摘要
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 conventionalhearing 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.
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 “3^rd 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.
