Dominant Hereditary Conductive Deafness Through Lack of Incus-Stapes Junction

Abstract

This report concerns an extensive family research in which a regular dominant conductive deafness can be traced in four generations. The middle-ear operation of four ears on three symptom-carriers showed a missing connection of the incudo-stapedial joint. Reference is made to the fundamental research work of Anson, Bast and collaborators, regarding the differentiated evolution of the middle-ear ossicles and the incudo-stapedial function. In this small malformation of the middle-ear ossicles it must be assumed that in affected members a dominant genetic factor prevents in the sixth to the seventh fetal week the rotation and the joint of the distal end of the incus with the blastema of the stapes, the result is an inborn conductive deafness.