Freeze-fracturing of vestibular sensory epithelia in a strain of the waltzing guinea pig.

The waltzing guinea pig exhibits genetically induced inner ear disease which develops into deafness and vestibular disorder. The type I hair cell in vestibular end organs shows specific pathology, featuring protrusion of the hair cell apex, sensory hair fusion and an intracellular actin filament rod. With the freeze-fracturing technique, consecutive stages of degeneration of the apical part of the type I hair cell are shown. Different possible mechanisms underlying these changes are discussed. It is probable that the genetically induced prenatally developed hair cell pathology is related to a pathological development of actin filaments and their connections in the apical cytoskeleton.