Progressive hearing loss from strial dysplasia.

An otherwise healthy male patient had a bilateral, slowly progressive hearing loss first noticed in early childhood and possibly present at birth. Audiometric studies at the age of 32 showed a moderately severe, bilateral, mixed type hearing loss. He died of unrelated causes at the age of 34. Studies of the temporal bones showed bilateral hypoplasia and atrophy of the striae vascularis as the cause of hearing loss. The history and findings are consistent with a genetically determined hypoplasia and atrophy of this structure.