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

OBJECTIVE: The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes. DESIGN: We report the audiological and clinical profile of a child who presented with a complaint of sudden loss of speech understanding associated with an atypical form of ANSD. He was later diagnosed with BVVL. STUDY SAMPLE: An 11-year-old boy with ANSD associated with BVVL. RESULTS: The patient's severe neurological symptoms improved within a year of supplementation with high doses of riboflavin. His fluctuating hearing loss and 0% WDS remained unchanged. The patient was able to use hearing aids without any discomfort after treatment initiation, but he stopped using them again due to a lack of benefit in speech understanding. Although cochlear implantation was recommended, the patient and his family decided not to consider it for another year since they still had hope for complete recovery. CONCLUSIONS: Sudden-onset ANSD can be the earliest sign of undetected BVVL syndrome. Early detection of BVVL is crucial since all symptoms can be reversible with an early intervention of high doses of riboflavin supplementation.

Determination of Anxiety, Health Anxiety and Somatosensory Amplification Levels in Individuals with Benign Paroxysmal Positional Vertigo.

OBJECTIVES: Psychiatric comorbidities may intensify peripheral vertigo and increase the number of repositioning maneuvers required. This study was designed to examine the relationship between benign paroxysmal positional vertigo (BPPV) and anxiety and assess its association with somatic amplification and health anxiety. MATERIALS AND METHODS: Sixty patients with BPPV (43 women, 17 men; age range: 24-81 years, mean age 40.4±13.3), and 60 healthy participants (29 women, 31 men; age range: 18-71, mean age 38.2±11.43) were prospectively enrolled. The participants completed the Beck Anxiety Inventory (BAI), Short Health Anxiety Inventory (SHAI), and Somatosensory Amplification Scale (SSAS) questionnaires. RESULTS: The BAI scores of the patients with BPPV were higher than those of the control group participants and were as follows: (16.4 vs. 12.7; p=0.01). The SHAI (p=0.44) and SSAS (p=0.60) scores were not significantly different between the two groups. The BAI scores were positively correlated with the SHAI (rho: 0.273, p=0.035) and SSAS (rho: 0.357, p=0.005) scores. Neither the number of BPPV attacks nor the number of Epley maneuvers required showed any correlation with the BAI [(rho: 0.208, p=0.11); (rho: -0.007, p=0.96)], SHAI [(rho: 0.068, p=0.06); (rho: 0.021, p=0.87)], and SSAS [(rho: -0.081, p=0.53); (rho: -0.012, p=0.92)] scores. CONCLUSION: Our findings indicate that patients with BPPV had higher anxiety scores than healthy participants. Although our findings indicated normal health anxiety and somatic amplification levels in patients with BPPV, regular evaluation of psychological status would be a good strategy to prevent chronic dizziness.