両側感音難聴を合併した表皮母斑症候群例

抄録

We report the first known case of epidermal nevus syndrome with bilateral sensorineural hearing loss. A 5-year-old boy with nevus extending from the right side of the neck to the thigh was brought to our department because of apparent speech delay since 3 years of age. Pure tone audiometry demonstrated bilateral moderate hearing loss, although development other than hearing and speech was within normal limits. CT scan did not show any evidence of abnormality in the intracranium or temporal bone. After providing hearing aids, language development was improved. Epidermal nevus syndrome is defined as one of neurocutaneous syndrome, and we should consider possible complication of hearing in neonates with extensive nevus.