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    SubjectsENTConnexin-26 GJB2 Congenital SNHL
    ClinicalENT

    Connexin-26 GJB2 Congenital SNHL

    1 MCQs in ENT for NEET PG

    1 Medium
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    A 3-month-old infant referred from newborn hearing screening shows bilateral symmetric sensorineural hearing loss of 85 dB across all frequencies on diagnostic ABR. Imaging (MRI temporal bones) is normal—no cochlear malformations, no enlarged vestibular aqueduct, no nerve aplasia. The audiologic pattern marked **A** in the diagram is consistent with connexin-26 (GJB2) mutation. Which of the following best explains the pathophysiology of hearing loss in this condition?

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