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The development of hair cells in both the auditory and vestibular sensory epithelia is a complex process that involves the coordinated expression of many regulatory proteins. Among these is the POU-domain transcription factor Brn-3.1. This factor is expressed in hair cell precursors immediately after commitment to the hair cell fate, and continues throughout life. Deletion of this factor in mice leads to failure of hair cell differentiation during development, and to the death of a majority of the undifferentiated cells. Normal expression of Brn-3.1 is required for adult hair cell survival as well, since a mutation in this gene causes dominant, late-onset, inherited hearing loss in humans. The timing of the onset of Brn-3.1 expression suggests that factors regulating its expression may be involved in fate determination of hair cells. Moreover, genes that are themselves directly regulated by Brn-3.1 appear to play critical roles in hair cell development and survival., (Copyright 2002 S. Karger AG, Basel)

In mice with genetically induced inner ear abnormalities it is conceivable that in the morphogenetic types and in mutants with the spotting kind of pigmentary anomaly, the genes act through the developing nervous system. It has been suggested that in degenerative (neuroepithelial) mutants the influence of the gene is also reflected in the inner ear through the agency of the nervous system. The jerker mouse belongs to the neuroepithelial type of mutants which in homozygotes results in early postnatal degeneration of the sensory epithelium of the inner ear, initially confined to the cuticular plate and the stereocilia. In spite of well-advanced hair cell degeneration, these mutants developed morphologically normal afferent and efferent nerve terminals at cochlear hair cells.

Human blood-group antigens are transiently expressed in developing cochlear hair cells. This temporal antigen expression seems to correspond to the main events of inner ear differentiation (e.g. hair cell development, synaptogenesis, ciliogenesis). In the present report, the cochlear expression of H human blood group antigen has been analyzed with the aid of the electron microscope. Ultrastructurally, cochleas stained with anti-H antibody exhibited an intense immunoreactivity on the apical hair cell membrane and on stereocilia. Cell surface glycoconjugates may serve a large number of functions and could be important during cell development. The temporal expression of H blood-group antigen in developing cochlear hair cells could be related to stereocilia differentiation.