Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function

Alexander Gow, Caroline Davies, Cherie M. Southwood, Gregory Frolenkov, Mark Chrustowski, Lily Ng, Daisuke Yamauchi, Daniel C. Marcus, Bechara Kachar

Research output: Contribution to journalArticlepeer-review

163 Citations (Scopus)

Abstract

Generation of a strong electrical potential in the cochlea is uniquely mammalian and may reflect recent evolutionary advances in cellular voltage-dependent amplifiers. This endocochlear potential is hypothesized to dramatically improve hearing sensitivity, a concept that is difficult to explore experimentally, because manipulating cochlear function frequently causes rapid degenerative changes early in development. Here, we examine the deafness phenotype in adult Claudin 11-null mice, which lack the basal cell tight junctions that give rise to the intrastrial compartment and find little evidence of cochlear pathology. Potassium ion recycling is normal in these mutants, but endocochlear potentials were below 30 mV and hearing thresholds were elevated 50 dB sound pressure level across the frequency spectrum. Together, these data demonstrate the central importance of basal cell tight junctions in the stria vascularis and directly verify the two-cell hypothesis for generation of endocochlear potential. Furthermore, these data indicate that endocochlear potential is an essential component of the power source for the mammalian cochlear amplifier.

Original languageEnglish
Pages (from-to)7051-7062
Number of pages12
JournalJournal of Neuroscience
Volume24
Issue number32
DOIs
Publication statusPublished - 2004 Aug 11
Externally publishedYes

Keywords

  • Evoked potentials
  • Freeze fracture
  • Homologous recombination
  • Oligodendrocyte-specific protein
  • Targeted deletion
  • β-galactosidase

ASJC Scopus subject areas

  • Neuroscience(all)

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