Progressive deafness with stapes fixation

Orpha code: 3235OMIM code: 601449

Definition

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).

Disease data
Classification

Malformation syndrome

Synonyms
Progressive hearing loss with stapes fixation
Ankyloza strzemiączkowo-przedsionkowa
Zespół Thiesa i Reisa
Stapedo-vestibular ankylosis
Thies-Reis syndrome
ORPHA code
3235
OMIM code
601449
ICD10 code
H74.3
ICD11 code
LD2H.2

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl