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December 1980

Hearing Patterns in Dominant Osteogenesis Imperfecta

Author Affiliations

From the Hearing and Speech Clinic, Departments of Otolaryngology (Drs Riedner Levin, and Holliday) and Medicine (Dr Levin), The Johns Hopkins University School of Medicine, Baltimore. Dr Riedner is now in private practice in Baltimore.

Arch Otolaryngol. 1980;106(12):737-740. doi:10.1001/archotol.1980.00790360015006

• Audiologic evaluations and impedance audiometry were obtained on persons from 13 families with dominant osteogenesis imperfecta (OI). Hearing impairment appeared in the late second or early third decade, usually with a conductive loss. Older patients had mixed or sensorineural losses. There was an increasing risk for hearing loss with advancing age. Impedance tests suggested that ossicular fixation may be the primary cause of middle ear disorders in patients with mixed or conductive hearing losses. The cause of sensorineural hearing loss could not be determined. The long-term success of otologic surgery in OI is uncertain. Amplification may be a reasonable alternative to surgery.

(Arch Otolaryngol 106:737-740, 1980)

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