AI Article Synopsis
- Audiograms were studied in 23 patients with Usher's syndrome from 10 Dutch families, focusing on the USH2A locus on chromosome 1q.
- A regression analysis revealed that 3 patients showed significant hearing loss progression, indicated by an annual threshold increase (ATI) of over 1 dB per year, while others displayed stable hearing.
- Compared to other reported cases linked to the USH3 locus, the progressive cases showed no significant variation in hearing loss progression.
Article 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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| http://dx.doi.org/10.1177/000348949610501206 | DOI Listing |
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