Abstract

Abstract Introduction In otosclerosis, spongiotic bone replaces normal bone in the ossicular chain. Focal deposits may also be found within the cochlea leading to sensorineural hearing loss refractory to conventional treatment. Cochlear implantation can play an important role in the management of otosclerosis in these patients. Our study objective is to establish hearing outcomes following cochlear implantation in patients with otosclerosis. Method Systematic review and narrative synthesis. Databases searched: Medline, PubMed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Results Searches identified 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. Significant heterogeneity existed in terms of outcomes and methods of reporting, which precluded a meta-analysis. Access to good rehabilitation support is essential to achieving the good hearing outcomes and Patient Reported Outcome Measures that can be expected by 12 months post-implantation in most cases. There was significant association between the radiological severity of otosclerosis and an increase in surgical and post-operative complications. Post-operative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment. Conclusions Hearing outcomes are typically good, but patients should be counselled on associated surgical complications which may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counselling.

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