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患者男,10岁。1997年8月21日因右耳听力减退而就诊。患儿出生后父母发现右颜面短小畸形,神态哭声正常。同时发现无肛门,即行肛门成形术。检查:颜面短小,双睑裂小,瞳孔正中,右颊部凹陷,颧弓存在,双侧下颌骨对称性发育不全,双外耳道正常,鼓膜内陷,标志不清,x线片示双下颌骨髁突发育差,下颌骨升支短且细,乳突为硬化型。纯音测听示右耳混合性聋,右耳气导平均损失50dB,左耳大致正常。脑干诱发电位检查示右耳听阈增高,Ⅰ～Ⅴ波潜伏期延长,波幅降低,波形差,提示:右耳听神经异
Patient male, 10 years old. August 21, 1997 due to hearing loss on the right ear and treatment. Children born after the discovery of short right facial deformity, dementia normal crying. At the same time found no anus, anal angioplasty. Check: short face, double palpebral fissure, pupil median, right cheek depression, zygomatic arch existence, bilateral symmetrical mandibular hypoplasia, double external auditory canal, eardrum, signs unclear, x-ray showed double mandible Condylar development is poor, mandibular ascending short and thin, mastoid sclerosis. Pure tone audiometry showed mixed right deafness, the average loss of right ear air conduction 50dB, left ear roughly normal. Brainstem evoked potentials showed right ear hearing threshold increased, Ⅰ ~ Ⅴ wave latency prolonged volatility, poor waveform, suggesting that: the right ear auditory nerve