Follow-up study in a family with dominant progressive hereditary sensorineural hearing impairment. I. Analysis of hearing deterioration.
An autosomal-dominant progressive hereditary sensorineural hearing loss with onset in the high frequencies was studied in a Dutch family with 105 affected individuals. The hearing loss was analyzed by a curve-fitting procedure. A hyperbolic tangent was fitted into the audiogram. Five characteristics were distinguished: the high-frequency plateau, the low-frequency plateau, the slope, the low cut-off frequency and the high cut-off frequency. Six phases are recognizable in the development of the hearing loss, which is characterized by a two-step progression, first in the high and then in the low frequencies. Great differences in the rate of the deterioration were observed. The age of onset is between 5 and 15 years.
D000367:Age Factors; D001299:Audiometry; D005500:Follow-Up Studies; D006316:Hearing Loss, High-Frequency / Q000175:diagnosis / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008433:Mathematics