Kyphoscoliotic EDS (kEDS), associated with PLOD1 or FKBP14 gene mutations, presents with severe congenital hypotonia, progressive kyphoscoliosis, ocular fragility (e.g., scleral rupture), joint contractures, and, in some PLOD1 cases, hearing loss (7). Here, PLOD1 is linked to hearing loss disorder.