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Cited by 15Hearing, Cochlea, Tinnitus, GeneticsVestibular and auditory disordersCongenital Ear and Nasal Anomalies

Abortive synaptogenesis as a factor in the inner hair cell degeneration in the bronx waltzer (bv) mutant mouse

Sobkowicz, H.M., Inagaki, M., August, B.K., Slapnick, S.M.·1999·Journal of Neurocytology
DOI 10.1023/a:1007059616607
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Abstract

The Bronx Waltzer (vb) mutation in the mouse results in the degeneration of most but not all of the primary auditory receptors, the inner hair cells, and their afferent neurons. We analyzed the ultrastructure of 94 inner hair cells in the intact postnatal mutant mouse and in neonatal cochleas in culture to understand the pathogenesis of hair cell death and to detect factors that may prevent it. The vb spiral neurons of the Bronx Waltzer display two distinctive features: some of them continue to divide mitotically for at least seven postnatal days, and the type I radial fibers that innervate inner hair cells display a deficiency in immunoexpression of GAD. The growing endings of spiral neurons converge around the inner hair cells or, in their absence, invade the outer hair cell region. Their profuse sprouting among inner spiral sulcus cells contributes to the characteristic ultrastructural picture of the bv cochlea. During the first three days after birth, 40% of the inner hair cells appear normal and innervated, 40% are mostly denervated and degenerating, and 20% are immature, with minimal or no neuronal appositions. However, in mutants 6 days and older only a few inner hair cells survive, and these show either normal or superfluous afferent innervation and axosomatic GABAergic efferent innervation. Degeneration of inner hair cells begins with a distention of the nuclear envelope and the ribosomal endoplasmic reticulum. The outer nuclear membrane eventually breaks, and exudate fills the cell interior. The cellular edema leads to cell death. We propose that success or failure in synaptic acquisition is a decisive factor in the survival or decline of the mutant inner hair cells. We also suggest that the developmental delay in maturation of the spiral ganglion neurons (type I) and the failure in their synaptogenesis may be caused by an impairment in neurotrophin (NT3/BDNF) synthesis by their mutant receptor cells.

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  • Bv/bv mice have defects in the differentiation and/or survival of inner hair cells and vestibular hair cells of the cochlea.

    “The bv/bv mice have defects in the differentiation and/or survival of inner hair cells and vestibular hair cells of the cochlea70,71.”

    From The neurogenetics of alternative splicing (2016) · cites this paper as [71]

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Authors
Sobkowicz, H. M., Inagaki, M., August, B. K., & Slapnick, S. M.
Affiliations
  • University of Wisconsin–Madison (US)
  • National Center of Neurology and Psychiatry (JP)
Corresponding author
Sobkowicz, H.M.
ORCID
Inagaki, M.
Venue
Journal of Neurocytology
Publisher
Springer Science and Business Media LLC
Published
1999-01
Volume
28
Issue
1
Pages
17-38
ISSN
  • 0300-4864 (ISSN-L)
  • 1573-7381
DOI
10.1023/a:1007059616607
OpenAlex
W1573510455
Cited by
15
References
63
Citations by year
2023: 1 · 2022: 1 · 2016: 2 · 2013: 1 · 2012: 1
FWCI
0.47
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Journal of Neurocytology (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Institutes of Health
  • National Institute on Deafness and Other Communication Disorders (5 RO1 DC/OD 00844-05)
Topics
  • Life Sciences › Neuroscience › Sensory Systems › Hearing, Cochlea, Tinnitus, Genetics
  • Life Sciences › Neuroscience › Neurology › Vestibular and auditory disorders
  • Health Sciences › Medicine › Genetics › Congenital Ear and Nasal Anomalies
Keywords
synaptogenesis, spiral ganglion neurons, neuronal ultrastructure, hair cell death, neurotrophin-3, brain-derived neurotrophic factor, cochlea, denervation, postnatal development
MeSH terms
Acetylcholinesterase, Animals, Animals, Newborn, Auditory Pathways, Cochlea, Hair Cells, Auditory, Inner, Mice, Mutant Strains, Nerve Degeneration, Nerve Endings, Nerve Fibers, Neurons, Organ Culture Techniques, Cell Death, Mice

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