Dysmorphic Features and Cochlear Implantation Outcome in Children with Sensorineural Hearing Loss; the Headmost Study

Introduction: Recently, treatment of children with severe-to-profound sensorineural hearing loss (SNHL) has been influenced by diagnostic improvements and technological treatment advances, specifically new cochlear implant prospects. Multiple handicaps children and children with syndromes and conditions resulting disabilities, such as dual sensory loss, cerebral palsy, somatic abnormalities, and autistic spectrum disorder, are now not routinely precluded from receiving a cochlear implant. The primary aim of this study was to determine the effects of dysmorphic feature on cochlear implant outcome. Materials and Methods: In this cohort study, we evaluated 336 cochlear implanted children from 2007 to 2009. The case group consists of 53 patients (15/7%) with dysmorphic features and control group consisted at 53 patients with normal features and without behavioral and developmental disorders. All patients received auditory and speech rehabilitation and we evaluated their speech and auditory outcome. One year after cochlear implantation, the patient was assessed by categories of auditory perception (CAP) and speech intelligibility rating (SIR) tests. Results: We included 106 out of 336 cochlear implanted children, with the mean age of 30.42 +/- 12.16 (maximum 48 months), 52 cases (49.1%) were girls and 54 (50.9%) were boys. There was a significant difference in SIR between case and control groups (3.26 +/- 0.98) versus (4.06 +/- 0.94) (P < 0.001), and a significant difference in CAP (4.09 +/- 1.26) versus (5.43 +/- 1.23) as well (P < 0.001). Conclusion: In this study, the prevalence of dysmorphic feature in children with severe-to-profound SNHL is 15.7%. One year after cochlear implant SIR and categories of auditory perception in these patients are significantly lower than children without dysmorphic feature, but cochlear implant will help these children.