Osteogenesis Imperfecta (OI) is the most common skeletal dysplasia that predisposes to recurrent fractures and bone deformities. In spite of significant advances in understanding the genetic basis of OI, there have been no large-scale natural history studies. To better understand the natural history and improve the care of patients, a network of Linked Clinical Research Centers (LCRC) was established. Subjects with OI were enrolled in a longitudinal study, and in this report, we present cross-sectional data on the largest cohort of OI subjects (n=544). OI type III subjects had higher prevalence of dentinogenesis imperfecta, severe scoliosis, and long bone deformities as compared to those with OI types I and IV. Whereas the mean LS aBMD was low across all OI subtypes, those with more severe forms had lower bone mass. Molecular testing may help predict the subtype in type I collagen-related OI. Analysis of such well-collected and unbiased data in OI can not only help answer questions that are relevant to patient care but also foster hypothesis-driven research, especially in the context of “phenotypic expansion” driven by next-generation sequencing.

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There is increased concern about excessive sound exposure, especially in students, resulting from the use of Ipods and similar devices. Such exposure needs to be considered in light of the daily noise dose such users experience. In this study five university students wore noise dosimeters for 10 h per day for 5 days, and noise levels were recorded every minute. The students also maintained a diary of their activities (activities were noted at least every half hour) during the time they wore the noise dosimeters. The diary was standardized so that each student used the same diary format. Results indicating the noise levels, noise dose, and the relationship of the levels to the students activities will be presented and discussed. In general the noise exposure experienced by these students was usually mild (less than 60 dBA) to moderate (between 60 and 75 dBA).

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