Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System

Otol Neurotol. 2018 Jul;39(6):e468-e474. doi: 10.1097/MAO.0000000000001844.


Objective: To describe the vestibular function in patients with superficial siderosis of the central nervous system (SSCN).

Study design: Retrospective analysis.

Setting: Tertiary referral center.

Patients: Ten consecutive patients with SSCN. This study is the largest case series of SSCN in which detailed neuro-otological findings, including electronystagmography recording, video head impulse test (vHIT), and posturography, were described.

Interventions: Audiological and neuro-otological examinations, including pure-tone audiometry, distortion product otoacoustic emissions, speech audiometry, auditory brainstem responses, electronystagmography recording, vHIT, and posturography.

Main outcome measures: Pure-tone average, DP level, maximum speech discrimination score, interpeak latency between auditory brainstem responses waves I and V, eye tracking test, examination of optokinetic nystagmus, caloric response, visual suppression, vestibulo-ocular reflex gains, total center of pressure path length, and Romberg's ratio.

Results: Audiological examinations suggested that the sensorineural hearing loss was of a cochlear etiology in 3 ears, a retrocochlear etiology in 11 ears, and a combined cochlear and retrocochlear etiology in 6 ears. Neuro-otological examinations revealed that eight out of nine patients had cerebellar disorders, while all patients also had peripheral vestibular dysfunction.

Conclusion: In addition to cerebellar disorders, SSCN patients suffer from severe peripheral vestibular dysfunction, which can exacerbate the patient's imbalance. When otolaryngologists encounter patients with distinctly progressive sensorineural hearing loss and imbalance, they should include SSCN in the differential diagnosis and perform neuro-otological examinations, including an electronystagmography recording and vHIT and brain magnetic resonance imaging.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Audiometry, Pure-Tone
  • Caloric Tests
  • Central Nervous System Diseases / complications*
  • Cochlear Diseases / complications
  • Electronystagmography
  • Evoked Potentials, Auditory, Brain Stem
  • Female
  • Head Impulse Test
  • Hearing Loss, Sensorineural / etiology
  • Humans
  • Male
  • Middle Aged
  • Otoacoustic Emissions, Spontaneous
  • Reflex, Vestibulo-Ocular
  • Retrospective Studies
  • Siderosis / complications*
  • Speech Discrimination Tests
  • Vestibular Diseases / etiology*