Cholesteatoma Causes, Symptoms & Clinical Presentation

[Dr. Gopi Shah]
We're going to talk today about a pretty big topic. We're going to talk about cholesteatoma, but before we get into the clinical nuts and bolts, can you go over the different types of cholesteatoma when you're discussing it with a family or maybe a medical student?

[Dr. Gauri Mankekar]
Yes. Cholesteatoma is traditionally classified as congenital cholesteatoma and acquired cholesteatoma. Congenital cholesteatoma is typically seen in children, and accounts for about 2% of all cholesteatomas, and they develop from the embryonic rest of the squamous cell, and in the middle ear. They're typically found in the front or the anterior superior part of the middle ear. Acquired cholesteatomas have traditionally been further divided into primary cholesteatoma and secondary types. The primary cholesteatoma develops in the attic or the pars flaccida, and are associated with perforations of that part, whereas the secondary cholesteatomas typically develop in the middle ear through the pars tensor retractions or perforations.

[Dr. Gopi Shah]
As we now talk about how patients present to you, we think of traditionally cholesteatoma as a painless, chronic draining ear. Are those some of the most common symptoms that you see, and does the type of cholesteatoma present differently in your experience?

[Dr. Gauri Mankekar]
Yes. I have seen in practice and in my experience, they present a little differently. For example, congenital cholesteatomas are incidental findings. So either a pediatric or otolaryngologist will notice it when they take up a child for either your microscopic or serum removal, or even the myringotomy, and they're seen as just white masses behind an intact eardrum. On the other hand, the cholesteatomas which are typically seen in adults, sometimes also in children, they present as chronic draining ears, and foul smelling drainage from the ears, hearing loss, and the drainage can wax and wane.

Sometimes it's a lot, and sometimes it's canty, and so that's typically the presentation, although sometimes there could be an acute inflammatory phase where they present with some bleeding from the ear, and there could be pain associated. Hearing loss is the main issue with the drainage.

[Dr. Gopi Shah]
I wanted to ask you, how often do you see your patients with cholesteatoma that present with tinnitus or vertigo?

[Dr. Gauri Mankekar]
So with children, they typically cannot tell you about tinnitus or vertigo, but the adults, they do present, often they will not mention it. It's only on inquiry that they will talk about the tinnitus. The vertigo though if there are complications, then, yes, some of them do present with vertigo, and they will talk about it.

[Dr. Gopi Shah]
In terms of risk factors, and it goes to the types of cholesteatomas, when I think of the primary acquired, I think of eustachian tube dysfunction, which could be due to different reasons, including craniofacial abnormalities with something more anatomic to what other risk factors for-- and we can just go through the risk factors for the acquired primary, acquired secondary. Are there any risk factors for congenital? I know sometimes, is it okay to say it's just bad luck?

[Dr. Gauri Mankekar]
Yes, I think so. With congenital cholesteatoma, it's quite often bad luck.

[Dr. Gopi Shah]
In terms of just getting into the history, I feel like cholesteatoma is something that we're taught that this is a physical exam, like you're going to see it on an exam, and it's a clinical diagnosis. I guess, first my question is-- and it might depend on the type, and you kind of talked about this with the congenital cholesteatomas behind the intact drum, anterior superior quadrant, the white pearl. What do you see with your acquired cholesteatomas depending on if it's primary or secondary?

[Dr. Gauri Mankekar]
First thing would be profuse drainage in the ear, and it's usually foul smelling. It could be greenish in color, and once I clear that drainage, then I might see squamous debris either in the middle ear through a perforation, or an attic perforation, or a pars flaccida perforation with a lot of debris. Sometimes I will see granulation inflammatory tissue, and that bleeds on touch, and it's somewhere along the bone, and there could be a marginal perforation associated with it. Then, sometimes through that perforation I can also see the incudostapedial joint if it's present. Sometimes just the stapes head, the eustachian tube opening, and very rarely even the round window membrane, or the niche.

[Dr. Gopi Shah]
Do you usually do the exam with microscopy? Is there ever a time where you're like, "Maybe I need to get a better look with auto endoscopy," or do you have a preference?

[Dr. Gauri Mankekar]
Right now in my practice, it's mainly a microscope in the clinic, and so, it's only in the OR that we are using endoscopes, but in the clinic, we are only using the microscope.

[Dr. Gopi Shah]
I find in kids, to get an exam, it can be pretty challenging. I could potentially hurt them more depending on the kid as well, but sometimes getting around the corners. Any tips or tricks in terms of getting a good exam? Because sometimes it's hard to tell, is it just wax on the drum? Is it a retraction pocket? Is the granulation I'm seeing? I touch it, it bleeds and that's it. Game over. I can't tell if there's a perf, how do you troubleshoot some of that? Every once in a while I've had to tell families like, "This is a pretty bad infection." The granulation tissue, and how long it's been going on for. These are the things I'm worried about, and I might include cholesteatoma differential, but I'm not 100% sure. What tips and tricks do you have for a good exam, or if you're not sure at what you're looking at?

[Dr. Gauri Mankekar]
Yes, children can be very challenging as you mentioned.

[Dr. Gopi Shah]
You have to gain their trust because it's going to be one of many exams.

[Dr. Gauri Mankekar]

Absolutely. If you hurt a child once, they will never trust you again. So, yes, it's very challenging. That's the first thing I try. I try to be friends with the child, and then see if they'll let me examine, if they don't, and if there's a lot of drainage that I have to clean out, sometimes I would wait until the second visit. So, because my first visit is just trying to get the child to know me and trust me. I would typically give them antibiotic ear drops, and see them maybe in a week or two weeks so I have gained their trust. Also, the drainage is a little less, and it helps me to clean out the ear. Very rarely I may have to profuse a kid, but kids are mini adults, and so, if you gain their trust, they do let you clean their ears, and it is easier to see inside.

As you mentioned, it's difficult to differentiate granulation tissue with an infected tube in place versus a cholesteatoma. It's very important to diagnose if there is a cholesteatoma hiding behind it. With kids who have craniofacial malformations, especially with Down syndrome kids, those are the kids that I would like to take under anesthesia and examine them. I do individualize the exam depending on the situation.

[Dr. Gopi Shah]
I'm glad you brought that up because, our children with Down syndrome, every once in a while it's a difficult exam. Sometimes a child with autism with a chronic draining ear, there's concerns of speech. Their hearing has been down for months now, we can't get it dry. Going to the OR, it happens, where it's an exam, and the exam though, if we don't get it right, we either aren't going to be able to treat it or missing the elephant, in the ear, in the room. That can cause more problems. I used to kick myself like, "I can't get the exam," but then it's like, "It's okay." There's going to be a handful of kids, and they don't all have to have Down syndrome or Autism. It might just be the four-year-old that just won't let you in their ear or whatnot.

I feel better about that now. Oh goodness. All right. In terms of, let's say you do see a cholesteatoma, let's say the ear is a little inflamed, that's your diagnosis. What are your next steps in terms of cooling it down, and then, your workup in terms of ideogram imaging, things like that?

[Dr. Gauri Mankekar]

I will typically get a culture from that drainage, and culture-directed antibiotics because most often the kids that I see have already been treated by their primary care pediatrician or a pediatric otolaryngologist. When I'm seeing the kid, I want to be sure it's culture-directed. That's what I start with. I do start with an antibiotic ear drop, and then the oral antibiotics as soon as I get the culture results back. But I will get an audiogram. If a child is older, then just a pure-tone audiogram, and then if I'm suspecting a CT scan, then I will get imaging studies. That would be my primary workup towards diagnosing the patient.