Auditory-Vestibular Outcomes in Superficial Siderosis

• Published in: Otolaryngology-head and neck surgery 2011-08, Vol.145 (2_suppl), p.P205-P205
• Main Author: Sydlowski, Sarah
• Format: Article
• Language: English
• ISSN: 0194-5998; 1097-6817
• DOI: 10.1177/0194599811415823a232

Objective: 1) Identify otologic clinical presentation of superficial siderosis of the central nervous system (SSCN). 2) Use audiologic assessment batteries to analyze variable presentation of SSCN. 3) Highlight the importance of site of lesion testing to identify and develop clinical management plans for affected individuals. Method: Ten participants with SSCN were recruited in 2009. Prospective study measures included audiologic and evoked potential battery, including audiometry, speech testing, immittance, otoacoustic emissions, auditory brainstem (ABR) testing, and vestibular evoked myogenic potential (VEMP). Analysis resulted in development of typical audiologic clinical presentation for this disorder. Results: This is the largest prospective audiovestibular study of SSCN to date. SSCN is a neurodegenerative disorder resulting from chronic, episodic subarachnoid hemorrhage, and hemosiderin deposition. Diffuse symptoms, including hearing loss, tinnitus, gait ataxia, and disordered balance result from preferential demyelination of the auditory-vestibular nerve and cerebellum. Sensorineural hearing loss was present in 100% of participants and may be due to cochlear and/or retrocochlear involvement. Study results suggest clinically characteristic findings that are relevant for otolaryngologists, otologists, and audiologists. SSCN may be underdiagnosed due to clinical presentation that resembles presbycusis and a long asymptomatic phase up to 30 years. Conclusion: Characteristic otologic findings in SSCN include hearing loss, tinnitus, and balance and/or gait disorder. Despite proclivity for audiovestibular damage, SSCN is not well-recognized by hearing health professionals. Due to certain involvement of central and peripheral auditory-vestibular structures, understanding of clinical presentation, typical progression, and audiovestibular prognosis is critical.