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    SubjectsOphthalmologyNorrie Disease X-Linked
    ClinicalOphthalmology

    Norrie Disease X-Linked

    1 MCQs in Ophthalmology for NEET PG

    1 Medium
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    Sample Questions

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    A 4-year-old boy born to a consanguineous family presents with congenital bilateral leukocoria and grayish-yellow retrolental masses. Genetic testing confirms Norrie disease (NDP gene mutation at Xp11.4). At age 16 years, he develops progressive high-frequency hearing loss that progresses to bilateral profound sensorineural hearing loss as shown by the audiogram marked **B**. Which of the following best explains the mechanism of hearing loss in this patient?

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