Decreased auditory function in the C3H/lpr autoimmune disease mouse

Trune, Dennis R.; Kempton, J. Beth; Mitchell, Curtin R.

doi:10.1016/0378-5955(96)00018-4

Cited by 21 publications

(23 citation statements)

References 26 publications

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“…The third function of steroids, as a regulator of intracellular sodium transport, has largely been overlooked in the ear. 3 Autoimmune mice with hearing loss have consistently demonstrated pathology confined to the stria vascularis, 4–6 with disruption of the blood–labyrinth barrier 7 and depression of the endocochlear resting potential. 8 Coupled with a complete lack of cochlear inflammation, the observed stria vascularis disease suggests that endolymph sodium–potassium imbalances underlie autoimmune hearing loss.…”

Section: Introductionmentioning

confidence: 99%

Spironolactone Blocks Glucocorticoid‐Mediated Hearing Preservation in Autoimmune Mice

2002

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Spironolactone effectively blocked prednisolone from improving hearing in MRL/MpJ-Faslpr autoimmune mice. This offers evidence that the inner ear mineralocorticoid receptor is the therapeutic target for corticosteroids used to treat autoimmune and sudden sensorineural hearing loss. Pharmacologic treatments that selectively target the mineralocorticoid receptor may provide greater clinical benefit with fewer systemic side effects than prednisone in patients with autoimmune sensorineural hearing loss.

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Section: Introductionmentioning

confidence: 99%

Spironolactone Blocks Glucocorticoid‐Mediated Hearing Preservation in Autoimmune Mice

2002

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“…The present study terminated treatment at 6 months of age, which is at the beginning of the cochlear manifestations due to systemic autoimmune disease (Trune et al, 1996b). The hearing loss presumably would have been more pronounced if mice were allowed to live another 2-3 months.…”

Section: Discussionmentioning

confidence: 98%

“…C3H/HeJ mice have normal auditory thresholds through 12-14 months of age (Trune et al, 1996a). Onset of systemic disease in the autoimmune mice occurs at 5-6 months of age and cochlear thresholds rise shortly thereafter (Trune et al, 1996b). Therefore, all mice were tested with auditory brainstem response (ABR) audiometry at 2-3 months of age to establish pretreatment baseline auditory thresholds.…”

Section: General Protocolmentioning

confidence: 99%

“…These Fas gene substrains still demonstrated signiWcant systemic autoimmune disease, but with increased survival due to reduced kidney disease (Kelley and Roths, 1985;Gilkeson et al, 1989). This laboratory Wrst described threshold elevations in the C3.MRL-Fas lpr mouse strain (formerly C3H/ lpr), which begin at approximately 5-6 months of age and increase with progression of systemic disease (Trune et al, 1989(Trune et al, , 1996b. This autoimmune mouse has a longer survival period (50% alive at 11-12 months) (Kelley and Roths, 1985;Gilkeson et al, 1989) and its C3H/HeJ parent strain oVers a normal hearing control (Trune et al, 1996a) that allows characterization of cochlear changes due only to Fas gene-induced inner ear disease.…”

Section: Introductionmentioning

confidence: 99%

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Glucocorticoid impact on cochlear function and systemic side effects in autoimmune C3.MRL-Faslpr and normal C3H/HeJ mice

et al. 2007

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“…One experimental SLE study involving C3H/lpr autoimmune strain mice showed accelerated age-related hearing loss, particularly in the high frequencies; cochlear dysfunction in the mice was the result of stria vascularis pathology (16). The atrophy of stria vascularis in SLE patients was reported (8); however, the control was not used in the study.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Histopathologic Findings in Patients With Systemic Lupus Erythematosus

Kariya¹,

Kaya

Hızlı

et al. 2016

Otology &Amp; Neurotology

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Hypothesis We hypothesized that, in archived human temporal bone samples from patients with systemic lupus erythematosus (SLE), a pathologic condition exists in the stria vascularis and cochlear hair cells. Background Sensorineural hearing loss is a common feature in SLE patients. However, the pathophysiologic mechanism of cochlear dysfunction is unclear. Methods We examined 15 temporal bone samples from 8 SLE patients, along with 17 samples from 10 age-matched healthy control patients. The samples were serially sectioned in the horizontal plane and stained with hematoxylin and eosin. We determined the area of the stria vascularis in a midmodiolar section of each cochlear turn. Then, we made cytocochleograms and calculated the percentage of missing inner and outer hair cells. Results The area of the stria vascularis in our SLE group was significantly smaller than in our control group. The number of remaining inner hair cells in our SLE group was smaller than in our control group; however, the difference did not reach statistical significance. The loss of outer hair cells in our SLE group was significantly higher than in our control group. There was a tendency toward a positive correlation between the loss of cochlear hair cells and the duration of SLE. Conclusion The stria vascularis and cochlear hair cells are affected in SLE patients. Our findings could provide the histopathologic basis for the cochlear dysfunction, including sensorineural hearing loss, experienced by SLE patients.

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Decreased auditory function in the C3H/lpr autoimmune disease mouse

Cited by 21 publications

References 26 publications

Spironolactone Blocks Glucocorticoid‐Mediated Hearing Preservation in Autoimmune Mice

Spironolactone Blocks Glucocorticoid‐Mediated Hearing Preservation in Autoimmune Mice

Glucocorticoid impact on cochlear function and systemic side effects in autoimmune C3.MRL-Faslpr and normal C3H/HeJ mice

Cochlear Histopathologic Findings in Patients With Systemic Lupus Erythematosus

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