In:
Annals of Otology, Rhinology & Laryngology, SAGE Publications, Vol. 105, No. 12 ( 1996-12), p. 962-967
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.
Type of Medium:
Online Resource
ISSN:
0003-4894
,
1943-572X
DOI:
10.1177/000348949610501206
Language:
English
Publisher:
SAGE Publications
Publication Date:
1996
detail.hit.zdb_id:
2033055-8
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