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Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation

Published online by Cambridge University Press:  21 May 2015

R Chandran ,
M Alexander ,
P Naina  and
A Balraj
R Chandran
Affiliation:
Audiovestibular Unit, Department of ENT, Christian Medical College, Vellore, India
M Alexander
Affiliation:
Department of Neurosciences, Christian Medical College, Vellore, India
P Naina
Affiliation:
Audiovestibular Unit, Department of ENT, Christian Medical College, Vellore, India
A Balraj*
Affiliation:
Audiovestibular Unit, Department of ENT, Christian Medical College, Vellore, India
*
Address for correspondence: Dr A Balraj, Audiovestibular Unit, Department of ENT, Christian Medical College, Vellore-632004, Tamil Nadu, India. Fax: +91-0416-2232035 E-mail: sujabalraj@gmail.com
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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.

Keywords

Brown–Vialetto–Van Laere SyndromeEvoked Potential, Auditory, BrainstemOtoacoustic Emissions, SpontaneousCochlear Implantation
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 129 , Issue 5 , May 2015 , pp. 504 - 508
Copyright
Copyright © JLO (1984) Limited 2015 

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