Details

Autor(en) / Beteiligte

• De Paolis, Annalisa
• Miller, Brendyn James
• Doube, Michael
• Bodey, Andrew John
• Rau, Christoph
• Richter, Claus-Peter
• Cardoso, Luis
• Carriero, Alessandra

Titel

Increased cochlear otic capsule thickness and intracortical canal porosity in the oim mouse model of osteogenesis imperfecta

Ist Teil von

• Journal of structural biology, 2021-06, Vol.213 (2), p.107708-107708, Article 107708

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2021

Quelle

MEDLINE

Beschreibungen/Notizen

• [Display omitted] •Synchrotron microtomography shows thicker and more porous oim otic capsule.•Oim otic capsule has a larger number and volume density of highly branched canals.•Osteocyte lacunae density and morphology did not differ in oim otic capsule.•Synchrotron microtomography shows a preserved cochlea morphology in oim mice.•The compromised oim otic capsule bone quality may contribute to hearing loss. Osteogenesis imperfecta (OI or brittle bone disease) is a group of genetic disorders of the connective tissues caused mainly by mutations in the genes encoding collagen type I. Clinical manifestations of OI include skeletal fragility, bone deformities, and severe functional disabilities, such as hearing loss. Progressive hearing loss, usually beginning in childhood, affects approximately 70% of people with OI with more than half of the cases involving the inner ear. There is no cure for OI nor a treatment to ameliorate its corresponding hearing loss, and very little is known about the properties of OI ears. In this study, we investigate the morphology of the otic capsule and the cochlea in the inner ear of the oim mouse model of OI. High-resolution 3D images of 8-week old oim and WT inner ears were acquired using synchrotron microtomography. Volumetric morphometric measurements were conducted for the otic capsule, its intracortical canal network and osteocyte lacunae, and for the cochlear spiral ducts. Our results show that the morphology of the cochlea is preserved in the oim ears at 8 weeks of age but the otic capsule has a greater cortical thickness and altered intracortical bone porosity, with a larger number and volume density of highly branched canals in the oim otic capsule. These results portray a state of compromised bone quality in the otic capsule of the oim mice that may contribute to their hearing loss.