BACKGROUND AND OBJECTIVES: Meniere's disease is a paroxysmal disorder with vertigo and hearing loss. Its episodic nature of vertigo attacks and responsiveness to diuretics is similar to several neurologic disorders with channelopathy. We performed this study to identify the possibility sodium channelopathy in the patients with Meniere's disease. MATERIALS AND METHOD: Thirty patients with definite Meniere's disease and 25 normal controls were included in this study. Clinical features of the patients with Meniere's disease were collected using dizziness chart. Whole blood was taken from all the patients with Meniere's disease and normal controls and used for DNA testing. alphaENaC gene was screened for mutations using direct DNA sequencing. RESULTS: Patients with Meniere's disease in this study showed the various clinical features of onset age, number of attacks and hearing levels. Many of them showed the caloric response reduction and abnormal electrocochleogram. Genetic analysis did not identify any mutations or differences in alphaENaC gene in normal controls and the patients with Meniere's disease. CONCLUSION: Na channelopathy due to alphaENaC gene mutation might not be a pathogenesis of Meniere's disease. Further studies with other subunits of ENaC and other ion channel genes are needed to explore the possibility of channelopathy as a pathogenesis of Meniere's disease.
Age of Onset ; Channelopathies* ; Diuretics ; Dizziness ; DNA ; Hearing ; Hearing Loss ; Humans ; Ion Channels ; Meniere Disease* ; Nervous System Diseases ; Sequence Analysis, DNA ; Sodium* ; Vertigo

BACKGROUND AND OBJECTIVES: Meniere's disease is a paroxysmal disorder with vertigo and hearing loss. Its episodic nature of vertigo attacks and responsiveness to diuretics is similar to several neurologic disorders with channelopathy. We performed this study to identify the possibility sodium channelopathy in the patients with Meniere's disease. MATERIALS AND METHOD: Thirty patients with definite Meniere's disease and 25 normal controls were included in this study. Clinical features of the patients with Meniere's disease were collected using dizziness chart. Whole blood was taken from all the patients with Meniere's disease and normal controls and used for DNA testing. alphaENaC gene was screened for mutations using direct DNA sequencing. RESULTS: Patients with Meniere's disease in this study showed the various clinical features of onset age, number of attacks and hearing levels. Many of them showed the caloric response reduction and abnormal electrocochleogram. Genetic analysis did not identify any mutations or differences in alphaENaC gene in normal controls and the patients with Meniere's disease. CONCLUSION: Na channelopathy due to alphaENaC gene mutation might not be a pathogenesis of Meniere's disease. Further studies with other subunits of ENaC and other ion channel genes are needed to explore the possibility of channelopathy as a pathogenesis of Meniere's disease.
Age of Onset ; Channelopathies* ; Diuretics ; Dizziness ; DNA ; Hearing ; Hearing Loss ; Humans ; Ion Channels ; Meniere Disease* ; Nervous System Diseases ; Sequence Analysis, DNA ; Sodium* ; Vertigo

BACKGROUND AND OBJECTIVES: Mast cells are the main effector cells in the immediate phase of the allergic responses. To evaluate the role of mast cells on the development of allergen-induced eosinophilic inflammation in a murine model of allergic rhinitis(AR), we compared the responses of mast cell deficient (W/Wv) mice with congenial normal (W/W+) mice. MATERIALS AND METHOD: Mice were sensitized intranasally with an extract of Aspergillus fumigatue (Af) for 3 weeks, and challenged intranasally with a single dose (acute model) or single dose every day for 5 consecutive days (chronic model). Interleukin (IL)-5 in nasal lavage fluids (NLF), histologic examination and Af-specific immunoglobulins (Ig) were examined in W/Wv mice and W/W+mice. RESULTS: In the sensitized W/Wv mice, the number of eosinophils in the nasal mucosa and IL-5 in NLF after repeated allergen challenge were not significantly different from those of W/W+ mice. In contrast, the number of eosinophils in the nasal mucosa and IL-5 in NLF after single allergen challenge were partially attenuated in W/Wv mice. The levels of Af-specific IgE and IgG1 in both acute and chronic models were not different between W/Wv mice and W/W+ mice. CONCLUSION: These findings suggest that eosinophilic inflammation caused by repeated allergen challenge are independent of mast cells in this AR model.
Animals ; Aspergillus* ; Eosinophils ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulins ; Inflammation ; Interleukin-5 ; Interleukins ; Mast Cells* ; Mice ; Models, Animal ; Nasal Lavage Fluid ; Nasal Mucosa ; Rhinitis*

No abstract available.
Diagnosis*

A congenital nasolacrimal duct cyst is a very rare cause of respiratory distress and feeding difficulty in the newborn who is an obligate nasal breather. A case of bilateral nasolacrimal duct cysts (NLDC) without an associated congenital dacryocele was first reported in 1993. The authors present a case of a newborn with bilateral NLDC with intranasal extension causing respiratory distress, but with no evidence of dacrocystitis. Diagnostic studies included intranasal endoscopy and CT scanning of the nasolacrimal system and nose. The patient was treated by endoscopic marsupialization of the cysts. After six months of treatment, there was no evidence of recurrence.
Dacryocystitis* ; Endoscopy ; Humans ; Infant, Newborn ; Lacrimal Duct Obstruction ; Nasolacrimal Duct* ; Nose ; Recurrence ; Respiratory Insufficiency ; Tomography, X-Ray Computed

BACKGROUND AND OBJECTIVES: We are developing an in vitro preparation of the mouse inner ear so as to study morphophysiologic character of primary vestibular afferents and synaptic transmission within the vestibular epithelium. MATERIALS AND METHOD: We have intra-axonally recorded from over 300 ampullary fibers, close to the base of their respective anterior and lateral crista (<500 micrometer from hair cell/afferent nerve synapse), and labelled as a sub-set of these with biocytin (n=71). Discharge activity can be classified as regular or irregular based on the variation of the interspike interval (coefficient of variation). Using a micropusher to indent exposed windows of membranous labyrinth, we have characterized the response properties of both anterior and horizontal canal afferents. We studied afferent activity in response to sinusoidal indentations of anterior and horizontal membranous canal. RESULTS: The majority of labelled units were dimorphic (56 out of 71), having both calyx and bouton terminals and there was no labelled bouton terminal. Whether action potentials (Aps) were spontaneous or elicited with current, a heterogeneity of discharge activity was observed and these were similar to those previously reported in in vivo recordings from other mammalian species. In recordings over a range of frequencies from 0.01 to 10.0Hz, afferents responded with sinusoidal changes at discharge rates and modulation of membrane potential in a predictable manner. The phase response of the afferent discharge was characterized by frequency-dependent shifts in peak activity. The peak activity of anterior canal was in advance of the maximum indentation (180dgrees out of phase), with largest phase leads at 0.01 Hz (59.2+/-14.1dgrees) and the smallest phase leads occurring at 1.0 Hz (13.4+/-9.3dgrees), while maximum indentation was in advance of the peak activity at 10.0 Hz (-17.6+/-9.1dgrees). These phase shifts were similar to those reported in in vivo recordings from mammals, despite our use of artificial rather than natural rotational stimuli. CONCLUSION: We developed an in-vitro mouse model to study morphophysiologic characteristics of primary vestibular afferent nerve and synaptic transmission.
Action Potentials ; Animals ; Ear, Inner* ; Electrophysiology ; Epithelium ; Hair ; Mammals ; Membrane Potentials ; Mice* ; Population Characteristics ; Semicircular Canals ; Synaptic Transmission

BACKGROUND AND OBJECTIVES: The effect of electrical stimulation (ES) on vestibular compensation was investigated for 28 days after unilateral labyrinthectomy (Lx) in 24 rats. MATERIALS AND METHOD: The rats were divided into 4 groups and were treated as follows: A) Lx only, B) Lx with ES (0.6 mA) for 2 days, C) Lx with ES (0.6 mA) for 7 days, D) Lx with ES (1.2 mA) for 2 days. ES with pulse wave (10 Hz) was applied to temporal portion bilaterally (8 hr/day). Cathodal currents were transmitted to the electrode on the destructive side, anodal to that on the intact side. Postoperatively, spontaneous nystagmus (SN) was recorded with the video camera and yaw and roll head tilt (YHT, RHT) were measured using photo images. Horizontal vestibulo-ocular reflex (VOR) was evaluated at various frequencies (0.05, 0.1, 0.5, 1, 2, 3 Hz with peak angular velocity of 40degrees/sec) with the magnetic search coil system. Locomotor movement was monitored with a video camera. RESULTS: The ES groups showed faster compensation in SN, YHT, and RHT than the Lx group. At 3 days after Lx, the ES groups showed significant improvement in the gain over the whole frequency and asymmetry at low frequency compared to that of the Lx group. But the ES group had no effect on compensation of gain and asymmetry on and after 7 days of post-labyrinthectomy. There was no significant difference in vestibular compensation with respect to duration and intensity of ES. ES had no significant effect on the mean velocity and mean deviation of locomotor movements. CONCLUSION: These results suggest that ES has a favorable effect on the suppression of early static symptoms but has no effect on the compensation of dynamic symptoms after the recovery of resting discharge of the vestibular nuclei neurons.
Animals ; Compensation and Redress* ; Ear, Inner ; Electric Stimulation* ; Electrodes ; Head ; Neurons ; Rats* ; Reflex, Vestibulo-Ocular ; Vestibular Nuclei

Small cell neuroendocrine carcinomas of the sinonasal tract are extremely uncommon tumors, and they have been recognized as a highly aggressive neoplasm with poor prognosis. The authors experienced a case of small cell neuroendocrine carcinoma of nasal cavity in a 72-year old male patient. Histopathologic findings showed a high nucleo/cytoplasmic ratio, and the nuclei were round or oval and hyperchromatic. Immunohistochemical study showed small cell neuroendocrine carcinoma. The chest X-ray and CT scan of chest showed no evidence of primary lung lesion. The patient received chemotherapy, but died from cerebral infarction and its complication. We report our case with a brief review of the literatures.
Aged ; Carcinoma, Neuroendocrine* ; Cerebral Infarction ; Drug Therapy ; Humans ; Lung ; Male ; Nasal Cavity* ; Prognosis ; Thorax ; Tomography, X-Ray Computed

BACKGROUND AND OBJECTIVES: This study was designed to investigate the influence of human leukocyte antigen (HLA) on genetic susceptibility to Meniere's disease and to evaluate the correlation between HLA genotypes and results of examination for various clinical factors of Meniere's disease. MATERIALS AND METHOD: The distribution of HLA class I was examined in 39 patients with MD and 199 healthy controls. For HLA-A and B, the serologic typing was performed according to a standard microlymphocytotoxicity technique. The HLA-C typing was performed by the ARMS-PCR method at DNA level. RESULTS: The frequencies of HLA-CwX0303 (RR=, p<0.02), and CwX0602 (RR=, p<0.03) were significantly increased in patients with Menieres disease, when compared to the controls. However, HLA-B44 (RR=, p<0.004) and CwX0102 (RR=, p<0.03) were significantly decreased in the patients compared to the controls. When an association between hearing level and the presence of HLA alleles was evaluated, the frequencies of HLA-B13 (RR=, p<0.004), CwX0303 (RR=5, p<0.02) and CwX0602 (RR=5, p<0.02) were significantly increased and the frequencies of B44 (RR=1, p*lt;0.02) and CwX0102 (RR=1, p<0.03) were significantly decreased in patients with the state of mild to profound hearing losses, compared to the controls. HLA-B13 showed a different distribution pattern between patients with and without hearing losses. CONCLUSION: These results suggest that some HLA alleles may be useful genetic markers in conferring the susceptibility to Meniere's disease and in implying a prognosis in Korean patients.
Alleles ; DNA ; Genetic Markers ; Genetic Predisposition to Disease ; Genotype ; Hearing ; Hearing Loss ; HLA-A Antigens ; HLA-B13 Antigen ; HLA-B44 Antigen ; HLA-C Antigens ; Humans ; Leukocytes ; Meniere Disease* ; Prognosis

Septic inflammation and thrombosis of the cavernous sinus was extremely fatal in the preantibiotic era. After the introduction of antibiotics, the incidence of septic cavernous thrombophlebitis has diminished significantly. It is, however, still a serious disease accompanied by a high morbidity and mortality rate. Therefore, a high index of suspicion and aggressive initiation of treatment is of the utmost importance for the prognosis. A case of cavernous sinus thrombophlebitis and abducence nerve palsy which takes place secondary to petrositis was diagnosed by magnetic resonance imaging (MRI), and is presented here. The resolution of septic thrombus and complete recovery of sixth (abducens) nerve palsy was observed with treatment with antibiotics and steroids accompanied by tympanotomy tube placement.
Anti-Bacterial Agents ; Cavernous Sinus Thrombosis* ; Cavernous Sinus* ; Incidence ; Inflammation ; Magnetic Resonance Imaging ; Mortality ; Paralysis* ; Petrositis* ; Prognosis ; Steroids ; Thrombophlebitis ; Thrombosis