title: Auditory neuropathy spectrum disorder with Brown-Vialetto-Van Laere syndrome: challenges in hearing rehabilitation.
abstract: BACKGROUND
  Brown-Vialetto-Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.
  
  
  METHODS
  We examined the clinical and audiological profiles of a case series comprising four siblings with Brown-Vialetto-Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.
  
  
  CONCLUSIONS
  The sensorineural hearing loss in Brown-Vialetto-Van Laere syndrome patients is a retrocochlear pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic origin. Early cochlear implantation along with high-dose riboflavin represents a possible rehabilitation therapy. However, further research is needed to confirm this. This report emphasises the need for a thorough neurological evaluation of auditory neuropathy spectrum disorder patients.
authors.affiliation: Audiovestibular Unit,Department of ENT,Christian Medical College,Vellore,India.
authors.forename: R
authors.initials: R
authors.lastname: Chandran
authors.affiliation: Department of Neurosciences,Christian Medical College,Vellore,India.
authors.forename: M
authors.initials: M
authors.lastname: Alexander
authors.affiliation: Audiovestibular Unit,Department of ENT,Christian Medical College,Vellore,India.
authors.forename: P
authors.initials: P
authors.lastname: Naina
authors.affiliation: Audiovestibular Unit,Department of ENT,Christian Medical College,Vellore,India.
authors.forename: A
authors.initials: A
authors.lastname: Balraj
mesh_terms: D000293:Adolescent; D010244:Bulbar Palsy, Progressive; D019929:Cochlear Implantation; D012048:Correction of Hearing Impairment; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006313:Hearing Loss, Central; D006319:Hearing Loss, Sensorineural; D006801:Humans; D008297:Male; D035781:Siblings; D013067:Speech Perception; D055815:Young Adult
keywords: Auditory; Brainstem; Brown–Vialetto–Van Laere Syndrome; Cochlear Implantation; Evoked Potential; Otoacoustic Emissions; Spontaneous