Vascular anomalies of the inner ear have been documented in only a few isol
ated case reports. The goal of our study was to describe, qualify and quant
ify vascular variations of the inner ear in 122 temporal bones from 64 pedi
atric subjects aged between 0 and 10 years. The average age was 11.6 months
. Horizontal sections of the temporal bone, examined by light microscopy, r
evealed vessels coursing freely through the perilymphatic space of the coch
lea, especially in the apical turn. Other findings included abnormally wide
vessels in the stria vascularis as well as a vascular malformation of the
internal auditory canal. Our study demonstrated more atypical Vessels in th
e cochlea than in the vestibular labyrinth. We found a statistically signif
icant positive correlation between vascular variations of the inner ear and
concomitant cardiac anomalies or endolymphatic hydrops. We also discuss th
e possible etiology and potential significance of these findings in terms o
f disturbances of the function of the inner ear.