A 9-year-old boy with inner ear malformation complained of slight deafness and unsteadiness. CT revealed a normal cochlea despite enlargement of the lateral semicircular canals. The vertical semicircular canals developed more or less normally. The caloric test showed complete canal paresis bilaterally; however, a horizontal rotational stimulus elicited a vestibulo-ocular response, which showed only rightward and downward nystagmus, and their maximal slow-phase velocities were low. In addition, the examination of the vertical semicircular canal function using the head-tilted rotation test revealed a more active response, and the maximal slow-phase velocities were higher than those of a standard horizontal rotational test. These results suggest that the function of the vertical semicircular canal was well preserved and that it may have perceived the horizontal acceleration instead of the lateral semicircular canal.

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