Background: Genetic characterization of hereditary hearing impairment has progressed considerably with the mapping of nine chromosomal loci for monosymptomatic autosomal-inherited hearing loss over the last three years.
Methods: Following thorough clinical evaluation, linkage analysis using microsatellite markers was performed in two large families from Westphalia/West Germany.
Results: For all the dominant (DFNA1--4) and three autosomal-recessive loci (DFNB1--3) described to date, linkage was finally excluded.
Conclusions: A high degree of genetic heterogeneity must be assumed. Identification of individual genes for monosymptomatic sensorineural hearing loss by linkage analysis in large pedigrees may help in molecular differentiation of hearing.
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