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Possibility of Epithelial Sodium Channelopathy as a Pathogenesis of Meniere's Disease
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Shi Nae Park, Byung Hoon Lee, Kyung Ho Park, Myoung Hwa Hong, Heung Youp Lee, Ki Hong Chang, Sang Won Yeo
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J Korean Bal Soc. 2006;5(1):9-14.
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 Abstract
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- 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. α-ENaC 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 α-ENaC gene in normal controls and the patients with Meniere's disease.
Conclusion
Na channelopathy due to α-ENaC 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.
Key Words : Meniere's disease, Channelopathy.
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Characteristics of the Extratympanic Electrocochleography in the Patients with Meniere's Disease
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Shi Nae Park, Hyun Jin Oh, Kyung Ho Park, Sang Won Yeo
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J Korean Bal Soc. 2004;3(2):367-371.
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Abstract
PDF
- Background
and Objectives : Electrocochleography is the only proven investigation and diagnostic tool to demonstrate the endolymphatic hydrops at this time. We designed this study to assess the characteristics of transtympanic electrocochleographic findings and to estimate the clinical value of the test results in the patients with Meniere's disease.
Materials and Method : Electrocochleographic duration and amplitude were measured in a clinically defined definite Meniere's disease group and the results were compared with those from a normal hearing control group.
Results
: The summating potential (SP) / action potential (AP) amplitude ratio was significantly different among study groups with 0.40±0.10 in the bilateral Meniere's group, 0.34±0.08 in the unilateral Meneire's group and 0.22±0.08 in the normal group (p<0.01). The elevated SP/AP ratio in the patients with such a relatively long duration of Meniere's disease in this study was mainly due to the decreased AP amplitude rather than the enhanced SP amplitude.
Conclusion
: Measuring electrocochleography and demonstrating the elevated SP/AP ratio could be quite valuable to diagnose the patients with Meniere's disease. The recognition of decreased AP amplitude might be helpful to understand the cochlear pathologic status in the patients with Meniere's disease.
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KBS
