Todd A Hardy1,2, Rachael L Taylor3, Jessica Qiu4, Billy O'Brien5, Sumana Gopinath6, Benjamin Trewin4, Penelope J Spring4, Mohamed Shaffi7, Sam J Bolitho8, Roger J Garsia9, Richard Roxburgh10, Deborah F Mason11, Jerome Ip12, Fiona Chan13, Luke Chen12, Ian Wilson14, Heidi N Beadnall12,9, Michael H Barnett12,15, John D E Parratt12,16, John D G Watson17,18, Miriam S Welgampola15, Stephen W Reddel4, Benjamin Tsang13, G Michael Halmagyi15. 1. Department of Neurology, Concord Hospital, University of Sydney, Sydney, NSW, Australia. thardy@med.usyd.edu.au. 2. Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia. thardy@med.usyd.edu.au. 3. Department of Physiology and Centre for Brain Research, The University of Auckland, Auckland, New Zealand. 4. Department of Neurology, Concord Hospital, University of Sydney, Sydney, NSW, Australia. 5. Department of Neurology, Gosford Hospital, Gosford, NSW, Australia. 6. Department of Neurology, Campbelltown Hospital, Sydney, NSW, Australia. 7. University of Notre Dame, Sydney, NSW, Australia. 8. Department of Neurology, St Vincent's Hospital, Sydney, NSW, Australia. 9. Department of Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. 10. Department of Neurology, Auckland City Hospital, Auckland, New Zealand. 11. Department of Neurology, Christchurch Hospital, Christchurch, New Zealand. 12. Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia. 13. Department of Neurology, Sunshine Coast Hospital, Sunshine Coast, Qld, Australia. 14. Department of Neurology, Cairns Hospital, Cairns, Qld, Australia. 15. Department of Neurology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. 16. Department of Neurology, Royal North Shore Hospital, Sydney, NSW, Australia. 17. Section of Neurology, Sydney Adventist Hospital, Sydney, NSW, Australia. 18. Discipline of Medicine, University of NSW, Sydney, Australia.
Abstract
OBJECTIVE: We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS: The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes. RESULTS: Our findings demonstrate that 29 of our 30 patients with Susac syndrome developed neuro-otological symptoms such as hearing loss, disequilibrium, tinnitus or vertigo during their disease course. Hearing loss was the most common neuro-otological symptom occurring in 93% of patients. A rising configuration of low-frequency greater than the high-frequency sensorineural hearing loss was the most characteristic finding on audiological testing (37% of reviewed audiograms). Disproportionately poor speech discrimination was identified in 20% of cases, and one case demonstrated a retrocochlear pattern on electrophysiological testing. Four patients required hearing aids and a further two patients required a cochlear implant due to severe hearing loss. Two out of two treated patients had improvements in hearing after the prompt administration of corticosteroids, indicating the potential for recoverable hearing loss if relapses are treated early. Effects on vestibular function were variable in ten patients who were tested, with most showing preservation of function despite significant hearing loss. CONCLUSIONS: Neuro-otological symptoms in Susac syndrome are almost universal. In the correct clinical context, a rising configuration of low to high-frequency sensorineural hearing loss should prompt consideration of Susac syndrome. Treatment of inner ear symptoms in Susac syndrome requires further research as early immunotherapy may be beneficial.
OBJECTIVE: We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS: The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes. RESULTS: Our findings demonstrate that 29 of our 30 patients with Susac syndrome developed neuro-otological symptoms such as hearing loss, disequilibrium, tinnitus or vertigo during their disease course. Hearing loss was the most common neuro-otological symptom occurring in 93% of patients. A rising configuration of low-frequency greater than the high-frequency sensorineural hearing loss was the most characteristic finding on audiological testing (37% of reviewed audiograms). Disproportionately poor speech discrimination was identified in 20% of cases, and one case demonstrated a retrocochlear pattern on electrophysiological testing. Four patients required hearing aids and a further two patients required a cochlear implant due to severe hearing loss. Two out of two treated patients had improvements in hearing after the prompt administration of corticosteroids, indicating the potential for recoverable hearing loss if relapses are treated early. Effects on vestibular function were variable in ten patients who were tested, with most showing preservation of function despite significant hearing loss. CONCLUSIONS: Neuro-otological symptoms in Susac syndrome are almost universal. In the correct clinical context, a rising configuration of low to high-frequency sensorineural hearing loss should prompt consideration of Susac syndrome. Treatment of inner ear symptoms in Susac syndrome requires further research as early immunotherapy may be beneficial.
Authors: James D Triplett; Jessica Qiu; Billy O'Brien; Sumana Gopinath; Benjamin Trewin; Penelope J Spring; Mohamed Shaffi; Jerome Ip; Fiona Chan; Luke Chen; Ian Wilson; Claire Muller; Heidi N Beadnall; Mike Boggild; Anneke Van der Walt; Richard Roxburgh; Nabil Seery; Tomas Kalincik; Michael H Barnett; John D E Parratt; Stephen W Reddel; Benjamin Tsang; Todd A Hardy Journal: Eur J Neurol Date: 2022-03-25 Impact factor: 6.288