A 7-year-old boy presents with bilateral profound sensorineural hearing loss detected on neonatal screening. Temporal bone MRI shows bilateral enlarged vestibular aqueducts (EVA) with incomplete cochlear partition (Mondini malformation). The structure marked **A** in the diagram demonstrates this classic imaging finding. At age 12, the child develops a palpable neck swelling. Thyroid function tests show normal TSH and fT4, but thyroid ultrasound confirms a diffuse goiter. Which of the following genetic and molecular defects best explains this clinical presentation?
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