Auditory brainstem responses and usefulness of hearing aids in hearing impaired children with Cornelia de Lange syndrome.
OBJECTIVE
To examine auditory brainstem evoked responses (ABRs) of children with Cornelia de Lange syndrome (CDLS) to evaluate hearing and the utility of hearing aids in hearing impaired cases.


METHODS
Thirteen Japanese infants and children with CDLS were studied. Behavioral observation audiometry and ABR were used to evaluate hearing.


RESULTS
Four different ABR patterns at 85 dB clicks were observed: no response in either ear (6 patients); clear ABRs in both ears (2 patients); no response in one ear but ABRs recorded in the other ear (3 patients); and no peaks after wave III in one ear and ABRs recorded in the other ear (2 patients). However, in 2 patients with no response in either ear at the first measurement, ABRs were recorded in one ear within 2 years. Three out of 13 patients exhibited better responses to sound through the use of hearing aids and auditory training.


CONCLUSIONS
The fitting of hearing aids and early consistent training have a significant effect on auditory development in CDLS children in terms of making them aware of sound localization and the different types of environmental sound.
D001299:Audiometry / Q000379:methods; D002675:Child, Preschool; D003635:De Lange Syndrome / Q000503:physiopathology*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D006310:Hearing Aids; D006319:Hearing Loss, Sensorineural / Q000503:physiopathology* / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D016896:Treatment Outcome