Cremers C, van Rijn P, ter Haar B
Department of Otorhinolaryngology, University of Nijmegen, The Netherlands.
Arch Otolaryngol Head Neck Surg. 1987 Dec;113(12):1319-24. doi: 10.1001/archotol.1987.01860120065010.
Autosomal recessive progressive high-frequency sensorineural deafness in childhood occurred in six patients from two families. This progressive sensorineural hearing loss starts mainly in the higher frequencies. There is an abrupt decline in the audiogram that slowly decreases with the increase of the hearing loss in the lower frequencies.
来自两个家族的6名患者出现了常染色体隐性遗传性儿童进行性高频感音神经性耳聋。这种进行性感音神经性听力损失主要始于高频。听力图上有一个突然下降,随着低频听力损失的增加而缓慢下降。