Potter's syndrome: a temporal bone histopathological study

Histopathological findings in seven temporal bones from four infants diagnosed as Potter's syndrome are described. The infants were labelled as Potter's syndrome after autopsy confirmed bilateral renal agenesis. Extrarenal manifestations included pulmonary hypoplasia and facial anomalies. The middle ear volume in infants with Potter's syndrome remained unchanged with age resulting in a significantly higher percentage of residual middle ear mesenchyme in these infants as compared to normal infants. Dehiscence of the facial nerve was seen in all the temporal bones studied. One ear showed the presence of eosinophilic effusion in the endolymphatic sac, an underdeveloped malleus and a wide facial nerve canal. The inner ear structures showed no significant anomalies except for the absence of the organ of Corti in the basal turn of the cochlea in one of the ears.