Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation
CHANDRAN, R
Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation
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.
Brown–Vialetto–Van Laere Syndrome; Evoked Potential, Auditory, Brainstem; Otoacoustic Emissions, Spontaneous; Cochlear Implantation
Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation
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.
Brown–Vialetto–Van Laere Syndrome; Evoked Potential, Auditory, Brainstem; Otoacoustic Emissions, Spontaneous; Cochlear Implantation