Any child who has unexplained congenital sensorineural hearing loss should be suspected of having an inner ear anomaly and other organ system anomalies evaluated. In recent years, high resolution computed tomograms are helpful in identifying inner ear anomalies, especially during the first trimester 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.