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J Laryngol Otol. 2015 May;129(5):504-8. doi: 10.1017/S0022215114003375.

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

Author information

1
Audiovestibular Unit,Department of ENT,Christian Medical College,Vellore,India.
2
Department of Neurosciences,Christian Medical College,Vellore,India.

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:

Auditory; Brainstem; Brown–Vialetto–Van Laere Syndrome; Cochlear Implantation; Evoked Potential; Otoacoustic Emissions; Spontaneous

PMID:
25994385
DOI:
10.1017/S0022215114003375
[Indexed for MEDLINE]

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