Cochlea implantation in patients with superficial hemosiderosis

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.

Aventricular hemispherotomy: technical note

Hemispheric disconnection in the form of hemispherectomy or hemispherotomy is the most effective way of treating intractable hemispheric epilepsy. Anatomical hemispherectomy approaches have largely been abandoned in most cases due to a higher risk of superficial hemosiderosis, intraoperative blood loss, hydrocephalus, prolonged hospital stay, and mortality compared to the variety of tissue-sparing hemispherotomy techniques. Disconnective hemispherotomy approaches utilize the lateral ventricle as a key component of the surgical corridor. Without a lateral ventricle, disconnective surgery becomes significantly challenging, typically leading to a hemispherectomy. The authors present the case of a patient with severe hemispheric dysplasia without a lateral ventricle on the pathologic side and detail a novel surgical technique for a prone, occipital interhemispheric, tissue-sparing, purely disconnective aventricular hemispherotomy with an excellent surgical outcome.

High noon back pain- severe pseudoradicular pain as a lead symptom of superficial siderosis: a case report

A superficial siderosis of the central nervous system following a traumatic cervical nerve root avulsion usually leads to gait difficulties and hearing loss, whereas back pain is described only rarely. Here we report on the first case with circadian occurrence of severe back pain as the only symptom of a superficial siderosis. We present a case with the most severe pseudoradicular lumbosacral pain occurring daily at noon for the past 5 weeks. The 48-year-old male white patient did not complain of pain in the morning. A traumatic root avulsion 26 years earlier led to a brachial plexus palsy and Horner’s syndrome in this patient. Superficial hemosiderosis in cranial MRI and examination of the cerebrospinal fluid revealing massive red blood cells as well as xanthochromia and elevated protein levels (742 mg/l) led to the diagnosis of a superficial siderosis. A pseudomeningocele caused by a cervical nerve root avulsion is described as a rare reason for superficial siderosis. Surgery on a pseudomeningocele, diagnosed by MRI, led to an immediate disappearance of complaints in our case. Regular neurological investigation and possibly repeated lumbar puncture to exclude superficial siderosis should be considered in cases with severe back pain and a history of traumatic root avulsion. Modern susceptibility weighted MR imaging (SWI) techniques, sensible to the detection of superficial hemosiderosis, might be helpful in the making of a diagnosis.