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    SubjectsENTOTOF Auditory Neuropathy
    ClinicalENT

    OTOF Auditory Neuropathy

    1 MCQs in ENT for NEET PG

    1 Medium
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    Sample Questions

    medium

    A 3-year-old child with prelingual severe-to-profound bilateral sensorineural hearing loss is evaluated. Behavioral audiometry confirms thresholds of 85–95 dB HL across frequencies. Otoacoustic emissions (OAE) are **present and robust**. Auditory brainstem response (ABR) shows **absent neural waves with a preserved cochlear microphonic**. MRI temporal bones is normal with intact cochlear nerves. The audiological pattern marked **A** in the diagram is consistent with auditory neuropathy spectrum disorder (ANSD). Genetic testing confirms biallelic OTOF mutations. Which of the following management strategies is most appropriate for this child?

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