Cochlear Implantation in Brown-Vialetto-Van-Laere syndrome

J Laryngol Otol. 2011 Mar;125(3):314-7. doi: 10.1017/S0022215110001982. Epub 2010 Oct 19.

Abstract

Objective: To report outcomes for the first known cochlear implantation procedures in two patients with Brown-Vialetto-Van-Laere syndrome.

Patients: Two adult patients (a brother and sister) with post-lingual sensorineural deafness associated with Brown-Vialetto-Van-Laere syndrome. The female patient presented with a milder form of the syndrome.

Intervention: Cochlear implantation.

Main outcome measure: Post-implantation speech discrimination scores.

Results: Auditory evoked potential testing suggested pathological changes in both patients' cochleae, auditory nerves, brainstem and (probably) central auditory pathways. In the male patient, despite implantation of the better ear, the Bamford-Kowal-Bench sentence score was zero at 21 months post-implantation. In the female patient, Bamford-Kowal-Bench sentence scores at six months post-implantation were 25 per cent in quiet and 3 per cent in noise.

Conclusion: These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Auditory Threshold / physiology
  • Bulbar Palsy, Progressive / pathology
  • Bulbar Palsy, Progressive / physiopathology
  • Bulbar Palsy, Progressive / surgery
  • Cochlear Implantation*
  • Cochlear Implants
  • Disease Progression
  • Evoked Potentials, Auditory*
  • Female
  • Hearing Aids
  • Hearing Loss, Sensorineural / pathology
  • Hearing Loss, Sensorineural / physiopathology
  • Hearing Loss, Sensorineural / surgery*
  • Humans
  • Male
  • Siblings
  • Speech Discrimination Tests
  • Speech Perception*
  • Treatment Outcome

Supplementary concepts

  • Brown-Vialetto-Van Laere syndrome