Increased cochlear otic capsule thickness and intracortical canal porosity in the oim mouse model of osteogenesis imperfecta

Paolis, Annalisa De; Miller, Bill; Doube, Michael; Bodey, Andrew J.; Rau, Christoph; Richter, Claus Peter; Cardoso, Luís; Carriero, Alessandra

doi:10.1016/j.jsb.2021.107708

Cited by 6 publications

(2 citation statements)

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“…Little data exist on the material properties of the ligaments and bones, and their effect on middle ear mechanics. The majority of these are also on mice models of the disease, such as those for OI ( Bonadio et al, 1990 ; Altschuler et al, 1991 ; Chen et al, 2007 ; Stankovic et al, 2007 ; Pokidysheva et al, 2013 ; De Paolis et al, 2021 ; Patel et al, 2022 ). Studies from small animals must then be translated to humans to be clinically relevant.…”

Section: Unsolved Questions On Middle Ear Mechanicsmentioning

confidence: 99%

Mammalian middle ear mechanics: A review

Ugarteburu

Withnell

Cardoso

et al. 2022

Front. Bioeng. Biotechnol.

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The middle ear is part of the ear in all terrestrial vertebrates. It provides an interface between two media, air and fluid. How does it work? In mammals, the middle ear is traditionally described as increasing gain due to Helmholtz’s hydraulic analogy and the lever action of the malleus-incus complex: in effect, an impedance transformer. The conical shape of the eardrum and a frequency-dependent synovial joint function for the ossicles suggest a greater complexity of function than the traditional view. Here we review acoustico-mechanical measurements of middle ear function and the development of middle ear models based on these measurements. We observe that an impedance-matching mechanism (reducing reflection) rather than an impedance transformer (providing gain) best explains experimental findings. We conclude by considering some outstanding questions about middle ear function, recognizing that we are still learning how the middle ear works.

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Section: Unsolved Questions On Middle Ear Mechanicsmentioning

confidence: 99%

Mammalian middle ear mechanics: A review

Ugarteburu

Withnell

Cardoso

et al. 2022

Front. Bioeng. Biotechnol.

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“…The stapes is the key ossicle affected by OI in humans and shows increased porosity of the crura (bend) and increased otosclerotic fixation of the stapedial footplate in those affected with OI (Waissbluth et al, 2020). Less is known about the pathological state of the cochlea, although increased bone density and porosity have been observed in the surrounding otic capsule of human patients (Swinnen et al, 2012) and mice with OI (de Paolis et al, 2021). Several hypotheses have been proposed regarding the cause of loss of cochlear function in OI, which include intrusion of petrous bone on the cochlea and subsequent soft tissue damage (i.e., hemorrhage of blood into the labyrinth, atrophy of the stria vascularis, the blood supply of the cochlea, or atrophy of the cochlear hair cells) (De Paolis et al, 2021;Shapiro et al, 1982).…”

Section: Introductionmentioning

confidence: 99%

Morphological variability in the inner ear of mice with osteogenesis imperfecta

Huston

Husain

Moore

et al. 2023

The Anatomical Record

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Osteogenesis imperfecta (OI) is known to cause hearing loss in ~60% of the affected human population. While OI‐related pathologies have been studied in the middle ear, the development of cochlear pathologies is less well understood. In this study, we examine OI‐related pathologies of the cochlea in a mouse model of OI to (1) document variation between OI and unaffected mice, and (2) assess the intrusion of the otic capsule onto the cochlea by analyzing differences in duct volumes. Juvenile and adult OIM C57BL/6mice were compared to unaffected wildtype (WT) mice using three‐dimensional models of the cochlea generated from high resolution micro‐CT scans. Two‐tailed Mann–Whitney U tests were then used to investigate duct volume differences both within and between the OI and WT samples. Areas of higher ossification were observed at the cochlear base in the OI sample. OI mice also had significant intraindividual differences in duct volume between right and left ears (4%–15%), an effect not observed in WT mice. WT and OI duct volumes showed a large degree of overlap, although the OIM volumes were more variable. Our findings indicate that OIM mice are likely to exhibit more asymmetry and variation in cochlear volume despite minor differences in sample cochlear volumes, possibly due to bony capsule intrusion. This suggests a potential mechanism of hearing loss, and a high potential for cochlear and otic capsule alteration in OIM mice.

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