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December 1987

Autosomal Recessive Progressive High-Frequency Sensorineural Deafness in Childhood

Author Affiliations

From the Departments of Otorhinolaryngology (Drs Cremers and van Rijn) and Human Genetics, Division of Clinical Genetics (Dr ter Haar), University of Nijmegen, the Netherlands.

Arch Otolaryngol Head Neck Surg. 1987;113(12):1319-1324. 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.

(Arch Otolaryngol Head Neck Surg 1987;113:1319-1324)

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