1309 Cochlear Implantation in Patients with Usher Syndrome: A Systematic Review of The Literature

Abstract Introduction Usher syndrome is a common cause of deaf-blindness characterised by progressive visual loss with congenital (types 1 and 2) or adult-onset (type 3) sensorineural hearing loss. Cochlear implantation one few effective options to enable patient access useful sound. Auditory rehabilitation after cochlear may be limited deterioration retinal function. Our objective was evaluate the auditory outcomes in patients syndrome. Method Systematic review Medline via PubMed, Ovid EMBASE, Web Science, CENTRAL clinicaltrials.gov performed up 30/03/2020, conducted accordance PRISMA statement. Patient demographics, comorbidity, details implantation, quality life (QOL) were extracted summarised. CRD 42020185102. Results 32 studies reported over 215 implants 186 syndrome, comprising subtypes (56 patients), 2 (9 3 (23 not specified (98 patients). Where reported, improved sound detection, speech perception, intelligibility, patient-reported majority Conclusions Outcomes comparable those without multiple sensory handicap. As clinical practice has evolved emphasise early, bilateral oral education it likely that these underestimate contemporary implant among young children To avoid multisensory deficits incurred poor secondary late syndrome-related loss, early crucial prelingually deaf group.