Send to

Choose Destination
See comment in PubMed Commons below
Int J Pediatr Otorhinolaryngol. 1995 Mar;31(2-3):137-46.

Auditory brainstem responses in children with Cornelia de Lange syndrome.

Author information

Department of Otolaryngology, Faculty of Medicine, University of Tokyo, Japan.


Auditory brainstem evoked responses (ABRs) were recorded in 10 Japanese infants and children with Cornelia de Lange syndrome to determine the level of the lesion causing their poor response to sound. Behavioral audiometry showed severe threshold elevation compared to an age-matched control group. ABR thresholds, peak latencies of wave I and IV and the peak interval latency of waves I-V were measured. ABR abnormalities of 4 types: no ABRs following 85 dB clicks in both ears (4 cases), no ABRs in one ear but normal ABR in the other (2 cases), mild wave V threshold elevation of ABR with a markedly prolonged wave V (2 cases) and normal ABR pattern in the one side and abnormal ABR pattern in the opposite side suggesting brainstem immaturity for age (2 cases). Our results demonstrate a high prevalence of peripheral sensorineural hearing loss in Cornelia de Lange syndrome, but brainstem immaturity was not ruled out in 2 cases.

[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Loading ...
    Support Center