A 6-year-old child from rural India presents with bilateral profound sensorineural hearing loss detected at age 2 years. Audiometry shows the pattern marked **A** in the diagram. High-resolution temporal bone CT reveals an enlarged vestibular aqueduct (≥1.5 mm) with incomplete partition of the cochlea. Thyroid examination reveals a firm, diffuse goiter. Which of the following genetic and molecular defects best explains this clinical presentation?
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