A 2-year-old child born to consanguineous parents presents with bilateral profound sensorineural hearing loss detected on newborn screening, delayed motor milestones (independent walking achieved only at 22 months), and recurrent falls with balance impairment that worsens in darkness. Ophthalmologic examination reveals bone-spicule pigmentation on fundus with attenuated retinal arterioles. The clinical and audiometric findings marked **A** in the diagram are most consistent with which genetic diagnosis?
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