 3. It is characteristic of lateral impact that, although the tolerance level appears to be high, there are diverse and characteristic pathological changes, and it is assumed that contusions such as gliding contusion and Zentrale Hirnschaden (or intermediary contusions) are apt to occur in cases of lateral impact.

The authors obtained the following results in this experiment in which monkeys were subjected to frontal and occipital blows with their heads in an unrestrained condition. 1) With respect to the impact to the head and the occurrence of concussion, translational acceleration is more important than rotational acceleration. 2) The concussion threshold of monkeys was an equal speed curve of 16 m/s for occipital blows, while in the case of frontal blows, a hyperbolic curve which is somewhat higher than that for occipital blows was obtained. This concussion tolerance curve was converted into the concussion tolerance curve for humans by means of dimensional analysis. 3) A close correlation was suggested between changes in the EEG amplitude and the severity of the concussion. The more severe the concussion, the greater the decrease in the EEG amplitude. 4) The concussions could be classified into three types pathologically. The first type showed capillary dilatation and vascular permeability. The second type showed hemorrhages at sites far from the cerebral cortex and the third type showed hemorrhages in the cerebral cortex. The second and third types were in parallel with the severity of the concussion.