Division of Genetics and Metabolism

Test module: Hearing Loss Syndromes

Estimated time to complete:5 min.
Number of questions: 6

Your name:

1. Hearing loss syndromes account for what percentage of all genetic-based hearing impairments?

Less than 2%
5-10%
20-30%
About 50%

Note: You must check your answer after each selection.

2. In the BOR syndrome, the “O” stands for:

The problem of cysts, sinuses or fistulas in the neck area
Represents “Other” features associated with hearing loss
Represents the “Oto” component of the syndrome illustrated by abnormally shaped ears, often with preauricular pits

3. Which ear is most typical for the CHARGE syndrome?

preauricular ear pit Protruding ear with preauricular pit
"pulled ear" appearance with hypoplasic ear lobe severe ear dysplasia and hypoplasia

4. Which hair pattern in most suggestive of the Waardenburg syndrome?

lightly pigmented hair and skin hypopigmented hair patch
widow peak pattern sparse, brittle hair

5. Which of the following is a component in the CHARGE syndrome?

Relatively shortened or fused fingers
Coloboma of the iris or retina
Ataxia and vestibular problems
Abnormal hair with white forelock

6. The skin problem of multiple lentigenes in combination with hearing loss and pulmonary stenosis is characteristic of which syndrome:

CHARGE
Waardenburg
Treacher Collins
LEOPARD
Pendred

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