Although absence of the olfactory bulbs is a relatively common occurre
nce seen in holoprosencephaly, in Kallman syndrome, and in a number of
malformation syndromes, the extent to which it determines olfactory n
erve development, as well as the part it plays in the morphogenesis of
the nasal structures, is unknown, Cases of arhinencephaly ascertained
at autopsy were studied in an effort to better understand the relatio
nships between the olfactory nerve, bulb, and facies. Based on these s
tudies, it is concluded that both olfactory receptor cells and olfacto
ry nerves are present in arhinencephaly, that the olfactory nerves did
not make contact with the brain in these cases, that the presence of
olfactory nerves is independent of the severity of the central nervous
system malformation, and that the shape of the nasal sturctures is no
t dependent on the presence of the olfactory nerve. (C) 1995 Wiley-Lis
s, Inc.