Familial pulmonary stenosis and deaf-mutism: Clinical and genetic considerations

Inherited diseases of the inner ear in man in the light of studies on the mouse.

“…Lewis et al (1958) have described a family in which the mother and 3 of the 5 children were affected.…”

Understanding Hereditary Nerve Deafness

“…Dementia slowly in¬ creases as the child develops massive myo¬ clonic jerks of the upper body.16,25 Deafness in association with myoclonic epilepsy is described by Latham The first attack of syncope occurs in early childhood, and is probably an AdamsStokes type of cardiac standstill. 48 The afflicted usually die in childhood, but post¬ mortem examination shows no gross abnor¬ malities of the heart. Electrocardiograms show marked prolongation of the Q-T inter¬ val, and large waves, but there is no clinical or x-ray evidence of organic heart disease.…”

Cardio-auditory syndromes. Cardiac and genetic study of 511 deaf-mute children.

“…In this connexion, it is interesting to consider the family reported first by Lewis et al (1958) and Koroxenidis et al (1966). Association of congenital heart disease with deaf-mutism in its members could be coincidental, but the possibility exists that it represents a second (Lewis) cardio-auditory syndrome, related again to cardiomyopathies, since one affected member presented subaortic obstruction plus infundibular stenosis as well as atrial septal defect.…”