PMID: 8973283Dec 1, 1996Paper

Stable and progressive hearing loss in type 2A Usher's syndrome

The Annals of Otology, Rhinology, and Laryngology
A Van AaremCor W Cremers

Abstract

Audiograms were traced or additionally performed on 23 Usher's syndrome patients in 10 Dutch multi-affected families, all linked to chromosome 1q (USH2A locus). Serial audiograms, available in 13 patients, were used for a regression analysis of binaural pure tone average on age (follow-up, 9 to 32 years) to test for "significant progression," ie, a significant regression coefficient, here called the "annual threshold increase" (ATI, expressed in decibels per year). A significant ATI (> 1 dB/y) was observed in 3 patients. Analysis of variance of ATI demonstrated significant heterogeneity; hearing loss was either stable or progressive. This implies a significant clinical heterogeneity. A similar analysis performed on our progressive USH2A cases and "type III" cases previously reported by others (ATI of 1 to 5 dB/y), some of which were recently linked to chromosome 3q (USH3 locus), failed to show any significant heterogeneity in the progression of hearing loss.

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Citations

Nov 8, 2003·Trends in Molecular Medicine·Tamar Ben-Yosef, Thomas B Friedman
Jun 17, 2004·International Journal of Audiology·Mehdi SadeghiClaes Möller
Jan 15, 2005·Audiology & Neuro-otology·R F PlantingaC W R J Cremers
Mar 17, 2007·International Journal of Audiology·Mazal CohenLinda Luxon
Dec 25, 2002·The Annals of Otology, Rhinology, and Laryngology·Christoph F V ReisserChristian R Otterstedde
Oct 29, 2013·International Journal of Audiology·André M SadeghiClaes Möller
Jan 25, 2003·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Ronald J E PenningsCor W R J Cremers
Sep 1, 1996·Journal of Medical Genetics·S Pieke-DahlW J Kimberling
Oct 11, 2003·The Annals of Otology, Rhinology, and Laryngology·Ronald J E PenningsW R J Cremers
Aug 1, 2021·American Journal of Medical Genetics. Part a·Alessandro IannacconeUNKNOWN Foundation Fighting Blindness Consortium Investigator Group

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