Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation (Record no. 2487)
[ view plain ]
| 000 -LEADER | |
|---|---|
| fixed length control field | 02063nam a22002057a 4500 |
| 003 - CONTROL NUMBER IDENTIFIER | |
| control field | OSt |
| 005 - DATE AND TIME OF LATEST TRANSACTION | |
| control field | 20151009140449.0 |
| 008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION | |
| fixed length control field | 151009b xxu||||| |||| 00| 0 eng d |
| 040 ## - CATALOGING SOURCE | |
| Transcribing agency | National Acoustic :Laboratories |
| 100 ## - MAIN ENTRY--PERSONAL NAME | |
| Personal name | CHANDRAN, R |
| 245 ## - TITLE STATEMENT | |
| Title | Auditory neuropathy spectrum disorder with Brown–Vialetto–Van Laere syndrome: challenges in hearing rehabilitation |
| 520 3# - SUMMARY, ETC. | |
| Summary, etc | Background: Brown–Vialetto–Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and<br/>sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.<br/>Case report: We examined the clinical and audiological profiles of a case series comprising four siblings with<br/>Brown–Vialetto–Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological<br/>investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes<br/>in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.<br/>Conclusion: The sensorineural hearing loss in Brown–Vialetto–Van Laere syndrome patients is a retrocochlear<br/>pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic<br/>origin. Early cochlear implantation along with high-dose riboflavin represents a possible rehabilitation therapy.<br/>However, further research is needed to confirm this. This report emphasises the need for a thorough neurological<br/>evaluation of auditory neuropathy spectrum disorder patients. |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM | |
| Topical term or geographic name as entry element | Brown–Vialetto–Van Laere Syndrome; Evoked Potential, Auditory, Brainstem; Otoacoustic Emissions, Spontaneous; Cochlear Implantation |
| 700 ## - ADDED ENTRY--PERSONAL NAME | |
| Personal name | M ALEXANDER, , |
| 700 ## - ADDED ENTRY--PERSONAL NAME | |
| Personal name | A BALRAJ |
| 700 ## - ADDED ENTRY--PERSONAL NAME | |
| Personal name | P NAINA |
| 773 0# - HOST ITEM ENTRY | |
| Relationship information | (2015), 129, 504–508 |
| Title | The Journal of Laryngology & Otology |
| 856 ## - ELECTRONIC LOCATION AND ACCESS | |
| Uniform Resource Identifier | <a href="http://dspace.nal.gov.au/xmlui/bitstream/handle/123456789/245/Auditory%20neuropathy%20spectrum%20disorder%20with.pdf?sequence=1&isAllowed=y">http://dspace.nal.gov.au/xmlui/bitstream/handle/123456789/245/Auditory%20neuropathy%20spectrum%20disorder%20with.pdf?sequence=1&isAllowed=y</a> |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) | |
| Source of classification or shelving scheme | Universal Decimal Classification |
| Koha item type | Journal article |
No items available.