IT IS often impossible to differentiate between secondary, acquired degenerative inner ear changes and the degenerative changes occurring on a genetic basis. Only recently has the presence of PAS-positive globules been noted in some cases of hereditary deafness and not, as yet, in acquired deafness.
The onset of hereditary deafness is often within the first ten years of life. However, in Teig's1 study of 72 family members, deafness usually began at about 20 years of age and in Mårtensson's2 study of five generations the onset of deafness varied from the second to the seventh decade.
The temporal bones under discussion are those of an 80-year-old grandmother, who was the first family member affected, presumably at the age of 65 years (Fig 1). Her parents, siblings, and husband all had normal hearing. Of four children in the second generation, only one daughter had the onset of gradually progressive sensorineural
Gussen R. Delayed Hereditary Deafness With Cochlear Aqueduct Obstruction. Arch Otolaryngol. 1969;90(4):429–436. doi:10.1001/archotol.1969.00770030431005
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