Dental Malocclusion and Craniofacial Development in OI
Dental Malocclusion and Craniofacial Development in OI
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.
Inclusion Criteria:

 -  Males and females with Clinical diagnosis of OI other than OI type I

 -  Individuals 10 years or older

 -  Participant of the Brittle Bone Disease (BBD) Longitudinal Study (7701)

 Exclusion Criteria:

 -  Individuals who cannot be correctly positioned for valid radiographic analysis (e.g.,
 due to severe scoliosis or short neck secondary to basilar invagination)

 -  Women who are pregnant