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Related Concept Videos

Unrenewable Cells00:50

Unrenewable Cells

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In humans, the photoreceptor cells of the eye and sensory hair cells of the ear lack stem cells. These cells are thus unrenewable and cannot be replaced when they are damaged or destroyed.
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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
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Outer hair cell function is normal in βV spectrin knockout mice.

Michael C Stankewich1, Jun-Ping Bai2, Paul R Stabach1

  • 1Department of Pathology, Yale School of Medicine, New Haven, CT, USA.

Hearing Research
|July 21, 2022
PubMed
Summary
This summary is machine-generated.

Beta V spectrin (Spnb5) is crucial for hearing, impacting auditory nerve fibers. Contrary to previous suggestions, this study finds Spnb5 is not essential for outer hair cell function.

Keywords:
Auditory brainstem responseCochleaCochlear nerveDistortion product otoacoustic emissionsElectromotilityPrestinRibbon synapseSpnb5

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Area of Science:

  • Neuroscience
  • Otoacoustic Emissions
  • Auditory System Research

Background:

  • Previous research suggested a role for beta V spectrin (Spnb5) in the function of outer hair cells (OHCs) within the cochlea.
  • The cytoskeleton's role in OHC electromotility is an area of ongoing investigation.

Purpose of the Study:

  • To investigate the function of beta V spectrin (Spnb5) in hearing and auditory function.
  • To determine the specific role of Spnb5 in outer hair cells and auditory neurons.

Main Methods:

  • Generation and analysis of Spnb5 knockout mice (Spnb5-/-).
  • Assessment of auditory thresholds, auditory brainstem response (ABR) wave 1-amplitudes, and otoacoustic emissions.
  • Evaluation of outer hair cell electromechanical activity and hair cell CtBP2 counts.
  • Analysis of afferent and efferent nerve fiber counts and Spnb5 mRNA expression in spiral ganglion neurons and OHCs.

Main Results:

  • Spnb5 knockout mice exhibited normal auditory thresholds, otoacoustic emissions, and non-linear capacitance, indicating unaffected outer hair cell function.
  • Auditory brainstem response (ABR) wave 1-amplitudes were significantly reduced in Spnb5 knockout mice.
  • No evidence of synaptopathy was found, but a decrease in afferent and efferent nerve fibers was observed.
  • Spnb5 mRNA was detected in spiral ganglion neurons but not in outer hair cells.

Conclusions:

  • Beta V spectrin (Spnb5) plays a critical role in auditory nerve structure and function, impacting overall hearing.
  • The findings challenge the previously proposed functional importance of beta V spectrin in outer hair cells.
  • Spnb5 is essential for neuronal integrity within the auditory system, rather than for outer hair cell electromotility.