Guinea pigs, aged from 3 weeks before term to 31 weeks after birth, we
re prepared for scanning electron microscopy. Examination of the extre
me apex of the cochlea showed apparently pathological hair cells, even
3 weeks before term. The pathologies included loss and fusion of ster
eocilia, and the formation of giant stereocilia. The pathologies were
most prevalent on row 3 of outer hair cells, declining to outer hair c
ell rows 2 and 1, with the inner hair cells being least affected. The
abnormalities increased with time, increasing rapidly over the first f
ew weeks of life, and more slowly thereafter. It is suggested that ear
ly degeneration in the extreme cochlear apex forms a novel model for s
pontaneous hair cell degeneration, with applicability to other types o
f spontaneous hair cell degeneration.