Cochlea Implantatation in Patients with Superficial Hemosiderosis

Introduction Superficial hemosiderosisis (SH) is a form of hemosiderosis, in which deposition of hemosiderin in the central nervous system damages the nerve cells. The cause of this form of siderose are chronic cerebral hemorrhage in particular subarachnoid bleeding. The diversity of symptoms depends on the damage in the brain, but in most cases it shows up as the beginning of uni- or bilateral hearing loss, ataxia and pyramidal signs. We investigate whether cochlear implantation (CI) is a treatment option for patients with SH.

Material and method: In the period from 2009 to 2018, we examined (N=10) patients with radiologically secured SH. Of these ten patients, five were treated with CI due to severe hearing loss. We compared pre- and postoperative speech comprehension (Freiburg speech intelligibility test for monosyllables and HSM sentence test).

Result Comparing the speech comprehension of these patients, the language comprehension improved by an average of 26 %  (Freiburg speech intelligibility test for monosyllables) and by 40 %  at rest (HSM sentence test).

Discussion The results show that patients with SH benefit from CI supply with better speech comprehension. The results are below the average of all postlingual deafened CI patients. The presence of a central hearing loss must be detected on the basis of the clinic and the diagnostics and no contraindications should be concluded from it. It is important to ensure that patients are properly preoperatively weighed on their expectations.

Poster-PDF A-1454.pdf

Publication History

Article published online:
13 May 2021