Abstract
Osteogenesis imperfecta (OI) is a genetic disorder that is known for its effects on bone fragility. Less is known regarding its significant impact on hearing. Fifty percent of people with OI over 50 years of age have a significant hearing loss. All types of hearing loss are found in patients with OI but conductive hearing loss is commonly reported in younger patients with OI and mixed hearing loss with increasing age. However, the presence of sensorineural hearing loss has also been reported. Changes in type I collagen lead to changes in the inner ear structures. Abnormal bony formation leads to ossicular changes that cause a conductive hearing loss. Changes in the inner ear bone lead to local environmental changes that likely cause progressive sensorineural hearing loss. This chapter will review the otologic changes seen in OI, the nature of the hearing loss in OI, and outline the treatment options available to overcome these hearing loss deficits.
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