• Pathophysiology: may be from abnormal tyrosine metabolism
• Otologic SSx: unilateral or bilateral SNHL, may have vestibular dysfunction
• Other SSx: pigmentary abnormalities (heterochromic iriditis, white forelock, patchy skin depigmentation), craniofacial abnormalities (dystopia canthorum [widely spaced inner canthi], synophrys [confluent eyebrows], flat nasal root)
I: presence of telecanthus, 20% have SNHL II: no telecanthus, 50% have SNHL
III: associated with a unilateral ptosis and skeletal abnormalities
• Dx: clinical exam and history, family history
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