Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV003442899 | SCV001906475 | pathogenic | not provided | 2021-07-29 | criteria provided, single submitter | clinical testing | Identified with another SPATA5L1 variant in a patient with sensorineural hearing loss and a neurodevelopmental disorder referred for genetic testing at GeneDx Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss-of-function is a known mechanism of disease Not observed at significant frequency in large population cohorts (Lek et al., 2016) |
OMIM | RCV001779315 | SCV002016205 | pathogenic | Neurodevelopmental disorder with hearing loss and spasticity | 2023-04-19 | no assertion criteria provided | literature only |