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

By: Contributor(s): Material type: TextTextSubject(s): Online resources: In: The Journal of Laryngology & Otology (2015), 129, 504–508Abstract: 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.
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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.

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