Molecular analysis of the heterogeneity of hereditary monosymptomatic sensorineural hearing loss

Author(s): Lamprechtdinnesen, A., Preisleradams, S., Deufel, T.

Journal/Book: Laryngo Rhino Otol. 1996; 75: P O Box 30 11 20, D-70451 Stuttgart, Germany. Georg Thieme Verlag. 141-147.

Abstract: 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.

Note: Article P Fiegert, Univ Munster, Poliklin Phoniatrie & Padaudiol, Kardinal Von Galen Ring 10, D-48129 Munster, Germany

Keyword(s): hereditary hearing impairment; non-syndromic hearing loss; linkage analysis; X-LINKED DEAFNESS; SYNDROME TYPE-I; LONG ARM; COLLAGEN; LINKAGE; GENE; CLONING; CHROMOSOME-11; LOCALIZATION; ALPHA-1(XVI)

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