Superficial Siderosis of the Central Nervous System: Neurotological Findings Related to Magnetic Resonance Imaging

摘要

Objective: To compare the neurotological results of five patients suffering from progressive hearing loss and ataxia due to superficial siderosis (SS) with the magnetic resonance imaging (MRI) findings. Study Design: Retrospective case review. Setting: Primary and hospital care center. Participants: Five adult patients with neurotological symptoms of SS underwent MRI with acquisition of our temporal bone protocol including 3D-constructive interference in steady state (3D-CISS) and susceptibility-weighted imaging (SWI). All patients underwent a complete neurotological examination, the results of which were compared with the imaging findings. Main Outcome Measures: Cochleovestibular deficits were present in all five patients as determined by uni- or bilateral bithermal caloric testing and/or video head impulse tests. Sacculocollic reflex was present with increased P1 and N1 latencies on both sides in all patients. MRI revealed an extensive hypointense SWI signal outlining the surface of the brain and the VIIIth cranial nerve in all five patients. Desynchronization of the brainstem auditory evoked potentials (BAEP) and partial or complete absence of the visual suppression of vestibulo-ocular reflex during the pendular rotatory test was particularly consistent with the lesions of the cochleovestibular nerves as well as the cerebellar atrophy seen on MRI. Conclusion: The MRI results with SWI were related to neurotological findings in patients suffering from sensorineural deafness with ataxia due to SS. Our findings support the integration of the SWI and 3D-CISS sequences into the MRI protocol for all patients referred for evaluation of the extent of SS.