Dr. Gopen:  Yes. They don’t complain about it and as they are growing up their brain takes a lot of what it’s given. And so they don’t understand that it’s not normal to hear their heartbeat in their ear or they don’t understand that their hearing should be better. So unlike an adult who complains about hearing loss pretty much immediately kids don’t tend to complain about hearing loss. And that does make it more tricky and difficult to diagnose the condition in the pediatric population.

Is it sometimes caught as a learning disability?

Dr. Gopen:  Certainly. Hearing loss often is perceived as a delay and so they think that the child is slow or there’s some developmental difficulty and it’s really just hearing loss. It tends to occur with some balance problems so the kid can be a late walker. Instead of walking at twelve months or fourteen months they might not walk until they’re two years of age. And no one really at the time thinks much of it but in retrospect in some of these cases it’s very clear that they walked very late and they do have hearing loss and it’s from this condition.

With Kerrie’s ear you said the hole was just two millimeters. Can you put that in perspective for people? How do you find a hole that small?

Dr. Gopen: It’s a pretty tiny little hole. When I’m seeing patients in the clinic I give them a demonstration. I say this hole is smaller than the tip of my pen. Two or three millimeters is a very small little pore that’s opening. So even on the CAT scans it’s hard to appreciate exactly how big that hole is. But it’s a very, very small little pore. One of the main challenges with the surgery is to find that little opening. Part of what helps is just knowing the anatomy and with the CT scan you can look at it ahead of time and see what is the topography of that person and the floor of the skull base how does it look and where are the peaks and valleys. And this particular opening is almost always in one of the main little prominences in the bone called the arcuate eminence and so you look for that. And there are things that you can use intraoperatively to find that spot. There is image guidance which helps you to precisely say okay, where am I at on the scans. And that can be helpful although there is some play with that system. But it’s just really knowing the anatomy, knowing where the structure is supposed to be and then looking for that little pore. In some cases it’s pretty obvious and in Kerrie’s it was very obvious. But in other cases especially when it’s smaller and not as big a defect as she has it can be challenging to find that little spot.

So what do you do once you find the hole?

Dr. Gopen: In order to block it there’s been many ways that have been described but the way that I prefer to do it is to take a little bone wax which is a sort of tacky like material and to gently force it in to that small opening and that’s a water tight seal. I then line the area above that with fascia which is some of the lining of muscle. Then I take a small piece of bone which I get from the craniotomy which is the little plate of bone that’s removed from the side of the head and I take a small chip of that and place that over the area. And the purpose of that is really to block a new pressure from eroding a new location in to the inner ear. And that seems to be very effective at solving this problem. The patients wake up pretty much immediately with the resolution of their symptoms.

So is it an invasive surgery because you have to go through the skull?

Dr. Gopen: Yes, it’s a craniotomy. That’s what the neurosurgeons use to do brain surgery. So you’re talking about making a window in the side of the bone here, retracting the brain over about a couple of centimeters to get access to the floor where the brain rests and find that little pore. You can use a different technique to come in through the ear but that is more dangerous to the hearing because you actually have to drill through the inner ear to get to that little opening and block it off. And not as physiologic as just covering up the top part of the opening. You have to block off the hole from the outside.

Does it fix the problem forever?

Dr. Gopen: It usually does. There have not been a lot of recurrences of this. The condition was discovered in nineteen ninety eight so we don’t have long term follow up data to know twenty, thirty years out how these patients are doing. But in my experience I haven’t seen patients that have had the repair done successfully and then relapse later on the same side. I’ve had some contralateral sides come up with a new condition on the opposite ear but not on the same side that’s been repaired.

There’s not a lot of surgeries that are that invasive that you can do and the person wakes up and the problem is completely gone.

Dr. Gopen: Usually it’s more of a waiting game that’s right. I’ve had times where people wake up in tears not from pain but from joy because the noise is gone immediately after the surgery. It’s rare that you know the solution will be such a quick and dramatic improvement.

You changed Kerrie’s life. She said she’s a different person.

Dr. Gopen: Yes. She’s much better off now than she was back then that’s for sure.

You get a person like Kerrie who said she was contemplating suicide and now she’s out playing Frisbee and eating ice cream with her daughter and back to normal.

Dr. Gopen: Well it’s nice, it’s nice to be able to see that. Obviously it’s not something that’s happening every single day but it’s worth the time and the effort for sure.

She went to doctors for years. Why was it so tough?

Dr. Gopen: It’s certainly not a common condition. The more common the condition you have the more likely you are to get the standard of care. Certainly if you have a heart attack and you go to the emergency room you’re going to get a standard set of blood work done and treatment for that. This is a condition that is not super rare but rare enough that most people don’t know to look for it. Also, being in a location like UCLA where we’re dealing with tertiary referrals from all over the world these rare conditions are bunched up and you see them from time to time and then you’re more prepared to deal with them and better off at identifying them. I would encourage patients that until they’re fixed keep seeking out treatment. Keep pushing until you get the answers that you need to get. In some cases it can be a little bit of a battle but the end point is making people better. And until you’re better it’s worth pushing.

 

 

FOR MORE INFORMATION, PLEASE CONTACT:

 

Quinton Gopen, MD

UCLA Medical Center