Cochlear implantation in superficial siderosis: a viable option?

OBJECTIVE

Superficial siderosis of the central nervous system is characterized by accumulation of haemosiderin in the subpial layers of the brain and spinal cord. The evidence largely suggests a retro-cochlear cause for hearing loss with questionable involvement of cochlea. We present our experience with two patients of superficial siderosis who underwent cochlear implantation, and discuss their outcomes and the underlying pathology.

METHODS

The first patient developed a gradually progressive, profound hearing loss over 25 years, clinical diagnosis being made on MRI scans. The second patient was referred to us with bilateral sensorineural hearing loss, tinnitus, ataxia, dementia, seizures, and visual impairment.

METHODS

Both underwent cochlear implantation for auditory rehabilitation. The first patient gained significant benefit, whereas the second patient has not developed any meaningful auditory stimulation at 9-month post-operative follow-up.

CONCLUSIONS

Hearing loss due to superficial siderosis even though predominantly retro-cochlear may be successfully rehabilitated with a cochlear implant. However, outcomes are variable and more evidence regarding experience with cochlear implant in such patients with long-term follow-up is desirable.