Dental Malocclusion and Craniofacial Development in OI

Overview

Osteogenesis imperfecta (OI) is a rare inherited disorder that causes bones to break easily. Individuals with osteogenesis imperfecta break bones often and may have other problems, including hearing loss and pain and difficulty getting around. People with moderate to severe OI may also be diagnosed with dentinogenesis imperfecta (DI). DI is characterized by grey or brown teeth that may chip and wear down and break easily. People with DI may also have skull and neck defects. These patients may have severe teeth misalignment resulting in clinically significant chewing problems. Teeth misalignment in OI is very hard to treat because of the quality and quantity of bone. The overall goal of this study is to improve dental health to improve the quality of life of people with OI.

Investigators will look at dental health in people with OI and will describe teeth misalignment and head and neck defects in individuals with moderate to severe Osteogenesis Imperfecta (OI). Investigators will look at results from the Longitudinal study of OI to complete the study evaluations. Several x-rays will be performed for this study. Participants will have a 3D scan of the mouth and a Cone Beam CT scan of the jaw at a baseline visit and at 3 years after the baseline visit. These study visits can be at the same time as the Longitudinal Study of OI study visits.

Conditions

Eligibility