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Miscellaneous Article

Follow-Up Study in a Family with Dominant Progressive Hereditary Sensorineural Hearing Impairment I. Analysis of Hearing Deterioration

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Pages 233-240 | Received 01 Oct 1984, Accepted 02 Jan 1985, Published online: 07 Jul 2009
 

Abstract

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.

Suivi ‘une famille ayant une surdité neurosensorielle héréditaire progressive dominante. I. Analyse de la deterioration de’ audition

Une atteinte auditive de perception héréditaire dominante commençant sur les aigus a été étudiée dans une famille dont 105 membres sont affectés. Les audiogrammes ont été analysés par ajustement de courbe (tangente hyperbolique). 5 caractéristiques étaient distinguées: les plateaux et les fréquences de coupure en hautes et basses fréquences et la pente. On reconnaiˇt 6 phases dans le développement de la surdité et une progression en deux étapes: ‘abord les hautes’, puis les basses fréquences. Il y a une grande variabilité dans la progressivité de la surdité, celle-ci débutant entre 5 et 15 ans.

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