The pattern of sensorineural degeneration in the cochlea of the deaf shaker-1 mouse: Ultrastructural observations

Experiments were done to extend existing knowledge on the nature and course of postnatal sensory and neural changes in the inner ear of shaker-1 mutant mice. Mice, 3-, 6-, 10-, 12-, 18-, and 30 days old, homozygous for the sh-1 gene, were studied using transmission electron microscopy. The data indicate retarded development coupled with the early onset of progressive degeneration in Corti's organ, its nerve supply, and the cells of the spiral ganglion. Especially noteworthy are the following: in 3-day-old mice both outer hair cells and spiral ganglion cells are already abnormal. The latter are especially loosely ensheathed by glial cells and are in direct contact with nerve fibers. Outer hair cells contain vacuoles and lysosomes. By 6 days of age inner hair cells come to be similarly affected. By 18 days most of the afferent nerve supply of the organ of Corti has degenerated. The behavior of efferents within the organ is complex. Efferents arrive late (day 12) at the outer hair cells, they are few in number, form only immature synapses with the cell, and they subsequently degenerate. In contrast, the efferent nerve supply of the inner hair cell appears normal, if not over-abundant. The simultaneous occurrence of organ of Corti and spiral ganglion cell anomalies is discussed in terms of the role of sensorineural interactions in the expression of genetic defects affecting the inner ear. The selective degeneration of efferents to outer hair cells is viewed as being consistent with the hypothesis that there are two independent efferent systems which innervate the two types of cochlear sensory hair cells.