Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
Progressive hearing loss stapes fixation
0.010 Biomarker disease BEFREE Mild PRS-I deficiency (DFNX-2) results in non-syndromic progressive hearing loss whereas moderate PRS-I deficiency (CMTX5) and severe PRS-I deficiency (Arts syndrome) present with peripheral or optic neuropathy, prelingual progressive sensorineural hearing loss, and central nervous system impairment. 26089585 2015