Major and Minor Temporal Bone Abnormalities in Children With and Without Congenital Sensorineural Hearing Loss

Open Access
Publisher Website
Google Scholar
Abstract
ABNORMALITIES OF the temporal bone have been associated with congenital sensorineural hearing loss (SNHL) since Mondini1 described a deformity of the osseous labyrinth in a child with congenital deafness in 1791. Since then, variations in the size and configuration of the cochlear and vestibular complex and abnormalities in the size and shape of the internal auditory canal (IAC) and the vestibular aqueduct (VA) have been reported to occur in up to 20% of children with SNHL evaluated using plain-film polytomography2 and computed tomography (CT).3,4 Because of improvements in high-resolution CT techniques and a heightened awareness of cochlear modiolar defects, this yield has increased to 30%.5 Currently, abnormal findings not only provide diagnostic information but also aid in genetic and lifestyle counseling.