Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss

Abstract

We present a 50-year-old man who was investigated for sensorineural hearing loss. On MRI of the brain superficial siderosis of the central nervous system was seen, while MRI of the spine revealed an ependymoma of the cauda equina. This case illustrates the importance of performing T2-weighted imaging of the brain and posterior fossa when sensorineural hearing loss is present. Spine imaging is mandatory when superficial siderosis of the brain is diagnosed without identification of a bleeding source in the brain.

Cochlear implantation in patients affected by superficial hemosiderosis of the central nervous system

“…Only three patients (patients no. 7,8,12) in two different reports [2,3] did not have any benefit from CI. Patient no.…”

“…Two types of SH-CNS are reported in the literature: idiopathic and secondary [12]. In the first one, there are no documented sources of bleeding.…”

“…The gradient-echo T2* and SE T2-weighted sequences are extremely sensitive to hemosiderin deposition due to the effect of magnetic susceptibility. This is shown as a marginal hypointense signal, which can outline the brainstem, cerebellum, cranial nerves, spinal cord and also the brain surface located within the subarachnoid space and the subpial tissue [6,12,16]. Neuroradiological evaluation (MRI, angio-MRI, angiography) is also recommended to identify the source of bleeding.…”

Superficial Siderosis of the Central Nervous System: A 70-Year-Old Man with Ataxia, Depression and Visual Deficits

“…1). The patient did not show a hearing loss otherwise occurring in 95% of patients with SSNS [2,4,11]. The main neurosensorial deficit presented, since the beginning of the patient's medical history, was a marked impairment of visual perception, due to unrecognized optical neuritis.…”

“…It is important to know the broad spectrum of unusual signs and symptoms associated with this markedly disabling disorder, in order to prevent a delay in diagnosis. A detailed neuropsychiatric evaluation along with visual acuity (not only hearing capacity) assessment and targeted brain and spine MR studies [11] should be done in patients with suspected SSNS.…”