Cochlear Implant

Question: We are currently being evaluated for the possibility of receiving a cochlear implant in one ear. His loss is presently mild on the left ear and severe on the right. My question is about the research regarding improvement in hearing? He currently has a significant amount of fluid and wax built up in his ears, as well as small ear canals. I am nervous about making such a big decision, like a cochlear implant, when I have heard from other parents that the hearing improvement is often significant. He has been diagnosed with the SMC3 variant of CdLS with a missense mutation, and the genetics team here has said it is a mild presentation. There doesn’t seem to be much research on SMC3. Is there anything you can tell me that I can’t find online? He also has ptosis and a mild form of nystagmus. Do you have information on the nystagmus? We are also going to have surgery for the ptosis in a few weeks. Is this something you would recommend?

Answer: The decision to do pros is surgery depends on symmetry (the more asymmetric the more likely it is to impact vision), the amount of chin lift (to see under the droopy lids), age (younger age with worse ptosis is more likely to affect vision development, and the vision ( if one eye is getting amblyopia (lazy vision) from ptosis we are more likely to do surgery) Nystagmus surgery is most often performed on people who wait till about 3 years old, although some believe earlier surgery is best.

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