Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000826050 | SCV000967544 | likely pathogenic | Rare genetic deafness | 2019-06-20 | criteria provided, single submitter | clinical testing | The p.Arg1888Gly variant in TECTA has been reported by our laboratory in 1 individual with mid-frequency moderately-severe sensorineural hearing loss in whom the variant was confirmed de novo. This variant was absent from large population studies. Computational prediction tools and conservation analysis suggest an impact to the protein. In summary, although additional studies are required to fully establish its clinical significance, this variant meets criteria to be classified as likely pathogenic for autosomal dominant nonsyndromic hearing loss. ACMG/AMP Criteria applied: PS2, PM2, PP3. |