Audiological findings in Brown Vialetto-Van-Laere Syndrome: A scoping review.

OBJECTIVE

This study aimed to characterize audiological porfile in inviduals with Brown-Vialetto-Van Laere syndrome (BVVLS).

METHODS

This is a scoping review following the methodological structure developed by the Joana Briggs Institute (JBI). The PCC mnemonic was used to elaborate the research question, which resulted in the research question: "What are the audiological findings in individuals with BVVLS?".

RESULTS

All of the studies included in this review were case reports. The main audiological findings are sensorineural hearing loss and Auditory Neuropathy Spectrum Disorder (ANSD).

CONCLUSION

All individuals presented a severe to profound bilateral hearing loss, related to ANSD.