Pathological changes during the development of the vestibular sensory and ganglion cells of the Bronx waltzer mouse. Scanning and transmission electron microscopy.

Vestibular receptors and ganglia of homozygous Bronx waltzer (bv/bv) mice were investigated by scanning and transmission electron microscopy at various stages between 3 days and 90 days after birth. Scanning electron microscopy revealed that there was already a considerable lack of hair bundles in the maculae utriculi, as well as in the cristae ampullares by the 3rd day after birth. During development, the growth of the remaining hair bundles was observed but the most of them exhibited morphological abnormalities. Transmission electron microscopy revealed early degeneration of sensory cells followed by delayed maturation of the remaining sensory cells. The sensory cells which seem unaffected displayed immature features in adult animals. In type I hair cells, the calyces were incomplete, contacts between the cell and the afferent calyces were immature and synaptic bodies persisted. In some type II hair cells, there was an abnormal overabundance of afferent nerve endings, which implies that these type II cells could be immature type I cells. Immature features were also observed in the vestibular ganglia, particularly the absence of the myelin sheath around the perikarya. We discuss the relationship between these vestibular morphogenetic abnormalities and those described in the cochlear system.