A 35-year-old man from rural India presents with progressive night blindness since age 16, followed by development of distal weakness and areflexia over the past 5 years. Audiometry reveals bilateral high-frequency sensorineural hearing loss as shown by the pattern marked **A** in the diagram. Plasma phytanic acid is markedly elevated at 280 µmol/L (normal <30). Which of the following enzyme deficiencies best explains the pathophysiology of this patient's presentation?
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