PONTINE HYPOPLASIA IN A CHILD WITH SENSORINEURAL DEAFNESS

A 2-year-old girl with bilateral sensorineural deafness showed pontine hypoplasia as well as a bulging contour of the pontine tegmentum on m agnetic resonance imaging (MRI). There were no bilateral responses of brainstem auditory-evoked potentials (BAEPs). The absent late componen ts of blink reflex (BR) indicated brainstem dysfunction. Chromosomal a bnormalities and neurodegenerative or neurometabolic disorders, which might have been the cause of the pontine hypoplasia, were ruled out. T he authors describe a rare case with pontine hypoplasia combined with sensorineural deafness and absent blink reflex and suggest that the br ainstem in this child may become involved in the early gestation perio d. (C) 1997 Elsevier Science B.V.