Cochlear Implant Outcomes in Patients with Neurosarcoidosis

Abstract

<b><i>Introduction:</i></b> Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. <b><i>Results:</i></b> Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. <b><i>Conclusion:</i></b> Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

Cochlear Implantation Outcomes in Patients With Retrocochlear Pathology: A Systematic Review and Pooled Analysis

“…One study suggested waiting over a year to ensure that the hearing will not recover (2), but to remain vigilant and perform serial imaging as cochlear ossification has been reported in this group of patients. Overall, excellent CI outcomes are reported in several small studies in the context of neurosarcoidosis, and these outcomes were stable over time (2,25,52,70).…”

“…Screening by title and abstract excluded 2,345 studies. Full-text review excluded 126 studies, and a total of 53 publications were included in the final analysis (2,5,6,8–10,15–61). A diagram of the search process can be found in Figure 1.…”