G Michael Halmagyi1,2, Geoffrey D Parker3, Luke Chen4, Miriam S Welgampola4,5, John D G Watson6, Michael H Barnett4,7, Michael J Todd4, Shadi El-Wahsh4, Victoria Rose8, Marcus A Stoodley9, Jeffrey W Brennan10. 1. Neurology Department, Royal Prince Alfred Hospital, Sydney, Australia. gmh@icn.usyd.edu.au. 2. Central Clinical School, University of Sydney, Sydney, Australia. gmh@icn.usyd.edu.au. 3. Radiology Department, Royal Prince Alfred Hospital, Sydney, Australia. 4. Neurology Department, Royal Prince Alfred Hospital, Sydney, Australia. 5. Central Clinical School, University of Sydney, Sydney, Australia. 6. Neurology Department, Sydney Adventist Hospital, Sydney, Australia. 7. Brain Mind Centre, University of Sydney, Sydney, Australia. 8. Audiology Unit, Royal Prince Alfred Hospital, Sydney, Australia. 9. Neurosurgery Department, Macquarie University Hospital, Sydney, Australia. 10. Neurosurgery Department, Royal Prince Alfred Hospital, Sydney, Australia.
Abstract
PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.
PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.
Authors: Duncan Wilson; Fiona Chatterjee; Simon F Farmer; Peter Rudge; Mark O McCarron; Peter Cowley; David J Werring Journal: Ann Neurol Date: 2017-01-28 Impact factor: 10.422
Authors: Jürgen Beck; Christian T Ulrich; Christian Fung; Jens Fichtner; Kathleen Seidel; Michael Fiechter; Kety Hsieh; Michael Murek; David Bervini; Niklaus Meier; Marie-Luise Mono; Pasquale Mordasini; Ekkehard Hewer; Werner J Z'Graggen; Jan Gralla; Andreas Raabe Journal: Neurology Date: 2016-08-26 Impact factor: 9.910
Authors: H H Weekamp; P L M Huygen; J L Merx; H P H Kremer; Cor W R J Cremers; Neil S Longridge Journal: Otol Neurotol Date: 2003-09 Impact factor: 2.311