Neurotologic and Functional MRI Findings in a Patient With Bilateral Profound Deafness Having Brown-Vialetto-Van Leare Syndrome.

Otol Neurotol. 2014 Sep 5;

Authors: Salmina C, Wagner F, Wiest R, Federspiel A, Senn P, Caversaccio M, Vibert D

Abstract
INTRODUCTION: We report the first findings of functional magnetic resonance imaging of the auditory cortex in a young woman with a bilateral cochleovestibular deficit as first manifestation of Brown-Vialetto-Van Leare syndrome. The patient had no open speech discrimination, even with hearing aids, and is depending on lip reading for communication.
METHODS: To evaluate the possible efficiency of a cochlear implantation, we investigated hemodynamic responses within the central auditory pathways using an auditory functional magnetic resonance imaging paradigm.
RESULTS: Blood oxygen level-dependent correlates were detected bilaterally along the auditory pathways after exposure to intermittent clicking tone stimulation at 2 kHz.
CONCLUSION: These results suggest integrity of the central auditory pathways and represent a positive argument to propose a cochlear implantation with the aim to restore hearing.

PMID: 25203559 [PubMed - as supplied by publisher]