The effects of the mutant gene shaker‐1 (sh‐1) in the mouse include degeneration of the neural epithelium in the inner ear, which in places is so constructed that degeneration of minute areas, even single cells, can be identified. In Cattanach's translocation the piece of autosome 1 that is translocated onto the X‐chromosome bears the normal allele of sh‐1, and so this gene would be expected to acquire the charateristics of sex‐linked genes in mammals. It was thought that a study of sh‐1/‐; Dp/X animals might illuminate some aspect of the development of the inner ear in normal and sh‐1/sh‐1 animals. Normal and affected regions were found to coexist in the organ of Corti but not in the macula of the saccule. The degree of abnormality of the macula differed from ear to ear but was quite uniform within each ear. This points to a radical difference in early development in the relationship between the otic epithelium and its innervation in the cochlea and the saccule. It also appears highly unlikely that abnormalities of the stria vascularis are responsible for the degeneration of the organ of Corti. Copyright © 1969 Wiley‐Liss, Inc., A Wiley Company