Diagnostic Imaging of Congenital Malformations of the Inner Ear
〜These malformations are not so rare〜

Kazutoshi Fujita
Department of Radiology, Kanagawa Children's Medical Center

Abstract
　Most causes of congenital sensorineural hearing loss are membranous malformations in which the bony architecture of the inner ear is normal. About 20% of patients with congenital sensorineural hearing loss have bony inner ear malformations, which are recognized by radiological imaging. The classification of bony inner ear malformations proposed by Jackler et al in 1987 has been accepted and widely used. According to the development of radiological imaging modalities and cochlear implantations, Sennalogu et al proposed a new classification for inner ear malformations in 2002. This paper summarizes the differences of each classification and outlines of the new classification. Although the new classification did not include the large vestibular aqueduct syndrome or semicircular canal dysplasia, these abnormalities are also explained in this paper.

Keywords : Diagnostic imaging, Inner ear, Congenital abnormalities