Autosomal recessive progressive high-frequency sensorineural deafness in childhood.
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.
Cremers
C
C
Department of Otorhinolaryngology, University of Nijmegen, The Netherlands.
van Rijn
P
P
ter Haar
B
B
D000328:Adult; D001301:Audiometry, Pure-Tone; D001302:Audiometry, Speech; D005260:Female; D005808:Genes, Recessive; D034381:Hearing Loss / Q000235:genetics*; D006316:Hearing Loss, High-Frequency / Q000235:genetics* / Q000503:physiopathology; D006319:Hearing Loss, Sensorineural / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D008297:Male; D010375:Pedigree