Kumar S, Mawby T, Sivapathasingam V, Humphries J, Ramsden J. X-linked deafness: A review of clinical and radiological findings and current management strategies. World J Otorhinolaryngol 2016; 6(1): 19-22 [DOI: 10.5319/wjo.v6.i1.19]
Corresponding Author of This Article
Sonia Kumar, PhD, SpR Otolaryngology, ENT Department, John Radcliffe Hospital, Headley Road, Oxford OX3 9DU, United Kingdom. soniakumar111@googlemail.com
Research Domain of This Article
Otorhinolaryngology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Otorhinolaryngol. Feb 28, 2016; 6(1): 19-22 Published online Feb 28, 2016. doi: 10.5319/wjo.v6.i1.19
X-linked deafness: A review of clinical and radiological findings and current management strategies
Sonia Kumar, Thomas Mawby, Vanaja Sivapathasingam, Jane Humphries, James Ramsden
Sonia Kumar, Thomas Mawby, Vanaja Sivapathasingam, Jane Humphries, James Ramsden, ENT Department, John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom
Author contributions: Kumar S wrote the manuscript; Humphries J gathered the audiometric data; Mawby T and Sivapathasingam V reviewed and amended the manuscript; Ramsden J is the senior author and gave overall final approval for submission.
Conflict-of-interest statement: The authors report no conflict of interest and have no financial disclosures.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Sonia Kumar, PhD, SpR Otolaryngology, ENT Department, John Radcliffe Hospital, Headley Road, Oxford OX3 9DU, United Kingdom. soniakumar111@googlemail.com
Telephone: +44-1865-741166
Received: September 29, 2015 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
Abstract
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.
