While hearing loss had not been reported in individuals with STLS, most of the reported patients are children and therefore it is plausible that in Puerto Rican STLS patients due to homozygosity for the missense c.2089G>C (p.Gly697Arg) variant, hearing loss may appear later in life versus early-onset hearing loss observed in individuals with more severe variants in COL27A1. Our data on the clinical characterization of adult STLS patients supports this conclusion. Here, COL27A1 is linked to Steel syndrome.