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KMID : 0387519940040010203
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Journal of Maryknoll Hospital
1994 Volume.4 No. 1 p.203 ~ p.210
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A Case of Mondini Dysplasia associated with Situs Inversus
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강준형
김영훈/김기철/조중환
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Abstract
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Any child who has unexplained congenital sensorineural hearing loss should be suspected of having an inner ear anomaly and evaluated other organ system anomalies.
In recent years, high resolution computed tomograms are helpful in identifying ear anomalies, especially first trimester inner ear malformation and inner ear malformations have been demonstrated by high resolution computed tomograms in 8% to 20%
of
children with sensorineural hearing loss.
The syndromes which show a high incidence of Mondini dysplasia are Klippel-Feil syndrome, Pendred's syndrome, trisomy syndrome, Digeorge's syndrome. It may also occur in association with other anomalies not yet recognized as specific syndromes.
The authors experienced a case of Mondini dysplasia with residual lower frequency hearing associated with situs inversus that there was no functional defects.
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