[Evaluation of hearing and speech rehabilitation after cochlear implantation in children with Waardenburg syndrome].

Objective: By comparing the hearing and speech rehabilitation effects of cochlear implantation （CI） in children with Waardenburg syndrome （WS） and children with common deafness genes （ SLC26A4 , GJB2 ） in the Chinese population, and the hearing and speech rehabilitation effects of bilateral CI and unilateral CI in children with WS, to provide a reference for clinical CIin children with WS. Methods: Follow up and return visit 72 pedestrian cochlear implant children with severe and above sensorineural hearing loss and clear gene mutation type diagnosed by Kunming Children's Hospital from 2017 to 2019, including 24 cases in the WS group, 24 cases in the control group （ SLC26A4 deafness group and GJB2 deafness group）. All enrolled children were evaluated for auditory and speech ability 12 months after startup. Results: The hearing aid threshold, the correct recognition rate of speech recognition ability evaluation, IT-MAIS / MAIS score rate, CAP score, SIR score, there was no significant difference（ P >0.05）. The correct recognition rates of IT-MAIS / MAIS score, SIR score, natural environment sound recognition, vowel recognition, tone recognition, monosyllabic word recognition, disyllabic word recognition and short sentence recognition in children with WS bilateral CI were significantly higher than those in children with WS unilateral CI （ P <0.05）. There was no significant difference in CAP score, initial recognition and correct recognition rate of trisyllabic words between children with WS bilateral CI and children with WS unilateral CI （ P >0.05）. Conclusion: Common deafness genes in children with WS and Chinese population （ SLC26A4 , GJB2 ） the effect of cochlear implantation on hearing and speech rehabilitation of sick children is equivalent. For children with severe and above sensorineural hearing loss associated with this syndrome, CI can be used clinically to improve their hearing and speech ability. WS bilateral CI has advantages in some hearing and speech abilities compared with unilateral CI, so those whomeet the conditions should be encouraged bilateral implantation.