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Related Experiment Videos

Strial dysfunction in the MRL-Fas mouse.

M J Ruckenstein1, M Milburn, L Hu

  • 1Department of Otolaryngology-Head and Neck Surgery, University of Tennessee, Memphis, USA.

Otolaryngology--Head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery
|October 3, 1999
PubMed
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The MRL-Fas(lpr) mouse model shows hearing loss due to reduced endocochlear potential (EP). This decline in EP is linked to stria vascularis degeneration, not a loss of Na, K-ATPase in autoimmune inner ear disease.

Area of Science:

  • Otolaryngology
  • Immunology
  • Genetics

Background:

  • The MRL-Fas(lpr) mouse model exhibits multisystemic autoimmune disease.
  • This model has been investigated for autoimmune inner ear disease, showing cochlear pathology like stria vascularis degeneration.
  • The stria vascularis is crucial for maintaining the endocochlear potential (EP).

Purpose of the Study:

  • To investigate the cause of diminished endocochlear potential (EP) in MRL-Fas(lpr) mice with autoimmune inner ear disease.
  • To determine if reduced EP is due to a loss of strial Na, K-ATPase activity.
  • To correlate auditory brain stem response (ABR) thresholds with EP and Na, K-ATPase levels.

Main Methods:

  • Auditory brain stem response (ABR) thresholds and endocochlear potentials (EPs) were recorded in MRL-Fas(lpr) mice and control MRL-+/+ mice.

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  • Cochleas were analyzed using immunohistochemistry to quantify Na, K-ATPase alpha1 and beta2 subunit levels.
  • Quantitative densitometry was used to evaluate the density of Na, K-ATPase staining.

    • Main Results:

    • MRL-Fas(lpr) mice demonstrated significantly elevated ABR thresholds and reduced EPs compared to controls.
    • Immunohistochemical analysis revealed no significant reduction in Na, K-ATPase levels in the stria vascularis of MRL-Fas(lpr) mice.
    • Despite reduced EP, Na, K-ATPase expression remained intact.

    Conclusions:

    • The reduction in EP in MRL-Fas(lpr) mice is likely caused by cellular degeneration within the stria vascularis.
    • The findings suggest that the breakdown of the strial perilymph/endolymph barrier, rather than diminished Na, K-ATPase, underlies the EP reduction.
    • This study highlights the importance of strial integrity for hearing function in autoimmune inner ear disease models.