CIborg bloggen til en ung voksen CI-bruker

In my last post I wrote why I want CI no 2 and explained why adults today are not offered two implants. I know others whose application for CI no 2 was declined, and had no expectations when I set up an appointment with my hearing doctor to form an application. My appointment was at August 24th and I spoke with dejected doctor who was beaten down, defeated and almost giving up the political fight to offer adults two implants.

The doctor showed me a note from all hospitals in Norway which do CI surgeries (which is Rikshospitalet in Oslo, Haukeland in Bergen and the hospital in Trondheim). The note stated that no adults (above 18 years) are offered CI no 2 unless they have:

• Meningitis.
• Severe tinnitus.
• Visual issues, for example Usher syndrome.

Even though my expectations were quite low at first, I was still a little defeated by the short list. I had hoped for some leeway for people with considerable profit by two implants or other medical reasons. I had a small amount of hope that my Pendred syndrome, which makes me gradually deaf, would be good enough a medical reason to get CI no 2.

The hearing doctor stated that this is a political fight, e.g. through associations such as Association for hard of hearing in Norway (HLF). Unfortunately it’s nothing the doctors can do, except help forming a good application (which probably are rejected nonetheless).

I expressed my concern that Pendred syndrome would suddenly “strike” and destroy all my hearing I have left on my right ear. There’s individual differences in the time aspect, but if you don’t use the hearing nerve over a long period of time (several years) the nerve will eventually die and stop working for good. When that’s happened there’s no use operating a CI, and I’d had to go the rest of my life hearing with only one ear.

My hearing doctor and I agreed to form and send an application for CI no 2, using Pendred syndrome as a medical reason. In writing I’m still anxiously waiting for a letter from one of Norway’s three hospitals. Unfortunately I’m 90% sure the letter is a rejection. In that case I’d have to start a hard battle in order to get optimal hearing.

When you (finally) receives a letter from the hospital the first step is to evaluate you. This spans in two phases and often happens with several weeks inbetween. (Call the hospital if it takes too long to receive info on the second phase).

Phase 1

In the first phase they will test your physical basis for CI. During a day on the hospital you get a CT/MR-scan, taken blood of and questioned about your medical condition. Completely safe tests. It’s mostly boring because of all the waiting. You don’t have the opportunity to ask someone about CI or surgery. The doctors you come in contact with in this phase has no connection whatsoever to the CI team, their job is just to perform the tests.

You are then sent home to wait a few weeks for a second letter for your second phase. This could take several weeks. I called the hospital when I didn’t receive a letter after a month or so. The hospital excused and sent the letter that very day.

Phase 2

During the second phase you will meet the CI team who will test your hearing as it works for you today, this normally take two days (you normally leave home the first day and come back the next). A person from the CI team is arranged to meet you, guide you and answer your questions during those two days. You will take a normal hearing test, other hearing tests and a chance to talk to a geneticist about your hearing loss. All the tests from phase one and two are then weighed up and decides whether you will benefit from CI. But the most important test is the sentence-test, where you sit in a echo-free room with speakers that play sentences you have to repeat.

My contact from the CI team came and brought me in a room to talk about CI. This is your chance to ask all your questions! After I completed the normal hearing test (where you sit in a soundproof room with earmuffs without hearing aids, and give signals when you hear something) my contact believed I would benefit well from CI because my speaking was well developed and my utilization of the remainder of my hearing was very good. People who are born deaf and have not used hearing aids the first years of childhood and not developed a good spoken language, are normally refused CI. In addition CI only help those who have a well-functioning hearing nerve but with damage in the inner ear.

But my contact changed her opinion after the sentence-test. I think I got 85% or more, which conflicted with my bad hearing. Even though the CI team and the doctors understood my good score was because of my ability to guess, I was now risking a rejection. I protested and asked for a more realistic test. Nothing is less similar to real life than a completely quiet and echo-free room where the sentences are pronounced slowly, clearly and loudly. During the second day I did another sentence-test with background noise, but my score was still too good.

My chance of getting CI was poor, but that changed after speaking to the geneticist. The geneticist had called experts on x-ray getting them to take a look at my CT-scans and confirmed I had an underdeveloped cochleas. Instead of the normal 2,5 turns I had only 1,2 on both of my cochlea. In addition I had something called enlarged vestibular aqueducts inside the cochleas. He diagnosed me with Pendred syndrome, a genetic disorder that in 50% of the cases combine goitre and those specific malfunctions in the cochleas. This also means that the increased pressure in my cochleas will gradually destroy all my hearing until there’s nothing left. That I’m gradually turning deaf didn’t grasp me before several weeks after this conversation.

Only five minutes after my conversation with the geneticist I had my final conversation with a chief surgeon and the CI team. They offered me a CI surgeon despite my good score at the sentence-test – Pendred syndrome alone was reason good enough. I was still bewildered and needed to think about it so I didn’t sign. I got the acceptance letter with me home and a new session a month later for signing and approving CI surgery.

Next phase: Getting CI: Deciding.