Abstract
Hearing loss in osteogenesis imperfecta (OI) typically begins in the third decade of life. OI rarely occurs in childhood. Case reports of hearing loss in pediatric patients with OI are considered as merely incidental, and until now pediatric OI cases have not been presented in the literature. Typically, hearing loss is conductive, but over time a sensorineural component also occurs. There are some reports of profound sensorineural hearing loss, but this type of loss is believed to be due to additional genetic factors. The two case reports described in this paper show how the hearing loss due to OI can be treated by means of stapedotomy and restapedotomy
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