Abstract
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. Subjects and methods: Thirteen Japanese infants and children with CDLS were studied. Behavioral observation audiometry and ABR were used to evaluate hearing. Result: 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.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callMore From: International Journal of Pediatric Otorhinolaryngology
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
