Hearing loss (in nonoperated ears) in relation to age in osteogenesis imperfecta type I.

Hearing loss was studied in relation to age in nonoperated ears in a group of 142 subjects with autosomal dominant osteogenesis imperfecta type I, which was compared to that in a random subsample of 70 subjects. In the n = 142 group, particularly below the age of 30 years, considerable selection (ie, for ear surgery) had occurred on hearing loss. The hearing threshold increased gradually with age. A hearing loss of greater than 30 dB (Fletcher index) was observed for 51% of the subjects older than 20 years and younger than 60 years. The median hearing loss progressed from the 10th to the 45th years of life with an average annual threshold increase (ATI) of 1 dB/y (0.5 to 4 kHz) up to 1.7 dB/y (8 kHz). Sensorineural loss accounted for 0.6 dB/y ATI at 0.5 to 4 kHz and 1.3 dB/y ATI at 8 kHz; conductive loss accounted for 0.4 dB/y ATI at all frequencies.