Cochlea implantation in patients with superficial hemosiderosis

Abstract

Introduction Superficial hemosiderosis is a sub-form of hemosiderosis in which the deposits of hemosiderin in the central nervous system damage the nerve cells. This form of siderosis is caused by chronic cerebral hemorrhages, especially subarachnoid hemorrhages. The diversity of symptoms depends on the respective damage to the brain, but in most of the cases it shows up as incipient unilateral or bilateral hearing loss, ataxia and signs of pyramidal tracts. We are investigating the question of whether cochlear implantation is a treatment option for patients with superficial hemosiderosis and which strategy of diagnostic procedure has to be ruled out preoperatively. Materials and methods In a tertiary hospital between 2009 and 2018, we examined (N = 5) patients with radiologically confirmed central hemosiderosis who suffered from profound hearing loss to deafness were treated with a cochlear implant (CI). We compared pre- and postoperative speech comprehension (Freiburg speech intelligibility test for monosyllables and HSM sentence test). Results Speech understanding improved on average by 20% (monosyllabic test in the Freiburg speech intelligibility test) and by 40% in noise (HSM sentence test) compared to preoperative speech understanding with optimized hearing aids. Discussion The results show that patients with superficial siderosis benefit from CI with better speech understanding. The results are below the average for all postlingual deaf CI patients. Superficial siderosis causes neural damages, which explains the reduced speech understanding based on central hearing loss. It is important to correctly weigh the patient's expectations preoperatively and to include neurologists within the therapy procedure.

Infratentorial superficial siderosis: report of six cases and review of the literature

“…Therefore, it is necessary to further study the risk of long-term treatment in the future; (3) Other symptomatic treatments: for patients with hearing loss, the suitableness of cochlear implantation can be evaluated. Although hearing loss is caused by post-cochlear lesions, some patients have different degrees of benefits after cochlear implantation (Artukarslan et al, 2022). In this study, 2 of the 6 patients with iSS were confirmed to have a dural tear, but both refused surgery and received deferiprone treatment, while the other patient without a dural tear was also treated with deferiprone.…”

A Case of Patient with Bilateral Cochleovestibular Function Loss due to Infratentorial Superficial Siderosis

“…Cochlear implantation could be considered, as evidenced by reported improvements in sentence and monosyllabic word tests in some cases [4]. Recently, studies emphasizing early diagnosis and active surgical rehabilitation have reported average improvements in tone discrimination of ＞20% following cochlear implantation [10]. The patient in this case, who initially presented with sudden hearing loss and imbalance, received systemic high-dose steroid therapy, intratympanic steroid injections, and hyperbaric oxygen therapy.…”

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

“…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.…”

“…The two who did not benefit had important disease progression resulting in poorer outcomes. One observation made when reviewing the granular individual SS data was that almost every small case series had at least one patient whose disease progressively worsened with concomitant decline in speech performance (15,17,29,42,59).…”