Ultrastructure of otic capsule sclerosis in Palmerston North autoimmune mice

Numerous temporal bone studies have reported a correlation between systemic autoimmune disease and osteogenic lesions within the inner ear. However, little is known of the cellular mechanisms that relate these two disease processes. The Palmerston North (PN) autoimmune strain mouse exhibits both spontaneous systemic autoimmune disease and otic capsule sclerotic lesions that are similar in many ways to those reported in humans. This suggests the PN mouse is a potential model in which to study the cellular events responsible for immune-related otic capsule lesions. Therefore, an evaluation of the fine structure of the PN modiolus was conducted to better understand these matrix changes of the inner ear.

Inner ears were collected from 15 PN mice at ages from 17 to 24 months and prepared for electron microscopy. The ears were ultrastructurally evaluated to characterize the lesions and their associated cytoarchitecture.

The sclerotic lesions consisted of an electron-dense mass that appeared lobulated or layered, usually adjacent to the modiolar bone and blood vessels. Immediately surrounding the lesions were activated fibroblasts and fine fibrillar material in the extracellular space between them. The sclerotic foci often were apposed to normal modiolar bone that never appeared degraded.

The similarities between these bony lesions and known human otic capsule diseases suggests parallel processes are involved. Thus, further study of the PN inner ear may provide insight into the cellular events that underlie otic capsule and other temporal bone alterations in systemic autoimmune diseases.