hereditary disease progressive deafness with stapes fixation https://rarediseases.info.nih.gov/diseases/5170/progressive-deafness-with-stapes-fixation https://www.malacards.org/card/deafness_progressive_with_stapes_fixation_2 GARD:0005170 icd11.foundation:1909954882 Thies-Reis syndrome MESH:C563316 OMIM:601449 Thies Reis syndrome Stapedo-vestibular ankylosis Orphanet:3235 Stapes fixation (stapedovestibular ankylosis) is a hearing loss condition that appears as a consequence of annular ligament destruction followed by excessive connective tissue production during the healing process. This condition is mainly observed in otosclerosis, but is also found in chronic otitis media with tympanosclerosis, and other rare bone diseases such as Paget's disease and osteogenesis imperfecta (Lobstein disease). SCTID:715529009 MEDGEN:330446 MONDO:0011080 UMLS:C1832354