So far, 37 different loci for autosomal dominant, sensorineural, non-syndromic hearing loss (ADSNSHL), have been mapped and 11 genes have been cloned.1 Among them, theTECTA gene, mapped to chromosome 11q22-24 (locus DFNA8/A12), encodes α-tectorin,2 a non-collagenous component of the cochlear tectorial membrane. This membrane is an extracellular matrix that covers the apical surface of the sensory epithelium in the cochlea and plays an important role in transmitting the mechanical energy of sound to the mechanosensitive stereociliary bundles of the hair cells, where the sound is transduced into neural potentials. In previous studies, five different missense mutations resulting in ADSNSHL have been described in the TECTA gene in fourDFNA8/A12 families, affecting different domains of the protein and showing different phenotypes (table 1): a prelingual, non-progressive hearing loss affecting mid frequencies or a postlingual, progressive, high frequency hearing impairment.

In this study, we report a Spanish family with a novel phenotype of postlingual, progressive, mid frequency hearing loss resulting from a new mutation in the TECTA gene.