Abstract
Osteogenesis imperfecta is a connective tissue disease manifested by abnormalities within organs and structures rich in collagen. Typically, symptoms arise from the osteoarticular system. Excessive brittleness of the bones causes multiple fractures. Among patients with hearing loss, osteogenesis imperfecta manifests as changes to the stapes: the legs are broken and the footplate is immobilized and thickened. Changes to the malleus and incus are rarely reported. This location is associated with difficult conditions during surgery, difficult access to the ossicles. This research presents the characteristics and hearing results of patients who have undergone ossiculoplasty as a treatment of hearing loss in osteogenesis imperfecta. Two case reports present massively altered conditions within the middle ear in patients with type III osteogenesis imperfecta – the most severe among live births, who have undergone multiple surgeries due to hearing loss, resulting in a moderate improvement in hearing.
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