This group of cases was first presented in an article1 which we published in December, 1954, reporting observations made in 1953. The 4 cases, a mother and 3 children, exhibited what was thought to be hereditary deafness due to a simple Mendelian dominant trait.
Seven years later, in February, 1960, we were fortunate in having an opportunity to examine again the 4 cases involved and to obtain this follow-up information. The deafness is still considered to be hereditary in origin. It is definitely perceptive in type. It is a question as to whether it is progressive.
Prof. Paul Manasse of Strassburg published several articles between the years 1903 and 1907. These dealt with the pathology of the labyrinth and the auditory nerve. In the paper on "Chronic Progressive Labyrinthine Deafness,"2 which relates to our cases, he described the pathological changes in acquired labyrinthine deafness and then compared those
WILLIAMS F, ROBLEE LA. Hereditary Nerve DeafnessA Follow-Up of Four Cases in One Family. Arch Otolaryngol. 1962;75(1):69–77. doi:10.1001/archotol.1962.00740040073007