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Vol. 9, Issue 1, 7-16, January 1999
REVIEW
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  B. Walmsley, A. Berntson, R. N. Leao, and R. E. W. Fyffe Activity-dependent regulation of synaptic strength and neuronal excitability in central auditory pathways J. Physiol., April 15, 2006; 572(2): 313 - 321. [Abstract] [Full Text] [PDF]
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R. N. Leao, H. Sun, K. Svahn, A. Berntson, M. Youssoufian, A. G. Paolini, R. E. W. Fyffe, and B. Walmsley Topographic organization in the auditory brainstem of juvenile mice is disrupted in congenital deafness J. Physiol., March 15, 2006; 571(3): 563 - 578. [Abstract] [Full Text] [PDF]
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R. N. Leao, A. Berntson, I. D. Forsythe, and B. Walmsley Reduced low-voltage activated K+ conductances and enhanced central excitability in a congenitally deaf (dn/dn) mouse J. Physiol., August 15, 2004; 559(1): 25 - 33. [Abstract] [Full Text] [PDF]
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 R. N. Leao, S. Oleskevich, H. Sun, M. Bautista, R. E.W. Fyffe, and B. Walmsley Differences in Glycinergic mIPSCs in the Auditory Brain Stem of Normal and Congenitally Deaf Neonatal Mice J Neurophysiol, February 1, 2004; 91(2): 1006 - 1012. [Abstract] [Full Text] [PDF]
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 P J Francis, S Johnson, B Edmunds, R E Kelsell, E Sheridan, C Garrett, G E Holder, D M Hunt, and A T Moore Genetic linkage analysis of a novel syndrome comprising North Carolina-like macular dystrophy and progressive sensorineural hearing loss Br. J. Ophthalmol., July 1, 2003; 87(7): 893 - 898. [Abstract] [Full Text] [PDF]
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 K. L. Mitchem, E. Hibbard, L. A. Beyer, K. Bosom, G. A. Dootz, D. F. Dolan, K. R. Johnson, Y. Raphael, and D. C. Kohrman Mutation of the novel gene Tmie results in sensory cell defects in the inner ear of spinner, a mouse model of human hearing loss DFNB6 Hum. Mol. Genet., August 1, 2002; 11(16): 1887 - 1898. [Abstract] [Full Text] [PDF]
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S. Oleskevich and B. Walmsley Synaptic transmission in the auditory brainstem of normal and congenitally deaf mice J. Physiol., April 15, 2002; 540(2): 447 - 455. [Abstract] [Full Text] [PDF]
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Head and
Neck Surgery, Center for Molecular and Human Genetics, and Kresge
Hearing Research Laboratory, Louisiana State University Medical Center,
New Orleans, Louisiana 70112 USA
-tectorin (TECTA), the transcription factor
POU4F3, connexin 26 (GJB2), and two unconventional
myosins (MYO7A and MYO15), and four novel proteins (PDS,
COCH, DFNA5, DFNB9). The same clinical
phenotype in hearing-impaired individuals, even those within the same
family, can result from mutations in different genes. Conversely,
mutations in the same gene can result in a variety of clinical
phenotypes with different modes of inheritance. For example, mutations
in the gene encoding MYO7A cause Usher syndrome type IB,
autosomal-recessive nonsyndromic hearing impairment (DFNB2),
and autosomal-dominant nonsyndromic hearing impairment (DFNA11). Additionally, the mouse ortholog of the
MYO7A gene is the shaker-1 gene. Mouse models such as
shaker-1 have facilitated the identification of genes that
cause hearing impairment in humans. The availability of high-resolution
maps of the human and mouse genomes and new technologies for gene
identification are advancing molecular understanding of hearing
impairment and the complex mechanisms of the auditory system.