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Objective: This study aimed to evaluate clinical musical perception, analyze the relationship between speech recognition and music perception, and investigate the effects of a three-month musical perception activities on these parameters in adult cochlear implant (CI) users with postlingual hearing loss. Methods: Free-field hearing and speech tests in a quiet environment, the Turkish matrix test, and the Turkish version of the clinical assessment of musical perception test were performed on 18 adult unilateral CI users before and after the three-month music training. Results were compared with those of 18 healthy controls. Results: Prior to the musical perception activities, word recognition scores, Turkish matrix test results, and 500, 1000, and 6000 Hz free-field hearing thresholds were significantly correlated with the clinical assessment of musical perception test scores in the CI group (p<0.047). Timbre recognition scores (p=0.019) had improved significantly in the CI group after the three-month musical perception activities. On the other hand, timbre recognition scores had significantly affected the Turkish matrix test results (R2adjusted=0.56). Conclusion: Our study showed that speech perception in noise and clinical musical perception measurements affected each other in CI users. The inclusion of musical perception activities to support an auditory rehabilitation program may contribute to increased speech recognition skills in noise. [ABSTRACT FROM AUTHOR]

The Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disorder that may present at all ages with sensorineural hearing loss, bulbar palsy and respiratory compromise. We describe a 6-yearold male patient who suffered bilateral sudden onset severe hearing loss for two years. Audiological investigations revealed sudden onset auditory neuropathy spectrum disorder bilaterally. He also had neurological complaints. During riboflavin therapy an improvement in hearing loss and the benefit of hearing aids were observed. In BVVLS, it is difficult to plan and apply auditory rehabilitation interventions and the results vary from patient to patient. In audiological evaluation, it should be borne in mind that subjective and objective tests are complemental. Early medical intervention and regular audiological follow-up are very important for effective hearing rehabilitation in the patients with BVVLS. [ABSTRACT FROM AUTHOR]