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

Abstract

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