CI candidate for cohlear and nerves hypoplasia?

I’m currently considering cochlear implant surgery due to bilateral hearing loss associated with cochlear hypoplasia type 4 (1 turn and half ) with nerves hypoplasia. However, I’m feeling uncertain about the potential success of the operation, particularly regarding whether the electrode (size 19 flex Medel) will fit properly in my small cochlea .

anyone who has undergone cochlear implantation under similar circumstances. If you or someone you know has experience with cochlear implants or similar conditions, I would love to hear about your journey and any insights you might have to offer.


any help pls ? any one experienced same case ?

I’ve had a CI but I don’t have your complications. Can I ask why you chose Med el? I’m just curious no other reason. Have you had your MRI and CT scan yet?

My only suggestion would be for you to take your scans and seek a second opinion from a very experienced CI surgeon. If that’s at all possible to do.
Good luck :four_leaf_clover: to you with your decision.


This is a copy and paste from a quick internet search.
Have you had your CI evaluation? The evaluation should answer your concerns. If you are questioning the that evaluation getting a second opinion might be in order, as deaf_piper mentioned.
@Neville might be able to help you.

“Cochlear nerve hypoplasia (VCN hypoplasia) is a common finding in unilateral sensorineural hearing loss (SNHL). VCN hypoplasia is when the cochlear nerve is smaller in diameter than the facial nerve. It can be accompanied by other inner ear malformations of varying severity, and can cause hearing loss ranging from moderate to profound”

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it s not my choice
it s the assurance choice so the cost will be from their own

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I think your have to talk to the surgical team? I don’t have experience with this; I don’t think we typically implant here when there is cochlear nerve hypoplasty. However, I’ve only seen unilateral hypoplasty so far, so those children can rely on their other ear. I have a vague memory of seeing someone in the USA discuss attempting CI for bilateral hypoplasty in advance of considering a brainstem implant.

I think you just need to have a serious talk with the ENT about outcome expectations.

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this is the response from my surgeon

"Thank you for your trust, but I would like to further clarify your situation:

  • we will never be sure of total or partial insertion of the electrode holder until intraoperatively, in fact the preoperative measurements of the diameter and length of the cochlea by CT and MRI are only approximate.
  • The response of your cochlear nerve during stimulation can only be assessed post-operatively during activation sessions, and the fact that you have a hypoplastic nerve can lead to a partial response, the degree of response after stimulation cannot in no case be guaranteed pre-operatively and therefore no one can guarantee you an optimal hearing restoration result.
    I hope it’s clear.
    Your situation is very rare and delicate, if you consent, and choose to be operated on, I ask you from this Monday to submit the request for treatment to the central CNAM Monplaisir, to carry out the vaccination with a doctor as soon as possible and carry out blood test, chest x-ray and ECG to be examined by the doctor.
    Good evening"

I don’t know anything about your specific condition. Only your doctor can give you information concerning how well a CI may work for you. And remember, there are no guarantees that it will work for any of us.

But I have a question. Can you hear anything now? I’m asking this because part of your decision to be implanted might be based on how much you have to lose. In other words, if you can’t hear now and it doesn’t help how much difference will there be? What if it only partly helps? Is the possible benefit worth it?

I was implanted last summer and I did this type of risk/benefit analysis before I decided. My ear was useless as it was. I decided that barring some very rare surgery complication, I had nothing to lose.

So really consider the possible outcomes in advance, and how they will effect you.


Okay, yeah, that response isn’t super helpful. I think what you sort of need to ask is, “have you implanted anyone with a similar case before or are you aware of anyone with a similar case have positive or negative outcomes?”

But I agree with db4art–what is your hearing function right now? How do you communicate now (spoken or sign language?)? And what are you hoping to improve?

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i m wearing phonak naida lumity up in both ears
i can hear well and i hve to face to read lips sometimes
unless i cannot understand speech in tv or any other electronics source like mobile etc
else i m struggle in noise so i have to read lips to understand

i m wish to have best hearing comprehension and up my Speech Recognition Scores
to understand speech well in any conditions
this is my current audiogram

Was there a time in your life when your high frequency hearing was better than this? Or is this essentially the loss you were born with? What country do you live in? If you take out your left hearing aid, do you feel like you lose much?

this is essencial hight frequescy loss from birthday
i m living in north africa
if i remove my hearing aid i dont hear anything

Does your lumity have bluetooth? That could help you a whole bunch with mobile phone, tv and other electronic devices. There are a whole lot of assistive devices that could help, too. TV connector. Roger devices. And struggling in noise can still be a problem after implants.

If I were you, I would want my doctor and audiologist to give me an honest assessment if a CI is right for you.

The doctor said his situation is very rare. Not sure if this answers your question.
If the OP can get a second opinion CI evaluation that might be helpful.
The high frequency loss from birth could be challenging.
If finances are not an issue, sure might be worth a try.

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Right, but does it make a difference to you? Do you hear just as well with only your right ear as your right and Left? Because chances are if you go forward with the surgery you will lose your residual hearing in that ear. But if that ear doesn’t contribute much anyway then the choice might be easier.