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.