The Brown-Vialetto-Van Laere syndrome (BVVLS)is a rare neurological disorder that may present atall ages with sensorineural hearing loss, bulbar palsyand respiratory compromise. We describe a 6-yearold male patient who suffered bilateral sudden onsetsevere hearing loss for two years. Audiological investigations revealed sudden onset auditory neuropathyspectrum disorder bilaterally. He also had neurological complaints. During riboflavin therapy an improvement in hearing loss and the benefit of hearing aidswere observed. In BVVLS, it is difficult to plan andapply auditory rehabilitation interventions and theresults vary from patient to patient. In audiologicalevaluation, it should be borne in mind that subjectiveand objective tests are complemental. Early medicalintervention and regular audiological follow-up arevery important for effective hearing rehabilitation inthe patients with BVVLS.