top of page

BackTable / ENT / Article

Cholesteatoma Causes, Symptoms & Clinical Presentation

Author Melissa Malena covers Cholesteatoma Causes, Symptoms & Clinical Presentation on BackTable ENT

Melissa Malena • Aug 30, 2023 • 38 hits

A cholesteatoma is a skin lined cyst within the ear that is especially susceptible to infection. Cholesteatomas are classified as either a congenital or acquired. 98% of all cholesteatoma cases are acquired and risk factors for development range from immune disorders to craniofacial malformations. According to expert ENT Dr. Gauri Mankekar, pediatric and adult cholesteatoma patients often have different clinical presentations and symptoms, with children at a higher risk for hearing loss and complications. This article features excerpts of the BackTable ENT Podcast. We’ve provided the highlight reel in this article, and you can listen to the full podcast below.

The BackTable ENT Brief

• Cholesteatomas are defined as congenital or acquired, and often present with chronic foul-smelling drainage and hearing loss.

• Risk factors for acquired cholesteatomas include eustachian tube dysfunction, cleft craniofacial malformations, turner syndrome and immune deficiencies leading to recurrent otitis media.

• In diagnosing acquired cholesteatomas, culture-directed antibiotic treatments are essential, especially if the patient has been pre-treated. An audiogram assists in assessing the impact on hearing, and imaging studies can be conducted if deemed necessary.

• Common microorganisms in ear cultures are pseudomonas, especially in cases with an infected PE tube. Other common organisms include staphylococcus and corynebacterium. Appropriate antibiotic treatment is essential when pseudomonas is detected.

Cholesteatoma Causes, Symptoms & Clinical Presentation

Table of Contents

(1) Cholesteatoma Classification & Presentation

(2) Acquired Cholesteatomas: Risk Factors & Presentation Differences in Adults vs Children

(3) Identifying Cholesteatoma in Pediatric Patients

(4) Medications for Cholesteatoma Treatment

Cholesteatoma Classification & Presentation

Cholesteatoma, can be primarily classified into two major categories: congenital and acquired. Congenital cholesteatomas, accounting for a mere 2% of cases, emerge from embryonic rest of the squamous cell in the middle ear and are usually identified incidentally during pediatric ear examinations. Contrarily, acquired cholesteatomas are further divided into primary and secondary types. While the former arises in the attic or pars flaccida, linked to perforations in that area, the latter emerges in the middle ear, associated with pars tensor retractions or perforations. The distinct clinical presentations also differentiate these types, with congenital forms often being asymptomatic and acquired types leading to symptoms like chronic draining ears, hearing loss, or even tinnitus and vertigo in some cases.

[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.

Listen to the Full Podcast

Evaluation and Management of Cholesteatoma with Dr. Gauri Mankekar on the BackTable ENT Podcast)
Ep 120 Evaluation and Management of Cholesteatoma with Dr. Gauri Mankekar
00:00 / 01:04

Earn CME

BackTable CMEfy button

Stay Up To Date

Follow:

Subscribe:

Sign Up:

Acquired Cholesteatomas: Risk Factors & Presentation Differences in Adults vs Children

According to Dr. Mankekar, acquired cholesteatomas can be attributed to a range of risk factors, from eustachian tube dysfunction to conditions like craniofacial malformations, Turner syndrome, and recurrent otitis media due to immune deficiencies. Clinically, it is vital to probe into the duration and onset of symptoms to paint an accurate picture of the ailment. Children, in particular, might manifest signs of hearing loss, such as raising the TV volume exceptionally high—a significant indicator of the ailment's magnitude. Furthermore, a history of multiple PE tubes might be present, though the connection between these tubes and cholesteatomas remains nuanced. Notably, the difference in presentations between children and adults is stark: children frequently show acute symptoms like mastoid abscesses or even meningitis, whereas adults present less commonly with such complications, though some might showcase labyrinthitis or vertigo.

[Dr. Gopi Shah]
In terms of kids, what percent of your practice are cholesteatomas in children after a history of ear tubes? What percent do you think makes up that pie?

[Dr. Gauri Mankekar]
I would say about 5% or so.

[Dr. Gopi Shah]
Okay. You're a referral center, so you're going to get more, but it is something that you're seeing.

[Dr. Gauri Mankekar]
Yes. There is definitely a history of multiple sets of PE tubes, but when I typically see them, there is no tube in place, and I do see in growing squam through the perforations where the PE tube was, but it's difficult to associate that as being related to the PE tube. It could be just that the eardrum did not heal, and it's trying to heal, and that's how the squam is trying to grow, but it's not growing towards the edges, it's growing inwards.

[Dr. Gopi Shah]
In terms of differences in presentation in your children and adults, with kids, we think about school, we think about grades. Adults, I think about work, but tell me what you see as some of the big differences in presentation.

[Dr. Gauri Mankekar]
With children, typically they will present with acute presentations. Sometimes I have seen more mastoid abscesses in children compared to adults, and they can also present with meningitis and facial palsy in adults. I haven't seen as many presenting with those complications, although some of them could present with meningitis, and also with vertigo. So labyrinthitis, for example, which is rare. Labyrinthitis in children, I haven't seen many.

Identifying Cholesteatoma in Pediatric Patients

In the diagnostic journey of cholesteatoma, physicians often rely on the power of physical examinations, which holds true both for congenital and acquired types. Dr. Mankekar emphasizes the significance of patient history and the clinical manifestations of acquired cholesteatoma, which may present as foul-smelling drainage or squamous debris in the ear. Notably, a clinician's skill in differentiating between common presentations, such as wax or retraction pockets, and cholesteatoma can be crucial, especially in pediatric patients. Dr. Mankekar advises the gradual building of trust with younger patients to facilitate more effective examinations. In cases with ambiguous findings, further investigation with audiograms and imaging can elucidate the diagnosis.

[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.

Medications for Cholesteatoma Treatment

Dr. Mankekar emphasizes the importance of tailoring ear treatments to the individual needs and circumstances of patients, particularly when affordability becomes a hurdle. Dr. Mankekar leans towards using Ofloxacin drops, keeping in mind the economic constraints of Medicaid patients, and offers steroids separately if necessary. Furthermore, a significant revelation made in the conversation is that cholesteatoma can sometimes have minimal effects on audiograms due to their disruptive yet subtle presence. They creep around the ossicles without causing extensive damage, occasionally resulting in almost normal hearing. Yet, the paradox is that clearing this disease might worsen the patient's hearing, making the physician-patient pre-surgical discussions complex and crucial.

[Dr. Gopi Shah]
In terms of the ear drops, do you prefer just ciprofloxacin with dex? It's expensive sometimes. Do you end up switching? Is floxin just as okay, or how strongly do you feel about having the steroid combo?

[Dr. Gauri Mankekar]
My patients are typically Medicaid patients, and they can't afford the drops. I have found that compliance is better if I just have them use the Ofloxacin drops. I just go with that, and if I have to use steroids, then I would give them as a separate, so it would be a prednisone or dexamethasone separately. That works out much cheaper, and so, it's less about ideals versus what's practical.

[Dr. Gopi Shah]
In terms of the steroid, is that like an ophthalmic drop that you use?

[Dr. Gauri Mankekar]
Yes.

[Dr. Gopi Shah]
What's the percentage? Is it just dexamethasone? Is there a certain percentage for that?

[Dr. Gauri Mankekar]
I think it's 0.01, but I may have to look it up.

[Dr. Gopi Shah]
No, that's a good trick, because I know we used to sometimes use Xylocaine, which I think was the ophthalmic Ciprodex, which is a little bit cheaper as well. Then there was the Otovel, the one with the ampules. The cost is cost prohibitive whether it's Medicaid or commercial, and obviously uninsured, and it can range, but we can get into that on a different podcast.

[Dr. Gauri Mankekar]
Yes. One of my residents found out he was prescribing Ciprodex, and I said, "Okay, check out the prices, and then you'll know."

[Dr. Gopi Shah]
In terms of oral antibiotics, when I saw granulation tissue, it definitely do some orals, but I wasn't culturing just with the hopes that if I could clear it with the suction, or every once in a while if it was significant thick drainage and I couldn't suction the ear, I'd have the families-- you know the baby blue bulb syringe that flares out for the nose?

[Dr. Gauri Mankekar]
Yes.

[Dr. Gopi Shah]
Because it flares out, I don't think they're going to be able to push it far deep. I would say, "Hey, you have about five to seven millimeters, and you can try to clear your ear and get the drops in hopefully." In terms of culture-directed antibiotics, what would be common microorganisms that you would find in your culture?

[Dr. Gauri Mankekar]
Sometimes it is pseudomonas, and especially if they have an infected PE tube, I do get pseudomonas, and then often it is just a staphylococcus, or corynebacterium. If it's just corynebacterium, the antibiotic sensitivity doesn't matter as much. Anything works, but, yes. If it turns out to be pseudomonas, then I have to be more vigilant about giving the culture-directed antibiotics, and for a longer duration.

[Dr. Gopi Shah]
Do you like oral cipro, or what do you normally do? What's the five antibiotics you end up having?

[Dr. Gauri Mankekar]
For pseudomonas, it could be ciprofloxacin or levofloxacin versus augmentin or amoxicillin and clavulanic acid.

[Dr. Gopi Shah]
In terms of the audiogram, every once in a while, and especially, and maybe the congenital cholesteatomas that are caught relatively early, sometimes the audiograms look pretty good, like normal. Do you find that too in cholesteatoma? It's maybe a mild PTA of 26 to 30?

[Dr. Gauri Mankekar]
Yes.

[Dr. Gopi Shah]
Then you do surgery, and it's like, "Ugh." How often do you see where the hearing is? It's maybe a little lost but it doesn't look too bad?

[Dr. Gauri Mankekar]
Yes. That is so amazing about cholesteatoma, because they are disruptive, but sometimes they creep around the ossicles, and so they don't destroy the ossicles. You may find, especially in congenital cholesteatoma, you might find intact ossicles, and that's why the hearing is almost near normal. The other thing about cholesteatomas is that they transmit sound. That's what causes us to believe there is no hearing loss. I will advise parents in these situations, families as well, that clearing the disease may worsen the hearing loss. There is an informed discussion before I recommend surgery discussing that the surgery and clearing the disease may worsen the hearing loss because the disease is what is helping them to hear. That's a difficult discussion.

Podcast Contributors

Dr. Gauri Mankekar discusses Evaluation and Management of Cholesteatoma on the BackTable 120 Podcast

Dr. Gauri Mankekar

Dr. Gauri Mankekar is an otolaryngolgist and head and neck surgeon with Ochsner LSU Health in Shreveport, Louisiana.

Dr. Gopi Shah discusses Evaluation and Management of Cholesteatoma on the BackTable 120 Podcast

Dr. Gopi Shah

Dr. Gopi Shah is a practicing ENT at UT Southwestern Medical Center in Dallas, TX.

Cite This Podcast

BackTable, LLC (Producer). (2023, July 18). Ep. 120 – Evaluation and Management of Cholesteatoma [Audio podcast]. Retrieved from https://www.backtable.com

Disclaimer: The Materials available on BackTable.com are for informational and educational purposes only and are not a substitute for the professional judgment of a healthcare professional in diagnosing and treating patients. The opinions expressed by participants of the BackTable Podcast belong solely to the participants, and do not necessarily reflect the views of BackTable.

backtable-earn-free-cme.jpg

Podcasts

Evaluation and Management of Cholesteatoma with Dr. Gauri Mankekar on the BackTable ENT Podcast)

Articles

Topics

Learn about Pediatric ENT on BackTable ENT

Get in touch!

We want to hear from you. Let us know if you’re interested in partnering with BackTable as a Podcast guest, a sponsor, or as a member of the BackTable Team.

Select which show(s) you would like to subscribe to:

Thanks! Message sent.

bottom of page