Is it necessary to screen for hearing loss in the paediatric population with osteogenesis imperfecta?

Abstract

The aim of the study was to assess the necessity of a screening service to detect early hearing loss in the paediatric population with osteogenesis imperfecta. Twenty-two children were assessed over a 5-year period. Five children (22.7%) had normal hearing. Fourteen (63.6%) had conductive hearing loss, with 12 children in this group having otitis media with effusion (OME); all had resolution of hearing loss with appropriate therapy. Two children had persistent conductive losses unrelated to OME. Three children (13.6%) had sensorineural hearing loss, with one being detected at the age of 1 year. Existing evidence suggests that hearing loss associated with osteogenesis imperfecta has its onset in the second to third decade of life. Contrary to this, hearing loss was detected in 77.3% (17) of this population with a median and mean age of 9 years. This study would suggest that routine screening is worthwhile in children with osteogenesis imperfecta.

Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

“…One recent study reported a prevalence of sensorineural hearing loss in 12% of ears, conductive hearing loss in 21% of ears, and mixed hearing loss in 29% of ears [18] in patients with OI. As has been reported in other investigations [17, 22], sensorineural hearing loss was present in a younger age group consisting of children (mean age = 9.87) and was found only in association with type I OI [18]. …”

“…Slightly lower prevalence of hearing loss (50%) [19, 20] or even a higher prevalence of hearing loss (65–72%) [16, 21] has been reported. Methodological or definitional differences may account for the varying results across studies [22]. Some investigators have excluded cases of conductive hearing loss believed to be secondary to otitis media with effusion (OME) in younger patients with OI [16, 23] which would lead to underestimation of the prevalence of hearing loss.…”

“…Some of these complications can result in hearing loss. Some studies have excluded patients with these or other complications believed to be unrelated directly to OI [17], whereas others have not [18, 22]. …”

“…However, hearing loss in younger patients with OI is not uncommon [17, 22], and mixed or sensorineural hearing loss may be observed at any age [17]. When sensorineural hearing loss is found in children, it is typically found in type I OI [17, 18].…”

“…In younger patients with OI, hearing loss was not as common but was still present in 38% of ears [18]. Other studies with pediatric patients with OI have reported prevalence rates ranging from 9.1% [35] to 77.3% [22]. When patients with a range of medical conditions including otitis media with effusion, skull fracture, and neonatal meningitis are included in the sample studied, higher prevalence rates are reported [22].…”