Neurosensory Development and Cell Fate Determination in the Human Cochlea

Overview

Journal Neural Dev

Publisher Biomed Central

Specialty Neurology

Date 2013 Oct 18

PMID 24131517

Citations 44

Authors

Heiko Locher

Johan H M Frijns

Liesbeth Van Iperen

John C M J de Groot

Margriet A Huisman

Susana M Chuva de Sousa Lopes

Affiliations

Soon will be listed here.

Abstract

Background: Hearing depends on correct functioning of the cochlear hair cells, and their innervation by spiral ganglion neurons. Most of the insight into the embryological and molecular development of this sensory system has been derived from animal studies. In contrast, little is known about the molecular expression patterns and dynamics of signaling molecules during normal fetal development of the human cochlea. In this study, we investigated the onset of hair cell differentiation and innervation in the human fetal cochlea at various stages of development.

Results: At 10 weeks of gestation, we observed a prosensory domain expressing SOX2 and SOX9/SOX10 within the cochlear duct epithelium. In this domain, hair cell differentiation was consistently present from 12 weeks, coinciding with downregulation of SOX9/SOX10, to be followed several weeks later by downregulation of SOX2. Outgrowing neurites from spiral ganglion neurons were found penetrating into the cochlear duct epithelium prior to hair cell differentiation, and directly targeted the hair cells as they developed. Ubiquitous Peripherin expression by spiral ganglion neurons gradually diminished and became restricted to the type II spiral ganglion neurons by 18 weeks. At 20 weeks, when the onset of human hearing is thought to take place, the expression profiles in hair cells and spiral ganglion neurons matched the expression patterns of the adult mammalian cochleae.

Conclusions: Our study provides new insights into the fetal development of the human cochlea, contributing to our understanding of deafness and to the development of new therapeutic strategies to restore hearing.

Citing Articles

Expression of Neurotrophins and Its Receptors During Fetal Development in the Human Cochlea.

Steinacher C, Nishio S, Usami S, Dudas J, Rieder D, Rask-Andersen H Int J Mol Sci. 2024; 25(23).

PMID: 39684722 PMC: 11641258. DOI: 10.3390/ijms252313007.

A primate model animal revealed the inter-species differences and similarities in the subtype specifications of the spiral ganglion neurons.

Hosoya M, Ueno M, Shimanuki M, Nishiyama T, Oishi N, Ozawa H Sci Rep. 2024; 14(1):25166.

PMID: 39448766 PMC: 11502759. DOI: 10.1038/s41598-024-76892-y.

SOX2, JAGGED1, β-Catenin, and Vitamin D Receptor Expression Patterns during Early Development and Innervation of the Human Inner Ear.

Mikulic P, Ogorevc M, Petricevic M, Kalicanin D, Tafra R, Saraga-Babic M Int J Mol Sci. 2024; 25(16).

PMID: 39201406 PMC: 11354891. DOI: 10.3390/ijms25168719.

Clinical Significance of SOX10 Expression in Human Pathology.

Bahmad H, Thiravialingam A, Sriganeshan K, Gonzalez J, Alvarez V, Ocejo S Curr Issues Mol Biol. 2023; 45(12):10131-10158.

PMID: 38132479 PMC: 10742133. DOI: 10.3390/cimb45120633.

Comprehensive Transcriptomic Profiling of m6A Modification in Age-Related Hearing Loss.

Feng M, Zhou X, Hu Y, Zhang J, Yang T, Chen Z Biomolecules. 2023; 13(10).

PMID: 37892219 PMC: 10605720. DOI: 10.3390/biom13101537.

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