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July 1969

Familial Progressive Sensorineural Deafness

Author Affiliations

From the Department of Otolaryngology, University of Minnesota Medical School, Minneapolis.

Arch Otolaryngol. 1969;90(1):44-51. doi:10.1001/archotol.1969.00770030046010

DEAFNESS resulting from genetic causes may be present at time of birth (congenital) or may develop subsequent to birth (acquired). In congenital deafness the end-or-gan of hearing may not develop in the first trimester (aplasia). It is also conceivable, but not proven, that a fully differentiated sense organ may degenerate during the second and third trimester of pregnancy. The aplasias are generally accepted as being congenital and nonprogressive. Genetic deafness may first become apparent during infancy, childhood, or early adult life and the deafness may be considered acquired although the genetic deficiency is obviously not.1

Goodhill2 has divided hereditary deafness occurring after birth into infantile and adult types. According to the age of onset of genetic deafness (heredodegenerative) Cawthorne3 believed there is a continuous distribution, beginning either in early infancy or throughout childhood, up to the age of 30 years. Heredodegenerative deafness is usually progressive and

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