The Ribbon Synapse Between Type I Spiral Ganglion Neurons and Inner Hair Cells

2015 ◽  
pp. 117-156 ◽  
Author(s):  
Mark A. Rutherford ◽  
Tobias Moser
Keyword(s):  
Hair Cells ◽  
Spiral Ganglion ◽  
Ribbon Synapse ◽  
Spiral Ganglion Neurons ◽  
Type I ◽  
Inner Hair Cells ◽  
Ganglion Neurons

scholarly journals ROR1 is essential for proper innervation of auditory hair cells and hearing in humans and mice

2016 ◽  
Vol 113 (21) ◽  
pp. 5993-5998 ◽  
Author(s):  
Oscar Diaz-Horta ◽  
Clemer Abad ◽  
Levent Sennaroglu ◽  
Joseph Foster ◽  
Alexandra DeSmidt ◽  
...  
Keyword(s):  
Tyrosine Kinase ◽  
Inner Ear ◽  
Hair Cells ◽  
Spiral Ganglion ◽  
Auditory Neuropathy ◽  
Mutant Mice ◽  
Spiral Ganglion Neurons ◽  
Type I ◽  
Auditory Hair Cells ◽  
Ganglion Neurons

Hair cells of the inner ear, the mechanosensory receptors, convert sound waves into neural signals that are passed to the brain via the auditory nerve. Little is known about the molecular mechanisms that govern the development of hair cell–neuronal connections. We ascertained a family with autosomal recessive deafness associated with a common cavity inner ear malformation and auditory neuropathy. Via whole-exome sequencing, we identified a variant (c.2207G>C, p.R736T) in ROR1 (receptor tyrosine kinase-like orphan receptor 1), cosegregating with deafness in the family and absent in ethnicity-matched controls. ROR1 is a tyrosine kinase-like receptor localized at the plasma membrane. At the cellular level, the mutation prevents the protein from reaching the cellular membrane. In the presence of WNT5A, a known ROR1 ligand, the mutated ROR1 fails to activate NF-κB. Ror1 is expressed in the inner ear during development at embryonic and postnatal stages. We demonstrate that Ror1 mutant mice are severely deaf, with preserved otoacoustic emissions. Anatomically, mutant mice display malformed cochleae. Axons of spiral ganglion neurons show fasciculation defects. Type I neurons show impaired synapses with inner hair cells, and type II neurons display aberrant projections through the cochlear sensory epithelium. We conclude that Ror1 is crucial for spiral ganglion neurons to innervate auditory hair cells. Impairment of ROR1 function largely affects development of the inner ear and hearing in humans and mice.

New insights into glutamate ototoxicity in cochlear hair cells and spiral ganglion neurons

2010 ◽  
Vol 130 (12) ◽  
pp. 1316-1323 ◽  
Author(s):  
Haitao Lu ◽  
Xiang Wang ◽  
Wenyan Sun ◽  
Yao Hu ◽  
Shusheng Gong
Keyword(s):  
Hair Cells ◽  
Spiral Ganglion ◽  
Spiral Ganglion Neurons ◽  
Cochlear Hair Cells ◽  
Ganglion Neurons

scholarly journals Pou4f1 Defines a Subgroup of Type I Spiral Ganglion Neurons and Is Necessary for Normal Inner Hair Cell Presynaptic Ca2+ Signaling

2019 ◽  
Vol 39 (27) ◽  
pp. 5284-5298 ◽  
Author(s):  
Hanna E. Sherrill ◽  
Philippe Jean ◽  
Elizabeth C. Driver ◽  
Tessa R. Sanders ◽  
Tracy S. Fitzgerald ◽  
...  
Keyword(s):  
Hair Cell ◽  
Spiral Ganglion ◽  
Spiral Ganglion Neurons ◽  
Type I ◽  
Inner Hair Cell ◽  
Ganglion Neurons
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