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Erwin D. Riedner, ScD; L. Stefan Levin, DDS, MSD; Michael J. Holliday, MD

Arch Otolaryngol. 1980;106(12):737-740.

Abstract
• 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)

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.

Footnotes
Accepted for publication March 20, 1980.

Read in part at the biennial meeting of The Johns Hopkins Medical and Surgical Association, Baltimore, Feb 23, 1979.

Reprint requests to Department of Otolaryngology, The Johns Hopkins Hospital, Osier 425, Baltimore, MD 21205 (Dr Levin).

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