Hearing impairment in two boys with Cornelia de Lange syndrome.
We analyzed brainstem auditory evoked potentials in 2 boys with Cornelia de Lange syndrome. One patient showed no responses with 100 dB hearing level (HL) stimuli. The other showed a normal latency of wave I, a normal I-V interpeak latency with 80 dB HL stimuli, and an elevated threshold of wave V (40 dB HL). It is likely that these abnormal findings reflect the presence of sensorineural hearing impairment.
Ichiyama
T
T
Department of Pediatrics, Yamaguchi University School of Medicine, Japan.
Hayashi
T
T
Tanaka
H
H
Nishikawa
M
M
Furukawa
S
S
D002675:Child, Preschool; D003635:De Lange Syndrome / Q000175:diagnosis*; D003638:Deafness / Q000175:diagnosis*; D016057:Evoked Potentials, Auditory, Brain Stem; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009474:Neurons / Q000473:pathology