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July 1969

Otosclerosis and Osteogenesis Imperfecta

Author Affiliations

From the Department of Otology, Rigshospitalet (University Hospital), Copenhagen.

Arch Otolaryngol. 1969;90(1):4-10. doi:10.1001/archotol.1969.00770030006004

IN THE EFFORT to find the early changes in otosclerosis and to view otosclerosis as a manifestation of generalized disease, attention has been focused, since the beginning of this century, on well-known bony dystrophies, such as osteitis fibrosa or Recklinghausen's disease, osteitis deformans or Paget's disease, and osteogenesis imperfecta.

Clinically and etiologically these diseases differ, but histologically they are similar in many respects.1

The bony labyrinthine capsule differs histologically from other bones of the skeleton. It is well-known that the bony labyrinthine capsule is preformed of cartilage (endochondral ossification). In the normal process of ossification, cartilaginous tissue is replaced entirely by bony tissue, but in the endochondral layer of the labyrinthine capsule islets of hyaline cartilage will persist. Thus, the ossification may be said to have been arrested at too early a stage.

In cases of incipient otosclerosis histological examination reveals osteoclastic destruction of the bony tissue in the

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