Mouse Genome Informatics
involves: 129S/SvEv * C57BL/6J
phenotype observed in females
phenotype observed in males
N normal phenotype
• limbal zone of tectorial membrane is extremely reduced, however hair bundle structure is normal
• Kimura's membrane is separated from the main body of the tectorial membrane
• the marginal band and Hensen's stripe are absent and there are large holes visible within the main body of the matrix
• enlargement of the subtectorial space in the vicinity of inner hair cell bundles
• striated-sheet matrix is not present in the sulcal zone and collagen fibrils are oriented almost perpendicular to its surface rather than projecting radially across the tectorial membrane
• neural response thresholds are elevated, neural tuning is broadened, and a sharp decrease in sensitivity is observed at the tip of the neural tuning curve, however the sensitivity and frequency tuning of the mechanical responses of the cochlea are little changed

Mouse Models of Human Disease
Deafness, Autosomal Dominant 12; DFNA12 601543 J:101691