Published online Feb 28, 2016. doi: 10.5319/wjo.v6.i1.19
Peer-review started: October 2, 2015
First decision: November 3, 2015
Revised: December 19, 2015
Accepted: January 16, 2016
Article in press: January 19, 2016
Published online: February 28, 2016
Processing time: 150 Days and 11.8 Hours
X-linked deafness is a rare genetic disorder causing a severe mixed hearing loss. This is due to an abnormal connection between the internal auditory meatus (IAM) and the basal turn of the cochlear leading to a “3rd window effect” and cochlear conductive hearing loss. Patients are traditionally treated with conventional hearing aids however these are often unsatisfactory. Cochlear implantation is a high-risk procedure in such cases due to the risk of inadvertent electrode placement in the IAM. We present three paediatric cases where the hearing loss was managed with a combination of a bone anchored hearing aid in combination with a conventional behind the ear hearing aid. We also present a review of the current literature regarding the management of X-linked deafness.
Core tip: X-linked deafness is a rare genetic disorder causing a severe mixed hearing loss. This is due to an abnormal connection between the internal auditory meatus (IAM) and the basal turn of the cochlear leading to a “3rd window effect” and cochlear conductive hearing loss. Patients are traditionally treated with conventional hearing aids however these are often unsatisfactory. Cochlear implantation is a high-risk procedure in such cases due to the risk of inadvertent electrode placement in the IAM.