Syndromes: Usher syndrome: 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 · Syndromes: Usher syndrome

Sample item

A boy with congenital sensorineural deafness develops night blindness and tunnel vision in adolescence. Which condition fits best?

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

The answer and the reasoning

A. Usher syndrome

Usher syndrome is autosomal recessive deaf-blindness, with retinitis pigmentosa causing night blindness and field loss. It is the commonest cause of combined deafness and blindness. Early cochlear implantation helps communication.

Option by option

  • A. Usher syndrome (correct)

    Correct. Deafness with retinitis pigmentosa is Usher syndrome.

  • B. Treacher Collins syndrome

    Treacher Collins causes conductive loss with facial dysmorphism.

  • C. Waardenburg syndrome

    Waardenburg has pigment anomalies, not retinitis pigmentosa.

  • D. Cogan syndrome

    Cogan syndrome involves interstitial keratitis in adults.

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, Usher syndrome. Usher syndrome is autosomal recessive deaf-blindness, with retinitis pigmentosa causing night blindness and field loss.

Why is "Treacher Collins syndrome" not the answer?

Treacher Collins causes conductive loss with facial dysmorphism.

Why is "Waardenburg syndrome" not the answer?

Waardenburg has pigment anomalies, not retinitis pigmentosa.

Why is "Cogan syndrome" not the answer?

Cogan syndrome involves interstitial keratitis in adults.

What concept does this question test?

It tests Syndromes: Usher syndrome in ENT. It is the commonest cause of combined deafness and blindness. Early cochlear implantation helps communication.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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 Syndromes: Usher syndrome?

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.