A case report of sudden-onset auditory neuropathy spectrum disorder associated with Brown-Vialetto-Van Laere syndrome (riboflavin transporter deficiency)

2021 ◽  
pp. 1-7
Author(s):  
Ozlem Gedik Soyuyuce ◽  
Elif Ayanoglu Aksoy ◽  
Zuhal Yapici
Keyword(s):  
Case Report ◽  
Sudden Onset ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Transporter Deficiency

scholarly journals A Case with Brown-Vialetto-Van Laere Syndrome: A Sudden Onset Auditory Neuropathy Spectrum Disorder

2019 ◽  
Vol 57 (4) ◽  
pp. 201-205 ◽  
Author(s):  
Basak Mutlu ◽  
◽  
Merve Torun Topcu ◽  
Ayca Ciprut ◽  
◽  
...  
Keyword(s):  
Sudden Onset ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

scholarly journals A case report-unilateral auditory neuropathy spectrum disorder

2014 ◽  
Vol 57 (2) ◽  
pp. 151-155
Author(s):  
Momoko Tsukahara ◽  
Miyako Sekiguchi ◽  
Yoko Oikawa ◽  
Mayuri Okami ◽  
Tomoe Okawa ◽  
...  
Keyword(s):  
Case Report ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

scholarly journals A Case Report on Familial origin of Auditory Neuropathy Spectrum Disorder

2020 ◽  
pp. 1-9
Author(s):  
Sabarish A ◽  
◽  
Harshavardhan Raje Urs P ◽  
Keyword(s):  
Case Report ◽  
Hair Cell ◽  
Outer Hair Cell ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Hearing Disorder ◽  
Inner Hair Cell ◽  
Genetic Trait ◽  
Auditory Neuropathy Spectrum Disorder ◽  
Related Risk

Auditory neuropathy Spectrum Disorder (ANSD) is a unique hearing disorder where outer hair cell status is found to be normal, but inner hair cell and/or the synaptic connections to the auditory nerve is disrupted. It is a diverse group of hearing disorder which can be manifested either congenitally or can be late in onset. However, there are various etiologies of auditory neuropathy which is represented in the literature, which includes birth related risk factors for hearing loss like prematurity, bilirubin synthesis issues, lack of oxygen supply to the baby during or after delivery, and genetic actors. It is estimated that approximately 40% of cases have an underlying causal factor as genetic origin, which can be inherited in either syndromic or non-syndromic way. The below case report provides an extra support for the fundamental genetic trait in ANSD. The study presents two congenital ANSD cases where, both children were diagnosed as congenital ANSD

Auditory testing outcomes with hearing aids in patients with auditory neuropathy spectrum disorder

2021 ◽  
Vol 42 (5) ◽  
pp. 103057
Author(s):  
Firas Sbeih ◽  
Donald M. Goldberg ◽  
Sara Liu ◽  
Maxwell Y. Lee ◽  
Gina Stillitano ◽  
...  
Keyword(s):  
Hearing Aids ◽  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder

Outcomes of audiometric testing in children with auditory neuropathy spectrum disorder

2020 ◽  
Vol 129 ◽  
pp. 109757 ◽  
Author(s):  
Joseph B. Meleca ◽  
Gina Stillitano ◽  
Maxwell Y. Lee ◽  
Whitney Lyle ◽  
Yi-Chun Carol Liu ◽  
...  
Keyword(s):  
Auditory Neuropathy ◽  
Spectrum Disorder ◽  
Auditory Neuropathy Spectrum Disorder
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