Cholesteatoma
Etiologia i przyczyny

Perlak (cholesteatoma) to nie nowotworowy rozrost nabłonka płaskiego w uchu środkowym, najczęściej nabyty, powstający w wyniku dysfunkcji trąbki Eustachiusza, przewlekłych infekcji lub urazów błony bębenkowej. Wyróżnia się trzy typy: wrodzony (2-4% przypadków), pierwotny nabyty (związany z retrakcją błony bębenkowej) oraz wtórny nabyty (po perforacji błony bębenkowej). Patogeneza obejmuje mechanizmy takie jak retrakcja błony, migracja nabłonka, metaplazja oraz implantacja, prowadząc do powstania cysty wypełnionej martwym nabłonkiem i woskiem. Perlak powoduje destrukcję kości ucha środkowego przez stały nacisk i aktywność osteoklastów, co skutkuje uszkodzeniem kosteczek słuchowych, ucha wewnętrznego, nerwu twarzowego oraz kości pokrywającej mózg, z ryzykiem poważnych powikłań neurologicznych. Epidemiologicznie perlak nabyty występuje częściej u mężczyzn (1,4x) i może mieć podłoże genetyczne. Czynniki ryzyka to m.in. nawracające zapalenia ucha środkowego, perforacja błony bębenkowej, dysfunkcja trąbki Eustachiusza, wcześniejsze zabiegi chirurgiczne oraz wady czaszkowo-twarzowe.

Etiopatogeneza perlaka (cholesteatoma)

Perlak (cholesteatoma) to nietypowy rozrost skóry w uchu środkowym, za błoną bębenkową. Jest to guz niepochodzenia nowotworowego, który przybiera formę cysty lub woreczka wypełnionego martwymi komórkami skóry i woskiem usznym, co nadaje mu perłowobiały, łojowaty wygląd. Mimo swojej nazwy perlak nie zawiera cholesterolu, a nazwa pochodzi od jego charakterystycznego perłowego wyglądu12.

Perlak może być obecny od urodzenia (wrodzony), jednak zdecydowanie częściej rozwija się jako powikłanie długotrwałych i nawracających stanów zapalnych ucha środkowego (nabyty). Niezależnie od etiologii, perlak rośnie stopniowo, wywierając destrukcyjny wpływ na struktury ucha środkowego i sąsiadujące tkanki, co może prowadzić do utraty słuchu, a w skrajnych przypadkach do poważnych powikłań neurologicznych34.

Klasyfikacja perlaka

Wyróżnia się trzy główne typy perlaka56:

  • Perlak wrodzony – występuje rzadko, obecny od urodzenia, powstaje w wyniku uwięzienia tkanki nabłonka płaskiego w uchu środkowym podczas rozwoju embrionalnego
  • Perlak pierwotny nabyty – najczęstsza postać, powstaje w wyniku retrakcji błony bębenkowej
  • Perlak wtórny nabyty – rozwija się jako bezpośredni skutek uszkodzenia błony bębenkowej przez infekcje, urazy lub interwencje chirurgiczne

Przyczyny powstawania perlaka

Patogeneza perlaka jest złożona i prawdopodobnie wynika z wzajemnego oddziaływania wielu mechanizmów, które mogą występować jednocześnie. Czynniki takie jak stan zapalny, ciśnienie miejscowe, specyficzne cytokeratyny oraz ziarnina produkująca różne enzymy i mediatory przyczyniają się do tworzenia perlaka i następującej po nim resorpcji kości7.

Perlak wrodzony

Perlak wrodzony powstaje w wyniku nieprawidłowego rozwoju embrionalnego, gdy komórki nabłonka płaskiego zostają uwięzione w uchu środkowym podczas formowania się struktur ucha8. Najczęściej teoria dotycząca etiopatogenezy perlaka wrodzonego wskazuje na obecność pozostałości komórek nabłonka w strukturach ucha środkowego9. Ten typ perlaka występuje niezwykle rzadko i stanowi tylko około 2-4% wszystkich przypadków10.

Dzieci z rozszczepem podniebienia mają znacznie wyższe ryzyko rozwoju perlaka niż inne dzieci. Badania pokazują, że tylko 24% perlaków u dzieci w wieku od 4 do 6 lat jest obecnych od urodzenia1112.

Perlak nabyty pierwotny

Perlak nabyty pierwotny rozwija się najczęściej w wyniku dysfunkcji trąbki Eustachiusza, która łączy ucho środkowe z nosogardłem. W prawidłowych warunkach trąbka Eustachiusza zapewnia równowagę ciśnień między uchem środkowym a otoczeniem. Jej zaburzenia prowadzą do powstania podciśnienia w jamie bębenkowej, co powoduje wciągnięcie błony bębenkowej1314.

Teoria kieszeni retrakcyjnej (lub inwaginacji) przypisuje powstawanie perlaka dysfunkcji trąbki Eustachiusza i nieprawidłowemu napowietrzaniu przestrzeni nadbębenkowej. Kieszeń retrakcyjna tworzy się w wyniku podciśnienia w uchu środkowym spowodowanego dysfunkcją trąbki Eustachiusza i utrzymującym się stanem zapalnym15.

Do czynników przyczyniających się do dysfunkcji trąbki Eustachiusza należą1617:

  • Alergie sezonowe
  • Infekcje górnych dróg oddechowych (przeziębienia)
  • Zapalenie zatok przynosowych
  • Przewlekłe zapalenie błony śluzowej nosa

Przewlekłe podciśnienie powoduje wciągnięcie błony bębenkowej, co prowadzi do utworzenia kieszeni lub woreczka, w którym gromadzą się martwe komórki skóry, łuszczący się nabłonek i woskowina. Z czasem te nagromadzone komórki tworzą perlaka1819.

Perlak nabyty wtórny

Perlak nabyty wtórny rozwija się zazwyczaj w wyniku ostrego zapalenia ucha środkowego, ale może powstać również w następstwie urazu, np. operacji chirurgicznej, obrażeń od fali uderzeniowej czy ciał obcych20.

Perforacja błony bębenkowej umożliwia migrację komórek nabłonka płaskiego z przewodu słuchowego zewnętrznego do jamy bębenkowej. Komórki te nie są usuwane przez naturalny mechanizm oczyszczania ucha środkowego, co prowadzi do ich nagromadzenia i powstania perlaka2122.

Uszkodzenie błony bębenkowej może nastąpić wskutek2324:

  • Przewlekłych lub nawracających infekcji ucha środkowego
  • Urazów mechanicznych błony bębenkowej
  • Zabiegów chirurgicznych w obrębie ucha

Inne teorie powstawania perlaka

Poza głównymi mechanizmami powstawania perlaka, istnieją również inne teorie wyjaśniające jego etiologię252627:

  • Teoria migracji nabłonka (teoria imigracji) – zakłada, że perlak powstaje w wyniku defektu błony bębenkowej, który umożliwia migrację keratynizującego nabłonka płaskiego do ucha środkowego
  • Teoria metaplazji płaskonabłonkowej – sugeruje, że perlak powstaje przed perforacją błony bębenkowej. Zgodnie z tą teorią, transformacja metaplastyczna błony śluzowej ucha środkowego, wywołana przewlekłym drażnieniem, prowadzi do powstania perlaka w wyniku hiperproliferacji
  • Teoria hiperplazji komórek podstawnych (teoria wrastania brodawkowatego) – zakłada, że przestrzeń Prussaka (zachyłek przyśrodkowy względem szyi młoteczka i boczny względem części wiotkiej) jest nachodzona przez wypełnione keratyną mikrotorbiele, pączki lub pseudopodium pochodzące z warstwy podstawnej nabłonka
  • Teoria implantacji – perlaki mogą wynikać z implantacji nabłonka płaskiego do ucha środkowego, potencjalnie z powodu urazu lub zabiegu chirurgicznego

Czynniki ryzyka rozwoju perlaka

Istnieje kilka czynników zwiększających ryzyko wystąpienia perlaka282930:

  • Nawracające infekcje ucha środkowego (ostre zapalenie ucha środkowego)
  • Perforacja błony bębenkowej
  • Dysfunkcja trąbki Eustachiusza
  • Wcześniejsze zabiegi chirurgiczne ucha
  • Historia zakładania drenów wentylacyjnych w dzieciństwie
  • Wady czaszkowo-twarzowe (rozszczep podniebienia)
  • Zespół Turnera
  • Niedobory odporności prowadzące do nawracającego zapalenia ucha środkowego

Badania epidemiologiczne wskazują, że perlak nabyty występuje częściej u mężczyzn niż u kobiet (1,4 razy częściej). Ze względu na to, że perlaki czasami występują rodzinnie, eksperci podejrzewają możliwe genetyczne podłoże tej choroby31.

Mechanizmy destrukcyjnego działania perlaka

Perlak powoduje niszczenie kości na drodze dwóch głównych mechanizmów32:

  • Stały nacisk wywierany przez rosnącego perlaka, prowadzący do przebudowy kości
  • Aktywność osteoklastyczna wzmocniona przez procesy enzymatyczne zachodzące na obrzeżach zakażonego perlaka

Badania z wykorzystaniem skaningowego mikroskopu elektronowego wykazały, że perlaki o nieregularnej strukturze macierzy mają tendencję do powodowania większych zniszczeń ścian kostnych ucha środkowego niż te o regularnej, warstwowej strukturze33.

Martwe komórki skóry gromadzące się w uchu tworzą idealne środowisko dla rozwoju bakterii i grzybów. Oznacza to, że cysta może ulec zakażeniu, powodując stan zapalny i ciągły wyciek z ucha34.

Z czasem perlak może zniszczyć otaczającą kość i uszkodzić struktury ucha środkowego, w tym3536:

Nawrotowy perlak

Nawrotowy perlak może wystąpić nawet u pacjentów operowanych przez najbardziej doświadczonych chirurgów. Jest to spowodowane agresywnym charakterem choroby. Nawroty perlaka występują w dwóch formach3738:

  • Perlak resztkowy (residual cholesteatoma) – powstaje, gdy mały fragment wyściółki perlaka zostaje pozostawiony podczas operacji i tworzy nową kulę skóry za błoną bębenkową
  • Prawdziwy nawrót perlaka (recurrent cholesteatoma) – powstaje, gdy nowy perlak tworzy się w uchu środkowym lub jamie sutkowej

Wskaźniki nawrotów sięgają do 30% u dorosłych i do 70% u dzieci39. Czynniki sprzyjające nawrotom perlaka to40:

  • Obecność nawracających infekcji ucha
  • Niepełne chirurgiczne usunięcie perlaka

Zapobieganie perlakowi

Nie ma możliwości zapobiegania wrodzonym przypadkom perlaka. Jednakże rodzice i opiekunowie powinni być świadomi tej choroby, aby dzieci mogły szybko otrzymać diagnozę i leczenie41.

W przypadku perlaka nabytego najlepszą profilaktyką jest właściwe leczenie infekcji ucha. Szybkie i dokładne leczenie przewlekłych infekcji ucha może pomóc zapobiec powstawaniu perlaka42. Mimo to perlaki mogą się nadal rozwijać43.

Podsumowanie

Perlak to niebezpieczne schorzenie ucha, które może prowadzić do poważnych powikłań, jeśli nie zostanie odpowiednio leczone. Powstaje głównie w wyniku dysfunkcji trąbki Eustachiusza, przewlekłych infekcji ucha lub urazów błony bębenkowej. Rzadko może być również wadą wrodzoną4445.

Patogeneza perlaka jest złożona i prawdopodobnie wynika z interakcji wielu mechanizmów, w tym retrakcji błony bębenkowej, migracji nabłonka czy metaplazji nabłonka. Niezależnie od mechanizmu powstawania, perlak jest chorobą postępującą, która z czasem może uszkodzić kość, tkanki miękkie i nerwy46.

Jedyną skuteczną metodą leczenia perlaka jest chirurgiczne usunięcie zmiany. Bez leczenia perlak będzie nadal rósł i powodował coraz większe zniszczenia w strukturach ucha, prowadząc do trwałej utraty słuchu, a w skrajnych przypadkach do powikłań zagrażających życiu47.

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

Materiały źródłowe

  • #1 Cholesteatoma: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/21535-cholesteatoma
    Cholesteatoma is an abnormal growth in your middle ear behind your eardrum. It can be congenital (present from birth), but typically happens because you have chronic ear infections. […] A cholesteatoma is a growth behind your eardrum (tympanic membrane). It develops when dead skin cells gather behind your eardrum to form a lump or cyst that may look like a pearl. […] One of the main causes is a retracted eardrum. This is when there’s an imbalance between the air inside and outside of your ear. This causes your middle ear to pull in your eardrum. When that happens, you may develop a cyst or pocket that fills up with skin cells and becomes a cholesteatoma. Chronic ear infections (acute otitis media) or ruptured eardrums can also lead to cholesteatomas. […] Treatments depend on the cause and symptoms, but surgeries like mastoidectomy and tympanoplasty are the only ways to remove a cholesteatoma.
  • #2 Cholesteatoma: Symptoms, Causes, and Treatment
    https://patient.info/ears-nose-throat-mouth/hearing-problems/cholesteatoma
    Cholesteatoma is the name given to a collection of skin cells that form a pearly-white greasy-looking lump deep in the ear, usually at the top of the area behind the eardrum. […] A cholesteatoma is a non-cancerous abnormal growth of skin-like tissue in the middle ear. Cholesteatomas are rare. It can be present at birth (congenital) but usually occurs as a complication of long-standing (chronic) changes to the pressure in the ear. […] Skin cells from the lining of the ear canal seem to get trapped in the middle ear. The middle ear would not normally contain these skin cells. […] If the dead cells become trapped and form a collection, this build-up of dead skin cells over time can form a cholesteatoma. […] No one quite knows why this happens but it is usually related to the eardrum being drawn inwards, deeper than it is meant to be (retracted).
  • #3 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Cholesteatomas cause bony erosion by either of the following mechanisms: Consistent pressure applied over time, resulting in bony remodeling […] Osteoclastic activity enhanced by enzymatic processes occurring at the margin of an infected cholesteatoma. […] Using a scanning electron microscope, a study by Wiatr et al found that cholesteatomas with an irregular matrix structure tend to cause more destruction to the middle ear bone walls than do those with a regular, layered structure. […] A study by Rosito et al suggested that in patients with posterior epitympanic cholesteatoma confined to the pars flaccida or a two-route cholesteatoma involving both the pars flaccida and pars tensa, the chance of having a labyrinthine fistula in the lateral semicircular canal is increased. […] Generally, three types of cholesteatoma are identified: congenital, primary acquired, and secondary acquired.
  • #4 Cholesteatoma | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/cholesteatoma.html
    A cholesteatoma is a growth in your ear made of trapped skin cells. This noncancerous (benign) ear cyst can invade your middle ear and nearby structures. Without treatment, cholesteatomas can cause swelling, infection, and damage to your ear and hearing. […] A cholesteatoma develops in your eardrum or middle ear, and can lead to damage of the hearing and middle ear bones from inflammation. It can grow into your mastoid (the bone behind your ear) if left untreated. The ear cyst can harm other nearby structures, including your inner ear, face, neck, and (in rare cases) brain. Untreated cholesteatomas are at high risk for infection, and can even lead to meningitis or facial paralysis. […] We dont fully understand why a cholesteatoma develops. Sometimes, the cyst is present from birth (congenital). Other times, ear injuries such as eardrum rupture (tympanic membrane perforation) can lead to cholesteatomas.
  • #5 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Cholesteatomas cause bony erosion by either of the following mechanisms: Consistent pressure applied over time, resulting in bony remodeling […] Osteoclastic activity enhanced by enzymatic processes occurring at the margin of an infected cholesteatoma. […] Using a scanning electron microscope, a study by Wiatr et al found that cholesteatomas with an irregular matrix structure tend to cause more destruction to the middle ear bone walls than do those with a regular, layered structure. […] A study by Rosito et al suggested that in patients with posterior epitympanic cholesteatoma confined to the pars flaccida or a two-route cholesteatoma involving both the pars flaccida and pars tensa, the chance of having a labyrinthine fistula in the lateral semicircular canal is increased. […] Generally, three types of cholesteatoma are identified: congenital, primary acquired, and secondary acquired.
  • #6 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Congenital cholesteatomas arise as a consequence of squamous epithelium trapped within the temporal bone during embryogenesis. […] A primary acquired cholesteatoma results from tympanic membrane retraction. […] Secondary acquired cholesteatomas result directly from injury to the tympanic membrane, frequently in the form of perforations caused by acute otitis media, trauma, or surgical manipulation.
  • #7 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Most cholesteatomas are unilateral, occurring either extradural in the middle ear or mastoid, or intradural at the cerebellopontine angle. Proposed etiologic mechanisms include the inclusion, migration, or invasion of squamous epithelium, epithelial nests from faulty embryogenesis, or metaplasia of normal epithelium. […] The pathogenesis of acquired cholesteatoma is likely the result of a complex interplay of mechanisms that may occur simultaneously. Factors such as inflammation, local pressure, specific cytokeratins, and granulation tissue which produces various enzymes and mediators are believed to contribute to cholesteatoma formation and subsequent bone resorption. […] The retraction-pocket (or invagination) theory attributes cholesteatoma formation to eustachian tube dysfunction and poor aeration of the epitympanic space. The retraction pocket forms due to negative pressure in the middle ear caused by eustachian tube dysfunction and persistent inflammation.
  • #8 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Congenital cholesteatomas arise as a consequence of squamous epithelium trapped within the temporal bone during embryogenesis. […] A primary acquired cholesteatoma results from tympanic membrane retraction. […] Secondary acquired cholesteatomas result directly from injury to the tympanic membrane, frequently in the form of perforations caused by acute otitis media, trauma, or surgical manipulation.
  • #9 Cholesteatoma: Causes, Treatment, and Complications | Ento Key
    https://entokey.com/cholesteatoma-causes-treatment-and-complications/
    Cholesteatoma causes significant morbidity and rare mortality in the modern era. […] Significant controversy exists regarding the etiology of this relatively rare entity (2 to 7% of all cholesteatomas). […] The most widely accepted theory currently is that of retained epithelial rests. […] Other theories have also been proposed. […] Similar to congenital cholesteatoma, controversy surrounds the exact pathophysiology of acquired cholesteatoma. […] The most common cause of primary acquired cholesteatoma is a retraction pocket in the tympanic membrane. […] It is postulated that lack of ventilation of the epitympanum and mastoid results in abnormal negative pressures and atelectasis of the relatively weaker pars flaccida and retraction into Prussak space. […] The exact mechanism by which the conversion between tympanic membrane retraction and cholesteatoma takes place is not clear. […] It is postulated that bacterial overgrowth and biofilm production induce an inflammatory reaction within the basal layer of the epithelium.
  • #10 Acquired cholesteatoma | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/acquired-cholesteatoma?lang=us
    Acquired cholesteatomas are far more common than congenital cholesteatomas and are almost always closely related to the tympanic membrane and pneumatized portion of the temporal bone from which most are thought to arise 9. […] Acquired cholesteatomas make up 98% of all middle ear cholesteatomas 9. […] The vast majority of acquired cholesteatomas develop due to chronic otitis media and are usually associated with perforation of the tympanic membrane. […] There are four hypotheses that relate to the formation of cholesteatomas; all may be true 1,6: […] probably the most common cause […] results from Eustachian tube dysfunction and tympanic membrane retraction, with debris and keratin eventually obstructing the neck of the retraction. […] in the setting of a previous perforation […] keratinized cells 'invade’ the middle ear through the perforation. […] invasive hyperplasia of the basal cell layer of the tympanic membrane as a result of infection. […] as a result of chronic irritation from middle ear infection.
  • #11 Cholesteatoma: Causes, symptoms, and treatment
    https://www.medicalnewstoday.com/articles/cholesteatoma
    Studies show that only 24% of cholesteatomas in children between the ages of 4 and 6 years are present at birth. Children with a cleft palate have a much higher risk of developing a cholesteatoma than other children. […] It is not possible to prevent congenital cases of cholesteatoma. However, it is worth parents and caregivers being aware of the condition, so that children can quickly receive diagnosis and treatment. […] For acquired cholesteatoma, properly treating ear infections is the best prevention. However, cholesteatomas may still develop. […] Without treatment, a severe cholesteatoma can be fatal.
  • #12 Cholesteatoma | What Causes This Growth in the Ear? | Buoy Health
    https://www.buoyhealth.com/learn/cholesteatoma
    Cholesteatomas are rare, but are one possible reason for hearing loss. You can be born with cholesteatomas. […] A cholesteatoma is a cyst-like collection of dead skin cells that can damage some of the middle ear bone and soft tissue. It generally starts in the middle ear, behind the eardrum. It may become larger as it fills with old skin cells. Occasionally, babies are born with it (congenital). […] The most common reason a cholesteatoma forms is from problems with your eustachian tubes. These are the passageways that connect your ears to your nose and throat and equalize pressure. When the eustachian tube doesn’t function properly, it causes negative pressure and pulls the eardrum backwards towards the inner ear. This leads to a mass of skin cells that can form the cholesteatoma. […] Eustachian tube dysfunction and sinusitis play significant roles in the development of cholesteatoma. The eustachian tube, which connects the middle ear to the back of the nose, is crucial for equalizing pressure in the ear. When this tube malfunctions, often due to conditions like sinusitis, it can create negative pressure in the middle ear. This negative pressure can cause the eardrum to retract, potentially leading to cholesteatoma formation.
  • #13 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Most cholesteatomas are unilateral, occurring either extradural in the middle ear or mastoid, or intradural at the cerebellopontine angle. Proposed etiologic mechanisms include the inclusion, migration, or invasion of squamous epithelium, epithelial nests from faulty embryogenesis, or metaplasia of normal epithelium. […] The pathogenesis of acquired cholesteatoma is likely the result of a complex interplay of mechanisms that may occur simultaneously. Factors such as inflammation, local pressure, specific cytokeratins, and granulation tissue which produces various enzymes and mediators are believed to contribute to cholesteatoma formation and subsequent bone resorption. […] The retraction-pocket (or invagination) theory attributes cholesteatoma formation to eustachian tube dysfunction and poor aeration of the epitympanic space. The retraction pocket forms due to negative pressure in the middle ear caused by eustachian tube dysfunction and persistent inflammation.
  • #14 Cholesteatoma – ENT Health
    https://www.enthealth.org/conditions/cholesteatoma/
    Cholesteatoma is an abnormal skin growth or skin cyst trapped behind the eardrum, or the bone behind the ear. […] Cholesteatomas begin as a build-up of ear wax and skin, which causes either a lump on the eardrum or an eardrum retraction pocket. Over time, the skin collects and eventually causes problems like infection, drainage, and hearing loss. […] The most common cause is poor ventilation of the middle ear space, which is called eustachian tube dysfunction. The eustachian tube is the natural tube that connects your middle ear space to your nose and sinuses, and helps regulate the pressure behind your eardrum. If the eustachian tube is not working properly, the middle ear space does not get ventilated. This creates negative pressure and ultimately causes the weakened eardrum to retract. This retraction collects skin and earwax, which leads to a cholesteatoma. Seasonal allergies, upper respiratory infections (cough/cold), or sinusitis may contribute to eustachian tube dysfunction. […] A cholesteatoma can develop when skin of the ear canal passes through a hole in the eardrum and into the middle ear space. Finally, another rare type of cholesteatoma is present at birth (congenital) and is related to how the ear develops.
  • #15 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Most cholesteatomas are unilateral, occurring either extradural in the middle ear or mastoid, or intradural at the cerebellopontine angle. Proposed etiologic mechanisms include the inclusion, migration, or invasion of squamous epithelium, epithelial nests from faulty embryogenesis, or metaplasia of normal epithelium. […] The pathogenesis of acquired cholesteatoma is likely the result of a complex interplay of mechanisms that may occur simultaneously. Factors such as inflammation, local pressure, specific cytokeratins, and granulation tissue which produces various enzymes and mediators are believed to contribute to cholesteatoma formation and subsequent bone resorption. […] The retraction-pocket (or invagination) theory attributes cholesteatoma formation to eustachian tube dysfunction and poor aeration of the epitympanic space. The retraction pocket forms due to negative pressure in the middle ear caused by eustachian tube dysfunction and persistent inflammation.
  • #16 Cholesteatoma – ENT Health
    https://www.enthealth.org/conditions/cholesteatoma/
    Cholesteatoma is an abnormal skin growth or skin cyst trapped behind the eardrum, or the bone behind the ear. […] Cholesteatomas begin as a build-up of ear wax and skin, which causes either a lump on the eardrum or an eardrum retraction pocket. Over time, the skin collects and eventually causes problems like infection, drainage, and hearing loss. […] The most common cause is poor ventilation of the middle ear space, which is called eustachian tube dysfunction. The eustachian tube is the natural tube that connects your middle ear space to your nose and sinuses, and helps regulate the pressure behind your eardrum. If the eustachian tube is not working properly, the middle ear space does not get ventilated. This creates negative pressure and ultimately causes the weakened eardrum to retract. This retraction collects skin and earwax, which leads to a cholesteatoma. Seasonal allergies, upper respiratory infections (cough/cold), or sinusitis may contribute to eustachian tube dysfunction. […] A cholesteatoma can develop when skin of the ear canal passes through a hole in the eardrum and into the middle ear space. Finally, another rare type of cholesteatoma is present at birth (congenital) and is related to how the ear develops.
  • #17 Cholesteatoma: Symptoms, Causes & Treatment | Baptist Health
    https://www.baptisthealth.com/care-services/conditions-treatments/cholesteatoma
    Cholesteatoma is caused by a combination of genetic factors and long-term ear infections. […] Cholesteatomas can develop because of a birth defect or chronic ear infections. […] The main cholesteatoma causes include: Congenital cholesteatoma – This type of cholesteatoma is present at birth and is caused by a developmental abnormality. […] Eustachian tube dysfunction – The Eustachian tube plays a crucial role in maintaining balance within the middle ear by regulating pressure. Dysfunction of this tube can cause negative pressure, leading to the formation of cholesteatoma. This is also known as primary acquired cholesteatoma. […] Chronic ear infections – Repeated ear infections and ruptured eardrums can lead to the formation of cholesteatoma, as the eardrum gets pulled inward, creating a pocket where skin cells can accumulate. This is sometimes called secondary cholesteatoma.
  • #18 What causes cholesteatoma?
    https://www.topdoctors.co.uk/medical-articles/what-causes-cholesteatoma
    A cholesteatoma is a cyst filled with dead skin cells that develops behind the eardrum. In order to understand how cholesteatoma develops, it is necessary to understand a little bit about the anatomy and physiology of the ear. The middle ear is ventilated by a tube connecting it with the back of the nose. This is the Eustachian tube. Normally the Eustachian tube opens when you swallow but in some people, especially children, it does not open very well. If it doesn’t open very well, over time there is a tendency for the atmospheric pressure in the middle ear to drop because the lining of the middle ear tends to absorb gases from the air and the gases are not replaced adequately because of the poor Eustachian tube function. This negative pressure causes the eardrum to be sucked inwards. This is termed retraction. The outer surface of the eardrum is lined with skin. Skin, as a natural process, tends to shed dead skin cells from its surface. Normally, in the ear, these dead skin cells migrate out of the ear canal with wax produced in the ear canal. If the eardrum becomes retracted, there is a lip around the edge of the retraction that the skin cannot migrate over. This means that the dead skin cells accumulate in the retracted part of the eardrum and, as the skin cells are constantly shed, they eventually build up into a cyst. This is the cholesteatoma. Over time, the cholesteatoma gradually expands into the middle ear space and wraps itself around and damages the tiny hearing bones that are found in the middle ear. These hearing bones (called ossicles) carry sound to the inner ear and when they become damaged this carriage of sound is no longer possible. It is therefore not uncommon for patients with a cholesteatoma to have a conductive hearing loss. Cholesteatomas are able to eat away at bone, hence the damage to the hearing bones. The walls of the middle ear are bony and cholesteatomas can erode these walls and damage the structures that are adjacent to the middle ear. This means that the ear discharges a smelly yellow or green liquid. Sometimes the ear can also be painful.
  • #19 Cholesteatoma: Causes, Symptoms, and Diagnosis
    https://www.healthline.com/health/cholesteatoma
    A cholesteatoma is a noncancerous skin growth in the middle section of your ear, behind the eardrum. It can be a birth defect or result from repeated middle ear infections. […] Besides repeated infections, a cholesteatoma may also be caused by a poorly functioning eustachian tube, which is the tube that leads from the back of the nose to the middle of the ear. […] In very rare cases, a baby may be born with a cholesteatoma. This is considered a birth defect. Congenital cholesteatomas can form in the middle ear or in other areas of the ear. […] In cases where children acquire ear infections repeatedly early in life, it is possible that cholesteatomas can develop from a young age. […] If your eustachian tube isnt working correctly, a partial vacuum might occur in your middle ear. This may cause a section of your eardrum to be pulled into the middle ear, creating a cyst that can turn into a cholesteatoma.
  • #20 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Nonretraction pocket cholesteatomas, also known as secondary-acquired cholesteatomas, typically result from acute otitis media but can also arise from trauma, such as surgery, blast injuries, or foreign bodies. […] Epithelial migration theory (immigration theory): This theory contradicts the retraction pocket theory. The theory posits that cholesteatomas form due to a defect in the tympanic membrane, allowing keratinizing squamous epithelium to migrate into the middle ear. […] Squamous metaplasia theory: This theory proposes that cholesteatomas originate before tympanic membrane perforation. The theory suggests that metaplastic transformation of middle ear mucosa, driven by chronic irritation, leads to cholesteatoma formation due to hyperproliferation. […] Basal cell hyperplasia (papillary ingrowth) theory: This theory proposes that the Prussak space (a recess medial to the neck of the malleus and lateral to the pars flaccida) is invaded by keratin-filled microcysts, buds, or pseudopods originating from the basal layer of the epithelium. […] Regardless of the etiology, once middle ear cholesteatomas have formed, they continue to proliferate and migrate, causing damage to surrounding structures in the middle ear. Cholesteatoma is a progressive condition that can eventually impact bone, soft tissue, and nerves.
  • #21 Cholesteatoma | UCI Health | Orange County, CA
    https://www.ucihealth.org/medical-services/ear-nose-throat-ent/hearing-ear-disorders/cholesteatoma
    When this skin grows behind the ear drum or it grows into the bone behind the ear (mastoid bone), it is called a cholesteatoma. […] Eardrum perforation. When the eardrum becomes perforated, skin can grow through the hole into the space behind the ear drum. […] Congential defect. Sometimes skin behind the ear drum is present at birth. […] Ear drum retraction. The ear drum can pull back when ear disease is present for a long time. As the ear drum retracts, skin grows into the space. […] As the cholesteatoma grows, it destroys the bone around it. […] The structures that can be invaded by the cholesteatoma include the little bones of the ear (ossicles), which causes hearing loss, inner ear (which would cause complete deafness and dizziness), the facial nerve (which would cause facial paralysis), or invade the bone covering the brain and cause meningitis. […] If left untreated, these complications will occur with time.
  • #22 Acquired cholesteatoma | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/acquired-cholesteatoma?lang=us
    Acquired cholesteatomas are far more common than congenital cholesteatomas and are almost always closely related to the tympanic membrane and pneumatized portion of the temporal bone from which most are thought to arise 9. […] Acquired cholesteatomas make up 98% of all middle ear cholesteatomas 9. […] The vast majority of acquired cholesteatomas develop due to chronic otitis media and are usually associated with perforation of the tympanic membrane. […] There are four hypotheses that relate to the formation of cholesteatomas; all may be true 1,6: […] probably the most common cause […] results from Eustachian tube dysfunction and tympanic membrane retraction, with debris and keratin eventually obstructing the neck of the retraction. […] in the setting of a previous perforation […] keratinized cells 'invade’ the middle ear through the perforation. […] invasive hyperplasia of the basal cell layer of the tympanic membrane as a result of infection. […] as a result of chronic irritation from middle ear infection.
  • #23 Cholesteatoma
    https://www.nhs.uk/conditions/cholesteatoma/
    A cholesteatoma can develop if your eardrum is damaged through injury, infection or after any kind of ear surgery. […] You can also be born with a cholesteatoma, but this is rare.
  • #24 Cholesteatoma: Causes, Symptoms, and Diagnosis
    https://www.healthline.com/health/cholesteatoma
    A cholesteatoma is a noncancerous skin growth in the middle section of your ear, behind the eardrum. It can be a birth defect or result from repeated middle ear infections. […] Besides repeated infections, a cholesteatoma may also be caused by a poorly functioning eustachian tube, which is the tube that leads from the back of the nose to the middle of the ear. […] In very rare cases, a baby may be born with a cholesteatoma. This is considered a birth defect. Congenital cholesteatomas can form in the middle ear or in other areas of the ear. […] In cases where children acquire ear infections repeatedly early in life, it is possible that cholesteatomas can develop from a young age. […] If your eustachian tube isnt working correctly, a partial vacuum might occur in your middle ear. This may cause a section of your eardrum to be pulled into the middle ear, creating a cyst that can turn into a cholesteatoma.
  • #25 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Nonretraction pocket cholesteatomas, also known as secondary-acquired cholesteatomas, typically result from acute otitis media but can also arise from trauma, such as surgery, blast injuries, or foreign bodies. […] Epithelial migration theory (immigration theory): This theory contradicts the retraction pocket theory. The theory posits that cholesteatomas form due to a defect in the tympanic membrane, allowing keratinizing squamous epithelium to migrate into the middle ear. […] Squamous metaplasia theory: This theory proposes that cholesteatomas originate before tympanic membrane perforation. The theory suggests that metaplastic transformation of middle ear mucosa, driven by chronic irritation, leads to cholesteatoma formation due to hyperproliferation. […] Basal cell hyperplasia (papillary ingrowth) theory: This theory proposes that the Prussak space (a recess medial to the neck of the malleus and lateral to the pars flaccida) is invaded by keratin-filled microcysts, buds, or pseudopods originating from the basal layer of the epithelium. […] Regardless of the etiology, once middle ear cholesteatomas have formed, they continue to proliferate and migrate, causing damage to surrounding structures in the middle ear. Cholesteatoma is a progressive condition that can eventually impact bone, soft tissue, and nerves.
  • #26 Cholesteatoma – Wittmaack, Ruedi, Habermann, Sade, Theories
    https://www.entlecture.com/cholesteatoma/
    Cholesteatomas most commonly originates from a retraction pocket in the pars flaccida or the posterosuperior part of the pars tensa. The initiating factor is likely the dysfunction of the Eustachian tube, resulting in negative middle ear pressure. […] The genesis of cholesteatoma is a subject of debate. Any theory must account for the appearance of squamous epithelium in the middle ear cleft. […] Various theories include: Presence of congenital epidermal cell rests: This forms congenital cholesteatoma. […] Invagination of tympanic membrane from the attic or posterosuperior part of pars tensa in the form of retraction pockets (Wittmaacks retraction pocket theory): The outer surface of the tympanic membrane, lined by stratified squamous epithelium, invaginates to form the cholesteatoma matrix, depositing keratin in the pocket.
  • #27 Cholesteatoma – Wittmaack, Ruedi, Habermann, Sade, Theories
    https://www.entlecture.com/cholesteatoma/
    Basal cell hyperplasia (Ruedis theory): Pseudopods are formed in the basal layer of the squamous epithelium of the pars flaccida epithelium which invade the sub-epithelial tissue, fuse together, resulting in the formation of cholesteatoma. […] Epithelial invasion (Habermanns migration theory): The epithelium from the bottom of the meatus or the outer epithelial surface of the tympanic membrane grows and migrates into the middle ear through a pre-existing perforation, especially of the marginal type where part of annulus tympanicus has already been destroyed. […] Squamous metaplasia (Sades theory): Middle ear mucosa undergoes metaplasia due to repeated infections, transforming from cuboidal to squamous epithelium. […] Implantation theory: Cholesteatomas may result from the implantation of squamous epithelium into the middle ear, potentially due to trauma or by surgery. This mechanism, however, likely accounts for only a small proportion of cholesteatomas.
  • #28 Cholesteatoma: Causes, symptoms, and treatment
    https://www.medicalnewstoday.com/articles/cholesteatoma
    Cholesteatomas are not common, with acquired forms (those not present at birth) having an annual incidence of 912.6 out of 100,000 adults and 315 out of 100,000 children. […] A study of adults found that acquired cholesteatoma was 1.4 times more common in men than in women. As cholesteatomas sometimes run in families, experts suspect a possible inherited genetic link. […] The majority of cholesteatoma cases are acquired. These growths can form because of the following: repeated ear infections, skull or facial bone birth abnormalities, an injury to the eardrum, negative pressure in the Eustachian tube (the tube connecting the middle ear to the back of the nose and throat), which makes the eardrum pull inward and allows keratin and dead skin cells to accumulate in the middle ear, dead skin cells and earwax moving into the middle ear through a hole in the eardrum.
  • #29 Cholesteatoma Causes, Symptoms & Clinical Presentation
    https://www.backtable.com/shows/ent/articles/cholesteatoma-causes-symptoms-clinical-presentation
    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. […] Risk factors for acquired cholesteatomas include eustachian tube dysfunction, cleft craniofacial malformations, turner syndrome and immune deficiencies leading to recurrent otitis media. […] 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. […] With congenital cholesteatoma, it’s quite often bad luck.
  • #30 Cholesteatoma | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/cholesteatoma.html
    Eustachian tube dysfunction may be a risk factor cholesteatomas by creating negative pressure in the middle ear space, but this is not always the case. […] Risk factors are things that increase your chances of having a cholesteatoma. Men have a slightly higher risk for acquired cholesteatoma than women. Other risk factors include: A history of needing an ear ventilation tube as a child, Eardrum rupture (tympanic membrane perforation), Eustachian tube dysfunction, Multiple ear infections (otitis media), especially during childhood.
  • #31 Cholesteatoma: Causes, symptoms, and treatment
    https://www.medicalnewstoday.com/articles/cholesteatoma
    Cholesteatomas are not common, with acquired forms (those not present at birth) having an annual incidence of 912.6 out of 100,000 adults and 315 out of 100,000 children. […] A study of adults found that acquired cholesteatoma was 1.4 times more common in men than in women. As cholesteatomas sometimes run in families, experts suspect a possible inherited genetic link. […] The majority of cholesteatoma cases are acquired. These growths can form because of the following: repeated ear infections, skull or facial bone birth abnormalities, an injury to the eardrum, negative pressure in the Eustachian tube (the tube connecting the middle ear to the back of the nose and throat), which makes the eardrum pull inward and allows keratin and dead skin cells to accumulate in the middle ear, dead skin cells and earwax moving into the middle ear through a hole in the eardrum.
  • #32 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Cholesteatomas cause bony erosion by either of the following mechanisms: Consistent pressure applied over time, resulting in bony remodeling […] Osteoclastic activity enhanced by enzymatic processes occurring at the margin of an infected cholesteatoma. […] Using a scanning electron microscope, a study by Wiatr et al found that cholesteatomas with an irregular matrix structure tend to cause more destruction to the middle ear bone walls than do those with a regular, layered structure. […] A study by Rosito et al suggested that in patients with posterior epitympanic cholesteatoma confined to the pars flaccida or a two-route cholesteatoma involving both the pars flaccida and pars tensa, the chance of having a labyrinthine fistula in the lateral semicircular canal is increased. […] Generally, three types of cholesteatoma are identified: congenital, primary acquired, and secondary acquired.
  • #33 Cholesteatoma: Practice Essentials, Background, Etiology and Pathophysiology
    https://emedicine.medscape.com/article/860080-overview
    Cholesteatomas cause bony erosion by either of the following mechanisms: Consistent pressure applied over time, resulting in bony remodeling […] Osteoclastic activity enhanced by enzymatic processes occurring at the margin of an infected cholesteatoma. […] Using a scanning electron microscope, a study by Wiatr et al found that cholesteatomas with an irregular matrix structure tend to cause more destruction to the middle ear bone walls than do those with a regular, layered structure. […] A study by Rosito et al suggested that in patients with posterior epitympanic cholesteatoma confined to the pars flaccida or a two-route cholesteatoma involving both the pars flaccida and pars tensa, the chance of having a labyrinthine fistula in the lateral semicircular canal is increased. […] Generally, three types of cholesteatoma are identified: congenital, primary acquired, and secondary acquired.
  • #34 Cholesteatoma: Causes, Symptoms, and Diagnosis
    https://www.healthline.com/health/cholesteatoma
    The dead skin cells that accumulate in the ear provide an ideal environment for bacteria and fungus to thrive. This means the cyst can become infected, causing inflammation and continual ear drainage. […] Over time, a cholesteatoma may also destroy the surrounding bone. It can damage the eardrum, the bones inside the ear, the bones near the brain, and the nerves of the face. […] Permanent hearing loss may occur if the bones within the ear are broken. […] Congenital cholesteatomas cannot be prevented, but parents should be aware of the condition so it can be quickly identified and treated when present. […] You can prevent cholesteatomas later in life by treating ear infections quickly and thoroughly. However, cysts may still occur.
  • #35 Cholesteatoma: Causes, Symptoms, and Diagnosis
    https://www.healthline.com/health/cholesteatoma
    The dead skin cells that accumulate in the ear provide an ideal environment for bacteria and fungus to thrive. This means the cyst can become infected, causing inflammation and continual ear drainage. […] Over time, a cholesteatoma may also destroy the surrounding bone. It can damage the eardrum, the bones inside the ear, the bones near the brain, and the nerves of the face. […] Permanent hearing loss may occur if the bones within the ear are broken. […] Congenital cholesteatomas cannot be prevented, but parents should be aware of the condition so it can be quickly identified and treated when present. […] You can prevent cholesteatomas later in life by treating ear infections quickly and thoroughly. However, cysts may still occur.
  • #36 Cholesteatoma Diagnosis & Treatment NYC | Mount Sinai – New York
    https://www.mountsinai.org/locations/ear-institute/conditions/cholesteatoma
    Cholesteatoma is a skin-lined cyst that begins at the margin of the eardrum and invades the middle ear and mastoid. […] Cholesteatoma grows aggressively. Because it retains bacteria, it is a commonly becomes infected. This infection may not go away until the cholesteatoma is removed. […] Cholesteatoma has the capacity to eat away bone. Bone erosion can lead to hearing loss by destroying the small hearing bones (malleus, incus, stapes) that carry sound in the middle ear. […] Bone erosion can also lead to more serious complications by allowing the spread of infection outside the middle ear. […] Recurrent cholesteatoma can occur even in the most experienced surgeon’s hands. This is because cholesteatoma is an aggressive disease. Recurrence comes in two forms: the first is when a tiny fragment of cholesteatoma lining is left behind („residual cholesteatoma”), that re-forms a new ball of skin behind the eardrum. The second is when a new cholesteatoma forms in the middle ear or mastoid cavity (a true „recurrence”).
  • #37 Cholesteatoma Diagnosis & Treatment NYC | Mount Sinai – New York
    https://www.mountsinai.org/locations/ear-institute/conditions/cholesteatoma
    Cholesteatoma is a skin-lined cyst that begins at the margin of the eardrum and invades the middle ear and mastoid. […] Cholesteatoma grows aggressively. Because it retains bacteria, it is a commonly becomes infected. This infection may not go away until the cholesteatoma is removed. […] Cholesteatoma has the capacity to eat away bone. Bone erosion can lead to hearing loss by destroying the small hearing bones (malleus, incus, stapes) that carry sound in the middle ear. […] Bone erosion can also lead to more serious complications by allowing the spread of infection outside the middle ear. […] Recurrent cholesteatoma can occur even in the most experienced surgeon’s hands. This is because cholesteatoma is an aggressive disease. Recurrence comes in two forms: the first is when a tiny fragment of cholesteatoma lining is left behind („residual cholesteatoma”), that re-forms a new ball of skin behind the eardrum. The second is when a new cholesteatoma forms in the middle ear or mastoid cavity (a true „recurrence”).
  • #38 Recurrent Cholesteatoma: Why It Occurs?
    https://www.tinnitusjournal.com/articles/recurrent-cholesteatoma-why-it-occurs-27916.html
    A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. […] Recurrence rates of up to 30% in adults and up to 70% in children have been reported. […] It is also very important to understand that cholesteatoma recidivism which includes both recurrent and residual Cholesteatoma. […] While residual cholesteatoma results from incomplete surgical removal of the cholesteatoma matrix, recurrent cholesteatoma results from the reformation of the retraction pocket after a complete previous surgical cholesteatoma removal and recidivism refers to the combination of recurrent and residual disease. […] Factors for development of recurrence of cholesteatoma in this case could be presence of recurrent infection of the ear.
  • #39 Recurrent Cholesteatoma: Why It Occurs?
    https://www.tinnitusjournal.com/articles/recurrent-cholesteatoma-why-it-occurs-27916.html
    A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. […] Recurrence rates of up to 30% in adults and up to 70% in children have been reported. […] It is also very important to understand that cholesteatoma recidivism which includes both recurrent and residual Cholesteatoma. […] While residual cholesteatoma results from incomplete surgical removal of the cholesteatoma matrix, recurrent cholesteatoma results from the reformation of the retraction pocket after a complete previous surgical cholesteatoma removal and recidivism refers to the combination of recurrent and residual disease. […] Factors for development of recurrence of cholesteatoma in this case could be presence of recurrent infection of the ear.
  • #40 Recurrent Cholesteatoma: Why It Occurs?
    https://www.tinnitusjournal.com/articles/recurrent-cholesteatoma-why-it-occurs-27916.html
    A cholesteatoma is an expansion of keratinizing squamous epithelium that enters the middle ear cleft from the outer layer of the tympanic membrane or ear canal. […] Recurrence rates of up to 30% in adults and up to 70% in children have been reported. […] It is also very important to understand that cholesteatoma recidivism which includes both recurrent and residual Cholesteatoma. […] While residual cholesteatoma results from incomplete surgical removal of the cholesteatoma matrix, recurrent cholesteatoma results from the reformation of the retraction pocket after a complete previous surgical cholesteatoma removal and recidivism refers to the combination of recurrent and residual disease. […] Factors for development of recurrence of cholesteatoma in this case could be presence of recurrent infection of the ear.
  • #41 Cholesteatoma: Causes, symptoms, and treatment
    https://www.medicalnewstoday.com/articles/cholesteatoma
    Studies show that only 24% of cholesteatomas in children between the ages of 4 and 6 years are present at birth. Children with a cleft palate have a much higher risk of developing a cholesteatoma than other children. […] It is not possible to prevent congenital cases of cholesteatoma. However, it is worth parents and caregivers being aware of the condition, so that children can quickly receive diagnosis and treatment. […] For acquired cholesteatoma, properly treating ear infections is the best prevention. However, cholesteatomas may still develop. […] Without treatment, a severe cholesteatoma can be fatal.
  • #42 Cholesteatoma: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001050.htm
    Cholesteatoma can be a birth defect (congenital). It more commonly occurs as a result of chronic ear infection. […] The eustachian tube helps equalize pressure in the middle ear. When it is not working well, negative pressure can build up and pull part of the eardrum (tympanic membrane) inward. This creates a pocket or cyst that fills with old skin cells and other waste material. […] Prompt and thorough treatment of chronic ear infection may help prevent cholesteatoma.
  • #43 Cholesteatoma: Causes, symptoms, and treatment
    https://www.medicalnewstoday.com/articles/cholesteatoma
    Studies show that only 24% of cholesteatomas in children between the ages of 4 and 6 years are present at birth. Children with a cleft palate have a much higher risk of developing a cholesteatoma than other children. […] It is not possible to prevent congenital cases of cholesteatoma. However, it is worth parents and caregivers being aware of the condition, so that children can quickly receive diagnosis and treatment. […] For acquired cholesteatoma, properly treating ear infections is the best prevention. However, cholesteatomas may still develop. […] Without treatment, a severe cholesteatoma can be fatal.
  • #44 Cholesteatoma: Causes, Symptoms, and Diagnosis
    https://www.healthline.com/health/cholesteatoma
    A cholesteatoma is a noncancerous skin growth in the middle section of your ear, behind the eardrum. It can be a birth defect or result from repeated middle ear infections. […] Besides repeated infections, a cholesteatoma may also be caused by a poorly functioning eustachian tube, which is the tube that leads from the back of the nose to the middle of the ear. […] In very rare cases, a baby may be born with a cholesteatoma. This is considered a birth defect. Congenital cholesteatomas can form in the middle ear or in other areas of the ear. […] In cases where children acquire ear infections repeatedly early in life, it is possible that cholesteatomas can develop from a young age. […] If your eustachian tube isnt working correctly, a partial vacuum might occur in your middle ear. This may cause a section of your eardrum to be pulled into the middle ear, creating a cyst that can turn into a cholesteatoma.
  • #45 Cholesteatoma and Otosclerosis: Two slowly progressive causes of hearing loss treatable through corrective surgery
    https://pmc.ncbi.nlm.nih.gov/articles/PMC1069039/
    Cholesteatoma is a unique disease of the ear in which a skin cyst grows into the middle ear and mastoid. […] There are several theories on how a cholesteatoma forms. Most evidence indicates that improper function of the Eustachian tube contributes to the formation of a cholesteatoma. […] Patients who develop a cholesteatoma commonly have had previous problems with middle ear fluid and/or infections. […] It is also important to understand that most patients with a history of ear fluid/infections do not develop a cholesteatoma. […] The cholesteatoma will usually grow or expand if not removed. […] The primary goal of the surgery is to remove the disease; the secondary goal is to restore or maintain hearing. […] Cholesteatoma and otosclerosis are two causes of progressive hearing loss less familiar to the general practitioner.
  • #46 Middle Ear Cholesteatoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448108/
    Nonretraction pocket cholesteatomas, also known as secondary-acquired cholesteatomas, typically result from acute otitis media but can also arise from trauma, such as surgery, blast injuries, or foreign bodies. […] Epithelial migration theory (immigration theory): This theory contradicts the retraction pocket theory. The theory posits that cholesteatomas form due to a defect in the tympanic membrane, allowing keratinizing squamous epithelium to migrate into the middle ear. […] Squamous metaplasia theory: This theory proposes that cholesteatomas originate before tympanic membrane perforation. The theory suggests that metaplastic transformation of middle ear mucosa, driven by chronic irritation, leads to cholesteatoma formation due to hyperproliferation. […] Basal cell hyperplasia (papillary ingrowth) theory: This theory proposes that the Prussak space (a recess medial to the neck of the malleus and lateral to the pars flaccida) is invaded by keratin-filled microcysts, buds, or pseudopods originating from the basal layer of the epithelium. […] Regardless of the etiology, once middle ear cholesteatomas have formed, they continue to proliferate and migrate, causing damage to surrounding structures in the middle ear. Cholesteatoma is a progressive condition that can eventually impact bone, soft tissue, and nerves.
  • #47 Cholesteatoma | Boston Medical Center
    https://www.bmc.org/patient-care/conditions-we-treat/db/cholesteatoma
    A cholesteatoma is a skin growth that occurs in the middle ear behind the eardrum. This condition usually results from poor Eustachian tube function concurrent with middle ear infection (otitis media), but can also be present at birth. […] Over time, untreated cholesteatoma can lead to bone erosion and spread of the ear infection to localized areas such as the inner ear and brain. […] If untreated, deafness, brain abscess, meningitis, and death can occur.