International Journal of Speech and Audiology
2022, Vol. 3, Issue 2, Part A
Brown-Vialetto-Van laere syndrome & auditory neuropathy spectrum disorder: A case study
Author(s): Nithin AK and Sagar Jose
Abstract: The Brown-Vialetto-Van Laere syndrome (BVVL) may be a rare neurological disease characterised by progressive pontobulbar palsy related to sensorineural hearing impairment. Cardinal cases are reportable in mere over one hundred years. The feminine to male quantitative relation is roughly 3:1[1]. The syndrome most often presents with sensorineural hearing impairment that is sometimes progressive and severe.Method: The patient conferred during this case study had undergone multiple tests tympanometry, acoustic reflex, pure tone audiometry, transient evoked otoacoustic emissions and auditory brain-stem response were used and continual 3 times throughout six years.Results: This study shows the clinical symptoms pertinent to auditory Neuropathy spectrum disorder (ANSD) for a 15-year-old woman with Brown-Vialetto-Van Laere Syndrome (BVVL). The results are followed for 6 years between 2016 and 2022. Within the 1st session, the results discovered bilaterally normal transient evoked otoacoustic emissions, absent acoustic reflexes (ipsilateral and contralateral), delicate to moderate low tone loss within the sound audiometry, no auditory brain stem responses at high input intensities and totally different polarities and gift tube-shaped structure micro phonic part in single polarities. Within the second session, sound audiometry showed slight to gentle high tone loss but the opposite tests incontestable their same results. Within the final session, the latter results were repeated. Conclusion: During this comprehensive follow up study, the patient with BVVL incontestable clinical symptoms of ANSD within which sensory system showed an impairment within the sense modality temporal synchronization and encoding.
Pages: 22-25 | Views: 532 | Downloads: 306
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How to cite this article:
Nithin AK and Sagar Jose. Brown-Vialetto-Van laere syndrome & auditory neuropathy spectrum disorder: A case study. International Journal of Speech and Audiology. 2022; 3(2): 22-25.
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