A patient with Paget's disease of the bone may develop the gradual onset of deafness. The most famous example is Ludwig van Beethoven.

Manifestations related to the ears:

(1) tinnitus

(2) vertigo

(3) bilateral loss of hearing (conductive and/or sensorineural)


Proposed mechanisms include:

(1) ankylosis of the ear ossicles

(2) compression of CN VIII

(3) cochlear damage (thickening of the labyrinthine capsule, other)

(4) distortion of the internal auditory meatus


Other findings in Paget's disease of the skull include:

(1) leonine facies

(2) palsy of other cranial nerves

(3) headache


Other manifestations of Paget's disease may include:

(1) bowing of the legs

(2) pathologic bone fractures

(3) peripheral neuropathy


Differential diagnosis:

(1) other causes of deafness

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