Ear and audiology: bilateral vestibular schwannoma: practice question

A ENT question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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ENT · Ear and audiology: bilateral vestibular schwannoma

Sample item

A 19-year-old man with bilateral progressive sensorineural deafness and tinnitus has a family history of similar illness. MRI shows bilateral cerebellopontine angle masses. Which gene is mutated?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

A. NF2 on chromosome 22, merlin

Bilateral vestibular schwannomas are diagnostic of neurofibromatosis type 2, an autosomal dominant disease due to mutation of the merlin gene on chromosome 22. Meningiomas and ependymomas are also associated. NF1 is on chromosome 17 and is not typically associated with bilateral schwannomas.

Option by option

  • A. NF2 on chromosome 22, merlin (correct)

    Correct. NF2 gives bilateral vestibular schwannomas.

  • B. VHL on chromosome 3

    VHL gives haemangioblastomas and endolymphatic sac tumours.

  • C. TSC1

    TSC gives tubers and angiomyolipomas.

  • D. NF1 on chromosome 17

    NF1 causes neurofibromas and cafe au lait spots.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this ENT question

What is the answer to this ENT question?

The best answer is A, NF2 on chromosome 22, merlin. Bilateral vestibular schwannomas are diagnostic of neurofibromatosis type 2, an autosomal dominant disease due to mutation of the merlin gene on chromosome 22.

Why is "VHL on chromosome 3" not the answer?

VHL gives haemangioblastomas and endolymphatic sac tumours.

Why is "TSC1" not the answer?

TSC gives tubers and angiomyolipomas.

Why is "NF1 on chromosome 17" not the answer?

NF1 causes neurofibromas and cafe au lait spots.

What concept does this question test?

It tests Ear and audiology: bilateral vestibular schwannoma in ENT. Meningiomas and ependymomas are also associated. NF1 is on chromosome 17 and is not typically associated with bilateral schwannomas.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Ear and audiology: bilateral vestibular schwannoma?

Learn tuning fork results and audiogram patterns by sketching them. Then use the flashcards and the other ENT questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.