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.

CASE REPORT:

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.

CONCLUSION:

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: R Chandran, M Alexander, P Naina, A Balraj
• Journal: The Journal of laryngology and otology (J Laryngol Otol) Vol. 129 Issue 5 Pg. 504-8 (May 2015) ISSN: 1748-5460 [Electronic] England
• PMID: 25994385 (Publication Type: Case Reports, Journal Article)

Topics

• Adolescent
• Bulbar Palsy, Progressive
• Cochlear Implantation
• Correction of Hearing Impairment
• Evoked Potentials, Auditory, Brain Stem
• Female
• Hearing Loss, Central
• Hearing Loss, Sensorineural
• Humans
• Male
• Siblings
• Speech Perception
• Young Adult