出汗性外胚叶发育不良并非少见,但合并先天性感音性聋罕见。我院曾遇1例,现报告如下。病历摘要男患,24岁。3岁时发现双掌跖微红并轻微增厚,逐年加重,粗糙角化,弥漫扩大至手、足背部。同时指(趾)甲增厚翘起,甲下堆积污黄色角化物,甲廓反复感染。4岁时发觉听力差。既往否认其它疾病史及药物中毒史。为早产儿。无家族史。体检:外耳及鼓膜未见异常。双耳不能听到表声。乳突无压痛。皮肤科所见:毛发稀疏、细软,左枕部见8×5cm椭圆形无发斑。双掌跖弥漫性角化至其背部,粗糙韧硬。双指(趾)甲萎缩并见纵嵴。实验室检查:血尿常规均为正 Sweating ectodermal dysplasia is not uncommon, but congenital sensory hearing loss rare. Our hospital had a case, the report is as follows now. Medical history male suffering, 24 years old. 3 years old found palmoplantar red and slightly thicker, increasing year by year, rough keratosis, diffuse to the hands and feet back. At the same time refers to (A) thickened tilt, nail polish accumulation of yellow keratin, A repeatedly infected. 4 years old found that poor hearing. Previously deny other medical history of illness and drug poisoning. For premature children. No family history. Physical examination: external ear and tympanic membrane no abnormalities. Binaural can not hear the sound of the table. Mastoid without tenderness. Dermatology seen: thin hair, thin, soft, see the left occipital 8 × 5cm oval without spot. Duplex plantar diffuse keratosis to its back, rough tough. Two fingers (toes) atrophy and see the longitudinal ridge. Laboratory tests: hematuria routine are positive