Abstract | BACKGROUND: METHOD: RESULTS: There was significant heterogeneity in vestibular function and in the benefit gained from cochlear implantation. The audiological response to riboflavin therapy was also variable, in contrast to generalised improvement in motor function. CONCLUSION: We suggest that comprehensive testing of vestibular function should be conducted in Brown-Vialetto-Van Laere syndrome, in addition to serial behavioural audiometry as part of the systematic examination of the effects of riboflavin.
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Authors | R Omar, K Rajput, T Sirimanna, S Rajput, W Pagarkar |
Journal | The Journal of laryngology and otology
(J Laryngol Otol)
Vol. 135
Issue 11
Pg. 1000-1009
(Nov 2021)
ISSN: 1748-5460 [Electronic] England |
PMID | 34496984
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Membrane Transport Proteins
- Receptors, G-Protein-Coupled
- SLC52A2 protein, human
- SLC52A3 protein, human
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Topics |
- Adolescent
- Audiometry
- Bulbar Palsy, Progressive
(complications, genetics, physiopathology)
- Child, Preschool
- Female
- Hearing
(genetics)
- Hearing Loss, Central
(genetics, physiopathology)
- Hearing Loss, Sensorineural
(complications, genetics, physiopathology)
- Humans
- Infant
- Male
- Membrane Transport Proteins
(genetics)
- Mutation
- Receptors, G-Protein-Coupled
(genetics)
- Vestibular Function Tests
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