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ENT General bb7c2072

Most common cause of syndromic deafness is:

A
Usher syndrome
B
Waardenburg syndrome
C
Pendred syndrome
D
All of the above
High-Yield Explanation
Pendred syndrome is the most common syndromic form of deafness, accounting for approximately 10% of cases. It presents with goiter and sensorineural hearing loss. Usually, the goiter is evident before pubey, but an adult onset has also been noted. Thyroid function is evenly divided, with 50% euthyroid patients and 50% hypothyroid patients. In most cases, the hearing loss is congenital, bilateral, moderate to profound, and sloping in the higher frequencies. An enlarged vestibular aqueduct is a consistent finding, and Mondini-type malformations have also been associated.

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