Labyrinthine Malformations Genetical and Radiological Aspects

Abstract

A family is described, three members of which presented progressive deafness, of mixed type, not present at birth. The two living, mother and daughter, exhibited a Mondini-like malformation of the labyrinth. The canaliculus cochleae was dilated in the daughter. In addition, a middle ear malformation was found in the mother. The findings, being unique, are discussed in the light of Brown-Farrior og Endicott's paper on progressive, non-congenital deafness, attributed to abnormally wide canaliculus cochlea.