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Progressive deafness with stapes fixation

ORPHA:3235· ICD-10 H74.3

Definition

Stapes fixation (stapediovestibular 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).

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Childhood