CHARGE Syndrome Canada Newsletter Summer 2008 Vol. 1 No.16 © copywrite |
MOBILITY OPPORTUNITIES VIA EDUCATON (M.O.V.E.)
TRANSITION RESOURCES
Past Edition of the Newsletter
From the mailbag:
Should we look further into the possibility of our child getting cochlear implants?
When parents find out their children have a hearing loss, one of the first questions is “what will the future hearing potential be”? The answers could be diverse. Each child will differ slightly in the ear anatomy that is affected, the hearing devise that has been prescribed, the age of diagnosis and therapy, and the amount of residual hearing combined with a varied degree of visual loss. All these factors affect outcome.
The state of the inner ear anatomy is now considered a key indicator that helps define this syndrome, because the unique radiological inner ear anatomy findings of the CT scan, does not occur in other syndromes. Because of the success of cochlear implants in other forms of hearing loss, we often receive questions on cochlear implantation and CHARGE syndrome.
A study done (Morimoto, A.K., et. al, 2006) looked at some of the specific inner ear conditions of children who were already diagnosed with CHARGE syndrome. This study confirms what many individual parents report about the findings of the inner ear. Radiological equipment has improved substantially in recent years, making it more possible to get specific evidence. Most of the children in the study had cochlea that were small or underdeveloped, and four had lacked a cochlea nerve. The round windows, oval windows and vestibules were also small or underdeveloped. The vestibular aqueducts were frequently affected, which may explain the sudden loss of hearing experienced by some older adults with CHARGE.
Again, as parents often report these unique findings, sometimes during surgical procedure itself, this study confirmed that in 88% of the cases, facial nerves were seen in the wrong location. Other findings included some cases where ear drums occasionally prolapsed, temporal bones were malformed, and small middle ear cavities or internal auditory canals.
The recommendation from this study, as well as other studies which also have documented some of these findings, is that cochlear implants would not be the best course of action for remediation of children with CHARGE syndrome.
Reference:
Absent Semicircular Canals in CHARGE Syndrome: Radiologic Spectrum of Findings, A.K. Morimotoa, R.H. Wiggins, IIIa, P.A. Hudginsb, G.L. Hedlundc, B. Hamiltona, S.K. Mukherjid, S.A. Teliane and H.R. Harnsbergera (2006) American Journal of Neuroradiology 27: 1663 – 1671, September.
~ Ann Gloyn, Education Specialist
newsletter submissions welcome - please send to ADMIN@chargesyndrome.ca