TY - JOUR

T1 - Cellular voids in the pathogenesis of otosclerosis

AU - Hansen, Lars Juul

AU - Bloch, Sune Land

AU - Sørensen, Mads Sølvsten

N1 - Funding Information: This research was supported by a donation from Iris Lundholm Iversen for research in otosclerosis, Grant number non existing. Publisher Copyright: © 2023 Acta Oto-Laryngologica AB (Ltd).

PY - 2023

Y1 - 2023

N2 - Background: Otosclerosis is a common ear disease that causes fixation of the stapes and conductive hearing impairment. However, the pathogenesis of otosclerosis is still unknown. Otosclerosis could be associated with the unique bony environment found in the otic capsule. Normal bone remodelling is almost completely absent around the inner ear after birth allowing degenerative changes and dead osteocytes to accumulate. High levels of inner ear anti resorptive osteoprotegerin (OPG) is most likely responsible for this capsular configuration. Studies have demonstrated how osteocyte lifespan variation creates occasional clusters of dead osteocytes, so-called cellular voids, at otosclerotic predilection sites in the human otic capsule. These cellular voids have been suggested as possible starting points of otosclerosis. Aim: To describe the cellular viability in otosclerotic lesions and compare it to that of cellular voids. Materials and Methods: The study was based on unbiased stereological quantifications in undecalcified human temporal bones with otosclerosis. Results: Osteocyte viability was found to vary within the otosclerotic lesions. Furthermore, the results presented here illustrate that inactive otosclerotic lesions consist of mainly dead interstitial bone, much like cellular voids. Conclusions and significance: Focal degeneration in the otic capsule may play an important role in the pathogenesis of otosclerosis.

AB - Background: Otosclerosis is a common ear disease that causes fixation of the stapes and conductive hearing impairment. However, the pathogenesis of otosclerosis is still unknown. Otosclerosis could be associated with the unique bony environment found in the otic capsule. Normal bone remodelling is almost completely absent around the inner ear after birth allowing degenerative changes and dead osteocytes to accumulate. High levels of inner ear anti resorptive osteoprotegerin (OPG) is most likely responsible for this capsular configuration. Studies have demonstrated how osteocyte lifespan variation creates occasional clusters of dead osteocytes, so-called cellular voids, at otosclerotic predilection sites in the human otic capsule. These cellular voids have been suggested as possible starting points of otosclerosis. Aim: To describe the cellular viability in otosclerotic lesions and compare it to that of cellular voids. Materials and Methods: The study was based on unbiased stereological quantifications in undecalcified human temporal bones with otosclerosis. Results: Osteocyte viability was found to vary within the otosclerotic lesions. Furthermore, the results presented here illustrate that inactive otosclerotic lesions consist of mainly dead interstitial bone, much like cellular voids. Conclusions and significance: Focal degeneration in the otic capsule may play an important role in the pathogenesis of otosclerosis.

KW - bone remodelling

KW - cellular voids

KW - Otosclerosis

KW - undecalcified histology

U2 - 10.1080/00016489.2023.2164904

DO - 10.1080/00016489.2023.2164904

M3 - Journal article

C2 - 36639139

AN - SCOPUS:85146685864

VL - 143

SP - 250

EP - 253

JO - Acta Oto-Laryngologica

JF - Acta Oto-Laryngologica

SN - 0001-6489

IS - 3

ER -