Clinical-pathologic correlation in a patient with selective loss of hair cells in the vestibular endorgans

We found a selective loss of vestibular hair cells in a patient followed for more than 10 years with imbalance and oscillopsia due to idiopathic progressive loss of vestibular function. Hearing function and cochlear hair cells were normal. The vestibulo-ocular reflex (VOR) gain at high frequencies was relatively maintained despite marked shortening of the dominant VOR time constant (to less than 500 ms). Ultrastructural examination of remaining hair cells showed mitochondrial abnormalities. The ultrashort VOR time constant probably resulted from changes in firing patterns of the primary afferent nerves due to loss of hair cells and impaired energy metabolism in remaining hair cells.