Recently, we experienced a case of myotonia dystrophy at Pediatric department of Yonsei Medical School. And we are presenting the case we experienced with reviewing the literatures.
Myotonia* ; Schools, Medical

BACKGROUND AND OBJECTIVES: Inner hair cells (IHCs) of the organ of Corti change the external sound stimulus into the electrical signal and transmit this signal to the auditory cortex through afferent nerve fibers. Outer hair cells (OHCs) control the sound transmission function of IHC. OHCs respond with a somatic shape change to alterations in their membrane potential and this electromotile response is believed to provide mechanical feedback to the basilar membrane. Efferent nerve fibers which arise from the superior olivary nucleus in the midbrain and transmit to OHCs through medial olivocochlear bundle use acetylcholine (ACh) as a neurotransmitter. The cholinergic response of OHCs' alpha-9 nicotinic ACh receptor increase the Ca2+ influx, which control OHCs' electromotility by changing a membrane potential. In this research, the effect of ACh on the K+ current in OHC of guinea pig was studied, and the change of OHC length by ACh was studied. MATERIALS AND METHODS: Using the extracted OHC from a guinea pig potassium currents induced by ACh were recorded using the whole-cell patch clamp technique. The change of OHC length when ACh was applied was observed. RESULTS: 1) ACh increases voltage-dependent K+ current in OHC. 2) In the condition, which Ca2+-dependent K+ current is blocked by removing Ca2+ from intra-cellular fluid, ACh has no effect on K+ current in OHC. 3) ACh increases OHC length. CONCLUSION: These experimental results show that ACh from the medial olivocochlear efferent system regulates mobility of OHC, increases the Ca2+-dependent K+ currents in OHC.
Acetylcholine* ; Animals ; Auditory Cortex ; Basilar Membrane ; Calcium ; Guinea Pigs ; Hair* ; Membrane Potentials ; Mesencephalon ; Nerve Fibers ; Neurotransmitter Agents ; Olivary Nucleus ; Organ of Corti ; Potassium Channels ; Potassium*