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      Vestibular function in superficial siderosis

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          Abstract

          Background

          Superficial siderosis (SS) is caused by repeated or continuous bleeding into the subarachnoid space that results in iron from hemoglobin (hemosiderin) being deposited on the surface of the brain. Clinically, the condition is characterized by sensorineural deafness, ataxia, and pyramidal signs. However the mechanism of peripheral vestibular disturbance was not revealed. We show the vestibular function of SS patients, and shed light on saccule-inferior vestibular nerve.

          Methods

          Over the past 9 years, 5 patients were definitively diagnosed with SS by MRI in our department. These patients were subjected to balance testing.

          Results

          Vestibular evoked myogenic potential (VEMP) was observed in patients who had suffered from SS for a short period but tended to be diminished or absent in patients who had suffered from the condition for a longer period.

          Conclusions

          These findings in SS patients suggest that saccule-inferior vestibular function is maintained at early stages of the disorder. Our study may help to clarify the mechanism of SS.

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          Most cited references37

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          Myogenic potentials generated by a click-evoked vestibulocollic reflex.

          N F Skuse, James Colebatch, G. Michael Halmagyi (1994)
          Electromyograms (EMGs) were recorded from surface electrodes over the sternomastoid muscles and averaged in response to brief (0.1 ms) clicks played through headphones. In normal subjects, clicks 85 to 100 dB above our reference (45 dB SPL: close to perceptual threshold for normal subjects for such clicks) evoked reproducible changes in the averaged EMG beginning at a mean latency of 8.2 ms. The earliest potential change, a biphasic positive-negativity (p13-n23), occurred in all subjects and the response recorded from over the muscle on each side was predominantly generated by afferents originating from the ipsilateral ear. Later potentials (n34, p44), present in most but not all subjects, were generated bilaterally after unilateral ear stimulation. The amplitude of the averaged responses increased in direct proportion to the mean level of tonic muscle activation during the recording period. The p13-n23 response was abolished in patients who had undergone selective section of the vestibular nerve but was preserved in subjects with severe sensorineural hearing loss. It is proposed that the p13-n23 response is generated by activation of vestibular afferents, possibly those arising from the saccule, and transmitted via a rapidly conducting oligosynaptic pathway to anterior neck muscles. Conversely, the n34 and p44 potentials do not depend on the integrity of the vestibular nerve and probably originate from cochlear afferents.
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            Vestibular evoked potentials in human neck muscles before and after unilateral vestibular deafferentation.

            G. Michael Halmagyi, James Colebatch (1992)
            Article 0 comments Cited 76 times – based on 0 reviews     Review now
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              Superficial siderosis of the central nervous system.

              Gonneke W. J. M. Stevens, P Rudge, J Fearnley (1995)
              There have been 87 cases of superficial siderosis of the CNS reported in the world literature and 63 cases had developed the clinical syndrome with sufficient details to be reviewed. It is a distinct clinical syndrome characterized by sensorineural deafness (95%), cerebellar ataxia (88%) and pyramidal signs (76%). Other features include dementia (24%), bladder disturbance (24%), anosmia (at least 17%), aniscoria (at least 10%) and sensory signs (13%). Less frequent features are extra-ocular motor palsies, neck or backache, bilateral sciatica and lower motor neuron signs (5-10% each). Males are more often affected than females (3:1). The age of onset ranged from 14 to 77 years, age at death from 29 to 78 years and duration until death from 1 to 38 years excluding premature death due to the underlying cause or as a result of surgery. Up to 27% become bed bound at 1-37 years from the first symptom due to either cerebellar ataxia, a myelopathic syndrome or both. Symptomatic subarachnoid haemorrhage occurred in 37% and the CSF was haemorrhagic and/or xanthochromic in 75%. It is now accepted that superficial siderosis is due to chronic subarachnoid haemorrhage and a source of bleeding has been reported in 54% of cases; it was either due to dural pathology (47%) including a CSF cavity lesion or cervical root lesion, a vascular tumour (35%) or a vascular abnormality (18%). Arguments are presented that the remaining cases were also due to chronic haemorrhage and that there is no evidence for a non-haemorrhagic form of superficial siderosis. There have been 14 incidental cases diagnosed by MRI or at post-mortem with no symptoms attributable to superficial siderosis during life, supporting the notion of a pre-symptomatic phase to the illness. In 22 patients who had developed the syndrome, the duration of this pre-symptomatic phase could be calculated and ranged from 4 months to 30 years with an average of 15 years. At present the most promising treatment for superficial siderosis is surgical ablation of the bleeding sources.
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                Author and article information

                Contributors
                Toru Miwa
                Ryosei Minoda
                Hidetake Matsuyoshi
                Journal
                Journal ID (nlm-ta): BMC Ear Nose Throat Disord
                Journal ID (iso-abbrev): BMC Ear Nose Throat Disord
                Title: BMC Ear, Nose, and Throat Disorders
                Publisher: BioMed Central
                ISSN (Electronic): 1472-6815
                Publication date Collection: 2013
                Publication date (Electronic): 23 April 2013
                Volume: 13
                Page: 5
                Affiliations
                [1 ]Department of Otolaryngology and Head and Neck Surgery, Kumamoto University, 1-1-1 Honjo, Kumamoto, Japan
                [2 ]Matsubase ENT Clinic, Kumamoto, Japan
                Article
                Publisher ID: 1472-6815-13-5
                DOI: 10.1186/1472-6815-13-5
                PMC ID: 3651722
                PubMed ID: 23617695
                SO-VID: 46617f63-0684-4d18-adf9-aac7ebbadf72
                Copyright © Copyright ©2013 Miwa et al.; licensee BioMed Central Ltd.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 12 July 2012
                Date accepted : 9 April 2013
                Categories
                Subject: Research Article

                ScienceOpen disciplines: Otolaryngology
                Keywords: hemosiderin,superficial siderosis,vertigo,vestibular function,clinical neurology examination
                Data availability:
                ScienceOpen disciplines: Otolaryngology
                Keywords: hemosiderin, superficial siderosis, vertigo, vestibular function, clinical neurology examination

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