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Publication : Abnormal outer hair cell efferent innervation in Hoxb1-dependent sensorineural hearing loss.

First Author  Di Bonito M Year  2023
Journal  PLoS Genet Volume  19
Issue  9 Pages  e1010933
PubMed ID  37738262 Mgi Jnum  J:340779
Mgi Id  MGI:7530351 Doi  10.1371/journal.pgen.1010933
Citation  Di Bonito M, et al. (2023) Abnormal outer hair cell efferent innervation in Hoxb1-dependent sensorineural hearing loss. PLoS Genet 19(9):e1010933
abstractText  Autosomal recessive mutation of HOXB1 and Hoxb1 causes sensorineural hearing loss in patients and mice, respectively, characterized by the presence of higher auditory thresholds; however, the origin of the defects along the auditory pathway is still unknown. In this study, we assessed whether the abnormal auditory threshold and malformation of the sensory auditory cells, the outer hair cells, described in Hoxb1null mutants depend on the absence of efferent motor innervation, or alternatively, is due to altered sensory auditory components. By using a whole series of conditional mutant mice, which inactivate Hoxb1 in either rhombomere 4-derived sensory cochlear neurons or efferent motor neurons, we found that the hearing phenotype is mainly reproduced when efferent motor neurons are specifically affected. Our data strongly suggest that the interactions between olivocochlear motor neurons and outer hair cells during a critical postnatal period are crucial for both hair cell survival and the establishment of the cochlear amplification of sound.
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