Riedner E D, Levin L S, Holliday M J
Arch Otolaryngol. 1980 Dec;106(12):737-40. 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 alterative to surgery.
对13个患有显性成骨不全（OI）家族的成员进行了听力学评估和声阻抗测听。听力障碍出现在第二个十年后期或第三个十年初期，通常为传导性听力损失。老年患者有混合性或感音神经性听力损失。随着年龄增长，听力损失风险增加。声阻抗测试表明，听骨固定可能是混合性或传导性听力损失患者中耳疾病的主要原因。感音神经性听力损失的原因无法确定。OI患者耳科手术的长期成功率尚不确定。放大助听器可能是手术的合理替代方案。
