title: Auditory profile in superficial siderosis of the central nervous system: a prospective study.
abstract: OBJECTIVE
  To identify auditory pathology resulting from superficial siderosis of the central nervous system (SSCN), auditory site of lesion, and a clinical profile for differential diagnosis and development of recommendations.
  
  
  METHODS
  Prospective study.
  
  
  METHODS
  Academic clinical center.
  
  
  METHODS
  Ten participants with SSCN (the largest prospective evaluation of audiologic status reported to date).
  
  
  METHODS
  Demographics, clinical characteristics and history, audiometric evaluation, and Tinnitus Handicap Inventory (THI).
  
  
  METHODS
  Type and degree of hearing loss, relationship to clinical course of SSCN, and expected results based on age and sex.
  
  
  RESULTS
  Sensorineural hearing loss (SNHL) is the most common symptom in SSCN (100%). Tinnitus (100%), imbalance (80%), and gait disorder (80%) were also frequently reported. Hearing loss is typically bilateral, asymmetric, progressive, sloping, and exceeds expected hearing loss related to age and sex. Hearing loss may be cochlear and/or retrocochlear in origin. Decreased word recognition is possible, and traditional amplification may offer limited benefit.
  
  
  CONCLUSIONS
  We observed significant audiometric findings in all participants. SSCN variably and pathologically targets the auditory system without regard for duration of disorder. A long asymptomatic phase and lack of predictive relationship between duration and severity of hearing loss makes suspicion of SSCN based solely on audiometric battery difficult; however, asymmetric hearing loss exceeding expectations, particularly with history of head trauma or previous neurosurgical procedure, should raise a red flag and trigger further medical evaluation including MRI. Diagnosis of SSCN may alter expectations for audiologic prognosis and is a critical component for comprehensive management of SSCN patients.
authors.affiliation: Head and Neck Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA. sydlows@ccf.org
authors.forename: Sarah A
authors.initials: SA
authors.lastname: Sydlowski
authors.forename: Michael
authors.initials: M
authors.lastname: Levy
authors.forename: Wendy D
authors.initials: WD
authors.lastname: Hanks
authors.forename: M Diane
authors.initials: MD
authors.lastname: Clark
authors.forename: R Steven
authors.initials: RS
authors.lastname: Ackley
mesh_terms: D000328:Adult; D000368:Aged; D001306:Auditory Pathways / Q000473:pathology / Q000503:physiopathology*; D001307:Auditory Perception / Q000502:physiology*; D002493:Central Nervous System Diseases / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D003051:Cochlea / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012806:Siderosis / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D014012:Tinnitus / Q000150:complications / Q000473:pathology / Q000503:physiopathology*