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KMID : 0391520160240030174
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Journal of the Korean Child Neurology Society
2016 Volume.24 No. 3 p.174 ~ p.177
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Monozygotic Twin Cases of Unilateral Sensorineural Hearing loss : Elderly One Accompanied with Internal Auditory Canal Stenosis and Facial Paralysis
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Suh Kyoo-Hyun
Son Su-Min
Kim Sae-Yoon
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Abstract
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We introduce the case of monozygotic twins with sensorineural hearing loss (SNHL). And one of them had congenital facial paralysis (CFP) accompanied by SNHL and internal auditory canal (IAC) stenosis. Most cases of IAC stenosis are accompanied by SNHL, but the patients in our cases showed CFP also. We confirmed the agenesis of facial and vestibulococchlear nerves with the T2-weighted constructive interference in steady-state magnetic resonance imaging (CISS MRI) scans. We should consider a variety causes including genetic problems or trauma. Although the mother did not have a specific trauma history, she may have prolonged exposure to traumatic events during the first trimester of pregnancy. Clinicians who examine the SNHL, should pay attention to the history, physical and neurologic examination, and audiologic and imaging findings in a patient with or without facial nerve paralysis
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KEYWORD
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Sensorineural hearing loss, Monozygotic twins, Facial paralysis, Magnetic resonance imaging
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