Description | Karen Steel has distinguished herself both by numerous contributions to the general problems of defining chromosomal homologies between mouse and man and the specific problems of genomic function and related phenotypes in deaf mice. She defined the position of several loci in mice and advanced knowledge of their mechanism by electrolyte responses within and between cells and electron microscopy of the causal lesion in the cochlea. She showed that structural disorganisation could be secondary to impaired function, rather than being primarily developmental, and that the well-known, but badly understood, relationship of deafness to both pigmentation and patchy pigmentation in humans, dalmatians and cats was associated, in mice, with reduced numbers of melanocytes in the inner ear. Her work has profoundly influenced the assumed nature of Mendelian forms of human deafness, a condition in which biopsy is impractical, autopsies rare, and rarely fresh, removal of the inner ear tedious and fixation with decalcification difficult |