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Hearing Loss in Children with Osteogenesis Imperfecta (OI) Treated with Bisphosphonates
Author(s) -
Arnerich Christi A.,
Moberly Aaron C.,
Dimeglio Linda A.,
Miyamoto Richard T.
Publication year - 2009
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.20441
Subject(s) - osteogenesis imperfecta , presentation (obstetrics) , medicine , section (typography) , library science , computer science , surgery , pathology , operating system
• 21.4% of children developed hearing loss, a percentage similar to previous population studies. • Since all received treatment, we cannot determine directly bisphosphonate effects on hearing loss over time. • However, it appears that bisphosphonates neither increase hearing loss nor stop progression in these children. CONCLUSIONS • Osteogenesis Imperfecta (OI) is a genetic disorder of collagen synthesis. It is commonly associated with increased fracture rate, decreased bone mineral density (BMD), and blue sclerae. • It is also associated with hearing loss, sensorineural and/or conductive, which progresses with age. Hearing loss is most prevalent in OI Type I. • There are several forms of OI ranging in severity: -Type I, mild with blue sclerae -Type II, perinatally lethal -Type III, progressively deforming with white sclerae -Type IV, severe with white sclerae -Additional severe forms also described (Types V, VI, VII) • Treatment includes IV or oral bisphosphonates to increase bone density, decrease fracture rate, and decrease bony pain. • Bisphosphonates have been associated with osteonecrosis of the mandible and osteopetrosis of long bones in some cases. • Effects of bisphosphonates on hearing, specifically the middle ear ossicles and cochlea, have not yet been determined. BACKGROUND Figure 2: Femoral bowing in an OI type III patient. Lucencies between lines along the epiphyses signify bone growth following treatment with bisphosphonates. METHODS