Abstract
Five Japanese girls of congenital deafness with blue iris were reported. Four of them had bilaterally isochromic blue irides and one had unilateral partial heterochromia iridis. The fundi of their eyes were reddish. Total or profound sensorineural deafness and canal paresis were found in all cases. Slight lateral displacement of the medial canthi of the eyes was observed in only one case. No prominent broad root of the nose, hyperplasia supercilii medialis or white forelock was found in the other cases. A father of one patient was deaf, but he had dark brown iris. No abnormity was found in the relatives of the other cases. These patients were discussed in relation to Waardenburg's syndrome, congenital deafness with total albinism and deaf white cats with blue iris.
