title: Monosymptomatic clinically isolated syndrome with sudden sensorineural hearing loss: case report and critical review of the literature.
abstract: BACKGROUND
  Isolated cranial nerve involvement is rare in patients with multiple sclerosis (10.4%) and extremely rare is an eighth nerve palsy, especially in the context of a clinically isolated syndrome (<1%).
  
  
  METHODS
  A 34-year-old male presented with a history of left-sided tinnitus and sudden sensorineural hearing loss (SSNHL). Magnetic resonance imaging of the brain revealed >9, nonenhancing periventricular and corpus callosum lesions. Brainstem auditory evoked potentials were abnormal, ipsilateral to the affected ear, consistent with the presumed underlying demyelinating pathology. Visual evoked potentials showed bilateral prolonged P100 latencies. Oligoclonal bands were not detected in the cerebrospinal fluid, but IgG index was marginally elevated. After administration of corticosteroids, the patient recovered auditory function over a several month period.
  
  
  CONCLUSIONS
  This report describes a case of SSNHL in the context of magnetic resonance imaging of the brain and electrophysiological findings consistent with a demyelinating etiology. SSNHL is a rare and possibly underrecognized manifestation of clinically isolated syndrome.
authors.affiliation: A' Department of Neurology, Medical School, National & Kapodistrian University of Athens, Aeginition Hospital, Athens, Greece. managnost@med.uoa.gr
authors.forename: Maria C
authors.initials: MC
authors.lastname: Anagnostouli
authors.forename: Elias S
authors.initials: ES
authors.lastname: Sotirchos
authors.forename: Ioannis
authors.initials: I
authors.lastname: Zalonis
authors.forename: Fotini
authors.initials: F
authors.lastname: Christidi
authors.forename: Stavros
authors.initials: S
authors.lastname: Korres
authors.forename: Michael
authors.initials: M
authors.lastname: Rentzos
authors.forename: Panagiota
authors.initials: P
authors.lastname: Davaki
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology; D003711:Demyelinating Diseases / Q000150:complications / Q000175:diagnosis*; D016057:Evoked Potentials, Auditory, Brain Stem; D005074:Evoked Potentials, Visual; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000209:etiology; D003639:Hearing Loss, Sudden / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D014012:Tinnitus / Q000175:diagnosis / Q000209:etiology