Abstract
Osteogenesis Imperfecta is a rare genetic disorder of connective tissue that is caused by an error in collagen formation. The disease is characterized by abnormal bone fragility, osteopenia, blue discoloration of the sclerae and hearing loss. Chronic non-suppurative otitis media is frequent in Osteogenesis Imperfecta patients and usually attributed to Eustachian tube dysfunction due to cranial molding and deformities. In some cases of severe Osteogenesis Imperfecta, the fragile bone of the petrous carotid canal can be broken down by the pulsations of the carotid artery, this may result in prolapse of the carotid artery into the protympanum with resultant Eustachian tube obstruction and tympanic membrane retraction with adhesion to prolapsed carotid artery, a condition called myringocarotidopexy.
Highlights
Osteogenesis Imperfecta (OI) is a rare genetic disorder of connective tissue that is caused by an error in collagen formation
In this report we present a 45-year-old lady with bilateral Chronic non-suppurative otitis media (COM) bilateral eustachian tube dysfunction caused by prolapsed Internal Carotid Arteries (ICA) with resultant adhesive otitis media and myringocarotidopexy
A 45-year-old Qatari female patient who is a known case of osteogenesis imperfecta, presented to the clinic complaining of reduced hearing in the right ear for long time, not associated with any other symptoms
Summary
Osteogenesis Imperfecta (OI) is a rare genetic disorder of connective tissue that is caused by an error in collagen formation. The disease is characterized by abnormal bone fragility, osteopenia, blue discoloration of the sclerae and hearing loss. (2015) Carotid Artery Prolapse and Myringocarotidopexy in Osteogenesis Imperfecta. Hearing loss is a common problem in patients with OI and it affects 30% - 60% of patients [1]-[5]. Conductive hearing loss (CHL) in OI is often attributed to otosclerosis-like stapes fixation, ossicular discontinuity and chronic otitis media [2]. Chronic non-suppurative otitis media (COM) is frequent in young OI patients and usually attributed to eustachian tube (ET) dysfunction cranial molding and deformities [9]. In this report we present a 45-year-old lady with bilateral COM bilateral eustachian tube dysfunction caused by prolapsed Internal Carotid Arteries (ICA) with resultant adhesive otitis media and myringocarotidopexy
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