Cochlear implantation in children with enlarged vestibular aqueduct (EVA): relationship to Pendred syndrome diagnosis, surgical outcomes, and radiological findings

Introduction The cochlear implant (CI) procedure in patients with inner ear malformations is challenging. aim of this study was to evaluate auditory perception and speech development children enlarged vestibular aqueduct (EVA) relate the results diagnosis Pendred syndrome (PS), imaging findings, surgical course, postoperative period. Material methods group consisted 49 EVA, aged 11 months 15 years, severe profound hearing loss. EVA included 22 PS 27 nonsyndromic (NSEVA). control 46 deafness. Outcomes after implantation were evaluated annually for at least 10 years. Auditory performance assessed by categories (CAP) Meaningful Integration Scale (MAIS). Speech outcomes Intelligibility Rating (SIR) Meaning Use (MUSS). Genetic counselling, studies, testing also when available. Results All benefited from implants, especially performed before age 3 After CI, (PS NSEVA) achieved a steeper rate increase intelligibility, demonstrating higher scores each follow-up point compared (NS). There no differences between NSEVA patients. In addition most commonly diagnosed malformation incomplete partition type 2 (IP-2), presence which negatively affected outcomes. During cochleostomy, cerebrospinal fluid (CSF)/perilymph leakage observed 50% implanted ears, but its did not affect final Conclusions Early associated satisfactory EVA. Due syndrome, detailed temporal bone indicated. Despite frequent occurrence CSF/perilymph during benefit satisfactorily implantation.