A 12-year-old boy from rural Maharashtra presents with progressive hearing loss over the past 5 years, now requiring hearing aids. Audiometry shows bilateral symmetric sensorineural hearing loss most marked at high frequencies, as marked **B** in the diagram. He also reports progressive visual loss with photophobia since age 3, truncal obesity, and recent diagnosis of type 2 diabetes mellitus with acanthosis nigricans. His parents are consanguineous. Which of the following best explains the underlying pathophysiology of the hearing loss pattern marked **B**?
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