Ślepogłuchota
Etiologia i przyczyny

Ślepogłuchota to złożona niepełnosprawność sensoryczna charakteryzująca się jednoczesnym uszkodzeniem słuchu i wzroku, które nie jest prostą sumą obu deficytów, lecz stanowi odrębny stan o poważniejszych konsekwencjach funkcjonalnych. Wyróżnia się ślepogłuchotę wrodzoną (kongenitalną) oraz nabytą, z licznymi wariantami pośrednimi. Etiologia jest heterogeniczna i obejmuje ponad 70 przyczyn, z których najczęstsze to powikłania wcześniactwa (urodzenie przed 37. tygodniem ciąży) oraz zespół CHARGE, odpowiadające za około 10% przypadków każda. Wcześniactwo zwiększa ryzyko retinopatii wcześniaków (ROP) i uszkodzenia słuchu. Zespół CHARGE, o częstości 1:10 000 urodzeń, manifestuje się m.in. wadami oka, serca, atrezją nozdrzy, opóźnieniem wzrostu, nieprawidłowościami narządów płciowych i ucha, często prowadząc do utraty słuchu i wzroku. Infekcje wewnątrzmaciczne, takie jak różyczka wrodzona, cytomegalowirus (CMV) i toksoplazmoza, również stanowią istotne przyczyny ślepogłuchoty wrodzonej. Ponadto, liczne zespoły genetyczne (np. zespół Downa, Goldenhara, Alströma, Sticklera) oraz czynniki ryzyka jak mózgowe porażenie dziecięce, alkoholowy zespół płodowy, wodogłowie i mikroencefalia mogą prowadzić do tego stanu.

Etiologia ślepogłuchoty

Ślepogłuchota (ang. deafblindness) to unikalna i złożona niepełnosprawność sensoryczna, charakteryzująca się jednoczesnym występowaniem uszkodzenia słuchu i wzroku w różnym stopniu nasilenia. Kombinacja tych dwóch deficytów sensorycznych powoduje znaczące trudności w komunikacji, dostępie do informacji, orientacji przestrzennej i wykonywaniu codziennych czynności życiowych. Należy podkreślić, że ślepogłuchota nie jest prostą sumą głuchoty i ślepoty, ale stanowi odrębną niepełnosprawność o charakterze mnożnym, której konsekwencje są znacznie poważniejsze niż suma problemów wynikających z izolowanych uszkodzeń poszczególnych zmysłów.12

Badania wykazały, że około 0,2% światowej populacji cierpi na ciężką ślepogłuchotę, a kolejne 2% doświadcza łagodniejszych form tej niepełnosprawności. W Stanach Zjednoczonych zidentyfikowano około 45 000-50 000 osób ze ślepogłuchotą, w tym ponad 11 000 to dzieci poniżej 21 roku życia.12

Klasyfikacja ślepogłuchoty

Ze względu na moment wystąpienia deficytów sensorycznych, ślepogłuchotę klasyfikuje się na dwa główne typy:12

  1. Ślepogłuchota wrodzona (kongenitalna) – występuje od urodzenia lub pojawia się we wczesnym dzieciństwie, przed rozwojem mowy (zazwyczaj przed 2 rokiem życia).
  2. Ślepogłuchota nabyta – rozwija się w późniejszym okresie życia, po opanowaniu języka, stopniowo lub nagle.

Dodatkowo, można wyróżnić pośrednie warianty:12

  • Osoby urodzone z uszkodzeniem słuchu, które później tracą wzrok
  • Osoby urodzone z uszkodzeniem wzroku, które później tracą słuch
  • Ślepogłuchota związana z wiekiem – najczęstsza postać ślepogłuchoty nabytej

Przyczyny ślepogłuchoty wrodzonej

W Narodowym Spisie Dzieci ze Ślepogłuchotą (National Deaf-Blind Child Count) zidentyfikowano ponad 70 różnych przyczyn (etiologii) ślepogłuchoty. Najczęstsze z nich to powikłania wcześniactwa oraz zespół CHARGE, z których każda odpowiada za około 10% wszystkich przypadków.12

Powikłania wcześniactwa

Wcześniactwo (urodzenie przed 37. tygodniem ciąży) stanowi jeden z głównych czynników ryzyka rozwoju ślepogłuchoty wrodzonej. U wcześniaków występuje zwiększone ryzyko retinopatii wcześniaków (ROP) oraz uszkodzenia słuchu z powodu niedorozwoju układów wzrokowego i słuchowego. Wcześniactwo nadal pozostaje najczęściej identyfikowaną etiologią ślepogłuchoty.123

Zespół CHARGE

Zespół CHARGE to złożony zespół genetyczny występujący u około 1 na 10 000 urodzeń na całym świecie. Nazwa stanowi akronim od angielskich określeń głównych objawów: Coloboma (wady oka dotyczące nerwu wzrokowego), Heart defects (wady serca), Atresia choanae (atrezja nozdrzy tylnych), Retarded growth (opóźnienie wzrostu), Genital abnormalities (nieprawidłowości narządów płciowych) oraz Ear abnormalities (nieprawidłowości ucha).12

Większość osób z zespołem CHARGE ma utratę słuchu, utratę wzroku i problemy z równowagą. Częstość występowania tego zespołu wzrasta, a w 2018 roku zidentyfikowano 933 dzieci z zespołem CHARGE w Stanach Zjednoczonych. Dokładna przyczyna zespołu CHARGE nadal nie jest w pełni poznana, ale badania sugerują, że różne czynniki mogą wywoływać nieprawidłowości. Istnieją pewne dowody na dziedziczenie cech zespołu CHARGE, a także pewne dowody wskazujące na delecję chromosomową w niektórych przypadkach.12

Zakażenia wrodzone

Infekcje wewnątrzmaciczne mogą być przyczyną ślepogłuchoty wrodzonej. Najważniejsze z nich to:

  • Różyczka wrodzona (Congenital Rubella Syndrome, CRS) – występuje, gdy kobieta w ciąży zarazi się różyczką (German measles) podczas pierwszego trymestru, co może prowadzić do urodzenia dziecka z głuchotą, ślepotą, problemami kardiologicznymi i innymi wadami wrodzonymi. Różyczka była kiedyś główną przyczyną ślepogłuchoty na świecie, ale dostępność szczepionki MMR od 1971 roku spowodowała drastyczny spadek liczby dzieci rodzących się z tym zespołem.12
  • Cytomegalowirus (CMV) – podobnie jak wirus różyczki, może przekraczać barierę łożyskową i wpływać na rozwijający się płód. Tylko pierwotne zakażenie matki podczas ciąży może powodować problemy. Rzadko zdarza się, by reaktywacja CMV w ciąży spowodowała uszkodzenia płodu.12
  • Toksoplazmoza – może zainfekować niemal wszystkie zwierzęta, w tym ludzi. U ciężarnej kobiety infekcja, choć nie stanowi dla niej ryzyka, może spowodować wrodzone nieprawidłowości u nienarodzonego dziecka w do 40% przypadków zakażeń. Niemowlęta urodzone z toksoplazmozą (zwykle zakażone między trzecim a szóstym miesiącem) mogą rozwinąć poważne objawy, takie jak wodogłowie, zwapnienia w mózgu i zapalenie siatkówki i naczyniówki. Może również wystąpić padaczka i głuchota.12

Zaburzenia genetyczne i zespoły wrodzone

Liczne zaburzenia genetyczne i chromosomowe mogą prowadzić do ślepogłuchoty, w tym:12

  • Zespół Downa (Trisomia 21) – charakteryzuje się zwiększonym ryzykiem problemów ze słuchem i wzrokiem oraz różnymi wadami wrodzonymi. Utrata słuchu może być czuciowo-nerwowa (wynikająca ze zmian w uchu wewnętrznym) lub przewodzeniowa (spowodowana nieprawidłowościami ucha środkowego).1
  • Zespół Goldenhara – występuje częściej u mężczyzn (70%) i charakteryzuje się asymetrycznymi, małymi uszami i ustami z hipoplazją szczęki, ust i oczu. Podstawowe cechy to asymetria twarzy, częściowo uformowane lub całkowicie nieobecne ucho, niezłośliwe guzy oka i nieprawidłowości kręgosłupa.12
  • Zespół Alströma – jest dziedziczony autosomalnie recesywnie. Oznacza to, że dziecko musi odziedziczyć po jednym zmutowanym genie od każdego z rodziców, aby rozwinąć ten stan. Został zmapowany do chromosomu 2p13, ale gen odpowiedzialny za to zaburzenie nie został zidentyfikowany.1
  • Zespół Stickler i zespół Dandy-Walker również stają się coraz częstszymi przyczynami ślepogłuchoty.1

Inne przyczyny ślepogłuchoty wrodzonej

Inne istotne czynniki ryzyka ślepogłuchoty wrodzonej obejmują:12

  • Mózgowe porażenie dziecięce – problem dotyczący mózgu i układu nerwowego, który głównie wpływa na ruch i koordynację, ale może również prowadzić do uszkodzeń sensorycznych, w tym słuchu i wzroku
  • Alkoholowy zespół płodowy (FAS) – problemy zdrowotne spowodowane spożywaniem alkoholu przez matkę w czasie ciąży
  • Wodogłowie – wysokie ciśnienie płynu mózgowo-rdzeniowego może uszkodzić wzrok z powodu nacisku na nerw wzrokowy lub inne nerwy. Może również powodować utratę słuchu, co jest prawdopodobnie bezpośrednim skutkiem wodogłowia lub efektem ubocznym chirurgicznego założenia zastawki.1
  • Mikroencefalia – objawy związane z mikroencefalią u niemowląt zależą od nasilenia stanu i obejmują drgawki, opóźnienie rozwoju, niepełnosprawność intelektualną, problemy z ruchem i równowagą, problemy z karmieniem oraz utratę wzroku i słuchu.1

Przyczyny ślepogłuchoty nabytej

W większości przypadków ślepogłuchota rozwija się później w życiu. Jest to tzw. ślepogłuchota nabyta. Osoba z nabytą ślepogłuchotą może urodzić się bez problemu ze słuchem lub wzrokiem, a następnie stracić częściowo lub całkowicie oba zmysły. Alternatywnie, ktoś może urodzić się z wadą słuchu lub wzroku, a następnie później w życiu stracić częściowo lub całkowicie drugi zmysł.1

Zespół Ushera

Zespół Ushera jest najczęstszą genetyczną przyczyną ślepogłuchoty, odpowiadającą za około 50% wszystkich przypadków ślepogłuchoty w Stanach Zjednoczonych u osób poniżej 60 roku życia.12

Zespół Ushera to stan charakteryzujący się częściową lub całkowitą utratą słuchu i utratą wzroku, która pogarsza się z czasem. Zazwyczaj osoba z zespołem Ushera rodzi się głucha, niedosłysząca lub z normalnym słuchem, a później doświadcza progresywnej utraty wzroku z powodu zwyrodnienia barwnikowego siatkówki (retinitis pigmentosa).12

Istnieją trzy typy zespołu Ushera:1

  • Usher 1 – osoba rodzi się głucha i zaczyna tracić wzrok zwykle w wieku nastoletnim
  • Usher 2 – osoba rodzi się niedosłysząca i zaczyna tracić wzrok później
  • Usher 3 – osoba zwykle rodzi się z normalnym wzrokiem i słuchem lub z łagodnym niedosłuchem i zaczyna tracić oba zmysły później w życiu

Naukowcy zidentyfikowali ponad 10 różnych genów, które mogą powodować zespół Ushera. Każda osoba dziedziczy dwie kopie każdego genu, po jednej od każdego rodzica. Błąd (mutacja genu) w obu kopiach któregokolwiek z tych 10 genów oznacza, że prawidłowe białko potrzebne w komórkach ucha wewnętrznego i siatkówki nie jest wytwarzane w organizmie.1

Problemy związane z wiekiem

Ślepogłuchota związana z wiekiem jest najczęstszą formą nabytej ślepogłuchoty. Częstość występowania ślepogłuchoty nabytej jest wyższa w starszym wieku (częstość występowania około 30% w wieku 80 lat). Problemy, które mogą przyczynić się do ślepogłuchoty związanej z wiekiem, obejmują:12

  • Utrata słuchu związana z wiekiem (presbycusis) – stopniowa utrata słuchu w obu uszach, powszechny problem związany ze starzeniem się. Jeden na trzech dorosłych powyżej 65 roku życia ma utratę słuchu.1
  • Problemy z oczami związane z wiekiem, takie jak:
    • Zwyrodnienie plamki żółtej związane z wiekiem (AMD)
    • Zaćma
    • Jaskra
  • Retinopatia cukrzycowa – powikłanie cukrzycy, w którym komórki wyściełające tylną część oka są uszkodzone przez wysoki poziom cukru we krwi1

Uszkodzenia mózgu i inne przyczyny nabyte

Uszkodzenie mózgu może prowadzić do ślepogłuchoty nabytej, na przykład w wyniku:12

  • Zapalenie opon mózgowo-rdzeniowych – infekcja może uszkodzić zarówno układ słuchowy, jak i wzrokowy, prowadząc do ślepogłuchoty
  • Zapalenie mózgu
  • Udar mózgu
  • Poważny uraz głowy – ciężkie urazy głowy mogą spowodować uszkodzenie części mózgu odpowiedzialnych za słuch i wzrok

Inne przyczyny ślepogłuchoty nabytej obejmują:12

  • Leki – niektóre leki, takie jak określone antybiotyki lub leki przeciwnowotworowe, zwłaszcza stosowane w wysokich dawkach lub przez długi czas, mogą powodować uszkodzenia słuchu i wzroku
  • Cukrzyca – długotrwała cukrzyca może prowadzić zarówno do retinopatii cukrzycowej (wpływającej na wzrok), jak i neuropatii (wpływającej na słuch)
  • Zaburzenia neurologiczne – stany takie jak stwardnienie rozsiane mogą wpływać na funkcje sensoryczne, potencjalnie prowadząc do kombinowanej utraty słuchu i wzroku
  • Narażenie na toksyny – długotrwałe narażenie na określone chemikalia lub toksyny środowiskowe może skutkować zaburzeniami sensorycznymi

Heterogeniczność ślepogłuchoty

Nie można przecenić heterogenicznego charakteru populacji osób ze ślepogłuchotą. Etiologie lub przyczyny ślepogłuchoty wiążą się z unikalnymi problemami i wyzwaniami. Niektóre są stosunkowo częstsze, takie jak zespół CHARGE, zespół Downa, zespół Ushera i Cytomegalowirus. Niektóre są bardzo rzadkie, z mniej niż pięcioma osobami zgłaszanymi na poziomie krajowym, takie jak choroba Battena, zespół Pradera-Williego i zespół Kearnsa-Sayre’a.1

Heterogeniczność populacji sprawia, że trudno jest badać osoby ze ślepogłuchotą jako jedną grupę. Różne poziomy utraty wzroku i słuchu, różne wykorzystanie modalności językowej, różne rodzaje i nasilenie dodatkowych niepełnosprawności oraz różna etiologia medyczna to niektóre zmienne dzielące grupę.1

Rozkład epidemiologiczny przyczyn ślepogłuchoty

Według danych z 2018 roku z National Deaf-Blind Child Count, rozkład etiologii ślepogłuchoty przedstawia się następująco:1

  • 44,8% – zespoły i zaburzenia dziedziczne (zwiększenie z 3 945 do 4 482 przypadków w ciągu pięciu lat)
  • 13,9% – powikłania wrodzone prenatalne
  • 11,6% – powikłania postnatalne nabyte
  • 10,5% – powikłania związane z wcześniactwem
  • 19,2% – brak zidentyfikowanej etiologii w momencie zbierania danych

Według innych źródeł, etiologie ślepogłuchoty u osób w różnym wieku obejmują zakażenia prenatalne, wcześniactwo, przyczyny związane z wiekiem i przyczyny genetyczne, przy czym przyczyny dziedziczne odpowiadają za około 27% przypadków.1

Diagnostyka i wczesna interwencja

Wczesna identyfikacja jest kluczowa dla zapewnienia interwencji i wsparcia dzieciom i rodzinom z kombinowaną utratą słuchu i wzroku. Z perspektywy medyczno-rozwojowej najważniejszymi aspektami ślepogłuchoty są wiek wystąpienia oraz nasilenie uszkodzeń słuchu i wzroku.1

W konsekwencji, im wcześniej wystąpią uszkodzenia słuchu i wzroku oraz im poważniejszy jest poziom każdego uszkodzenia, tym większe są konsekwencje dla rozwoju. Ślepogłuchota prawdopodobnie wpłynie na rozwój poznawczy, językowy, społeczny i emocjonalny.1

Zrozumienie przyczyny ślepogłuchoty u danej osoby często wymaga szczegółowej historii medycznej, badań genetycznych i dokładnego badania przez specjalistów. Wiedza o tym, co dana osoba jest w stanie zobaczyć lub usłyszeć, jest niezbędna do nawiązania jasnej komunikacji, zapewnienia bezpiecznej mobilności, oceny potrzeb wsparcia i wyznaczania rozsądnych celów w zakresie zatrudnienia, warunków życia, transportu i wielu innych aspektów życia.12

Korzystne dla rozwoju niemowląt ze ślepogłuchotą jest wczesne zdiagnozowanie i rozpoczęcie stymulacji odpowiedniej do wieku. Czynniki środowiskowe odgrywają zasadniczą rolę w określaniu późniejszych wyników u dzieci. Jest to szczególnie ważne w przypadku dzieci ze ślepogłuchotą.1

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Deaf-blindness | Causes, Treatment & Support | Britannica
    https://www.britannica.com/science/deaf-blindness
    deaf-blindness, disability in which an individual has both a hearing impairment and a visual impairment. Deaf-blind individuals form a highly heterogeneous group, in which hearing and visual impairments are expressed to varying degrees. […] The causes of deaf-blindness vary greatly among the population of deaf-blind individuals. A genetic syndrome known as Usher syndrome is the most frequent genetic cause of deaf-blindness. However, other genetic syndromes, such as CHARGE syndrome and Goldenhar syndrome, can also cause the condition. Other causes include illnesses or diseases of the pregnant mother or her child (e.g., rubella, meningitis, cytomegalovirus, and tumours) or accidents (e.g., head injury). A combination of any of the causes mentioned above is also possible. For example, an individual may be born deaf because of a genetic syndrome and may later lose vision as a result of an accident or illness. Deaf-blindness is also associated with premature birth.
  • #1 Understanding deafblindness – Sense International
    https://www.senseinternational.org.uk/our-work/understanding-deafblindness/
    Research has shown that 0.2% of the world’s population is living with severe deafblindness. A further 2% of people around the world are living with milder forms of deafblindness. […] There are many causes of deafblindness and multi-sensory impairment, including: Congenital Rubella Syndrome – where a mother contracts rubella during pregnancy and her unborn child suffers birth defects, such as vision and hearing impairments. Genetic conditions – these include Usher syndrome, CHARGE syndrome, and Down Syndrome. Medical complications during pregnancy and birth – including poor nutrition and trauma. Premature birth. Illness and accidents – including meningitis and cerebral malaria. Sensory loss as a result of ageing. […] According to the World Health Organisation (WHO), rubella is the leading cause of birth defects that can be prevented by vaccination.
  • #1 Deafblindness – Wikipedia
    https://en.wikipedia.org/wiki/Deafblindness
    Deafblindness is the condition of little or no useful hearing and little or no useful sight. Different degrees of vision loss and auditory loss occur within each individual. […] The medical condition of deafblindness occurs in different forms. For some, this condition might happen congenitally from birth as a result of genetic defect, for others it happens suddenly due to a form of illness or accident that results in a modality deprivation of either vision or hearing, or both. A person might be born deaf and become blind at a later stage in life, or vice versa. In any given case of deafblindness, many possible onsets and causes of this condition exist; some happen gradually, others happen unexpectedly and suddenly. The diagnosis of deafblindness could be medically classified into specific types based on one’s symptoms and causes. […] The two overarching types of deafblindness are congenital and acquired. […] Congenital deafblindness: the condition of deafblindness from birth. […] Acquired deafblindness: condition of deafblindness developed later in life.
  • #1 Unit 5: Deaf-blindness
    https://specialeducationnotes.co.in/B7unit5.htm
    Deafblindness is the combination of significant auditory and visual impairments in a person. These dual sensory losses vary in severity from person to person and do not necessarily lead to total deafness and/or total blindness. […] Deafblindness is a unique disability; it has its own concepts and terminology, its own methods of assessment and means of education, and its own modes of communication, which distinguishes deafblindness from deafness and blindness. […] Deafblindness is not a medical concept, surprisingly; medical literature makes few references to deafblindness. It is a developmental concept which helps us to understand the nature and the extent of a disability consequent to deafblindness. […] Deafblind people fall into four groups: 1. Those who are born deaf and blind, which can happen if the mother, inter alia, contacted Rubella (German Measles) during pregnancy. 2. Those who were born deaf and then lost their sight. This is often caused by the Usher Syndrome deafness followed by a decrease in sight because of retinitis pigmentosa (tunnel vision). 3. Those who were born blind and then lost their hearing. 4. The adventitious deafblind, as a result of old age, or through an illness or accident later in life.
  • #1 Causes of Deafblindness | National Center on Deafblindness
    https://www.nationaldb.org/info-center/overview/causes/
    More than 70 causes (also known as etiologies) of deafblindness are identified in National Deaf-Blind Child Count. The most common are complications of prematurity and CHARGE syndrome, with each causing approximately 10% of cases. […] Usher syndrome is a condition characterized by partial or total hearing loss and vision loss that worsens over time. […] Individuals with Down syndrome have an increased risk of hearing and vision problems and a variety of birth defects. […] The hearing loss may be sensorineural, meaning that it results from changes in the inner ear, or conductive, meaning that it is caused by abnormalities of the middle ear. […] The primary characteristics are facial asymmetry, a partially formed or totally absent ear, noncancerous growths of the eye, and spinal abnormalities.
  • #1
    https://www.nhs.uk/conditions/deafblindness/causes/
    There are many possible causes of deafblindness. The condition can either be present at birth or develop later in life. […] Deafblindness from birth is known as congenital deafblindness. […] It can be caused by: problems associated with premature birth (birth before 37 weeks of pregnancy), an infection in a baby in the womb, such as rubella (german measles), toxoplasmosis or cytomegalovirus (CMV), genetic conditions, such as Down’s syndrome or CHARGE syndrome, cerebral palsy a problem with the brain and nervous system that mainly affects movement and co-ordination, foetal alcohol spectrum disorder health problems caused by drinking alcohol in pregnancy. […] In most cases, deafblindness develops later in life. This is known as acquired deafblindness. […] Problems that can contribute to acquired deafblindness include: age-related hearing loss, Usher syndrome, a genetic condition that affects hearing, vision and balance, eye problems associated with increasing age, such as age-related macular degeneration (AMD), cataracts and glaucoma, diabetic retinopathy a complication of diabetes where the cells lining the back of the eye are damaged by high blood sugar levels, damage to the brain, such as from meningitis, encephalitis, a stroke or severe head injury.
  • #1 Deaf-blindness causes complex problems – Bonner General Health
    https://bonnergeneral.org/deaf-blindness-causes-complex-problems/
    Today, theyve identified more than 70 causes of deaf-blindness. The most common of which are complications of premature births and CHARGE syndrome. CHARGE is an acronym for several features common in disorder: Coloboma (an eye abnormality affecting the optic nerve); Heart defects; Atresia choanae (a congenital disorder where the back of the nasal passage is blocked); Growth retardation; genital abnormalities, and Ear abnormalities. […] Other conditions such as Usher syndrome, Down syndrome, stickler syndrome, dandy-walker syndrome, Goldenhar syndrome, Zellweger spectrum disorder, and Cornelia de Lange syndrome can be causes of deaf-blindness. Prenatal and congenital complications such as very common cytomegalovirus, hydrocephalus, and microcephaly (we heard a lot about this condition when a babys head is much smaller than normal during the Zika virus outbreak),
  • #1 Encyclopedia of deafblindness – Deafblind International DbI
    https://www.deafblindinternational.org/encyclopedia-of-deafblindness/
    The exact cause of CHARGE Association is still unknown but research suggests a variety of catalysts may induce the irregularities present in the CHARGE sequence of anomalies. […] There is some evidence in the literature of characteristics of CHARGE being inherited, and also some evidence to suggest chromosome deletion in some cases. […] It seems likely that multiple genetic and environmental factors play a role in the aetiogenesis of the CHARGE Association. […] Toxoplasmosis can affect almost all animals, including humans, but most animals carry it in their bodies. […] However, in a pregnant woman the infection, although not a risk to her, can cause congenic abnormality in the unborn child in up to 40% of infections. […] If the disease is caught in early pregnancy it is less likely to cross the placenta to the foetus but, if it does, the effects are more serious.
  • #1 Causes & Conditions – Anne Sullivan Centre for People who are Deafblind
    https://www.annesullivan.ie/advice-information/causes-conditions/
    Over 80 different causes and conditions of deafblindness have been identified and are listed at the end of this page. Some of the most common causes are: Age related deafblindness, Congenital Rubella Syndrome (CRS), CHARGE Syndrome, Usher Syndrome. […] Congenital Rubella Syndrome was once the leading cause of deafblindness around the world but the availability of the MMR vaccine, since 1971 onwards, has resulted in a dramatic decrease in the number of children being born with CRS. […] CHARGE is a rare condition that can affect different parts of the body. It estimated that 4 6% of the deafblind population are deafblind as a result of CHARGE Syndrome. […] Usher Syndrome is now the most common cause of deafblindness worldwide, after age-related deafblindness. Typically, a person with Usher Syndrome is born Deaf or Hard of Hearing and experiences progressive sight loss due to Retinitis Pigmentosa (RP).
  • #1 Encyclopedia of deafblindness – Deafblind International DbI
    https://www.deafblindinternational.org/encyclopedia-of-deafblindness/
    Various syndromes and congenital conditions affect both the eye and the ear. This can be attributed in part to the development in the embryo of the eye and ear during the same time period, during the first 12 weeks of pregnancy. There are also similarities between the two organs, such as their common origin. As a result there are many diseases and symptom complexes which affect both sense organs and result in dual sensory impairment or deafblindness. It follows that prenatal causes of deafness may be associated with visual impairment whereas with postnatal causes there will be hardly any association. […] Prenatal causes of deafblindness include rubella, cytomegalovirus and toxoplasmosis. […] Cytomegalovirus or CMV is a potential prenatal cause of deafblindness. […] CMV, like the rubella virus, can cross the placenta and affect the developing foetus. It is only the first, or primary infection of a person during pregnancy which can cause problems. It is very rare that reactivation of CMV in pregnancy causes damage to the foetus.
  • #1 Encyclopedia of deafblindness – Deafblind International DbI
    https://www.deafblindinternational.org/encyclopedia-of-deafblindness/
    Babies that are born with toxoplasmosis (usually when infected between the third and sixth month) may develop severe symptoms. These include hydrocephalus, calcification in the brain and chorioretinitis (damage to the retina). Epilepsy and deafness can also result. […] Goldenhar is prevalent in males (70%) and affected individuals may have asymmetrical small ears and mouth with hypoplasia of the jaw, mouth and eyes (with epibular dermoids). […] There is very little evidence to explain why Goldenhar Syndrome occurs. In most cases, Goldenhar Syndrome appears to occur randomly, with no apparent cause; nothing similar has ever happened in the family before, and there is little chance of it happening again. […] Alstrm Syndrome is autosomal recessive and even though it has been mapped to chromosome 2p13, the gene responsible for the disorder has not been identified. […] Alstrm syndrome is autosomal recessive which means that for a child to have the condition they must inherit one mutated gene from their mother and one from their father.
  • #1 Etiologies | National Center on Deafblindness
    https://www.nationaldb.org/products/national-child-count/report-2018/etiologies/
    Over 70 specific etiologies are identified in the 2018 National Deaf-Blind Child Count. Some are exceedingly rare, with less than five cases. Others are much more prevalent. There are 12 etiologies with at least 100 cases. […] Complications from prematurity continues to be the most commonly identified etiology. The prevalence of CHARGE syndrome continues to increase. In 2018 there were 933 children and youth identified as having CHARGE syndrome. While still relatively rare, the prevalence of children with Stickler syndrome and Dandy Walker syndrome has also increased. About 19% of the children and youth have no determined etiology. […] Overall, the total number of children and youth identified with a prenatal congenital or postnatal non-congenital etiology has declined from 2,732 to 2,551 over the past five years, while the number with hereditary syndromes and disorders has increased from 3,945 to 4,482. Hereditary syndrome and disorders now account for 44.8% of all identified etiologies. Prenatal congenital complications account for 13.9% of identified etiologies and postnatal non-congenital complications account for 11.6% of identified etiologies. Complications from prematurity account for 10.5% of identified etiologies and 19.2% of the children and youth had no identified etiology at the time data was collected.
  • #1 Causes of Deafblindness | National Center on Deafblindness
    https://www.nationaldb.org/info-center/overview/causes/
    The most common long-term issue is hearing loss, which may be present at birth or develop later in childhood. […] High CSF pressure can damage vision due to pressure on the optic nerve or other nerves. It may also cause hearing loss, believed to be due directly to hydrocephalus or as a side effect of surgical shunt placement. […] Signs and symptoms associated with microcephaly in infants depend on the severity of the condition and include seizures, developmental delay, intellectual disability, problems with movement and balance, feeding problems, and vision and hearing loss.
  • #1 Deafblindness | NHS inform
    https://www.nhsinform.scot/illnesses-and-conditions/eyes/deafblindness/
    In most cases, deafblindness develops later in life. This is known as acquired deafblindness. A person with acquired deafblindness may be born without a hearing or sight problem and then later loses part or all of both senses. Alternatively, someone may be born with either a hearing or vision problem, and then later loses part or all of the other sense later on. […] Problems that can contribute to acquired deafblindness include: age-related hearing loss, Usher syndrome a genetic condition that affects hearing, vision and balance, eye problems associated with increasing age, such as age-related macular degeneration (AMD), cataracts and glaucoma, diabetic retinopathy a complication of diabetes where the cells lining the back of the eye are damaged by high blood sugar levels, damage to the brain, such as from meningitis, encephalitis, a stroke or severe head injury.
  • #1 DeafBlind 101: An Introduction to Combined Hearing and Vision Loss
    https://pathlore.dhs.mn.gov/courseware/DisabilityServices/DeafBlind_101/intro_causes1.html
    There are many causes of congenital deafblindness, including Usher syndrome, which accounts for more than half of all deafblindness in the United States. […] Usher Syndrome is a genetic condition that accounts for more than 50 percent of the deafblindness in the United States. […] People can be born deafblind or born with a congenital syndrome, such as Usher Syndrome, that eventually leads to deafblindness. […] CHARGE Syndrome is a complex genetic syndrome that occurs in about one of every 10,000 births worldwide and affects many areas of the body. Most people with CHARGE Syndrome have hearing loss, vision loss, and balance problems. […] Congenital Rubella Syndrome (CRS) occurs when a woman contracts rubella, or German measles, during the first trimester of her pregnancy and passes it on to the developing fetus, causing the child to be born deaf, blind, with cardiac problems, developmental delays and other medical conditions.
  • #1 American Association of the Deaf-Blind
    https://www.aadb.org/FAQ/faq_DeafBlindness.html
    No. Most people who are deaf-blind have a combination of vision and hearing loss. They usually have some useful but not always reliable vision and hearing. Some people have little or no useable hearing and vision. […] About 50 percent of people in the deaf-blind community have Usher Syndrome. This is a genetic condition where a person is born deaf or hard of hearing, or with normal hearing, and loses his or her vision later on in life from retinitis pigmentosa (RP). There are three kinds of Usher Syndrome. If a person has Usher 1, she is born deaf, and starts to lose her vision usually in the teen years. If a person has Usher Syndrome 2, he is born hard of hearing and starts to lose his vision later on. With Usher 3, a person is usually born with normal vision and hearing, or with a mild hearing loss, and start to lose both senses later in life.
  • #1 Causes & Conditions – Anne Sullivan Centre for People who are Deafblind
    https://www.annesullivan.ie/advice-information/causes-conditions/
    Scientists have identified more than 10 different genes that can cause Usher Syndrome. Each person inherits two copies of each gene, one from each parent. A misprint (a gene mutation) in both copies of any one of those 10 genes means that the correct protein that is needed in cells of the inner ear and in the retina is not made in the body. […] Currently, there is no cure for Usher syndrome. However, scientific researchers around the world are making significant advances in treating hearing loss and the retinal degeneration experienced by people with Usher Syndrome.
  • #1 Deaf-Blind
    https://education.mn.gov/MDE/dse/sped/cat/db/
    Early identification is critical for providing interventions and support to children and families with combined hearing and vision loss. […] For early identification of infants and children with combined hearing and vision loss, DeafBlind Quick Cards summarize the most important information about risk factors, concerns, and next steps for referrals (DeafBlind (DB) Quick Card) and also focus on identification, evaluation, program planning, collaborative partners and resources (Deafblindness Referral Quick Card). […] The Combination of Deaf-Blindness Causes Severe Communication and Other Developmental and Educational Needs That Should be Addressed Jointly.
  • #1 Deafblindness | Deafblind Scotland
    https://www.dbscotland.org.uk/deafblindness
    Congenital Infections Infections such as rubella (German measles), cytomegalovirus (CMV), and toxoplasmosis during pregnancy can lead to deafblindness in the human infant. […] Premature Birth Premature infants are at a higher risk for various sensory impairments, including deafblindness. […] Meningitis: This infection can damage both the auditory and visual systems, leading to deafblindness. […] Head Trauma: Severe head injuries can result in damage to the parts of the brain responsible for hearing and vision. […] Medications: Some medications, such as certain antibiotics or cancer medications, especially when used in high doses or for prolonged periods, can cause damage to hearing and vision. […] Genetic Disorders Usher Syndrome: The most common genetic cause of acquired deafblindness, characterised by hearing loss and progressive vision loss due to retinitis pigmentosa.
  • #1 Etiologies | National Center on Deafblindness
    https://www.nationaldb.org/products/national-child-count/report-2018/etiologies/
    The heterogeneous nature of the population cannot be overstated. The etiologies or causes of deaf-blindness bring unique issues and challenges. Some are relatively more common, such as CHARGE syndrome, Down syndrome, Usher syndrome, and Cytomegalovirus. Some are very rare, with less than five individuals reported nationally, such as Batten disease, Prader-Willi, and Kearns-Sayre syndrome.
  • #1 Deafblindness and dual sensory loss research: Current status and future directions
    https://www.wjgnet.com/2218-6247/full/v5/i2/37.htm
    Second, the heterogeneity of the population makes it difficult to study people with deafblindness as one single group. Different levels of vision and hearing loss, different use of language modality, different kinds and severity of additional disabilities, and different medical aetiology are some of the variables splitting the group. […] Third, due to the dual sensory loss, it is generally very difficult to use traditional functional assessment procedures and psychological tests, since these often require full sensory functioning as a prerequisite. […] Forth, it is often difficult to communicate or even cooperate with a person with congenital deafblindness even for a researcher mastering both the local oral, signed, and tactile languages. […] Fifth, interpretation of deafblind behaviour can be very challenging.
  • #1 Hiding in plain sight: genetic deaf-blindness is not always Usher syndrome
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8327880/
    Hearing loss (HL) is the most common congenital sensory impairment. Usher syndrome (USH) is the leading genetic etiology of congenital deafness combined with progressive vision loss, and individuals presenting with these symptoms are often assumed to have USH. […] However, there are additional genetic causes of deaf-blindness. […] The leading genetic etiology of deaf-blindness is Usher syndrome (USH), a form of hearing loss inherited in an autosomal recessive pattern with vestibular dysfunction and progressive vision loss. […] Importantly, there are a number of other frequently overlooked genes associated with both hearing loss diagnosed in early childhood and visual impairment that may warrant distinct clinical management. […] Etiologies of deaf-blindness across age groups include prenatal infection, prematurity, age-related causes, and genetic causes, with heritable causes estimated to be responsible in 27% of affected individuals.
  • #1 Deaf-blindness – Children, Causes, Treatment | Britannica
    https://www.britannica.com/science/deaf-blindness/Deaf-blindness-in-children
    When considering deaf-blindness from a developmental perspective, it is also possible to consider deaf-blindness from both the medical and the cultural or social perspectives. […] From a developmental medical point of view, the most important aspects of deaf-blindness are the age of onset and the severity of the hearing and visual impairments. […] Consequently, the earlier the auditory and the visual impairments occur and the more severe the level of each impairment, the greater the consequences on development. […] Deaf-blindness is likely to affect cognitive, language, social, and emotional development. […] It is therefore beneficial for the development of deaf-blind infants that they are diagnosed early and that age-appropriate stimulation is started young. […] Environmental factors play an essential role in determining later outcomes in children. This is the case particularly for deaf-blind children.
  • #1 Deafblindness | Deafblind Scotland
    https://www.dbscotland.org.uk/deafblindness
    Diabetes: Long-term diabetes can lead to both diabetic retinopathy (affecting vision) and neuropathy (affecting hearing). […] Neurological Disorders: Conditions like multiple sclerosis can affect sensory functions, potentially leading to combined hearing and vision loss. […] Exposure to Toxins: Prolonged exposure to certain chemicals or environmental toxins can result in sensory impairments. […] Understanding the cause of deafblindness in an individual often requires a detailed medical history, genetic testing, and a thorough examination by specialists.
  • #2 Preventive audiology in the context of deafblindness – Preventive Audiology – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK601308/
    Deafblindness is a distinctive multisensory disability characterised by varying degrees of hearing and visual impairment. […] Deafblindness is a lifelong and progressive condition that can be congenital or acquired in nature, has multiple causes and can be categorised into four distinct categories. […] The contribution of deafblindness on the developing child is multiplicative in that it is a combination of two vital senses vision and hearing which are foundations for communication, socialisation, orientation and mobility, access to information and daily living. […] Unfortunately, not much is known about South African deaf-blind children. […] The authors of this chapter argue that although quantitatively, the impact of primary prevention on reducing disability is not known, it cannot be disputed that efforts put towards controlling potential disabilities (e.g. immunisation drives) have positively and significantly achieved the desired outcomes.
  • #2 Deaf-Blindness – Center for Parent Information and Resources
    https://www.parentcenterhub.org/deafblindness/
    There are approximately roughly 45,000 to 50,000 individuals in the U.S who are deaf-blind. […] According to the 2018 National Deaf-Blind Child Count, over 11,000 are children under the age of 21. […] The term actually describes a person who has some degree of loss in both vision and hearing. […] The National Center on Deaf-Blindness observes that the key feature of deaf-blindness is that the combination of losses limits access to auditory and visual information. […] Because deaf-blindness causes severe communication and other developmental and educational needs, its very important for children with deaf-blindness to receive special education and related services to address their individual needs.
  • #2 Causes & Conditions – Anne Sullivan Centre for People who are Deafblind
    https://www.annesullivan.ie/advice-information/causes-conditions/
    Deafbindness can occur for a variety of reasons. Some people are born deafblind while others loose one or both senses later in life, either suddenly or gradually. […] People who are congenitally deafblind are hearing and vision impaired from birth. Some causes of Congenital deafblindness include prematurity pre and post-natal complications, viral infections such as rubella and rare genetic conditions including CHARGE Syndrome. […] Acquired deafblindness occurs when a person loses their vision and hearing, either gradually or suddenly, during development or in adulthood. Acquired deafblindness can be related to a specific genetic condition from birth that wasnt detected until later in life, or occur as a result of illness or an accident. Age-related deafblindness is the most common cause of acquired deafblindness followed by Usher Syndrome.
  • #2 Deafblindness – when one can neither hear nor see
    https://www.enableme.ug/en/article/deafblindness-when-one-can-neither-hear-nor-see-10416
    Generally, there is a distinction made between congenital and acquired deafblindness. Congenital deafblindness refers to cases when the double sensory impairment exists from birth or since early childhood. […] Acquired deafblindness refers to cases where the disability initially develops after the acquisition of speech. […] The causes are diverse for both acquired and congenital deafblindness. In the past, the main cause of congenital hearing impairment was rubella during pregnancy. Today, very early premature births and various genetic syndromes such as CHARGE syndrome are the main causes of deafblindness in newborns. Besides age-related impairment, Usher syndrome is the most common cause of acquired hearing loss. This is a congenital hearing impairment to which a gradual reduction in vision arises over the years.
  • #2 Etiologies | National Center on Deafblindness
    https://www.nationaldb.org/products/national-child-count/report-2018/etiologies/
    Over 70 specific etiologies are identified in the 2018 National Deaf-Blind Child Count. Some are exceedingly rare, with less than five cases. Others are much more prevalent. There are 12 etiologies with at least 100 cases. […] Complications from prematurity continues to be the most commonly identified etiology. The prevalence of CHARGE syndrome continues to increase. In 2018 there were 933 children and youth identified as having CHARGE syndrome. While still relatively rare, the prevalence of children with Stickler syndrome and Dandy Walker syndrome has also increased. About 19% of the children and youth have no determined etiology. […] Overall, the total number of children and youth identified with a prenatal congenital or postnatal non-congenital etiology has declined from 2,732 to 2,551 over the past five years, while the number with hereditary syndromes and disorders has increased from 3,945 to 4,482. Hereditary syndrome and disorders now account for 44.8% of all identified etiologies. Prenatal congenital complications account for 13.9% of identified etiologies and postnatal non-congenital complications account for 11.6% of identified etiologies. Complications from prematurity account for 10.5% of identified etiologies and 19.2% of the children and youth had no identified etiology at the time data was collected.
  • #2 Preventive audiology in the context of deafblindness – Preventive Audiology – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK601308/
    Primary prevention within the context of deafblindness, may, among others, include immunisation campaigns against communicable diseases, genetic counselling, prenatal and postnatal care at the primary health care level and measures to control endemic diseases. […] Considering the causes (prenatal viral infections, premature birth, genetic conditions and age-related conditions) and categories of deafblindness (congenital, acquired, congenital, and acquired and age-related), primary prevention has a significant role to play in reducing the incidence of deafblindness. […] Prematurity is one of the main contributors to acquired deafblindness. […] In cases of hereditary causes of deafblindness, the WHO recommended that families be referred for genetic counselling where concerns about current or future pregnancies may be discussed.
  • #2 DeafBlind 101: An Introduction to Combined Hearing and Vision Loss
    https://pathlore.dhs.mn.gov/courseware/DisabilityServices/DeafBlind_101/intro_causes1.html
    There are many causes of congenital deafblindness, including Usher syndrome, which accounts for more than half of all deafblindness in the United States. […] Usher Syndrome is a genetic condition that accounts for more than 50 percent of the deafblindness in the United States. […] People can be born deafblind or born with a congenital syndrome, such as Usher Syndrome, that eventually leads to deafblindness. […] CHARGE Syndrome is a complex genetic syndrome that occurs in about one of every 10,000 births worldwide and affects many areas of the body. Most people with CHARGE Syndrome have hearing loss, vision loss, and balance problems. […] Congenital Rubella Syndrome (CRS) occurs when a woman contracts rubella, or German measles, during the first trimester of her pregnancy and passes it on to the developing fetus, causing the child to be born deaf, blind, with cardiac problems, developmental delays and other medical conditions.
  • #2 Causes of deafblindness – Deafblind Australia
    https://www.deafblind.org.au/causes-of-deafblindness/
    Some of the most common causes of deafblindness are Rubella, CHARGE syndrome, Usher syndrome and ageing. […] Usher syndrome is the most common genetic cause of combined vision and hearing impairment and deafblindness. […] CHARGE syndrome is a recognisable genetic pattern of birth defects which occurs in an estimated one in every nine to 10,000 births worldwide. […] Babies born with Congenital Rubella Syndrome (CRS) can be affected differently. […] Some of the problems associated with CRS include sensorineural hearing loss, visual problems such as cataract, inflammation of the retina (retinopathy), nystagmus, small eyes (microphthalmia), and occasionally optic atrophy, corneal haze, and glaucoma. […] Most have hearing loss, vision loss, and balance problems which delay their development and communication.
  • #2 Understanding deafblindness – Sense International
    https://www.senseinternational.org.uk/our-work/understanding-deafblindness/
    Research has shown that 0.2% of the world’s population is living with severe deafblindness. A further 2% of people around the world are living with milder forms of deafblindness. […] There are many causes of deafblindness and multi-sensory impairment, including: Congenital Rubella Syndrome – where a mother contracts rubella during pregnancy and her unborn child suffers birth defects, such as vision and hearing impairments. Genetic conditions – these include Usher syndrome, CHARGE syndrome, and Down Syndrome. Medical complications during pregnancy and birth – including poor nutrition and trauma. Premature birth. Illness and accidents – including meningitis and cerebral malaria. Sensory loss as a result of ageing. […] According to the World Health Organisation (WHO), rubella is the leading cause of birth defects that can be prevented by vaccination.
  • #2
    https://www.nhs.uk/conditions/deafblindness/causes/
    There are many possible causes of deafblindness. The condition can either be present at birth or develop later in life. […] Deafblindness from birth is known as congenital deafblindness. […] It can be caused by: problems associated with premature birth (birth before 37 weeks of pregnancy), an infection in a baby in the womb, such as rubella (german measles), toxoplasmosis or cytomegalovirus (CMV), genetic conditions, such as Down’s syndrome or CHARGE syndrome, cerebral palsy a problem with the brain and nervous system that mainly affects movement and co-ordination, foetal alcohol spectrum disorder health problems caused by drinking alcohol in pregnancy. […] In most cases, deafblindness develops later in life. This is known as acquired deafblindness. […] Problems that can contribute to acquired deafblindness include: age-related hearing loss, Usher syndrome, a genetic condition that affects hearing, vision and balance, eye problems associated with increasing age, such as age-related macular degeneration (AMD), cataracts and glaucoma, diabetic retinopathy a complication of diabetes where the cells lining the back of the eye are damaged by high blood sugar levels, damage to the brain, such as from meningitis, encephalitis, a stroke or severe head injury.
  • #2 Encyclopedia of deafblindness – Deafblind International DbI
    https://www.deafblindinternational.org/encyclopedia-of-deafblindness/
    Babies that are born with toxoplasmosis (usually when infected between the third and sixth month) may develop severe symptoms. These include hydrocephalus, calcification in the brain and chorioretinitis (damage to the retina). Epilepsy and deafness can also result. […] Goldenhar is prevalent in males (70%) and affected individuals may have asymmetrical small ears and mouth with hypoplasia of the jaw, mouth and eyes (with epibular dermoids). […] There is very little evidence to explain why Goldenhar Syndrome occurs. In most cases, Goldenhar Syndrome appears to occur randomly, with no apparent cause; nothing similar has ever happened in the family before, and there is little chance of it happening again. […] Alstrm Syndrome is autosomal recessive and even though it has been mapped to chromosome 2p13, the gene responsible for the disorder has not been identified. […] Alstrm syndrome is autosomal recessive which means that for a child to have the condition they must inherit one mutated gene from their mother and one from their father.
  • #2 Causes of Deafblindness – Support for schools and settings
    https://www.devon.gov.uk/support-schools-settings/send/areas-of-need/sensory-and-or-physical/multi-sensory-impairment/causes-of-deafblindness/
    Usher syndrome is a genetic condition that affects hearing, vision, and balance. […] It can result in progressive hearing loss and retinitis pigmentosa, leading to night blindness and loss of peripheral vision. […] Infections such as rubella (German measles), toxoplasmosis, and cytomegalovirus (CMV) can be transmitted from mother to foetus. […] These infections can cause congenital hearing loss and vision problems, including cataracts and retinal damage. […] Down syndrome is a naturally occurring genetic condition caused by an extra chromosome. […] There is an increased risk of hearing and vision problems, including congenital cataracts and conductive hearing loss. […] Cerebral Palsy is a group of disorders affecting movement and muscle tone, often caused by damage to the developing brain. […] It can include sensory impairments such as hearing and vision loss due to neurological damage. […] Brain injuries can result in sensory impairments, including hearing and vision loss.
  • #2 Deaf-Blind
    https://education.mn.gov/MDE/dse/sped/cat/db/
    Deafblindness is defined under the Individuals with Disabilities Education Act (IDEA) as: „concomitant (simultaneous) hearing and visual impairments, the combination of which causes such severe communication and other developmental and educational needs that they cannot be accommodated in special education programs solely for children with deafness or children with blindness.” […] If a student meets criteria for deaf/hard of hearing and blind/visually impaired, then the student meets eligibility for deafblindness. […] IEP teams should also consider evaluating hearing and vision levels when a student has at-risk potential, such as an identified condition or syndrome, including extreme prematurity, CHARGE Syndrome, or Usher Syndrome. […] „Deafblindness is a unique disability and not the sum total of vision and hearing loss.” (Miles, D. and Riggio, M. (1999))
  • #2 Deafblindness and dual sensory loss research: Current status and future directions
    https://www.wjgnet.com/2218-6247/full/v5/i2/37.htm
    Several causes are also known for acquired (post-lingual) deafblindness with Usher syndrome being the most dominant (about half of all cases) among people below 60 years of age. Usher syndrome is an autosomal recessive disorder characterized by congenital or progressive hearing loss and progressive vision impairment due to the eye disease Retinitis Pigmentosa and involve several subtypes. Other causes are head injuries, tumors, blindness following diabetes, as well as hearing impairment caused by noise exposure. […] The lack of published research may have at least seven explanations. First, the rareness of the condition makes it difficult to even gather just a small group to study. Prevalence of congenital deafblindness and acquired deafblindness among people below 60 years of age is below the 0.1 percent level. Acquired deafblindness is more frequent at higher ages (prevalence around 30 percent at age 80) and may therefore be more researched.
  • #2 American Association of the Deaf-Blind
    https://www.aadb.org/FAQ/faq_DeafBlindness.html
    No. Most people who are deaf-blind have a combination of vision and hearing loss. They usually have some useful but not always reliable vision and hearing. Some people have little or no useable hearing and vision. […] About 50 percent of people in the deaf-blind community have Usher Syndrome. This is a genetic condition where a person is born deaf or hard of hearing, or with normal hearing, and loses his or her vision later on in life from retinitis pigmentosa (RP). There are three kinds of Usher Syndrome. If a person has Usher 1, she is born deaf, and starts to lose her vision usually in the teen years. If a person has Usher Syndrome 2, he is born hard of hearing and starts to lose his vision later on. With Usher 3, a person is usually born with normal vision and hearing, or with a mild hearing loss, and start to lose both senses later in life.
  • #2 Deafblindness | Deafblind Scotland
    https://www.dbscotland.org.uk/deafblindness
    Congenital Infections Infections such as rubella (German measles), cytomegalovirus (CMV), and toxoplasmosis during pregnancy can lead to deafblindness in the human infant. […] Premature Birth Premature infants are at a higher risk for various sensory impairments, including deafblindness. […] Meningitis: This infection can damage both the auditory and visual systems, leading to deafblindness. […] Head Trauma: Severe head injuries can result in damage to the parts of the brain responsible for hearing and vision. […] Medications: Some medications, such as certain antibiotics or cancer medications, especially when used in high doses or for prolonged periods, can cause damage to hearing and vision. […] Genetic Disorders Usher Syndrome: The most common genetic cause of acquired deafblindness, characterised by hearing loss and progressive vision loss due to retinitis pigmentosa.
  • #2 Deafblindness | Deafblind Scotland
    https://www.dbscotland.org.uk/deafblindness
    Diabetes: Long-term diabetes can lead to both diabetic retinopathy (affecting vision) and neuropathy (affecting hearing). […] Neurological Disorders: Conditions like multiple sclerosis can affect sensory functions, potentially leading to combined hearing and vision loss. […] Exposure to Toxins: Prolonged exposure to certain chemicals or environmental toxins can result in sensory impairments. […] Understanding the cause of deafblindness in an individual often requires a detailed medical history, genetic testing, and a thorough examination by specialists.
  • #2 Introduction to Deaf-Blindness and Etiologies – Helen Keller Services
    https://www.helenkeller.org/courses/introduction-to-deafblindness-and-etiologies/
    Knowing what a person is able to see or hear is essential in establishing clear communication, ensuring safe mobility, assessing support needs and setting reasonable goals for employment, living situations, transportation and many other aspects of life. […] Common Etiologies of DeafBlindness. […] Genetic Disorders. […] Prenatal, Perinatal, and Congenital Complications. […] Adventitious Conditions. […] Age Related DeafBlindness.
  • #3 Causes of Deafblindness – Support for schools and settings
    https://www.devon.gov.uk/support-schools-settings/send/areas-of-need/sensory-and-or-physical/multi-sensory-impairment/causes-of-deafblindness/
    Deafblindness can result from a variety of congenital (present at birth) and acquired (developed later in life) causes. […] Understanding these causes can help in early diagnosis and support. […] Here are details of some of the main causes of Deafblindness, in order of prevalence. […] Complications of prematurity can lead to underdeveloped organs, including the eyes and ears, resulting in sensory impairments. […] There is increased risk of retinopathy of prematurity (ROP) and hearing loss due to underdeveloped auditory and visual systems. […] CHARGE syndrome is a complex genetic condition referring to a set of specific physical and sensory features. […] The main input senses of vision and hearing, as well as the vestibular and proprioceptive senses are usually impacted on for children with CHARGE syndrome.