A Case of Congenital Inner Ear Malformation

Abstract

We report a case of inner ear malformation (Michel dysplasia) associated with left total deafness in an 8-year-old boy. He had normal vestibular function and right normal hearing. The dysplasia of the left temporal bone and inner ear was revealed by a radiological study, especially computed tomography. A left cochlear aplasia, and dysplasia of left vestibule and semicircular canals which made a vacant cavity, and a narrowed internal auditory canal were demonstrated.
Our case could n o t be classified according to the reported radiological and pathological classifications. We would like to advocate that inner ear malformations should be classified based on anatomical constitution determined by modern radiologic techniques, rather than denoting them with eponym.
It should beem phasized that, there is a poor correlation between the osseous radiographic changes and vestibular function of the severe case of aplasia or dysplasia in cochlea, vestible and semicircular canals. Therefore, we considered that there is little significance clinically in making a detailed classification.