We report the progressive otic capsule demineralization around the membranous labyrinth
and facial nerve in an adult patient with osteogenesis imperfecta tarda. Whereas the initial …

Osteogenesis imperfecta (OI) is an autosomal-dominantly inherited connective tissue
disorder characterized by abnormal bone fragility combined with blue sclerae. The …

DISCUSSION The differential diagnosis of symmetric bilateral otic capsule demineralization,
as demonstrated in this case, would include osteogenesis imperfecta (OI), diffuse …

The histopathology of the temporal bones in a 59-year-old female with osteogenesis
imperfecta tarda (OIT), complicated by otosclerosis, is presented. Advanced conduction and …

Osteogenesis imperfecta, often called the syndrome of Van der Hoeve-de Kleyn, consists of
blue sclerae, fragilitas ossium and hardness of hearing. Previous authors have considered …

Osteogenesls Imperfecta Tarda: Appearance of the Temporal Bones at CT’ Page 1 Osteogenesls
Imperfecta Tarda: Appearance of the Temporal Bones at CT’ Ellen K. Tabor, MD #{149}Hugh …

• Osteogenesis imperfecta (01) is an inherited connective-tissue disorder of variable
penetrance. With 01, the triad of blue sclera, osseous fragility, and a conductive hearing loss …

The histopathologic findings in 8 temporal bones from 5 patients with osteogenesis
imperfecta congenita are reported. The otic capsule, bony walls of middle ear, and ossicles …

This temporal bone report describes the inner ear deformities which were found in addition
to the bony pathology in a case of osteogenesis imperfecta congenita. The labyrinthine …

Osteogenesis imperfecta (OI) is an inherited generalized disorder of type-I collagen
synthesis often associated with hearing loss. We present a case of OI type I in which hearing …