내이도협착 및 전정계이형성을 동반한 와우무형성 1예

Congenital anomalies of the inner ear are rare disease and the majority of them are difficult to diagnose during one’s life time because they are malformations limited to the membranous labyrinth. However, inner ear anomalies involving bony labyrinth can be detected in life time through radiogaphic imaging and it has been known to occupy 8-20% of patients with congenital sensorineural hearing loss. We experienced a rare case of inner ear anomaly, in which the cochlea was not formed, the lateral semicircular canal was not developed, the vestibule was dilated and the internal auditory canal was narrowed. (J Clinical Otolaryngol 2002;13:122-126)