A full-term male newborn with a white forelock and bilateral sensorineural hearing loss is most likely diagnosed with which condition?

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The presentation of a full-term male newborn with a white forelock and bilateral sensorineural hearing loss is characteristic of Waardenburg syndrome. This genetic disorder is associated with specific clinical features, including pigmentary abnormalities (such as a white forelock or patches of reduced pigmentation in the skin and hair) and hearing loss due to neural defects affecting the auditory system.

Waardenburg syndrome results from mutations in genes involved in melanocyte development, which explains the white forelock due to lack of melanin in the hair. The bilateral sensorineural hearing loss is a common manifestation and occurs due to abnormalities in the development or function of the inner ear elements related to neural pathways.

In contrast, oculocutaneous albinism primarily involves a lack of pigmentation in the skin, hair, and eyes, without a specific association with hearing loss. Phenylketonuria (PKU) is a metabolic disorder resulting from a deficiency of the enzyme phenylalanine hydroxylase, leading to intellectual disability if untreated, but does not present with the specific features mentioned. Cystic fibrosis is a genetic disorder that affects the lungs and digestive system and is not associated with the clinical findings described.

Thus, the distinct combination of a white forelock and bilateral

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