Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients' sensory perception and lifestyle.

Objectives/Hypothesis Facial nerve stimulation (FNS) can occur after cochlear implantation for a small number of recipients. This study aimed to investigate if a correlation exists between the variables involved in FNS. Study Design Retrospective cohort review. Methods There were 32 out of 1,100 cochlear implant recipients who experienced FNS in our clinic between 2010 and 2019. The following variables were recorded from a retrospective chart review: grade of FNS, onset of FNS, the number of channels stimulating FNS, and radiological findings of abnormalities in the inner ear. Statistical analyses were performed to identify a correlation between any of the variables involved. The techniques used to reduce FNS were analyzed. Results Eleven adult ears had progressive hearing loss, three had idiopathic sudden sensorineural hearing loss (SNHL), and one congenital SNHL. All pediatric ears were diagnosed with congenital SNHL, except for one ear with idiopathic sudden SNHL. The grade of FNS ranged from mild stimulation or slight motion in the eye, mouth, nasolabial, or forehead regions (n = 8) to total severe stimulation of the facial musculature and/or severe pain (n = 3). The onset of FNS occurred immediately after activation for nine ears, and up to 16 months later for the other subjects. A significant correlation was observed between the number of channels stimulating FNS, the grade of FNS, and the radiological findings of the inner ear. FNS was completely resolved for 30 ears and partially resolved for two ears. Conclusions FNS can occur any time after cochlear implantation and can affect both adult and pediatric. However, it can be effectively resolved using specific fitting techniques. Level of Evidence 2c Laryngoscope, 131:374–379, 2021

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