Origin and growth of otosclerosis

The external layer of the otic capsule arises from periosteal osteoblasts, which produce large numbers of Volkmann's canals as well as lamellar bone. The main plaque of otosclerosis is a histologic replica of the external layer and seems to arise from similar cells in the periosteum and to follow a defined invasive course into the footplate of the stapes, the basal coil of the cochlea and the saccule.

To determine by histologic study of the developing otic capsule and temporal bones with otosclerosis the site, tissue of origin, and pathways of growth of the disease.

Step sections of 60 celloidin-embedded temporal bones from fetuses and 24 from patients aged between 1 and 52 years were examined in the study of the development of the otic capsule. Step sections of 65 temporal bones each with 2 or more deposits of otosclerosis were surveyed to obtain data on the site, tissue of origin, and pathways of its growth.

The otic capsule differs from other bones in that the formation of the ultimate lamellar bone tissue is accompanied by very numerous intercommunicating channels. In the middle (cartilage origin) layer these are chondro-osseous canals and Volkmann's canals (like Haversian canals, but multidirectional). In the external (periosteal origin) layer these are Volkmann's canals only. In all, 63 of the 65 temporal bones with otosclerosis that were studied showed a prominent posterior otic capsule plaque. Evidence that this is derived from the periosteum of the external canal is as follows. (a) The otosclerotic tissue of the plaque--like all otosclerotic tissue--is composed of Volkmann's canals and lamellar bone only, as does external layer tissue. (b) All posterior plaques have an edge at the periosteum bordering the processus cochleariformis and tensor tympani muscle. The presumed invasive edge of the plaque on the opposite (cochlear) side shows a variable level of its front. (c) The tissue on the cochlear side of the plaque has a darkly stained appearance with large numbers of osteoblasts and poorly differentiated Volkmann's canals, suggesting that this is an invasive front. The otosclerosis becomes progressively better differentiated away from the darkly stained zone, indicating increasing maturation, which is greatest in the suggested origin of the plaque at the processus/tensor tympani muscle region because this would be the oldest region of the plaque. The pathway of the growth indicated by this study suggests a possible time sequence in the symptomatology of otosclerosis as it moves first to stapes footplate and then through the spiral ligament of cochlea to the saccule. An anterior plaque was seen in 42 of the 65 temporal bones with multiple sites of otosclerosis examined. These showed features similar to those listed above for the posterior plaque, with a base on the periosteum bordering the canal for the internal carotid artery, dark zonation at the invasive front near the cochlea, and increasing differentiation towards the base.