Cholesteatoma
Epidemiologia

Perlak ucha środkowego (cholesteatoma) to niszczące schorzenie o zachorowalności w krajach rozwiniętych na poziomie 3-14/100 000 rocznie, z wyższą częstością u dorosłych (9,2-13/100 000) niż u dzieci (~3/100 000). Perlaki nabyte stanowią 98% przypadków, a wrodzone są znacznie rzadsze (~0,12/100 000 dzieci), choć w Japonii obserwuje się wzrost wykrywalności do 26,44/100 000 urodzeń. Średni wiek diagnozy to 9,7 lat dla perlaka nabytego i 4,5 lat dla wrodzonego, z przewagą mężczyzn (M:K=1,4:1) w przypadku perlaka nabytego. Główne czynniki ryzyka to wywiad rodzinny (4-krotnie zwiększone ryzyko), dysfunkcja trąbki Eustachiusza, rozszczep podniebienia, przewlekłe zapalenie ucha środkowego (72,4% pacjentów z perlakiem miało wcześniejsze epizody) oraz alergiczny nieżyt nosa. Epidemiologicznie obserwuje się spadek częstości perlaka nabytego, co przypisuje się wcześniejszej diagnostyce i leczeniu, natomiast wzrasta wykrywalność perlaka wrodzonego dzięki postępom diagnostycznym.

Epidemiologia perlaka

Perlak ucha środkowego (cholesteatoma) jest stosunkowo częstym, niszczącym schorzeniem, które może prowadzić do poważnych powikłań. Mimo że jest znany w literaturze medycznej od ponad 300 lat, dokładne dane epidemiologiczne na jego temat są nadal ograniczone, ponieważ w wielu krajach nie jest to choroba podlegająca obowiązkowej rejestracji. 12

Częstotliwość występowania perlaka

Zachorowalność na perlaka w krajach rozwiniętych szacuje się na poziomie 3-14 przypadków na 100 000 mieszkańców rocznie. Dokładniej, badania wskazują na następujące wartości:12

  • U dzieci: około 3 przypadki na 100 000 rocznie12
  • U dorosłych: 9,2-13 przypadków na 100 000 rocznie12

W badaniu przeprowadzonym w Finlandii, średnia roczna zachorowalność wynosiła 9,2 na 100 000 mieszkańców (zakres 3,7-13,9) i znacząco spadała w okresie badania.1 Z kolei w Japonii, badanie epidemiologiczne w prefekturze Miyagi wykazało roczną zachorowalność na poziomie 4,2 przypadków na 100 000 osób.1

Interesujące są również dane z Korei Południowej, gdzie całkowita roczna liczba operacji perlaka zmniejszyła się z 3 502 w 2006 roku do 3 199 w 2018 roku (6 na 100 000). Średni wskaźnik operacji perlaka wynosił 0,006% i zmniejszał się w skali roku.12

Perlak wrodzony vs nabyty

Perlaki nabyte stanowią około 98% wszystkich perlaków ucha środkowego.1 Częstość występowania perlaka wrodzonego jest znacznie niższa i wynosi około 0,12 na 100 000 dzieci.1 Jednak w Japonii zaobserwowano, że całkowita częstość występowania perlaka wrodzonego wynosiła 26,44 na 100 000 urodzeń i miała tendencję wzrostową. Badacze przypisują to raczej zwiększeniu wykrywalności niż rzeczywistemu wzrostowi zachorowalności.12

Rozkład demograficzny

Perlak występuje we wszystkich grupach wiekowych, ale istnieją pewne wzorce demograficzne:12

  • Średni wiek diagnozy perlaka nabytego wynosi 9,7 lat, a wrodzonego 4,5 lat1
  • Szczyt zachorowań przypada na drugą dekadę życia1
  • W przypadku perlaka nabytego obserwuje się przewagę płci męskiej (stosunek M:K wynosi 1,4:1)12
  • U pacjentów poniżej 50 roku życia perlak ucha środkowego występuje częściej1

Co ciekawe, chociaż częściej chorują mężczyźni, to u kobiet częściej występuje choroba obustronna oraz nawroty choroby podczas wizyt kontrolnych.1 Według danych z Korei Południowej z 2018 roku, operacje perlaka były najczęściej wykonywane u pacjentów w wieku 50-59 lat.1

Czynniki ryzyka

Do głównych czynników ryzyka rozwoju perlaka należą:12

W badaniu brazylijskim stwierdzono, że częstość występowania perlaka u pacjentów z przewlekłym zapaleniem ucha środkowego wynosiła 24,5%, przy czym był on częstszy u dorosłych niż u dzieci.12 Z kolei badania w Stanach Zjednoczonych wykazały, że spośród 67 661 dzieci obserwowanych przez 10 lat, 36,5% miało zapalenie ucha środkowego, a 12,0% zdiagnozowano przewlekłe zapalenie ucha środkowego; spośród tych z przewlekłym zapaleniem, 5,1% dzieci miało perlaka.1

Prewalencja jest wyższa u dorosłych: spośród 1 006 dorosłych z zapaleniem ucha środkowego, 24,4% miało przewlekłe zapalenie; a spośród tych z przewlekłym zapaleniem, 39,2% miało perlaka.1

Trendy epidemiologiczne

W ostatnich dekadach zaobserwowano pewne zmiany w epidemiologii perlaków:12

  • Ogólna częstość występowania perlaka nabytego zmniejsza się, co przypisuje się wcześniejszemu rozpoznaniu, wcześniejszej interwencji chirurgicznej i powszechnemu stosowaniu drenażu wentylacyjnego w leczeniu zapalenia ucha środkowego12
  • Wykrywalność perlaka wrodzonego wzrasta, prawdopodobnie dzięki postępom technologicznym w diagnostyce1
  • Śmiertelność z powodu powikłań wewnątrzczaszkowych perlaka jest obecnie rzadka, co można przypisać wcześniejszemu rozpoznaniu, terminowej interwencji chirurgicznej i wspomagającej antybiotykoterapii1

Nadzór i monitorowanie perlaka

Ze względu na charakter choroby i wysokie ryzyko nawrotów, długoterminowy nadzór nad pacjentami z perlakiem jest niezbędny.12

Znaczenie długoterminowej obserwacji

Perlak wymaga długoterminowego nadzoru w celu sprawdzenia nawrotów, ponieważ:12

  • Nawroty mogą pojawić się nawet 5-8 lat po operacji pierwotnej, a czasem nawet później12
  • Wskaźniki nawrotów po operacji zgłaszano jako wysokie – od 5% do 13%, a w niektórych badaniach nawet do 80% dla pewnych typów operacji12
  • Około 11% pacjentów będzie wymagało wielu operacji rewizyjnych w celu całkowitego wyeliminowania choroby1

Metody nadzoru

Do monitorowania pacjentów z perlakiem stosuje się różne metody:12

  • Coroczne kontrole kliniczne są niezbędne ze względu na możliwość późnych nawrotów1
  • Obrazowanie MRI w pierwszym roku, a następnie potencjalnie w trzecim roku po operacji1
  • Nadzór obrazowy zwykle przeprowadza się co najmniej rok po początkowej operacji i kontynuuje przez co najmniej 5 lat1
  • MRI z techniką dyfuzji (DWI) z uzupełniającymi sekwencjami anatomicznymi odgrywa kluczową rolę w obrazowaniu nadzorczym pooperacyjnego perlaka1

Alternatywą dla rutynowej operacji typu „second-look” jest nadzór za pomocą MRI u pacjentów, którzy przeszli techniki chirurgiczne, które ograniczyłyby kliniczne wykrywanie pozostałości choroby.1

Koszty i efektywność nadzoru

Badania porównujące koszty planowanej operacji „second-look” i nadzoru obrazowego z wykorzystaniem MRI wykazały, że mają one podobne koszty.1 Jednak wybór metody nadzoru powinien uwzględniać:2

  • Agresywny charakter perlaka, zwłaszcza u dzieci
  • Wysokie wskaźniki nawrotów (23-53% w przypadku operacji „second-look” u dzieci)
  • Ryzyko opóźnionego wykrycia nawrotu w przypadku fałszywie negatywnego wyniku MRI

Ostatecznie, decyzja powinna być podejmowana wspólnie przez pacjenta/opiekuna i lekarza, biorąc pod uwagę indywidualne czynniki ryzyka i preferencje.1

Implikacje dla zdrowia publicznego

Perlak pozostaje istotnym problemem zdrowia publicznego na całym świecie, mimo że jego częstość występowania jest stosunkowo niska.12

Obciążenie systemu opieki zdrowotnej

Perlaki są stosunkowo częstym powodem operacji otologicznych (co dwa tygodnie w praktykach otologicznych trzeciego stopnia referencyjności).1 Powodują one znaczne obciążenie systemu opieki zdrowotnej ze względu na:12

  • Konieczność interwencji chirurgicznej w leczeniu
  • Wysokie wskaźniki nawrotów wymagające ponownych operacji
  • Długoterminowy nadzór trwający wiele lat
  • Potencjalne poważne powikłania wymagające dalszego leczenia

Perlak pozostaje stosunkowo częstą przyczyną trwałego, umiarkowanego niedosłuchu przewodzeniowego u dzieci i dorosłych.1 Ponadto może powodować poważne powikłania, takie jak erozja kanału nerwu twarzowego (33,1%), erozja płytki opony twardej (4,8%) i przetoka błędnikowa (10,3%).1

Badania i rejestry

Ze względu na znaczenie kliniczne perlaka, prowadzone są różne inicjatywy badawcze i rejestrowe:12

  • Ponad 330 ekspertów medycznych zajmujących się perlakiem ucha środkowego w 36 krajach
  • Co najmniej 4 badania kliniczne zarejestrowane w ClinicalTrials.gov, w tym 3 zakończone
  • Duński Narodowy Rejestr Szpitalny (DNHR) wykorzystywany do badań epidemiologicznych nad perlakiem z pozytywną wartością predykcyjną 85,8% i stopniem kompletności 91,0%

Dokładne rejestrowanie przypadków perlaka jest istotne dla poprawy naszego zrozumienia tej choroby i opracowania lepszych strategii jej zapobiegania i leczenia.1

Przyszłe kierunki badawcze

Obecne badania nad perlakiem koncentrują się na kilku obiecujących obszarach:12

  • Techniki lepszej lokalizacji perlaka śródoperacyjnie podczas operacji pierwotnych
  • Potencjał selektywnej fotoaktywacji w celu ablacji perlaka
  • Lepsze zrozumienie patologii perlaka, aby pomóc we wdrożeniu standardowych klasyfikacji zmian
  • Doskonalenie protokołów leczenia perlaka i przewlekłego zapalenia ucha środkowego w warunkach ograniczonych zasobów opieki zdrowotnej

Mimo 50 lat badań nad histologią perlaka, nadal nie ma konsensusu co do sposobu jego powstawania, co podkreśla potrzebę dalszych badań epidemiologicznych i klinicznych w tej dziedzinie.1

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 SciELO Brazil – Epidemiology of middle ear and mastoid cholesteatomas: study of 1146 cases Epidemiology of middle ear and mastoid cholesteatomas: study of 1146 cases
    https://www.scielo.br/j/bjorl/a/kBHjH66wHwhrYFGmZZvZzck/?lang=en
    Middle ear cholesteatoma is an important and relatively common disorder which may have serious consequences. […] The etiology of cholesteatomas remains unknown. Epidemiological and statistical data, surgical reports, and conclusions of experimental studies are welcome, as they may provide support for clarifying the pathogenesis of cholesteatoma. […] Except for histological studies of temporal bones, the epidemiology of cholesteatomas has been poorly studied in Brazil. As it is not a disease that has to be notified, the true incidence of middle ear cholesteatoma is unknown in this country. […] Based on a survey of 1,146 cholesteatoma surgery cases we attempted to add knowledge about the epidemiology of this condition in the Brazilian context, given that published papers on this specific topic are rare.
  • #1 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Cholesteatoma has traditionally not been considered a hereditary condition, but recent findings suggest otherwise. A population-based case-control study conducted over 30 years in Sweden reported nearly a 4-fold increased risk in patients with a first-degree relative who had previously undergone surgery for cholesteatoma removal. […] The incidence of cholesteatoma is reported to be 6 to 9 per 100,000 people in developed countries, with a higher incidence among children. Patients with cleft palate are at an increased risk of developing cholesteatoma. Recent studies have demonstrated a statistically significant decrease in the annual incidence rate of cholesteatoma surgery with a slight male-to-female predominance of 1.4:1. […] Socioeconomic disparities among cholesteatoma patients may also contribute to the higher incidence. Potential factors include increased use of pressure-equalization tubes, adenoidectomy, access to otomicroscopy, and more targeted antibiotic treatment for otitis media.
  • #1 Neuroradiology of Cholesteatomas | American Journal of Neuroradiology
    http://www.ajnr.org/content/32/2/221
    Cholesteatoma has been known for more than 300 years in the medical literature; still its precise detection with the use of cross-sectional imaging techniques remains challenging. […] The annual incidence of cholesteatoma is reported as 3 per 100,000 in children and 9.2 per 100,000 in adults with a male predominance of 1.4:1. Middle ear cholesteatomas have a higher incidence in individuals younger than 50 years of age, whereas EAC cholesteatomas present predominantly at 40-70 years of age. Hereditary predisposition is probable. There is a high prevalence among white individuals, and cholesteatoma is rarely detected in the Asian, American Indian, and Alaskan Eskimo populations.
  • #1 Epidemiology and Aetiology of Middle Ear Cholesteatoma
    https://www.periodicos.capes.gov.br/index.php/acervo/buscador.html?task=detalhes&id=W2119895516
    A total of 500 patients with cholesteatoma diagnosed and operated during 1982-91 in the region of Tampere University Hospital and Mikkeli Central Hospital in Finland were analysed retrospectively. The mean annual incidence was 9.2 per 100,000 inhabitants (range 3.7-13.9) and during the study period the annual incidence decreased significantly. The incidence was higher among males under the age of 50 years. […] The majority (72.4%) of cholesteatoma patients had suffered from otitis media episodes. Tympanostomy was carried out in 10.2% and adenoidectomy or adenotonsillectomy in 15.9% of all cholesteatoma ears prior to cholesteatoma surgery. Cholesteatoma behind an intact tympanic membrane with no history of otitis media was verified in 0.6% of patients and in cleft palate patients in 8%. In this study, 13.2% of patients had ear trauma or ear operation in anamnes.
  • #1 Epidemiological study on cholesteatoma in Japan
    https://www.jstage.jst.go.jp/article/otoljpn1991/8/3/8_3_165/_article/-char/en
    In order to determine the incidence of cholesteatoma in Japan, an epidemiological study was conducted in Miyagi Prefecture which is located in northern part of Japan, of approximately 2, 300, 000 population. […] One hundred and ninety cases (199 ears) underwent surgery for cholesteatoma at hospitals in Miyagi Prefecture. […] Including these cases the annual incidence of cholesteatoma was calculated as 4.2 cases per 100, 000 persons per year.
  • #1 Rate of chronic otitis media operations and cholesteatoma surgeries in South Korea: a nationwide population-based study (2006–2018) | Scientific Reports
    https://www.nature.com/articles/s41598-020-67799-5
    The aim of this study was to estimate the total number and rate of chronic otitis media (COM) operations and cholesteatoma surgeries in South Korea, using a nationwide survey which analysed a 13-year trend (20062018). […] The total annual number of cholesteatoma surgeries decreased from 3,502 in 2006 to 3,199 in 2018 (6 in 100,000). […] According to the 2018 data, cholesteatoma surgery was most commonly performed in patients in their 50s. […] Cholesteatoma surgery rates increased dramatically from 2006 to 2018 in patients aged 010 years due to congenital cholesteatoma. […] The mean rate of cholesteatoma surgery was 0.006%, and decreased annually. […] The total number of cholesteatoma surgeries was highest in 2006 (3,502 individuals, 0.00715%), lowest in 2018 (3,199 individuals, 0.00617%), and decreased gradually until 2018.
  • #1 Acquired cholesteatoma | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/acquired-cholesteatoma?lang=us
    Acquired cholesteatomas make up 98% of all middle ear cholesteatomas 9. […] Cholesteatomas occur far more commonly in the middle ear than in the external auditory canal. This article relates to middle ear acquired cholesteatomas.
  • #1 Cholesteatoma | 5-Minute Clinical Consult
    https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/1688269/all/Cholesteatoma
    Cholesteatomas are found in all age groups. Congenital cholesteatomas are usually found only in pediatric patients, whereas acquired cholesteatomas can be found in any individual with middle ear disease (1,2,3). […] The average age for diagnosis of cholesteatoma is 9.7 years for acquired and 4.5 years for congenital (1). There exists a slight male preponderance for the disease. However, female patients are more likely to have bilateral disease and exhibit recurrence of disease in subsequent follow-up appointments (1). Overall, the incidence of acquired cholesteatoma is decreasing, secondary to the widely accepted and implemented early use of pressure equalization (PE) tubes for otitis media and other middle ear disease (1). […] The prevalence of acquired cholesteatoma is approximately 15 cases per 100,000 children and 12 cases per 100,000 adults. By comparison, congenital cholesteatomas are rare only affecting approximately 0.12 per 100,000 children. Congenital cholesteatoma is becoming more commonly reported because recognition and screening of the disease has become more sensitive (1).
  • #1 Epidemiology of Congenital Cholesteatoma: Surveys of the Last 17 Years in Japan
    https://www.mdpi.com/2077-0383/13/5/1276
    The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. […] The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence. […] The incidence of CC has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. […] The number of surgeries performed was higher in the second half of the study period. […] The mean incidence rate per 100,000 newborn births was 28.09 and 24.80 in the Oita and Miyazaki prefectures, respectively. Consequently, the total disease incidence rates in both prefectures during the study period were calculated to be 26.44. […] There was a significant positive correlation between the year of birth and the total disease incidence. Spearman’s rank correlation coefficient was 0.53 (p = 0.03). Therefore, its incidence increased from 2001 to 2017.
  • #1 Cholesteatoma – Wikipedia
    https://en.wikipedia.org/wiki/Cholesteatoma
    In one study, the number of new cases of cholesteatoma in Iowa was estimated in 1975-76 to be just under one new case per 10,000 citizens per year. Cholesteatoma affects all age groups, from infants through to the elderly. The peak incidence occurs in the second decade.
  • #1 Pathology Outlines – Cholesteatoma
    https://www.pathologyoutlines.com/topic/earcholesteatoma.html
    Annual incidence ranges between 3 – 14 per 100,000 children and 9.2 – 13 per 100,000 adults, with slight male predilection (M:F = 1.4:1) (Acta Otolaryngol 1999;119:568, Eur Arch Otorhinolaryngol 2004;261:6) […] Incidence and detection of congenital cholesteatoma is on the rise, possibly due to technological advances (Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:143) […] Due to treatment advances in otic pathology, acquired cholesteatoma is decreasing due to adequate treatment of otitis media (Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:143) […] Conditions involving cleft palate or ear anomalies show increased tendency for cholesteatoma formation (Auris Nasus Larynx 2014;41:101, Int J Pediatr Otorhinolaryngol 2013;77:1403) […] Association with allergic rhinitis (Laryngoscope 2018;128:547)
  • #1
    https://step1.medbullets.com/neurology/113065/cholesteatoma
    Epidemiology […] incidence […] unknown […] […] […] demographics […] acquired cases occur in children and adults […] congenital cases begin in childhood […] […] […] location […] soft tissue and bony structures of the temporal bone […] […] […] risk factors […] family history […] long-standing Eustachian tube dysfunction […] cleft palate and other craniofacial anomalies
  • #1 Characteristics of 419 patients with acquired middle ear cholesteatoma | Brazilian Journal of Otorhinolaryngology
    https://www.elsevier.es/en-revista-brazilian-journal-otorhinolaryngology-english-edition–497-articulo-characteristics-419-patients-with-acquired-S1808869416300556
    Cholesteatoma is a destructive lesion that can result in life-threatening complications. Typically, it presents with hypoacusis and continuous otorrhea as symptoms. Because it is a rare disease, there are few studies in Brazil describing the characteristics of patients with the disease. […] This study aimed to determine the prevalence of cholesteatoma in patients with chronic otitis media and describe clinical, audiological and surgical characteristics of patients with acquired middle ear cholesteatoma treated at a referral hospital in the public health system. […] Of the patients with chronic otitis media, 419 (24.5%) had cholesteatoma; mean age of 34.49 years; 53.5% female and 63.8% adults. […] The prevalence of cholesteatoma in patients with chronic otitis media was 24.5% and it was more common in adults than in children.
  • #1 Frontiers | Microbiota Associated With Cholesteatoma Tissue in Chronic Suppurative Otitis Media
    https://www.frontiersin.org/journals/cellular-and-infection-microbiology/articles/10.3389/fcimb.2022.746428/full
    Otitis media (OM), defined as infection or inflammation of the middle ear (ME), remains a major public health problem worldwide. Cholesteatoma is a non-cancerous, cyst-like lesion in the ME that may be acquired due to chronic OM and cause disabling complications. […] Cholesteatoma was estimated to develop in 10-24% of OM cases. […] In a tertiary hospital setting in Colorado, out of 67,661 children seen over 10 years, 36.5% had OM and 12.0% were diagnosed with COM; of those with COM, 5.1% of children presented with cholesteatoma. […] Prevalence is higher in adults: of 1,006 adults with OM, 24.4% had COM; of those with COM, 39.2% had cholesteatoma. […] These data show that while COM and cholesteatoma are prevalent in lower income countries such as the Philippines, these disease entities are also significant health issues in high-income countries including the United States.
  • #1 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Cholesteatomas are a relatively common reason for otologic surgery (biweekly in tertiary otologic practices). […] Death from intracranial complications of cholesteatoma is now uncommon, with this change being attributable to earlier recognition, timely surgical intervention, and supportive antibiotic therapy. Cholesteatomas remain a relatively frequent cause of permanent, moderate conductive hearing loss in children and adults. […] The exact worldwide incidence of cholesteatomas is unknown, although the incidence has reportedly declined in recent decades; this can be attributed to earlier recognition, timely surgical intervention, and the widespread use of ventilation tubes. The majority of cholesteatoma cases are acquired, with boys making up the preponderance of these.
  • #1
    https://med.uth.edu/orl/2020/01/10/cholesteatoma-2/
    Cholesteatoma requires long-term surveillance to check for recurrence. […] The primary goal of cholesteatoma surgery is to remove the skin, clear the infection, and create a dry, safe ear.
  • #1 Cholesteatoma – Ear, Nose, and Throat Doctors in Munster, IN
    https://www.arthurhkatzmd.com/cholesteatoma/
    Cholesteatoma requires long-term surveillance to check for recurrence. […] Without proper treatment cholesteatoma will cause recurrent ear infections. Chronic infection of the ear can lead to progressive hearing loss and even deafness.
  • #1 Evaluation and Management of Cholesteatoma | BackTable ENT Podcast
    https://www.backtable.com/shows/ent/podcasts/120/evaluation-management-of-cholesteatoma
    Dr. Mankekar underscores the importance of tailoring the physical exam to each patients history and needs. […] All patients with cholesteatoma require long-term surveillance, as recurrence can happen more than five years after initial treatment. […] Dr. Mankekar summarizes her strategy for patient-centered, long-term management of cholesteatoma. […] long-term surveillance is absolutely essential. Sometimes, we can see a recurrence as late as five to even eight years down the line, even longer, and so, we do need to see these patients in the long term.
  • #1 Clinical and Surgical Features of Acquired Middle Ear Cholesteatoma: A 10 Years Population-Based Study
    https://brieflands.com/articles/semj-133899
    Cholesteatoma was seen in about a quarter of patients with chronic otitis media. […] Based on our findings, cholesteatoma can be associated with serious complications such as facial nerve canal erosion (33.1%), dural plate erosion (4.8%), and labyrinthine fistula (10.3%). […] Regarding the functional importance of the hearing system and the high prevalence of disease complications, middle ear cholesteatoma needs long-term follow-up. […] In this survey, we reported our experience in epidemiology, the signs and symptoms, the various effect of the disease on middle ear structures and mastoid, and the type of surgery in patients with middle ear cholesteatoma over 10 years. […] The progressive nature of cholesteatoma, its fatal consequences, and the lack of long-term studies in Iran underline the need for a population-based investigation of this disease. […] The recurrence rate of this disease is estimated at 5 to 13%. Although this recurrence usually occurs in the first 5 years, some studies suggest that recurrence is possible even 14 years after primary surgery.
  • #1 A fluorescent photoimmunoconjugate for imaging of cholesteatoma | Scientific Reports
    https://www.nature.com/articles/s41598-022-22072-9
    Cholesteatoma is a potentially serious complication of chronic ear infections and requires surgical intervention for definitive management. […] Cholesteatoma is a common complication of chronic ear infections and typically requires repeat surgical intervention to eradicate entirely. […] Rates of cholesteatoma recurrence after surgery have been reported as high as 30%, and 11% of patients will require multiple revision surgeries to eradicate the disease. […] Techniques to better localize cholesteatoma intraoperatively during primary surgeries have the potential to eliminate need for revision surgeries, and to increase surgeon confidence to perform primary ossicular chain reconstruction in some cases. […] A promising candidate marker for intraoperative localization of cholesteatoma is EGFR, since skin cysts are known to overexpress this marker compared to adjacent tissues of the head and neck.
  • #1 The Great Debate: MRI Versus Second-Look Surgery for Cholesteatoma – ENTtoday
    https://www.enttoday.org/article/the-great-debate-mri-versus-second-look-surgery-for-cholesteatoma/
    There are different imaging options for cholesteatoma surveillance. Non-contrast CT scan of the temporal bones is often used to delineate disease and bony involvement, as well as to look for the status of ossicles, tegmen, inner ear, and facial nerve canal. […] MRI scans of the temporal bones are increasingly used in cholesteatoma surveillance as the techniques advance, with increasing accuracy and the ability to avoid radiation exposure.
  • #1 Cholesteatoma Surgery: Expert Strategies for Optimal Outcomes
    https://www.backtable.com/shows/ent/articles/cholesteatoma-surgery-expert-strategies-optimal-outcomes
    Annual clinical checks are vital due to the potential for late recurrences in cholesteatoma patients. […] MRI surveillance at the first year and then possibly at the three-year mark is beneficial. […] Dr. Gauri Mankekar further explains why long-term clinical surveillance is a necessary component of cholesteatoma management. While MRIs play a crucial role in the early postoperative years, she strongly advocates for annual clinical checks as recurrences can arise even eight years post-surgery. […] In cholesteatoma, long-term surveillance is absolutely essential. Sometimes, we can see a recurrence as late as five to even eight years down the line, even longer, and so, we do need to see these patients in the long term.
  • #1
    https://link.springer.com/article/10.1007/s00330-024-11021-x
    MRI surveillance represents an alternative to routine second-look surgery in patients who have undergone CWUM or obliterative or reconstructive techniques that would limit clinical detection of residual disease. […] Surveillance imaging is typically carried out at least 1 year after initial surgery and continued for at least 5 years.
  • #1
    https://link.springer.com/article/10.1007/s00330-024-11021-x
    Although non-malignant, middle ear cholesteatoma can result in significant complications due to local bone erosion and infection. […] Imaging plays a key role in the evaluation of middle ear cholesteatoma, supporting the clinical diagnosis, demonstrating complications, planning surgery, and detecting residual disease at follow-up. […] Non-EPI (or multi-shot EPI) DWI with supplementary anatomical sequences plays a key role in imaging surveillance of postoperative cholesteatoma and this should be continued for at least 5 years (moderate evidence). […] Cholesteatomas most commonly occur within the middle ear and affect approximately 69 per 100,000 individuals in developed countries. […] Imaging plays a significant role in the appropriate management of patients with middle ear cholesteatomas.
  • #1 The Great Debate: MRI Versus Second-Look Surgery for Cholesteatoma – Page 3 of 3 – ENTtoday
    https://www.enttoday.org/article/the-great-debate-mri-versus-second-look-surgery-for-cholesteatoma/3/?singlepage=1
    Imaging is also presumed to be a more cost-effective alternative to second-look surgeries; however, in a study that compared these costs, planned second-look surgery and imaging surveillance with non-EP DW MRI had similar costs. […] Second-look surgeries are the gold standard in the management of cholesteatoma and are used to evaluate for recurrence/recidivism and perform ossicular chain reconstruction if needed. When the surgery is staged, the second look is ideally performed at four to 12 months, depending on the extent and location of the disease and clinical suspicion. It is well accepted that childhood cholesteatoma is more aggressive, and rates of cholesteatoma detected in second-look surgeries range from 23% to 53%. […] Knowing the aggressive nature of cholesteatoma and high rates of recidivism in children, however, an 18-month delay places the child at significant risk of disease that may surpass the extent of the original disease if there is a false-negative MRI scan. […] Ultimately, this lends itself to shared decision making between the patient/caregiver and the physician.
  • #1 Top Published Expert Doctors for Middle Ear Cholesteatoma
    https://findexpertmd.com/d/Middle_Ear_Cholesteatoma?physician=physician
    330 top medical experts on Middle Ear Cholesteatoma across 36 countries and 27 U.S. states, including 215 MDs (Physicians). This is based on an objective analysis of their Scientific Publications, Clinical Trials, Medicare, and NIH Grants. […] Clinical Trials ClinicalTrials.gov: at least 4 including 3 Completed.
  • #1
    https://ugeskriftet.dk/dmj/validation-cholesteatoma-diagnosis-danish-national-hospital-register
    The degree of completeness of the cholesteatoma diagnosis in combination with a relevant surgical code was estimated applying the capture-recapture method. […] The PPV of the cholesteatoma diagnosis in the DNHR combined with relevant surgery was 85.8% and the degree of completeness was 91.0%. […] We thus conclude that the first occurrence of the cholesteatoma diagnosis combined with relevant surgery in the DNHR may be used as an estimated surrogate for the diagnosis of cholesteatoma. […] We found the PPV and the degree of completeness of the DNHR to be within an acceptable range. Hence, we conclude that the DNHR can be used as a valuable tool for epidemiological research on cholesteatoma.
  • #1 A fluorescent photoimmunoconjugate for imaging of cholesteatoma | Scientific Reports
    https://www.nature.com/articles/s41598-022-22072-9
    This study is the first to demonstrate the potential for intraoperative imaging of cholesteatoma, and the first application in the head and neck region overall, using a novel application of an established imaging agent from the field of oncology. […] The next steps in investigation should seek to evaluate the potential for selective photoactivation for cholesteatoma ablation, above and beyond the ability for selective imaging demonstrated in this study.
  • #1 Frontiers | Microbiota Associated With Cholesteatoma Tissue in Chronic Suppurative Otitis Media
    https://www.frontiersin.org/journals/cellular-and-infection-microbiology/articles/10.3389/fcimb.2022.746428/full
    Cholesteatoma has a propensity for insidious growth and erosion of the ossicles or temporal bone that houses neural structures and may lead to disabling complications such as hearing loss, facial nerve palsy, vertigo, or intracranial extension. […] Surgery is required for treatment, with high rates of recurrence even after >10 years. […] Fifty years after its histology was resolved, there is still no consensus on how a cholesteatoma forms, except that the process is probably a hybrid of theories. […] Better understanding of cholesteatoma pathology will aid in implementation of standard classifications of lesions to guide management and predict surgical outcomes. […] Our findings will be useful for fine-tuning treatment protocols for cholesteatoma and chronic OM in settings with limited health care resources.
  • #2 SciELO Brazil – Epidemiology of middle ear and mastoid cholesteatomas: study of 1146 cases Epidemiology of middle ear and mastoid cholesteatomas: study of 1146 cases
    https://www.scielo.br/j/bjorl/a/kBHjH66wHwhrYFGmZZvZzck/
    Middle ear cholesteatoma is an important and relatively common disorder which may have serious consequences. […] The etiology of cholesteatomas remains unknown. Epidemiological and statistical data, surgical reports, and conclusions of experimental studies are welcome, as they may provide support for clarifying the pathogenesis of cholesteatoma. Our results were compared with internationally published papers. We found no published papers on the epidemiology of cholesteatoma in the Brazilian literature. […] Except for histological studies of temporal bones, the epidemiology of cholesteatomas has been poorly studied in Brazil. As it is not a disease that has to be notified, the true incidence of middle ear cholesteatoma is unknown in this country. […] Based on a survey of 1,146 cholesteatoma surgery cases we attempted to add knowledge about the epidemiology of this condition in the Brazilian context, given that published papers on this specific topic are rare.
  • #2 Pathology Outlines – Cholesteatoma
    https://www.pathologyoutlines.com/topic/earcholesteatoma.html
    Annual incidence ranges between 3 – 14 per 100,000 children and 9.2 – 13 per 100,000 adults, with slight male predilection (M:F = 1.4:1) (Acta Otolaryngol 1999;119:568, Eur Arch Otorhinolaryngol 2004;261:6) […] Incidence and detection of congenital cholesteatoma is on the rise, possibly due to technological advances (Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:143) […] Due to treatment advances in otic pathology, acquired cholesteatoma is decreasing due to adequate treatment of otitis media (Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:143) […] Conditions involving cleft palate or ear anomalies show increased tendency for cholesteatoma formation (Auris Nasus Larynx 2014;41:101, Int J Pediatr Otorhinolaryngol 2013;77:1403) […] Association with allergic rhinitis (Laryngoscope 2018;128:547)
  • #2 Rate of chronic otitis media operations and cholesteatoma surgeries in South Korea: a nationwide population-based study (2006–2018) | Scientific Reports
    https://www.nature.com/articles/s41598-020-67799-5
    Recently, however, cholesteatoma surgery rates were higher in the male population (1.2 times higher than in females in 2018). […] The rate of cholesteatoma surgery was highest in 2006 (3,502 individuals, 0.00715%), lowest in 2015 (3,154 individuals, 0.00612%), and generally decreased from 2006 to 2018 (0.007150.00617%). […] The total number of cholesteatoma surgeries in South Korea determined using a nationwide survey to analyse a 13-year trend (20062018) according to age groups. […] From 2006 to 2018, the prominent age groups were the 50s and 60s (1st, 3rd rank in 2018, respectively) due to acquired cholesteatoma. […] In conclusion, the annual rate of cholesteatoma surgery was 0.006%, and decreased annually.
  • #2 Epidemiology of Congenital Cholesteatoma: Surveys of the Last 17 Years in Japan
    https://www.mdpi.com/2077-0383/13/5/1276/xml
    The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. […] The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence. […] The incidence of CC has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. […] The number of surgeries performed was higher in the second half of the study period. […] The mean incidence rate per 100,000 newborn births was 28.09 and 24.80 in the Oita and Miyazaki prefectures, respectively. Consequently, the total disease incidence rates in both prefectures during the study period were calculated to be 26.44. […] There was a significant positive correlation between the year of birth and the total disease incidence.
  • #2
    https://step1.medbullets.com/neurology/113065/cholesteatoma
    Epidemiology […] incidence […] unknown […] […] […] demographics […] acquired cases occur in children and adults […] congenital cases begin in childhood […] […] […] location […] soft tissue and bony structures of the temporal bone […] […] […] risk factors […] family history […] long-standing Eustachian tube dysfunction […] cleft palate and other craniofacial anomalies
  • #2 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Cholesteatoma has traditionally not been considered a hereditary condition, but recent findings suggest otherwise. A population-based case-control study conducted over 30 years in Sweden reported nearly a 4-fold increased risk in patients with a first-degree relative who had previously undergone surgery for cholesteatoma removal. […] The incidence of cholesteatoma is reported to be 6 to 9 per 100,000 people in developed countries, with a higher incidence among children. Patients with cleft palate are at an increased risk of developing cholesteatoma. Recent studies have demonstrated a statistically significant decrease in the annual incidence rate of cholesteatoma surgery with a slight male-to-female predominance of 1.4:1. […] Socioeconomic disparities among cholesteatoma patients may also contribute to the higher incidence. Potential factors include increased use of pressure-equalization tubes, adenoidectomy, access to otomicroscopy, and more targeted antibiotic treatment for otitis media.
  • #2
    https://step2.medbullets.com/ear-nose-throat/121789/cholesteatoma
    Epidemiology […] Incidence […] unknown […] […] […] Demographics […] acquired cases occur in children and adults […] congenital cases begin in childhood […] […] […] Risk factors […] family history […] long-standing Eustachian tube dysfunction […] cleft palate and other craniofacial anomalies […] […] […] Prognosis […] There may be recurrence of cholesteatoma post-surgery
  • #2 Characteristics of 419 patients with acquired middle ear cholesteatoma | Brazilian Journal of Otorhinolaryngology
    https://www.elsevier.es/en-revista-brazilian-journal-otorhinolaryngology-english-edition–497-articulo-characteristics-419-patients-with-acquired-S1808869416300556
    The estimated incidence in the general population is 3.7-13.9/100,000. This incidence is lower in children (3/100,000) than in adults (9/100,000). […] The prevalence of bilateral cholesteatoma in this study was 17.1%; similar to previous studies. […] The main complaints of patients at the time of the first evaluation in this service are shown in Fig. 2. Hypoacusis, with or without otorrhea, was the main complaint of 84.4% of the study population, and otorrhea was observed in 87%. […] The prevalence of the different routes of cholesteatoma formation in this population is shown in Fig. 1. […] In the present study, a frequency of 3.8% of patients with palatal defects was observed, with no difference between children and adults. […] The present results are contrary to the international trend, currently more favorable to conservative surgeries, using closed tympanomastoidectomy or channel reconstruction technique. […] The prevalence of cholesteatoma in patients with COM was 24.5%; it was more frequently found in adults than in children. Bilateral disease was found in 17.1% of patients with cholesteatoma.
  • #2 Cholesteatoma
    https://www.midwestent.com/cholesteatoma
    Cholesteatoma requires long-term surveillance to check for recurrence. […] Cholesteatoma can be managed in a variety of ways, but definitive removal of the skin or cyst typically requires surgical intervention. […] The primary goal of cholesteatoma surgery is to remove the skin, clear the infection, and create a dry, safe ear.
  • #2 Cholesteatoma Surgery: Expert Strategies for Optimal Outcomes
    https://www.backtable.com/shows/ent/articles/cholesteatoma-surgery-expert-strategies-optimal-outcomes
    Annual clinical checks are vital due to the potential for late recurrences in cholesteatoma patients. […] MRI surveillance at the first year and then possibly at the three-year mark is beneficial. […] Dr. Gauri Mankekar further explains why long-term clinical surveillance is a necessary component of cholesteatoma management. While MRIs play a crucial role in the early postoperative years, she strongly advocates for annual clinical checks as recurrences can arise even eight years post-surgery. […] In cholesteatoma, long-term surveillance is absolutely essential. Sometimes, we can see a recurrence as late as five to even eight years down the line, even longer, and so, we do need to see these patients in the long term.
  • #2 The Great Debate: MRI Versus Second-Look Surgery for Cholesteatoma – ENTtoday
    https://www.enttoday.org/article/the-great-debate-mri-versus-second-look-surgery-for-cholesteatoma/
    Unfortunately, it is a well-recognized aphorism that to have a busy surgical otology practice, one needs only 10 children with cholesteatoma as patients. This highlights the high rates of recidivism (a term used to describe both recurrent and residual cholesteatoma) of this condition after surgical treatment. A meta-analysis by Yasser Shewel, MD, and colleagues in 2020 showed recidivism rates ranged from 5.26% to 80% (average of 27.6%) in individuals with canal wall up (CWU) mastoidectomies and from 0% to 48% (average of 17.4%) when the canal wall was down (CWD) […] Even with endoscopes, the reported rates of recidivism are still high, ranging from 18%-24% […] With this in mind, the matter up for discussion here is HOW do we monitor patients ears most effectively via imaging with computed tomography/magnetic resonance imaging (CT/MRI) or via second-look surgery? This remains a Great Debate.
  • #2
    https://link.springer.com/article/10.1007/s00330-024-11021-x
    Although non-malignant, middle ear cholesteatoma can result in significant complications due to local bone erosion and infection. […] Imaging plays a key role in the evaluation of middle ear cholesteatoma, supporting the clinical diagnosis, demonstrating complications, planning surgery, and detecting residual disease at follow-up. […] Non-EPI (or multi-shot EPI) DWI with supplementary anatomical sequences plays a key role in imaging surveillance of postoperative cholesteatoma and this should be continued for at least 5 years (moderate evidence). […] Cholesteatomas most commonly occur within the middle ear and affect approximately 69 per 100,000 individuals in developed countries. […] Imaging plays a significant role in the appropriate management of patients with middle ear cholesteatomas.
  • #2 The Great Debate: MRI Versus Second-Look Surgery for Cholesteatoma – Page 3 of 3 – ENTtoday
    https://www.enttoday.org/article/the-great-debate-mri-versus-second-look-surgery-for-cholesteatoma/3/?singlepage=1
    Imaging is also presumed to be a more cost-effective alternative to second-look surgeries; however, in a study that compared these costs, planned second-look surgery and imaging surveillance with non-EP DW MRI had similar costs. […] Second-look surgeries are the gold standard in the management of cholesteatoma and are used to evaluate for recurrence/recidivism and perform ossicular chain reconstruction if needed. When the surgery is staged, the second look is ideally performed at four to 12 months, depending on the extent and location of the disease and clinical suspicion. It is well accepted that childhood cholesteatoma is more aggressive, and rates of cholesteatoma detected in second-look surgeries range from 23% to 53%. […] Knowing the aggressive nature of cholesteatoma and high rates of recidivism in children, however, an 18-month delay places the child at significant risk of disease that may surpass the extent of the original disease if there is a false-negative MRI scan. […] Ultimately, this lends itself to shared decision making between the patient/caregiver and the physician.
  • #2 Frontiers | Microbiota Associated With Cholesteatoma Tissue in Chronic Suppurative Otitis Media
    https://www.frontiersin.org/journals/cellular-and-infection-microbiology/articles/10.3389/fcimb.2022.746428/full
    Cholesteatoma has a propensity for insidious growth and erosion of the ossicles or temporal bone that houses neural structures and may lead to disabling complications such as hearing loss, facial nerve palsy, vertigo, or intracranial extension. […] Surgery is required for treatment, with high rates of recurrence even after >10 years. […] Fifty years after its histology was resolved, there is still no consensus on how a cholesteatoma forms, except that the process is probably a hybrid of theories. […] Better understanding of cholesteatoma pathology will aid in implementation of standard classifications of lesions to guide management and predict surgical outcomes. […] Our findings will be useful for fine-tuning treatment protocols for cholesteatoma and chronic OM in settings with limited health care resources.
  • #2
    https://ugeskriftet.dk/dmj/validation-cholesteatoma-diagnosis-danish-national-hospital-register
    Cholesteatoma can cause serious complications such as hearing loss, facial paresis, dizziness and intracranial infections. […] Previous studies, which were primarily based on local hospital districts, have demonstrated annual incidence rates of cholesteatoma ranging from three to 17 per 100,000 person-years. […] The purpose of this study was to validate the cholesteatoma diagnosis in the Danish National Hospital Register (DNHR) and to estimate the degree of completeness, hereby answering the question whether the DNHR may be used for epidemiological research on cholesteatoma. […] The positive predictive value (PPV) of the cholesteatoma diagnosis in the DNHR in combination with a relevant surgical code was calculated as a proportion by dividing the number of registered cases with a confirmed diagnosis after surgical chart review by the total number of retrievable surgical charts.