Anomalies of the auditory organ in Potter's syndrome. Histopathological findings in the temporal bone

Abstract

Histopathological findings in the temporal bone are described in a newborn infant, diagnosed as having Potter's syndrome. The infant has severely malformed low-set ears bilaterally and a small lower jaw; autopsy findings showed bilateral renal agenesis and pulmonary hypoplasia. The temporal bone indicated the deformities of the inner ear, classified as Mondini-type, complicated by extensive deformities to the external ear and middle ear, including absence of auditory ossicles, atresia of the oval window, abnormal course of the facial nerve, and hypoplastic external auditory canal. The cochlear membranous labyrinth showed nearly normal form in the upper turn, but severe hypoplasia in the basal turn, which was an unusual cochlear anomaly.