000			02063nam a22002057a 4500
003			OSt
005			20151009140449.0
008			151009b xxu||||| |||| 00| 0 eng d
040			_cNational Acoustic :Laboratories
100			_aCHANDRAN, R
245			_aAuditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation
520	3		_aBackground: 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.
650			_aBrown–Vialetto–Van Laere Syndrome; Evoked Potential, Auditory, Brainstem; Otoacoustic Emissions, Spontaneous; Cochlear Implantation
700			_aM ALEXANDER, ,
700			_aA BALRAJ
700			_aP NAINA
773	0		_g (2015), 129, 504–508 _tThe Journal of Laryngology & Otology
856			_uhttp://dspace.nal.gov.au/xmlui/bitstream/handle/123456789/245/Auditory%20neuropathy%20spectrum%20disorder%20with.pdf?sequence=1&isAllowed=y
942			_2udc _cARTICLE
999			_c2487 _d2487