Cochlear implantation in children with internal ear malformations.

摘要: OBJECTIVE To evaluate surgical aspects and results of cochlear implantation in inner ear malformations. STUDY DESIGN Retrospective cohort study. SETTING Ear, nose, throat department a tertiary referral hospital. PATIENTS Out 260 implanted children, 18 (6.9%) had malformations: complex cochleovestibular malformation (n = 11), common cavity 1), enlarged vestibular aqueduct (EVA) 6). Deafness was progressive 12 cases (G1) congenital 6 (G2). Genetics lead to diagnosis 13 cases: PSD mutation Waardenburg syndrome negative (1). Mean age at implant 7.8 years. follow-up period 48 months. MAIN OUTCOME MEASURES Medical outcomes were reported. Closed (CSW) open (OSW) set word perception level speech production evaluated each year. The compared pre- postoperatively between the two groups. RESULTS Gusher surgery observed 50% cases, with persistent leak one case. No facial injury or infectious complications observed. At months, 83% population achieved more than 75% recognition CSW, versus 16% before (p 0.001). After 2 years, 64% patients OSW. Good oral language seen 76% years 100% 3 55% (respectively, p > 0.05 0.03). 1 year after implant, G1 20% G2 OSW 0.02). 24 40% 0.05). Before 0% good 0.01). year, One child no improvement implantation. CONCLUSIONS major complication seen. Perceptive linguistic variable depended on type deafness. In deafness, perceptive result are expected be good. variable.