Authors: Giampietro Ricci Franco Trabalzini Mario Faralli Luca D’ascanio Cristina Cristi Egisto Molini
Publish Date: 2013/03/24
Volume: 271, Issue: 3, Pages: 489-493
Abstract
CHARGE syndrome is a rare polymalformative disease representing one of the major causes of associated blindness and deafness Bilateral severeprofound sensorineural hearing loss is common in CHARGE children Aim of this study is to present our results in children with “CHARGE syndrome” submitted to cochlear implantation CI The frequency of anatomic anomalies possible variations in the surgical technique of CI and the audiological/rehabilitative benefits attained in our patients are reported we submitted 5 children affected by CHARGE syndrome with profound bilateral sensorineural hearing loss to CI Otoacoustic emissions auditory brainstem response acoustic impedance testing cranial computed tomography and magnetic resonance were carried out preoperatively in all children CI was performed using the mastoidotomy–posterior tympanotomy approach in two cases and the suprameatal approach in three children Infant toddlermeaningful auditory integration scale was used to evaluate kid’s audiological performance before and after CI Intraoperatory findings and postsurgical complications were evaluated Among our patients intraoperative anatomical malformations were cochlear hypoplasia 100 ossicles malformations 100 semicircular canals aplasia 100 oval window atresia 60 round window atresia 40 widening of the aqueduct of the vestibule 20 and aberrant course of the facial nerve 20 No intra or postoperative complication was recorded in relation to implant positioning After a followup ranging from 1 to 45 years only 2/5 patients used oral language as the sole mean of communication 1 started utilizing oral language as the main mean of communication while 2 patients did not develop any linguistic ability In conclusion CI in patients with CHARGE association is feasible and despite results variability it should be carried out in CHARGE children with severe hearing loss as soon as possible Although the selection of a specific surgical technique does not seem to influence the audiological outcome the suprameatal access is valuable when important surgical landmarks ie lateral semicircular canal and incus are absent
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