Otoskleroza
Etiologia i przyczyny

Otoskleroza to schorzenie charakteryzujące się nieprawidłową remodelacją kości w uchu środkowym, głównie wokół strzemiączka, prowadzącą do postępującego niedosłuchu przewodzeniowego. Etiologia jest wieloczynnikowa, obejmująca predyspozycje genetyczne (około 60% przypadków), zidentyfikowane loci na chromosomach 6p, 9p, 1q, 3q, 6q, 7q, 15q, 16q oraz mutacje w genie SERPINF1 i locus 7q22.1. Infekcja wirusem odry, wykrywana w 59-100% ognisk otosklerotycznych, oraz czynniki hormonalne, zwłaszcza ciąża, znacząco wpływają na rozwój i progresję choroby. Dodatkowo, mechanizmy autoimmunologiczne i zaburzenia metabolizmu kości (w tym geny COL1A1, TGF-beta 1, BMP2, BMP4) mogą przyczyniać się do patologii. Epidemiologicznie otoskleroza dotyczy 0,3-0,4% populacji kaukaskiej, z dwukrotną przewagą u kobiet i typowym początkiem między 15 a 35 rokiem życia.

Otoskleroza – Etiologia, przyczyny powstawania

Otoskleroza to schorzenie charakteryzujące się nieprawidłowym wzrostem kości w uchu środkowym, głównie wokół strzemiączka, co prowadzi do postępującego niedosłuchu przewodzeniowego. Dokładna etiologia tej choroby pozostaje niejasna, jednak badania wskazują na złożony mechanizm obejmujący czynniki genetyczne, środowiskowe, hormonalne i immunologiczne.12

Czynniki genetyczne

Predyspozycje genetyczne odgrywają kluczową rolę w rozwoju otosklerozy. Szacuje się, że około 60% przypadków otosklerozy ma podłoże genetyczne.34 W ponad połowie przypadków pacjenci zgłaszają występowanie otosklerozy w rodzinie, co sugeruje dziedziczny charakter choroby.5 Transmisja genów odpowiedzialnych za otosklerozę jest złożona i nie każda osoba posiadająca predyspozycje genetyczne rozwinie objawy kliniczne.6

Ryzyko wystąpienia otosklerozy zwiększa się przy dodatnim wywiadzie rodzinnym:

  • Osoba z jednym rodzicem z otosklerozą ma około 25% szans na rozwój tego schorzenia78
  • Przy obojgu rodzicach z otosklerozą ryzyko wzrasta do 50%910

Badania genetyczne zidentyfikowały wiele loci chromosomowych związanych z otosklerozą, w tym obszary na chromosomach 6p, 9p, 1q, 3q, 6q, 7q, 15q i 16q.11 Niedawno zidentyfikowano również zmiany w genie SERPINF1, które mogą powodować otosklerozę.12 W otwartej analizie całego genomu zidentyfikowano nowy locus na chromosomie 7q22.1.13

Czynniki wirusowe

Infekcje wirusowe, szczególnie wirus odry, mogą odgrywać istotną rolę w patogenezie otosklerozy.14 RNA wirusa odry wykryto w ogniskach otosklerotycznych w płytce strzemiączka u 59-100% pacjentów z otosklerozą.1516 Występuje także wyraźna korelacja między szczepionkami przeciwko odrze a zmniejszoną częstością występowania otosklerozy:

  • Badania wykazały, że osoby zaszczepione przeciwko odrze mają około 25% niższy wskaźnik leczenia otosklerozy w porównaniu z nieszczepionymi17
  • Wirus odry może aktywować gen odpowiedzialny za otosklerozę18

Teoria ta sugeruje, że wirus odry może być czynnikiem wyzwalającym u osób genetycznie predysponowanych do rozwoju otosklerozy.19

Czynniki hormonalne

Obserwacje kliniczne wyraźnie wskazują na rolę czynników hormonalnych w rozwoju i progresji otosklerozy.20 Choroba występuje dwa razy częściej u kobiet niż u mężczyzn, co sugeruje udział hormonów płciowych w patogenezie.2122

Szczególną uwagę zwraca się na wpływ ciąży na przebieg otosklerozy:

  • Ciąża może nasilać objawy otosklerozy i przyspieszać utratę słuchu2324
  • U kobiet podatnych na otosklerozę, choroba może rozwinąć się podczas ciąży25
  • Badania sugerują, że zmiany poziomu estrogenu podczas ciąży mogą odgrywać rolę w nasileniu objawów26
  • Kobiety z większą liczbą dzieci wymagały operacji strzemiączka w młodszym wieku27

Oprócz ciąży, inne stany związane ze zmianami hormonalnymi mogą wpływać na rozwój otosklerozy, w tym menopauza i stosowanie antykoncepcji hormonalnej.2829

Czynniki autoimmunologiczne

Istnieją dowody sugerujące, że otoskleroza może mieć podłoże autoimmunologiczne.30 Badacze zwracają uwagę na rolę nieprawidłowej odpowiedzi immunologicznej w patogenezie choroby:

  • Zaburzenia autoimmunologiczne, w których układ odpornościowy atakuje własne tkanki organizmu, mogą być powiązane z otosklerozą31
  • Reakcja autoimmunologiczna może zakłócać normalny proces przebudowy kości32
  • Cytokiny, będące komórkami układu immunologicznego, odgrywają istotną rolę w zdrowej przebudowie kości, a ich zaburzenie może prowadzić do nieprawidłowej remodelacji kości typowej dla otosklerozy3334

W badaniach genetycznych zidentyfikowano także warianty genów związanych z układem immunologicznym, które mogą przyczyniać się do rozwoju otosklerozy.35

Inne czynniki ryzyka

Oprócz głównych czynników etiologicznych, zidentyfikowano również inne potencjalne czynniki ryzyka rozwoju otosklerozy:

  • Pochodzenie etniczne: otoskleroza występuje częściej u osób rasy kaukaskiej, zwłaszcza pochodzenia europejskiego lub indyjskiego, natomiast rzadziej występuje u osób pochodzenia afrykańskiego363738
  • Wiek: otoskleroza najczęściej rozwija się u młodych dorosłych, zazwyczaj między 15 a 35 rokiem życia3940
  • Osteogenesis imperfecta (wrodzona łamliwość kości): osoby z tym schorzeniem mają zwiększone ryzyko rozwoju otosklerozy4142
  • Uraz i mikrourazy: urazy oraz mikrourazy tkanki kostnej otaczającej ucho wewnętrzne mogą zwiększać ryzyko rozwoju otosklerozy4344
  • Niski poziom fluoru w wodzie pitnej: niektóre badania epidemiologiczne wykazały związek między otosklerozą a niską zawartością fluoru w wodzie pitnej4546
  • Zaburzenia metabolizmu kości: inne nieprawidłowości metabolizmu kości, w tym geny związane z remodelowaniem kości (COL1A1, TGF-beta 1, BMP2, BMP4), mogą być potencjalnymi przyczynami otosklerozy47

Patofizjologia otosklerozy

Patofizjologia otosklerozy jest złożona i nie do końca poznana. Kluczową cechą tej choroby są wieloogniskowe obszary sklerotyzacji w obrębie torebki kostnej ucha wewnętrznego.4849

Fazy rozwoju choroby

W przebiegu otosklerozy wyróżnia się dwie główne fazy:

  • Faza wczesna/aktywna (otospongioza): charakteryzuje się występowaniem wysoce unaczynionych zmian, które resorbują kość otaczającą ucho wewnętrzne. Zmiany te składają się głównie z histiocytów, osteoblastów i osteocytów, przy czym osteocyty są najbardziej aktywną grupą komórek.5051
  • Faza późna/nieaktywna (otoskleroza właściwa): aktywne zmiany dojrzewają do zwapniałych blaszek otosklerotycznych, które są odpowiedzialne za unieruchomienie strzemiączka.52

Podczas tego procesu prawidłowa dojrzała kość blaszkowata zostaje zastąpiona kością o charakterze gąbczastym, która jest bardziej porowata i mniej gęsta.53 Zmiany te występują w sposób plamkowy (nieregularny).54

Mechanizm powstawania niedosłuchu

Otoskleroza prowadzi do zaburzeń słuchu poprzez kilka mechanizmów:

  1. Unieruchomienie strzemiączka: nieprawidłowy wzrost kości najczęściej występuje wokół podstawy strzemiączka, powodując jego unieruchomienie i uniemożliwiając prawidłowe drgania. W rezultacie fale dźwiękowe nie mogą być efektywnie przekazywane z ucha środkowego do ucha wewnętrznego.5556
  2. Zaburzenia w przemianie kości: w otosklerozie występuje zaburzony metabolizm tkanki kostnej w okolicy okienka owalnego, co prowadzi do nieprawidłowego wzrostu kości i ograniczenia ruchomości łańcucha kosteczek słuchowych.57
  3. Progresja do ucha wewnętrznego: w zaawansowanych przypadkach choroba może rozprzestrzeniać się do ucha wewnętrznego, powodując dodatkowe uszkodzenie komórek sensorycznych, co prowadzi do niedosłuchu odbiorczego.58

W typowym obrazie otosklerozy występuje różnica między progiem przewodnictwa kostnego a powietrznego, gdzie próg przewodnictwa kostnego jest na niższych poziomach niż próg przewodnictwa powietrznego, co określa się jako niedosłuch przewodzeniowy.59

Epidemiologia i grupy ryzyka

Otoskleroza jest jedną z najczęstszych przyczyn postępującego niedosłuchu u młodych dorosłych.60 Dane epidemiologiczne wskazują na specyficzny wzorzec występowania tej choroby:

Rozpowszechnienie

Częstość występowania otosklerozy w populacji ogólnej wynosi około 0,3-0,4% w populacji kaukaskiej.61 Jest to przyczyna około 5% wszystkich przypadków niedosłuchu.62 Chorobę stwierdza się w badaniach autopsyjnych u 5-10% osób rasy kaukaskiej, u 25-30% mieszkańców południowych Indii oraz u 60-70% Etiopczyków.63

Dystrybucja demograficzna

  • Płeć: otoskleroza występuje dwa razy częściej u kobiet niż u mężczyzn6465
  • Wiek: objawy najczęściej pojawiają się między 15 a 35 rokiem życia6667
  • Rasa: choroba występuje najczęściej u osób rasy kaukaskiej, szczególnie pochodzenia europejskiego lub indyjskiego, rzadziej u osób pochodzenia afrykańskiego6869

Grupy szczególnego ryzyka

Określone grupy osób są szczególnie narażone na rozwój otosklerozy:

  • Osoby z rodzinnym występowaniem otosklerozy: przy jednym rodzicu z otosklerozą ryzyko wynosi około 25%, przy obojgu rodzicach wzrasta do 50%7071
  • Kobiety w ciąży: ciąża może przyspieszać rozwój lub nasilać objawy otosklerozy7273
  • Osoby z historią zachorowania na odrę: przebyta infekcja wirusem odry zwiększa ryzyko rozwoju otosklerozy7475
  • Osoby z osteogenesis imperfecta: choroba powodująca wrodzoną łamliwość kości zwiększa ryzyko otosklerozy7677
  • Osoby z zaburzeniami autoimmunologicznymi: choroby autoimmunologiczne mogą predysponować do rozwoju otosklerozy7879

Interakcje między czynnikami etiologicznymi

Aktualne dane naukowe sugerują, że otoskleroza jest prawdopodobnie wynikiem interakcji między różnymi czynnikami, a nie pojedynczej przyczyny.80 Wystąpienie i ciężkość choroby mogą zależeć od kombinacji czynników genetycznych, środowiskowych i wewnętrznych.

Obecne rozumienie etiologii otosklerozy wskazuje na następujące interakcje:

  • Komponenta genetyczna może nie być wystarczająca do zapoczątkowania choroby – prawdopodobnie wymagane są dodatkowe czynniki wyzwalające81
  • Wirus odry może działać jako czynnik uaktywniający u osób genetycznie predysponowanych8283
  • Zmiany hormonalne (np. podczas ciąży) mogą przyspieszać progresję choroby u osób z istniejącymi predyspozycjami84
  • Mechanizmy autoimmunologiczne mogą przyczyniać się do zaburzenia równowagi metabolicznej kości85

Mimo licznych badań, dokładny mechanizm interakcji między tymi czynnikami pozostaje niejasny, a etiologia otosklerozy jest prawdopodobnie heterogenna, z różnym udziałem czynników środowiskowych i genetycznych u poszczególnych pacjentów.86

Perspektywy badawcze w etiologii otosklerozy

Badania nad etiologią otosklerozy są ciągle rozwijane, z kilkoma obiecującymi kierunkami:

  • Badania genetyczne: identyfikacja nowych genów i mutacji związanych z otosklerozą, w tym analiza całego genomu w poszukiwaniu markerów predykcyjnych8788
  • Rola wirusów: dalsze badania nad związkiem między wirusem odry a otosklerozą, w tym skuteczność szczepień jako czynnika ochronnego89
  • Mechanizmy molekularne: badanie szlaków sygnałowych i mechanizmów komórkowych zaangażowanych w nieprawidłową przebudowę kości90
  • Czynniki środowiskowe: analiza roli fluoru i innych czynników środowiskowych w patogenezie choroby91
  • Potencjalne metody profilaktyki: rozwój strategii zmniejszających ryzyko rozwoju lub progresji otosklerozy, szczególnie u osób z czynnikami genetycznymi92

Lepsze zrozumienie etiologii otosklerozy może prowadzić do opracowania skuteczniejszych metod zapobiegania i leczenia tej choroby, wykraczających poza obecne opcje chirurgiczne i akustyczne.93

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

Materiały źródłowe

  • #1 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Otosclerosis is a pathological bone remodeling process in which the normal, dense endochondral bone of the otic capsule is replaced by one or more foci of irregularly laid spongy bone that subsequently hardens abnormally and impairs function; the condition most commonly involves the stapes region. […] The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. […] In more than half of patients presenting with otosclerosis, there is a family history of the condition. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. […] Otosclerosis is more common in patients of European or Indian ancestry.
  • #2 Otosclerosis – UF Health
    https://ufhealth.org/conditions-and-treatments/otosclerosis
    Otosclerosis is an abnormal bone growth in the middle ear that causes hearing loss. […] The exact cause of otosclerosis is unknown. It may be passed down through families. […] Risks for this condition include pregnancy and a family history of hearing loss. White people are more likely to develop this condition than people of other races.
  • #3 Otosclerosis – ENT Carolina
    https://entcarolina.com/patient-center/patient-education/otosclerosis/
    Otosclerosis usually causes conductive hearing loss, which means there is a problem with how the ear transmits sound vibrations. […] Approximately 60 percent of otosclerosis has an underlying genetic cause. Transmission of the gene that causes otosclerosis is complex and not everyone who has the gene will develop the condition.
  • #4 Otosclerosis
    https://dariuskohanmd.com/otosclerosis
    About ten percent of the adult Caucasian population is affected by otosclerosis. […] Approximately 60 percent of otosclerosis cases are genetic in origin. On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent. Rubella virus has been implicated in some cases of otosclerosis.
  • #5 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Otosclerosis is a pathological bone remodeling process in which the normal, dense endochondral bone of the otic capsule is replaced by one or more foci of irregularly laid spongy bone that subsequently hardens abnormally and impairs function; the condition most commonly involves the stapes region. […] The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. […] In more than half of patients presenting with otosclerosis, there is a family history of the condition. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. […] Otosclerosis is more common in patients of European or Indian ancestry.
  • #6 Otosclerosis
    https://ent-hns.net/otosclerosis
    Otosclerosis describes a condition of abnormal bone growth around one of the three small bones in the middle ear space called the stapes. […] Approximately 60 percent of otosclerosis has an underlying genetic cause. Transmission of the gene that causes otosclerosis is complex and not everyone who has the gene will develop the condition.
  • #7 Otosclerosis – American Hearing Research Foundation
    https://www.american-hearing.org/disease/otosclerosis/
    Otosclerosis affects the bones of the middle ear that conduct sound. Sometimes called otospongiosis, its one of the most common causes of progressive hearing loss in young adults. What triggers otosclerosis is still uncertain. But it often runs in families, and experts consider it an inherited disease. Still, there isnt always a previous family history and developing otosclerosis when a family history does exist isnt absolute. […] Known to have a genetic component, otosclerosis predominantly affects Caucasians of European decent, and white women in particular. In the United States, about one in 10 Caucasian adults develop otosclerosis, with the risk to white women twice as high. Generally, if someone has a parent with otosclerosis, they have a 25 percent chance of developing it as well. The risk jumps to 50 percent if both parents have the disorder, experts approximate.
  • #8 Otosclerosis | Shohet Ear Associates | Audiologist, Hearing Aids in Orange County
    https://www.eardoctor.org/conditions-and-treatments/other-conditions/otosclerosis/
    The exact cause of otosclerosis is unknown but approximately 60 percent of otosclerosis cases have a genetic predisposition. […] On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent.
  • #9 Otosclerosis – Brigham and Women’s Faulkner Hospital
    https://www.brighamandwomensfaulkner.org/programs-and-services/otolaryngology/ear/otosclerosis
    Otosclerosis, which affects more than three million Americans, is caused by an abnormal growth of sponge-like bone in the middle ear. Many cases of otosclerosis are thought to be inherited. On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent. […] Otosclerosis is most often caused when one of the bones in the middle ear, the stapes, becomes stuck in place due to the abnormal bone growth. When this bone is unable to vibrate, sound is unable to travel through the ear and hearing becomes impaired. […] The hallmark of otosclerosis is a slowly worsening ability to hear.
  • #10 Otosclerosis – Ear, Nose, and Throat Disorders – Merck Manual Consumer Version
    https://www.merckmanuals.com/home/ear-nose-and-throat-disorders/middle-ear-disorders/otosclerosis
    Otosclerosis causes abnormal bone to form and accumulate in the bones of the ear. The abnormal bone interferes with the transmission of sound from the middle ear to the inner ear. […] Otosclerosis tends to run in families, and half of all cases are inherited. Children have a 25% chance of developing otosclerosis if one parent has it and a 50% chance if both parents have it.
  • #11 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Otosclerosis is a pathological bone remodeling process in which the normal, dense endochondral bone of the otic capsule is replaced by one or more foci of irregularly laid spongy bone that subsequently hardens abnormally and impairs function; the condition most commonly involves the stapes region. […] The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. […] In more than half of patients presenting with otosclerosis, there is a family history of the condition. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. […] Otosclerosis is more common in patients of European or Indian ancestry.
  • #12 Otosclerosis – Balance & Dizziness Canada
    https://balanceanddizziness.org/disorders/vestibular-disorders/otosclerosis/
    Contributing reasons may include: A faulty gene inherited from a parent. Researchers have recently identified a range of changes in the SERPINF1 gene that can cause otosclerosis. […] Exposure to the measles virus may play an important role in activating a gene responsible for otosclerosis. […] Some studies suggest an autoimmune response in the body, possibly related to environmental or genetic factors, may be linked to otosclerosis. […] Changes in levels of the hormone estrogen during pregnancy may play a role in worsening otosclerosis. […] Otosclerosis often occurs along with a rare disorder called brittle bone disease (osteogenesis imperfecta).
  • #13 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. In an open genome-wide analysis, a new locus on chromosome 7q22.1 has also been identified. Additionally, other bone metabolism abnormalities, such as osteogenesis imperfecta, and multiple genes associated with bone remodeling have been considered potential causes of otosclerosis, including type I collagen (COL1A1), TGF-beta 1 (BMP2 and BMP4), angiotensin II (AGT M235T and ACE I/D), autoimmune reactions, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone and expression of parathyroid hormone-related peptide receptors, and oxidative stress.
  • #14 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear. […] A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #15 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. Measles virus ribonucleic acid has been detected in the footplate of the stapes on electron microscopy and immunohistochemical studies in 59%-100% of patients. Moreover, vaccination against the measles virus appears to be a protective factor against otosclerosis: in 2007, Arnold et al demonstrated a ~25% lower rate of treatment for otosclerosis in Germany for the population vaccinated against measles compared with patients who were not. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #16 Otosclerosis: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/859760-overview
    The exact cause of otosclerosis is unknown. Measles virus RNA is found in otosclerotic foci in footplates removed during surgery. Measles virus infection may activate the gene responsible for otosclerosis. Otosclerosis, however, is not responsible for all cases of stapes ankylosis. A heterogeneous group of disorders, including other bone degenerative disorders, appears to cause stapes fixation and conductive hearing loss. […] Evidence has recently mounted that the measles virus plays an important role in gene activation of otosclerosis. This hypothesis is supported by a declining incidence of otosclerosis since measles vaccinations became widespread as well as finding measles virus RNA in the footplate of otosclerosis specimens.
  • #17 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. Measles virus ribonucleic acid has been detected in the footplate of the stapes on electron microscopy and immunohistochemical studies in 59%-100% of patients. Moreover, vaccination against the measles virus appears to be a protective factor against otosclerosis: in 2007, Arnold et al demonstrated a ~25% lower rate of treatment for otosclerosis in Germany for the population vaccinated against measles compared with patients who were not. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #18 Otosclerosis: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/859760-overview
    The exact cause of otosclerosis is unknown. Measles virus RNA is found in otosclerotic foci in footplates removed during surgery. Measles virus infection may activate the gene responsible for otosclerosis. Otosclerosis, however, is not responsible for all cases of stapes ankylosis. A heterogeneous group of disorders, including other bone degenerative disorders, appears to cause stapes fixation and conductive hearing loss. […] Evidence has recently mounted that the measles virus plays an important role in gene activation of otosclerosis. This hypothesis is supported by a declining incidence of otosclerosis since measles vaccinations became widespread as well as finding measles virus RNA in the footplate of otosclerosis specimens.
  • #19 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    A common disease characterized by a disordered bone remodeling in the otic capsule, otosclerosis has a prevalence of about 0.3 percent to 0.4 percent in the Caucasian population. […] Although many environmental and genetic factors have been suggested for the development of otosclerosis, a great part of the etiology remains a mystery. […] The data suggest that measles virus infection is probably a susceptibility factor involved in otosclerosis but not the single cause or necessary for the disease to manifest. Endocrine factors could be involved because of the large difference in prevalence between males and females (1:2). […] In addition, epidemiological studies show otosclerosis associated with low fluoride content in the drinking water. […] Evidence for genetic variants in otosclerosis has been discovered in different parts of the spectrum, but the extent to which these contribute to the disease is unclear since only a limited number of genetic studies have been done.
  • #20 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Otosclerosis is a pathological bone remodeling process in which the normal, dense endochondral bone of the otic capsule is replaced by one or more foci of irregularly laid spongy bone that subsequently hardens abnormally and impairs function; the condition most commonly involves the stapes region. […] The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. […] In more than half of patients presenting with otosclerosis, there is a family history of the condition. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. […] Otosclerosis is more common in patients of European or Indian ancestry.
  • #21 Otosclerosis: Symptoms, Complications, and Treatment
    https://www.healthline.com/health/otosclerosis
    Otosclerosis is a condition of the middle ear that involves abnormal bone remodeling. Otosclerosis causes tissues to harden around three bones called the stapes, incus, and malleus. […] The exact cause is unknown, but early detection of otosclerosis is key to helping you access treatments that can improve your hearing. […] While certain types of infections, immune-mediated diseases, and stress fractures may be possible risk factors for developing otosclerosis, the exact cause of this ear condition isn’t clear. Scientists believe that otosclerosis may run in families. […] There’s also a theory and anecdotal reporting that pregnancy may increase the risk of otosclerosis. In fact, a 2020 review found that females with a higher number of children needed surgery of the stapes at a younger age overall. […] But the authors also note that there’s not enough evidence to draw a definitive link between pregnancy and otosclerosis. It’s also possible that hormone fluctuations in pregnancy could worsen pre-existing cases.
  • #22 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    In more than half of patients presenting with otosclerosis, there is a family history of the condition. Additionally, patients with family histories of otosclerosis tend to demonstrate earlier symptom onset. Genetic studies show that an autosomal dominant mode of inheritance with reduced penetrance (40%) and variable expressivity is found in most cases. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. However, because of the elasticity that estrogens provide to ligaments, women seem to suffer less hearing loss than men with the same degree of otosclerosis. […] Otosclerosis is more common in patients of European or Indian ancestry. The condition is seen less frequently in individuals of African descent. […] Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear.
  • #23 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear. […] A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #24 Otosclerosis – ENT Doctor | Ear and Tinnitus Specialist | Hamid Djalilian MD
    https://hamiddjalilianmd.com/conditions/otosclerosis/
    Otosclerosis is a condition caused by abnormal growth of the bone of the inner ear. […] Otosclerosis is thought to be hereditary, which means it tends to run in families. Having a close relative with otosclerosis thus increases the chances of developing it. Certain viruses are also thought to be a potential cause of otosclerosis. The hormones produced during pregnancy cause the disease to progress more quickly, so a pregnant woman with otosclerosis is at a greater risk of developing hearing loss than is a man or a woman who is not pregnant.
  • #25 Otosclerosis: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22033-otosclerosis
    Otosclerosis happens when irregular bone remodeling/growth occurs in your middle ear or, more rarely, your inner ear. […] Otosclerosis most often develops when the stapes bone (a small, triangular bone in your middle ear) fuses with the surrounding bone tissue. […] Possible risk factors for otosclerosis include: Family history. Youre more likely to develop otosclerosis if a parent, sibling or grandparent has it. […] Females are more prone to developing otosclerosis. […] Overall, white people have a higher chance of getting otosclerosis. […] People who are already prone to otosclerosis may develop the condition while theyre pregnant. […] Osteogenesis imperfecta (OI). Also known as brittle bone disease, OI increases your risk of otosclerosis.
  • #26 Otosclerosis – Balance & Dizziness Canada
    https://balanceanddizziness.org/disorders/vestibular-disorders/otosclerosis/
    Contributing reasons may include: A faulty gene inherited from a parent. Researchers have recently identified a range of changes in the SERPINF1 gene that can cause otosclerosis. […] Exposure to the measles virus may play an important role in activating a gene responsible for otosclerosis. […] Some studies suggest an autoimmune response in the body, possibly related to environmental or genetic factors, may be linked to otosclerosis. […] Changes in levels of the hormone estrogen during pregnancy may play a role in worsening otosclerosis. […] Otosclerosis often occurs along with a rare disorder called brittle bone disease (osteogenesis imperfecta).
  • #27 Otosclerosis: Symptoms, Complications, and Treatment
    https://www.healthline.com/health/otosclerosis
    Otosclerosis is a condition of the middle ear that involves abnormal bone remodeling. Otosclerosis causes tissues to harden around three bones called the stapes, incus, and malleus. […] The exact cause is unknown, but early detection of otosclerosis is key to helping you access treatments that can improve your hearing. […] While certain types of infections, immune-mediated diseases, and stress fractures may be possible risk factors for developing otosclerosis, the exact cause of this ear condition isn’t clear. Scientists believe that otosclerosis may run in families. […] There’s also a theory and anecdotal reporting that pregnancy may increase the risk of otosclerosis. In fact, a 2020 review found that females with a higher number of children needed surgery of the stapes at a younger age overall. […] But the authors also note that there’s not enough evidence to draw a definitive link between pregnancy and otosclerosis. It’s also possible that hormone fluctuations in pregnancy could worsen pre-existing cases.
  • #28 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. Measles virus ribonucleic acid has been detected in the footplate of the stapes on electron microscopy and immunohistochemical studies in 59%-100% of patients. Moreover, vaccination against the measles virus appears to be a protective factor against otosclerosis: in 2007, Arnold et al demonstrated a ~25% lower rate of treatment for otosclerosis in Germany for the population vaccinated against measles compared with patients who were not. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #29 Otosclerosis | South Valley Ear Nose & Throat
    https://southvalleyent.com/ent/otosclerosis/
    Otosclerosis is an abnormal growth of bone in the middle ear that causes hearing loss. […] The exact cause of otosclerosis is not known, but evidence suggests a genetic link passed down from parent to child. Middle-aged Caucasian women are most at risk and hormonal changes seem to be a contributing factor including those produced by pregnancy and menopause. […] This bone growth usually occurs around the stapes bone in the middle ear, preventing it from moving freely, essential to proper hearing.
  • #30 Otosclerosis | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/otosclerosis.html
    Otosclerosis occurs due to irregular turnover of the bone that surrounds the inner ear. […] Doctors know that otosclerosis develops due to abnormal bone growth but do not know what triggers this process. It is likely a complex combination of genetic, environmental, and hormonal factors. […] Potential factors associated with otosclerosis include: Family history: About half of people who develop otosclerosis have a family member with the condition. But not all people with a family history of otosclerosis develop symptoms. Researchers are trying to better understand the genetic mechanisms that control family inheritance. […] Immune response: Autoimmunity occurs when your immune system becomes confused and attacks your body. An autoimmune response may cause otosclerosis by disrupting the normal bone growth process.
  • #31 Otosclerosis and Hearing Loss: Risk Factors, Symptoms, and Treatment
    https://www.webmd.com/cold-and-flu/ear-infection/otosclerosis-facts
    Otosclerosis affects more than 3 million Americans. Experts aren’t sure exactly what causes it. But they do know these risk factors may make you more likely to get it: […] Certain medical problems can raise your chances of otosclerosis. For example, if you had measles at any time, your risk may go up. Stress fractures to the bony tissue around your inner ear also might make it more likely to happen. And immune disorders, in which your immune system mistakenly attacks parts of your body, also can be linked to the condition.
  • #32 Otosclerosis | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/otosclerosis.html
    Otosclerosis occurs due to irregular turnover of the bone that surrounds the inner ear. […] Doctors know that otosclerosis develops due to abnormal bone growth but do not know what triggers this process. It is likely a complex combination of genetic, environmental, and hormonal factors. […] Potential factors associated with otosclerosis include: Family history: About half of people who develop otosclerosis have a family member with the condition. But not all people with a family history of otosclerosis develop symptoms. Researchers are trying to better understand the genetic mechanisms that control family inheritance. […] Immune response: Autoimmunity occurs when your immune system becomes confused and attacks your body. An autoimmune response may cause otosclerosis by disrupting the normal bone growth process.
  • #33 What Is Otosclerosis? Symptoms & Diagnosis| NIDCD
    https://www.nidcd.nih.gov/health/otosclerosis
    Otosclerosis is most often caused when one of the bones in the middle ear, the stapes, becomes stuck in place. When this bone is unable to vibrate, sound is unable to travel through the ear and hearing becomes impaired (see illustration). […] Why this happens is still unclear, but scientists think it could be related to a previous measles infection, stress fractures to the bony tissue surrounding the inner ear, or immune disorders. Otosclerosis also tends to run in families. […] It may also have to do with the interaction among three different immune-system cells known as cytokines. Researchers believe that the proper balance of these three substances is necessary for healthy bone remodeling and that an imbalance in their levels could cause the kind of abnormal remodeling that occurs in otosclerosis.
  • #34 What is otosclerosis and how can you treat it?
    https://www.hearclear.com/hearing-health/otosclerosis/
    Otosclerosis causes hearing loss. […] If you have otosclerosis, remodeling of the stapes bone in the ear goes wrong and new bone forms abnormally. […] Its not known why this happens, or even why it affects the stapes in particular. […] One possibility is that its related to cytokines (cells in the immune system) that are thought to be important in healthy bone remodeling. […] Its likely theres a genetic factor, too, because otosclerosis appears to run in families. […] Otosclerosis has been associated with viral infection, particularly the measles virus. […] Experts think its likely that a combination of genetic and environmental factors determine whether you develop otosclerosis.
  • #35 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    Most cases of otosclerosis, however, do not have a clear familial background. […] The authors noted that recently they have been able to provide strong evidence for T cell receptor beta as the responsible gene at the OTSC2 region. […] The authors also discussed association studies for otosclerosis, including genes in the immune system, in bone remodeling and in hormonal and other pathways. […] Regarding otosclerosis as an autoimmune disease, the authors said the precise underlying mechanism might vary among otosclerosis patients as several factors can influence and contribute to a similar disease outcome and it is unclear whether an autoimmune pathology underlies disease development. […] Overall, studies suggest a heterogeneous etiology for otosclerosis with the extent of involvement of environmental versus genetic factors unclear.
  • #36 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Otosclerosis is a pathological bone remodeling process in which the normal, dense endochondral bone of the otic capsule is replaced by one or more foci of irregularly laid spongy bone that subsequently hardens abnormally and impairs function; the condition most commonly involves the stapes region. […] The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. […] In more than half of patients presenting with otosclerosis, there is a family history of the condition. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. […] Otosclerosis is more common in patients of European or Indian ancestry.
  • #37 Otosclerosis – UF Health
    https://ufhealth.org/conditions-and-treatments/otosclerosis
    Otosclerosis is an abnormal bone growth in the middle ear that causes hearing loss. […] The exact cause of otosclerosis is unknown. It may be passed down through families. […] Risks for this condition include pregnancy and a family history of hearing loss. White people are more likely to develop this condition than people of other races.
  • #38 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    In more than half of patients presenting with otosclerosis, there is a family history of the condition. Additionally, patients with family histories of otosclerosis tend to demonstrate earlier symptom onset. Genetic studies show that an autosomal dominant mode of inheritance with reduced penetrance (40%) and variable expressivity is found in most cases. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. However, because of the elasticity that estrogens provide to ligaments, women seem to suffer less hearing loss than men with the same degree of otosclerosis. […] Otosclerosis is more common in patients of European or Indian ancestry. The condition is seen less frequently in individuals of African descent. […] Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear.
  • #39 What Is Otosclerosis and What Causes It? | Bay Audio
    https://www.bayaudio.com.au/types-of-hearing-loss/conductive-hearing-loss/what-is-otosclerosis/
    Otosclerosis occurs when abnormal bone growth stops these tiny bones from vibrating. […] The precise cause of otosclerosis remains unclear, but research has linked it to factors such as: […] Genetics: while its possible to develop otosclerosis with no genetic history, the condition often runs in families. […] Age: unlike most other forms of hearing loss, otosclerosis is statistically more likely to appear in younger age brackets particularly those in their 20s. […] Ethnicity and gender: caucasian women appear to be most susceptible to otosclerosis. […] Prior health issues: while research is still being conducted, doctors have found some evidence linking otosclerosis to measles, immune conditions, and stress fractures to the bony tissue surrounding the inner and middle ears. […] Despite these links, otosclerosis can still develop without any known underlying factors.
  • #40 Otosclerosis causes | Otosclerosis childhood deafness treatment
    https://www.ndcs.org.uk/information-and-support/childhood-deafness/causes-of-deafness/otosclerosis/
    Otosclerosis can be a hereditary (genetic) disorder, but in many cases where there is no family history, it is thought to be caused by a virus. […] Otosclerosis usually develops between the ages of 15 to 35 but has been known to occur in younger children.
  • #41 Otosclerosis: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22033-otosclerosis
    Otosclerosis happens when irregular bone remodeling/growth occurs in your middle ear or, more rarely, your inner ear. […] Otosclerosis most often develops when the stapes bone (a small, triangular bone in your middle ear) fuses with the surrounding bone tissue. […] Possible risk factors for otosclerosis include: Family history. Youre more likely to develop otosclerosis if a parent, sibling or grandparent has it. […] Females are more prone to developing otosclerosis. […] Overall, white people have a higher chance of getting otosclerosis. […] People who are already prone to otosclerosis may develop the condition while theyre pregnant. […] Osteogenesis imperfecta (OI). Also known as brittle bone disease, OI increases your risk of otosclerosis.
  • #42 Otosclerosis – Balance & Dizziness Canada
    https://balanceanddizziness.org/disorders/vestibular-disorders/otosclerosis/
    Contributing reasons may include: A faulty gene inherited from a parent. Researchers have recently identified a range of changes in the SERPINF1 gene that can cause otosclerosis. […] Exposure to the measles virus may play an important role in activating a gene responsible for otosclerosis. […] Some studies suggest an autoimmune response in the body, possibly related to environmental or genetic factors, may be linked to otosclerosis. […] Changes in levels of the hormone estrogen during pregnancy may play a role in worsening otosclerosis. […] Otosclerosis often occurs along with a rare disorder called brittle bone disease (osteogenesis imperfecta).
  • #43 Otosclerosis – American Hearing Research Foundation
    https://www.american-hearing.org/disease/otosclerosis/
    Measles and/or other viral infections: Some experts believe there may be a viral link to otosclerosis and to the measles in particular. […] Trauma and stress fractures: Experts have suggested that stress fractures to bones in the ear, and to the bony tissue surrounding the inner ear in particular, may put people at an increased risk of developing otosclerosis. […] Autoimmunity: Some researchers believe that otosclerosis may be linked to an autoimmune response by the body.
  • #44 Otosclerosis and Hearing Loss: Risk Factors, Symptoms, and Treatment
    https://www.webmd.com/cold-and-flu/ear-infection/otosclerosis-facts
    Otosclerosis affects more than 3 million Americans. Experts aren’t sure exactly what causes it. But they do know these risk factors may make you more likely to get it: […] Certain medical problems can raise your chances of otosclerosis. For example, if you had measles at any time, your risk may go up. Stress fractures to the bony tissue around your inner ear also might make it more likely to happen. And immune disorders, in which your immune system mistakenly attacks parts of your body, also can be linked to the condition.
  • #45 Ears – otosclerosis | Better Health Channel
    https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/ears-otosclerosis
    Otosclerosis is a form of abnormal bone growth within the middle ear that causes progressive hearing loss. […] The cause is unknown, but risk factors include family history, gender and pregnancy. […] The cause of otosclerosis is a mystery, but researchers have uncovered a number of risk factors including: family history otosclerosis tends to run in families, which suggests a genetic susceptibility or hereditary component to the disorder; gender women are more likely to develop otosclerosis than men; pregnancy susceptible women may develop otosclerosis during pregnancy; race Caucasians (white people) tend to be most commonly affected; osteogenesis imperfecta this genetic disorder is characterised by abnormally brittle bones. People with osteogenesis imperfecta are at increased risk of developing otosclerosis; non-fluoridated water some evidence suggests that drinking non-fluoridated water may increase the risk of otosclerosis in susceptible people; measles virus there is some evidence that viruses may be contributory to developing otosclerosis, in particular the measles virus; unknown causes some people develop otosclerosis even though they dont have any of the known risk factors.
  • #46 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    A common disease characterized by a disordered bone remodeling in the otic capsule, otosclerosis has a prevalence of about 0.3 percent to 0.4 percent in the Caucasian population. […] Although many environmental and genetic factors have been suggested for the development of otosclerosis, a great part of the etiology remains a mystery. […] The data suggest that measles virus infection is probably a susceptibility factor involved in otosclerosis but not the single cause or necessary for the disease to manifest. Endocrine factors could be involved because of the large difference in prevalence between males and females (1:2). […] In addition, epidemiological studies show otosclerosis associated with low fluoride content in the drinking water. […] Evidence for genetic variants in otosclerosis has been discovered in different parts of the spectrum, but the extent to which these contribute to the disease is unclear since only a limited number of genetic studies have been done.
  • #47 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. In an open genome-wide analysis, a new locus on chromosome 7q22.1 has also been identified. Additionally, other bone metabolism abnormalities, such as osteogenesis imperfecta, and multiple genes associated with bone remodeling have been considered potential causes of otosclerosis, including type I collagen (COL1A1), TGF-beta 1 (BMP2 and BMP4), angiotensin II (AGT M235T and ACE I/D), autoimmune reactions, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone and expression of parathyroid hormone-related peptide receptors, and oxidative stress.
  • #48 Otosclerosis – Wikipedia
    https://en.wikipedia.org/wiki/Otosclerosis
    Otosclerosis can be caused by both genetic and environmental factors, such as a viral infection (like measles). Ribonucleic acid of the measles virus has been found in stapes footplate in most patients with otosclerosis. Populations that have been vaccinated against measles had a significant reduction in otosclerosis. While the disease is considered to be hereditary, its penetrance and the degree of expression is so highly variable that it may be difficult to detect an inheritance pattern. Most of the implicated genes are transmitted in an autosomal dominant fashion. One genome-wide analysis associates otosclerosis with variation in the RELN gene. […] The pathophysiology of otosclerosis is complex. The key lesions of otosclerosis are multifocal areas of sclerosis within the endochondral temporal bone. These lesions share some characteristics with Paget’s Disease, but they are not thought to be otherwise related. Histopathological studies have all been done on cadaveric temporal bones, so only inferences can be made about progression of the disease histologically. It seems that the lesions go through an active „spongiotic” or hypervascular phase before developing into „sclerotic” phase lesions. There have been many genes and proteins identified that, when mutated, may lead to these lesions. Also there is mounting evidence that measles virus is present within the otosclerotic foci, implicating an infectious etiology (this has also been noted in Paget’s Disease).
  • #49 Otospongiosis | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/otospongiosis-1?lang=us
    Otospongiosis, also known as otosclerosis, is a primary osteodystrophy of the otic capsule (bony labyrinth of the inner ear). It is one of the leading causes of deafness in adults. […] The pathophysiology of otosclerosis is multifactorial and incompletely understood, with genetic, viral, inflammatory and autoimmune components. […] Two phases are described: early/active (otospongiosis) and late/inactive (otosclerosis). In the early phase, lesions consist predominantly of histiocytes, osteoblasts and osteocytes, the latter being the most active cell group. […] Retrofenestral otosclerosis usually occurs with fenestral involvement, so the two entities are considered a continuum rather than distinct.
  • #50 Otospongiosis | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/otospongiosis-1?lang=us
    Otospongiosis, also known as otosclerosis, is a primary osteodystrophy of the otic capsule (bony labyrinth of the inner ear). It is one of the leading causes of deafness in adults. […] The pathophysiology of otosclerosis is multifactorial and incompletely understood, with genetic, viral, inflammatory and autoimmune components. […] Two phases are described: early/active (otospongiosis) and late/inactive (otosclerosis). In the early phase, lesions consist predominantly of histiocytes, osteoblasts and osteocytes, the latter being the most active cell group. […] Retrofenestral otosclerosis usually occurs with fenestral involvement, so the two entities are considered a continuum rather than distinct.
  • #51 Otosclerosis | Otolaryngology⁠ — Head & Neck Surgery | Stanford Medicine
    https://med.stanford.edu/ohns/OHNS-healthcare/earinstitute/conditions-and-services/conditions/otosclerosis.html
    Otosclerosis is an inherited disease that affects the bone of the ear. […] The exact cause of the disease remains unknown. […] Otosclerosis is a disease of the otic capsule. In its active phase, known as otospongiosis, highly vascular lesions resorb bone surrounding the inner ear, most often in a patchy pattern. The active lesions mature into calcified otosclerotic plaques which are responsible for stapes fixation.
  • #52 Otosclerosis | Otolaryngology⁠ — Head & Neck Surgery | Stanford Medicine
    https://med.stanford.edu/ohns/OHNS-healthcare/earinstitute/conditions-and-services/conditions/otosclerosis.html
    Otosclerosis is an inherited disease that affects the bone of the ear. […] The exact cause of the disease remains unknown. […] Otosclerosis is a disease of the otic capsule. In its active phase, known as otospongiosis, highly vascular lesions resorb bone surrounding the inner ear, most often in a patchy pattern. The active lesions mature into calcified otosclerotic plaques which are responsible for stapes fixation.
  • #53 Otosclerosis is a treatable cause of hearing loss.
    https://lmhofmeyr.co.za/conditions/hearing/otosclerosis/
    Otosclerosis is a genetic localized bone disease that causes hearing loss. It occurs only in humans and affects the otic capsule (bony labyrinth) of the temporal bone and the ossicles. In this disorder, the normal mature lamellar bone is absorbed and replaced by woven and more porous less dense bone. Otospongiosis (softening) of bone is actually what occurs. […] Although otosclerosis is present in 10% of the population, only 1% of people will develop hearing loss. The development of hearing loss depends on the location of the osteosclerotic focus. It is mainly inherited in an autosomal dominant way with invariable penetrance, but other forms of inheritance are possible. Half of the patients with clinical otosclerosis have a positive family history. In 30% of cases, it can also arise sporadically due to non-genetic causes. The trigger that sets the development in these patients is unknown. Hormonal, metabolic, viral infections (measles, mumps, and rubella), vascular and autoimmune causes have been implicated.
  • #54 Otosclerosis – Wikipedia
    https://en.wikipedia.org/wiki/Otosclerosis
    Otosclerosis can be caused by both genetic and environmental factors, such as a viral infection (like measles). Ribonucleic acid of the measles virus has been found in stapes footplate in most patients with otosclerosis. Populations that have been vaccinated against measles had a significant reduction in otosclerosis. While the disease is considered to be hereditary, its penetrance and the degree of expression is so highly variable that it may be difficult to detect an inheritance pattern. Most of the implicated genes are transmitted in an autosomal dominant fashion. One genome-wide analysis associates otosclerosis with variation in the RELN gene. […] The pathophysiology of otosclerosis is complex. The key lesions of otosclerosis are multifocal areas of sclerosis within the endochondral temporal bone. These lesions share some characteristics with Paget’s Disease, but they are not thought to be otherwise related. Histopathological studies have all been done on cadaveric temporal bones, so only inferences can be made about progression of the disease histologically. It seems that the lesions go through an active „spongiotic” or hypervascular phase before developing into „sclerotic” phase lesions. There have been many genes and proteins identified that, when mutated, may lead to these lesions. Also there is mounting evidence that measles virus is present within the otosclerotic foci, implicating an infectious etiology (this has also been noted in Paget’s Disease).
  • #55 What Is Otosclerosis? Symptoms & Diagnosis| NIDCD
    https://www.nidcd.nih.gov/health/otosclerosis
    Otosclerosis is most often caused when one of the bones in the middle ear, the stapes, becomes stuck in place. When this bone is unable to vibrate, sound is unable to travel through the ear and hearing becomes impaired (see illustration). […] Why this happens is still unclear, but scientists think it could be related to a previous measles infection, stress fractures to the bony tissue surrounding the inner ear, or immune disorders. Otosclerosis also tends to run in families. […] It may also have to do with the interaction among three different immune-system cells known as cytokines. Researchers believe that the proper balance of these three substances is necessary for healthy bone remodeling and that an imbalance in their levels could cause the kind of abnormal remodeling that occurs in otosclerosis.
  • #56 Otosclerosis | Red River ENT Associates
    https://redriverent.com/ear/otosclerosis/
    Otosclerosis is an abnormal growth of bone in the middle ear that causes hearing loss. […] The exact cause of otosclerosis is not known, but evidence suggests a genetic link passed down from parent to child. Middle-aged Caucasian women are most at risk, and hormonal changes seem to be a contributing factor including pregnancy and menopause. […] This bone growth usually occurs around the stapes bone in the middle ear, preventing it from moving freely, essential to proper hearing.
  • #57
    https://mysecondear.lv/blogs/wissen/otosklerose
    Otosclerosis is a middle ear disease. In advanced stages, the disease can also spread to the inner ear. […] In otosclerosis, the metabolism of the bone tissue at the oval window is disturbed. Growths occur that restrict the ability to move. As a result, not every movement of the eardrum reaches our inner ear. This is how conductive hearing loss develops. […] Progression of the disease causes the ossicular chain to stiffen completely, which can then even lead to deafness. […] If there are multiple cases of otosclerosis in your family tree, the risk that you will also develop the disease increases. […] Typically in otosclerosis, there is a difference between these two hearing thresholds. The bone conduction threshold is at lower levels than the air conduction threshold. This is referred to as conductive hearing loss.
  • #58 Otosclerosis | Tampa Bay Hearing and Balance Center
    https://www.tampabayhearing.com/ear-education/auditory-education/otosclerosis/
    Otosclerosis may cause hearing loss in children and adults with an incidence of about 8 per 100,000 population per year. In otosclerosis, abnormal bone grows around the footplate of the stapes. […] The abnormal bone growth dampens the vibrations of the stapes bone, causing a hearing loss. The hearing loss type is called conductive because it affects the mechanism that conducts sound from the ear drum membrane to the inner ear. Sometimes, otosclerosis may also damage the sensory hair cells in the inner ear, causing sensorineural hearing loss. Sensorineural loss occurs in about 20-30% of patients that have otosclerosis. […] Otosclerosis seems to be an inherited problem that is triggered by a viral illness, perhaps the measles virus. While otosclerosis tends to run in families, most blood relatives are free of the disorder. Some research has shown that Caucasian middle-aged women are at most risk. Asians and people from the Indian subcontinent are more prone to develop the disease, as well. Pregnancy related hormone changes worsen pre-existing otosclerosis. Some other causes of stapes bone fixation may be corrected at least in part with surgery, such as osteogenesis imperfecta.
  • #59
    https://mysecondear.lv/blogs/wissen/otosklerose
    Otosclerosis is a middle ear disease. In advanced stages, the disease can also spread to the inner ear. […] In otosclerosis, the metabolism of the bone tissue at the oval window is disturbed. Growths occur that restrict the ability to move. As a result, not every movement of the eardrum reaches our inner ear. This is how conductive hearing loss develops. […] Progression of the disease causes the ossicular chain to stiffen completely, which can then even lead to deafness. […] If there are multiple cases of otosclerosis in your family tree, the risk that you will also develop the disease increases. […] Typically in otosclerosis, there is a difference between these two hearing thresholds. The bone conduction threshold is at lower levels than the air conduction threshold. This is referred to as conductive hearing loss.
  • #60 Otosclerosis – Balance & Dizziness Canada
    https://balanceanddizziness.org/disorders/vestibular-disorders/otosclerosis/
    Otosclerosis (oh-toe-skler-OH-suhs) a complex disorder of abnormal bone growth in the middle ear. It most often happens when the tiny stapes bone knits with surrounding bone. Otosclerosis usually results in slow, progressive conductive hearing loss. […] When the stapes is unable to vibrate, hearing becomes impaired. Soundwaves no longer move efficiently from the eardrum to the inner ear. Far advanced otosclerosis can cause dizziness and affect balance, likely when abnormal bone growth extends into the inner ear. […] Otosclerosis is one of the most common causes of hearing impairment in young adults. Most often, hearing loss is first noticed during the teens and twenties. […] Bone is living tissue that is continually being broken down and remade. In otosclerosis, the process for remodeling bone does not work properly and abnormal bone forms. Scientists do not fully understand why this happens.
  • #61 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    A common disease characterized by a disordered bone remodeling in the otic capsule, otosclerosis has a prevalence of about 0.3 percent to 0.4 percent in the Caucasian population. […] Although many environmental and genetic factors have been suggested for the development of otosclerosis, a great part of the etiology remains a mystery. […] The data suggest that measles virus infection is probably a susceptibility factor involved in otosclerosis but not the single cause or necessary for the disease to manifest. Endocrine factors could be involved because of the large difference in prevalence between males and females (1:2). […] In addition, epidemiological studies show otosclerosis associated with low fluoride content in the drinking water. […] Evidence for genetic variants in otosclerosis has been discovered in different parts of the spectrum, but the extent to which these contribute to the disease is unclear since only a limited number of genetic studies have been done.
  • #62 Otosclerosis is a treatable cause of hearing loss.
    https://lmhofmeyr.co.za/conditions/hearing/otosclerosis/
    Otosclerosis is a genetic localized bone disease that causes hearing loss. It occurs only in humans and affects the otic capsule (bony labyrinth) of the temporal bone and the ossicles. In this disorder, the normal mature lamellar bone is absorbed and replaced by woven and more porous less dense bone. Otospongiosis (softening) of bone is actually what occurs. […] Although otosclerosis is present in 10% of the population, only 1% of people will develop hearing loss. The development of hearing loss depends on the location of the osteosclerotic focus. It is mainly inherited in an autosomal dominant way with invariable penetrance, but other forms of inheritance are possible. Half of the patients with clinical otosclerosis have a positive family history. In 30% of cases, it can also arise sporadically due to non-genetic causes. The trigger that sets the development in these patients is unknown. Hormonal, metabolic, viral infections (measles, mumps, and rubella), vascular and autoimmune causes have been implicated.
  • #63 Otosclerosis | Ear Associates & Rehabilitation Services of San Jose California
    https://www.earassociates.com/conditions-otosclerosis-san-jose-ca.html
    Otosclerosis is a hereditary condition that causes the abnormal changes of the bone of the middle ear and inner ear. […] Otosclerosis is a hereditary condition with a highly variable gene frequency across the world. It is found on autopsy in 5-10 percent of Caucasians, in up to 25-30% of Southern Indians and up to 60-70% in Ethiopians. […] On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent. […] Some research suggests a relationship between otosclerosis and hormonal changes.
  • #64 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Otosclerosis is a pathological bone remodeling process in which the normal, dense endochondral bone of the otic capsule is replaced by one or more foci of irregularly laid spongy bone that subsequently hardens abnormally and impairs function; the condition most commonly involves the stapes region. […] The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. […] In more than half of patients presenting with otosclerosis, there is a family history of the condition. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. […] Otosclerosis is more common in patients of European or Indian ancestry.
  • #65 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    In more than half of patients presenting with otosclerosis, there is a family history of the condition. Additionally, patients with family histories of otosclerosis tend to demonstrate earlier symptom onset. Genetic studies show that an autosomal dominant mode of inheritance with reduced penetrance (40%) and variable expressivity is found in most cases. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. However, because of the elasticity that estrogens provide to ligaments, women seem to suffer less hearing loss than men with the same degree of otosclerosis. […] Otosclerosis is more common in patients of European or Indian ancestry. The condition is seen less frequently in individuals of African descent. […] Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear.
  • #66 Otosclerosis: Symptoms, Diagnosis and Treatment Options
    https://www.healthyhearing.com/report/53072-Otosclerosis
    Otosclerosis is a form of hearing loss that occurs due to abnormal bone growth in your middle ear. […] Often, its unknown why people develop the condition, says Dr. Kim. […] Some known risk factors of otosclerosis include: Genetics: If theres a family history of otosclerosis, youre at an increased risk of developing it, Dr. Kim notes. […] Pregnancy is considered an otosclerosis risk factor, according to the U.S. National Library of Medicine (NLM). […] White women are particularly at risk for otosclerosis, notes the NIDCD. The condition tends to start in your 20s and 30s, says Dr. Kim. […] Otosclerosis will still progress if you dont treat it, notes Dr. Kim.
  • #67 Otosclerosis causes | Otosclerosis childhood deafness treatment
    https://www.ndcs.org.uk/information-and-support/childhood-deafness/causes-of-deafness/otosclerosis/
    Otosclerosis can be a hereditary (genetic) disorder, but in many cases where there is no family history, it is thought to be caused by a virus. […] Otosclerosis usually develops between the ages of 15 to 35 but has been known to occur in younger children.
  • #68 Otosclerosis – UF Health
    https://ufhealth.org/conditions-and-treatments/otosclerosis
    Otosclerosis is an abnormal bone growth in the middle ear that causes hearing loss. […] The exact cause of otosclerosis is unknown. It may be passed down through families. […] Risks for this condition include pregnancy and a family history of hearing loss. White people are more likely to develop this condition than people of other races.
  • #69 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    In more than half of patients presenting with otosclerosis, there is a family history of the condition. Additionally, patients with family histories of otosclerosis tend to demonstrate earlier symptom onset. Genetic studies show that an autosomal dominant mode of inheritance with reduced penetrance (40%) and variable expressivity is found in most cases. […] The prevalence of otosclerosis is twice as great in women compared to men, suggesting the role of sex hormones. However, because of the elasticity that estrogens provide to ligaments, women seem to suffer less hearing loss than men with the same degree of otosclerosis. […] Otosclerosis is more common in patients of European or Indian ancestry. The condition is seen less frequently in individuals of African descent. […] Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear.
  • #70 Otosclerosis | Shohet Ear Associates | Audiologist, Hearing Aids in Orange County
    https://www.eardoctor.org/conditions-and-treatments/other-conditions/otosclerosis/
    The exact cause of otosclerosis is unknown but approximately 60 percent of otosclerosis cases have a genetic predisposition. […] On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent.
  • #71 Otosclerosis – Ear, Nose, and Throat Disorders – Merck Manual Consumer Version
    https://www.merckmanuals.com/home/ear-nose-and-throat-disorders/middle-ear-disorders/otosclerosis
    Otosclerosis causes abnormal bone to form and accumulate in the bones of the ear. The abnormal bone interferes with the transmission of sound from the middle ear to the inner ear. […] Otosclerosis tends to run in families, and half of all cases are inherited. Children have a 25% chance of developing otosclerosis if one parent has it and a 50% chance if both parents have it.
  • #72 Otosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK560671/
    Pregnancy appears to exacerbate hearing loss in osteosclerosis; however, the exact nature of the relationship between pregnancy and otosclerosis remains unclear. […] A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #73 What is Otosclerosis? | Barrow Neurological InstituteGroup 9Group 49Group 49
    https://www.barrowneuro.org/condition/otosclerosis/
    Otosclerosis is a condition in which bone tissue grows abnormally in the middle ear—usually around the smallest hearing bone, called the stapes. […] The condition is more common in women but does occur in men. It may have some link to hormone circulation in the body. As such, many female patients note that hearing loss caused by otosclerosis tends to occur during, or in the years immediately following, a pregnancy. In other cases no known trigger is discovered. […] Patients with otosclerosis typically do not have a history of other ear-related conditions, infections, or trauma. The hearing loss of otosclerosis can occur alone or be coupled with an unrelated nerve-hearing loss.
  • #74 Otosclerosis and Hearing Loss: Risk Factors, Symptoms, and Treatment
    https://www.webmd.com/cold-and-flu/ear-infection/otosclerosis-facts
    Otosclerosis affects more than 3 million Americans. Experts aren’t sure exactly what causes it. But they do know these risk factors may make you more likely to get it: […] Certain medical problems can raise your chances of otosclerosis. For example, if you had measles at any time, your risk may go up. Stress fractures to the bony tissue around your inner ear also might make it more likely to happen. And immune disorders, in which your immune system mistakenly attacks parts of your body, also can be linked to the condition.
  • #75 Otosclerosis | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/otosclerosis.html
    Measles infection: Viral illnesses, especially the measles, may play a role in otosclerosis. Researchers have found the measles virus in bone samples taken from the ears of people with otosclerosis. Studies have also reported that people vaccinated against the measles have lower rates of otosclerosis.
  • #76 Otosclerosis: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22033-otosclerosis
    Otosclerosis happens when irregular bone remodeling/growth occurs in your middle ear or, more rarely, your inner ear. […] Otosclerosis most often develops when the stapes bone (a small, triangular bone in your middle ear) fuses with the surrounding bone tissue. […] Possible risk factors for otosclerosis include: Family history. Youre more likely to develop otosclerosis if a parent, sibling or grandparent has it. […] Females are more prone to developing otosclerosis. […] Overall, white people have a higher chance of getting otosclerosis. […] People who are already prone to otosclerosis may develop the condition while theyre pregnant. […] Osteogenesis imperfecta (OI). Also known as brittle bone disease, OI increases your risk of otosclerosis.
  • #77 Otosclerosis | Tampa Bay Hearing and Balance Center
    https://www.tampabayhearing.com/ear-education/auditory-education/otosclerosis/
    Otosclerosis may cause hearing loss in children and adults with an incidence of about 8 per 100,000 population per year. In otosclerosis, abnormal bone grows around the footplate of the stapes. […] The abnormal bone growth dampens the vibrations of the stapes bone, causing a hearing loss. The hearing loss type is called conductive because it affects the mechanism that conducts sound from the ear drum membrane to the inner ear. Sometimes, otosclerosis may also damage the sensory hair cells in the inner ear, causing sensorineural hearing loss. Sensorineural loss occurs in about 20-30% of patients that have otosclerosis. […] Otosclerosis seems to be an inherited problem that is triggered by a viral illness, perhaps the measles virus. While otosclerosis tends to run in families, most blood relatives are free of the disorder. Some research has shown that Caucasian middle-aged women are at most risk. Asians and people from the Indian subcontinent are more prone to develop the disease, as well. Pregnancy related hormone changes worsen pre-existing otosclerosis. Some other causes of stapes bone fixation may be corrected at least in part with surgery, such as osteogenesis imperfecta.
  • #78 Otosclerosis and Hearing Loss: Risk Factors, Symptoms, and Treatment
    https://www.webmd.com/cold-and-flu/ear-infection/otosclerosis-facts
    Otosclerosis affects more than 3 million Americans. Experts aren’t sure exactly what causes it. But they do know these risk factors may make you more likely to get it: […] Certain medical problems can raise your chances of otosclerosis. For example, if you had measles at any time, your risk may go up. Stress fractures to the bony tissue around your inner ear also might make it more likely to happen. And immune disorders, in which your immune system mistakenly attacks parts of your body, also can be linked to the condition.
  • #79 Otosclerosis – Vestibular Disorders Association
    https://vestibular.org/article/diagnosis-treatment/types-of-vestibular-disorders/otosclerosis/
    Otosclerosis is defined by abnormal and unregulated growth of bone within the bony structures that make up the middle ear. […] There are a variety of factors that can lead to otosclerosis of bone. External environmental factors such as sodium fluoride, viral antigen exposure, and exogenous hormone intake have been shown to cause dysregulation of bone formation. […] Internal factors such as autoimmune/inflammatory diseases, hormone dysregulation, and genetic anomalies can also lead to otosclerosis. […] About 50-60% of patients suffering from otosclerosis have a genetic predisposition for the disease and a majority of patients report a family history of otosclerosis. […] Multiple genes have been identified and implicated in the incidence of otosclerosis emphasizing the importance of family history. Genome wide studies have identified 23 loci which are associated with an increased risk of otosclerosis.
  • #80 Otosclerosis – American Hearing Research Foundation
    https://www.american-hearing.org/disease/otosclerosis/
    While the medical and research communities understand what happens to the ear as a result of otosclerosis, what actually triggers the disorder remains uncertain. Current thinking leans toward the belief that there is likely an interplay among multiple factors and that the genetic component may not be enough to set the disease in motion. Many experts believe that otosclerosis must be triggered by a combination of genetic, environmental, hormonal, and/or other factors. […] Research suggests that otosclerosis may be linked in some way to the following: Genetics: Otosclerosis tends to run in families. Estimates vary, but many (perhaps up to 50%) of those with the disorder have a gene linked to it. […] Pregnancy: There appears to be a pattern in which women first show signs of otosclerosis during or immediately after pregnancy.
  • #81 Otosclerosis – American Hearing Research Foundation
    https://www.american-hearing.org/disease/otosclerosis/
    While the medical and research communities understand what happens to the ear as a result of otosclerosis, what actually triggers the disorder remains uncertain. Current thinking leans toward the belief that there is likely an interplay among multiple factors and that the genetic component may not be enough to set the disease in motion. Many experts believe that otosclerosis must be triggered by a combination of genetic, environmental, hormonal, and/or other factors. […] Research suggests that otosclerosis may be linked in some way to the following: Genetics: Otosclerosis tends to run in families. Estimates vary, but many (perhaps up to 50%) of those with the disorder have a gene linked to it. […] Pregnancy: There appears to be a pattern in which women first show signs of otosclerosis during or immediately after pregnancy.
  • #82 Otosclerosis: Practice Essentials, History of the Procedure, Epidemiology
    https://emedicine.medscape.com/article/859760-overview
    The exact cause of otosclerosis is unknown. Measles virus RNA is found in otosclerotic foci in footplates removed during surgery. Measles virus infection may activate the gene responsible for otosclerosis. Otosclerosis, however, is not responsible for all cases of stapes ankylosis. A heterogeneous group of disorders, including other bone degenerative disorders, appears to cause stapes fixation and conductive hearing loss. […] Evidence has recently mounted that the measles virus plays an important role in gene activation of otosclerosis. This hypothesis is supported by a declining incidence of otosclerosis since measles vaccinations became widespread as well as finding measles virus RNA in the footplate of otosclerosis specimens.
  • #83 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    A common disease characterized by a disordered bone remodeling in the otic capsule, otosclerosis has a prevalence of about 0.3 percent to 0.4 percent in the Caucasian population. […] Although many environmental and genetic factors have been suggested for the development of otosclerosis, a great part of the etiology remains a mystery. […] The data suggest that measles virus infection is probably a susceptibility factor involved in otosclerosis but not the single cause or necessary for the disease to manifest. Endocrine factors could be involved because of the large difference in prevalence between males and females (1:2). […] In addition, epidemiological studies show otosclerosis associated with low fluoride content in the drinking water. […] Evidence for genetic variants in otosclerosis has been discovered in different parts of the spectrum, but the extent to which these contribute to the disease is unclear since only a limited number of genetic studies have been done.
  • #84 Otosclerosis: Symptoms, Complications, and Treatment
    https://www.healthline.com/health/otosclerosis
    Otosclerosis is a condition of the middle ear that involves abnormal bone remodeling. Otosclerosis causes tissues to harden around three bones called the stapes, incus, and malleus. […] The exact cause is unknown, but early detection of otosclerosis is key to helping you access treatments that can improve your hearing. […] While certain types of infections, immune-mediated diseases, and stress fractures may be possible risk factors for developing otosclerosis, the exact cause of this ear condition isn’t clear. Scientists believe that otosclerosis may run in families. […] There’s also a theory and anecdotal reporting that pregnancy may increase the risk of otosclerosis. In fact, a 2020 review found that females with a higher number of children needed surgery of the stapes at a younger age overall. […] But the authors also note that there’s not enough evidence to draw a definitive link between pregnancy and otosclerosis. It’s also possible that hormone fluctuations in pregnancy could worsen pre-existing cases.
  • #85 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. In an open genome-wide analysis, a new locus on chromosome 7q22.1 has also been identified. Additionally, other bone metabolism abnormalities, such as osteogenesis imperfecta, and multiple genes associated with bone remodeling have been considered potential causes of otosclerosis, including type I collagen (COL1A1), TGF-beta 1 (BMP2 and BMP4), angiotensin II (AGT M235T and ACE I/D), autoimmune reactions, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone and expression of parathyroid hormone-related peptide receptors, and oxidative stress.
  • #86 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    Most cases of otosclerosis, however, do not have a clear familial background. […] The authors noted that recently they have been able to provide strong evidence for T cell receptor beta as the responsible gene at the OTSC2 region. […] The authors also discussed association studies for otosclerosis, including genes in the immune system, in bone remodeling and in hormonal and other pathways. […] Regarding otosclerosis as an autoimmune disease, the authors said the precise underlying mechanism might vary among otosclerosis patients as several factors can influence and contribute to a similar disease outcome and it is unclear whether an autoimmune pathology underlies disease development. […] Overall, studies suggest a heterogeneous etiology for otosclerosis with the extent of involvement of environmental versus genetic factors unclear.
  • #87 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    The etiopathogenesis of otosclerosis remains unclear. Some of the multiple hypotheses that have been postulated include: […] Many loci on chromosomes 6p, 9p, 1q, 3q, 6q, 7q, 15q, and 16q have been associated with otosclerosis. In an open genome-wide analysis, a new locus on chromosome 7q22.1 has also been identified. Additionally, other bone metabolism abnormalities, such as osteogenesis imperfecta, and multiple genes associated with bone remodeling have been considered potential causes of otosclerosis, including type I collagen (COL1A1), TGF-beta 1 (BMP2 and BMP4), angiotensin II (AGT M235T and ACE I/D), autoimmune reactions, human leucocyte antigen, inflammatory and regulatory cytokines, parathyroid hormone and expression of parathyroid hormone-related peptide receptors, and oxidative stress.
  • #88 Otosclerosis – Vestibular Disorders Association
    https://vestibular.org/article/diagnosis-treatment/types-of-vestibular-disorders/otosclerosis/
    Otosclerosis is defined by abnormal and unregulated growth of bone within the bony structures that make up the middle ear. […] There are a variety of factors that can lead to otosclerosis of bone. External environmental factors such as sodium fluoride, viral antigen exposure, and exogenous hormone intake have been shown to cause dysregulation of bone formation. […] Internal factors such as autoimmune/inflammatory diseases, hormone dysregulation, and genetic anomalies can also lead to otosclerosis. […] About 50-60% of patients suffering from otosclerosis have a genetic predisposition for the disease and a majority of patients report a family history of otosclerosis. […] Multiple genes have been identified and implicated in the incidence of otosclerosis emphasizing the importance of family history. Genome wide studies have identified 23 loci which are associated with an increased risk of otosclerosis.
  • #89 Otosclerosis | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26432
    A role for measles virus infection in the pathogenesis of otosclerosis has been postulated. Measles virus ribonucleic acid has been detected in the footplate of the stapes on electron microscopy and immunohistochemical studies in 59%-100% of patients. Moreover, vaccination against the measles virus appears to be a protective factor against otosclerosis: in 2007, Arnold et al demonstrated a ~25% lower rate of treatment for otosclerosis in Germany for the population vaccinated against measles compared with patients who were not. […] In addition to the above factors, menopause, trauma, and major surgery have been reported as either causing or aggravating otosclerosis.
  • #90 What Is Otosclerosis? Symptoms & Diagnosis| NIDCD
    https://www.nidcd.nih.gov/health/otosclerosis
    Otosclerosis is most often caused when one of the bones in the middle ear, the stapes, becomes stuck in place. When this bone is unable to vibrate, sound is unable to travel through the ear and hearing becomes impaired (see illustration). […] Why this happens is still unclear, but scientists think it could be related to a previous measles infection, stress fractures to the bony tissue surrounding the inner ear, or immune disorders. Otosclerosis also tends to run in families. […] It may also have to do with the interaction among three different immune-system cells known as cytokines. Researchers believe that the proper balance of these three substances is necessary for healthy bone remodeling and that an imbalance in their levels could cause the kind of abnormal remodeling that occurs in otosclerosis.
  • #91 Understanding Otosclerosis Etiology and Impacting Factors – ENTtoday
    https://www.enttoday.org/article/understanding-otosclerosis-etiology-and-impacting-factors/
    A common disease characterized by a disordered bone remodeling in the otic capsule, otosclerosis has a prevalence of about 0.3 percent to 0.4 percent in the Caucasian population. […] Although many environmental and genetic factors have been suggested for the development of otosclerosis, a great part of the etiology remains a mystery. […] The data suggest that measles virus infection is probably a susceptibility factor involved in otosclerosis but not the single cause or necessary for the disease to manifest. Endocrine factors could be involved because of the large difference in prevalence between males and females (1:2). […] In addition, epidemiological studies show otosclerosis associated with low fluoride content in the drinking water. […] Evidence for genetic variants in otosclerosis has been discovered in different parts of the spectrum, but the extent to which these contribute to the disease is unclear since only a limited number of genetic studies have been done.
  • #92 Otosclerosis | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/ear-nose-and-throat/otosclerosis.html
    Measles infection: Viral illnesses, especially the measles, may play a role in otosclerosis. Researchers have found the measles virus in bone samples taken from the ears of people with otosclerosis. Studies have also reported that people vaccinated against the measles have lower rates of otosclerosis.
  • #93 Otosclerosis – Vestibular Disorders Association
    https://vestibular.org/article/diagnosis-treatment/types-of-vestibular-disorders/otosclerosis/
    Otosclerosis is defined by abnormal and unregulated growth of bone within the bony structures that make up the middle ear. […] There are a variety of factors that can lead to otosclerosis of bone. External environmental factors such as sodium fluoride, viral antigen exposure, and exogenous hormone intake have been shown to cause dysregulation of bone formation. […] Internal factors such as autoimmune/inflammatory diseases, hormone dysregulation, and genetic anomalies can also lead to otosclerosis. […] About 50-60% of patients suffering from otosclerosis have a genetic predisposition for the disease and a majority of patients report a family history of otosclerosis. […] Multiple genes have been identified and implicated in the incidence of otosclerosis emphasizing the importance of family history. Genome wide studies have identified 23 loci which are associated with an increased risk of otosclerosis.