患儿男,2岁。第1胎,足月顺产,母孕期无异常。生后发现患儿头发颜色不一致,有片状色素缺失区,枕部最为显著。5～6个月时发现听觉不灵敏,但对高声呼喊尚有反应。14个月会走,能说简单话,但对大人的指令反应较迟钝。曾在当地做脑干诱发电位检查示听力异常。未用过氨基甙类抗生素。家族中无类似患者。查体:前额及枕部有大小不等的片状白发区,该处头皮 Children male, 2 years old. The first child, term full-term pregnancy, no abnormalities during pregnancy. After birth, children found that hair color is inconsistent, a piece of pigmented missing area, occipital most significant. 5 to 6 months found hearing insensitive, but shout loud response. 14 months will go, to be able to say simple words, but the response to the instructions of adults dull. Had a local brain stem evoked potential test showed abnormal hearing. Not used aminoglycoside antibiotics. There are no similar patients in the family. Physical examination: forehead and occipital size flaky white hair area, where the scalp