Deafblindness

Causes and onset

Deafblindness has heterogeneous causes and may be present from birth, develop during childhood or adulthood, or arise later in life when hearing and vision impairments coexist. The relative importance of different causes varies substantially with age, population and the definition of deafblindness used.

Genetic and congenital causes

Genetic disorders are important causes of both congenital and progressive deafblindness. Usher syndrome combines sensorineural hearing loss with progressive retinitis pigmentosa and is one of the best-characterized inherited causes of deafblindness. Its clinical course varies according to genetic subtype; hearing loss may be congenital or progressive, while retinal degeneration usually develops later.

CHARGE syndrome, most often associated with pathogenic variants in CHD7, is an important cause of congenital deafblindness. Visual impairment commonly results from ocular coloboma, while hearing impairment may arise from abnormalities of the external, middle or inner ear and from cranial nerve dysfunction. Vestibular impairment and multiple additional medical and developmental conditions are also common.

Other genetic conditions can produce combined hearing and visual impairment through different mechanisms, including retinal dystrophy, optic neuropathy, structural ocular abnormalities or neurodegenerative disease. Examples include some forms of Alström syndrome, Wolfram syndrome, Stickler syndrome, mitochondrial disorders, peroxisomal disorders and other rare syndromic conditions.

Congenital deafblindness may also result from prenatal infections or developmental injury. Historically, congenital rubella syndrome was an important cause, producing combinations of sensorineural hearing loss, cataract or other ocular abnormalities and additional systemic manifestations. Congenital cytomegalovirus infection can also cause sensorineural hearing loss and visual impairment, particularly when central nervous system or ocular involvement is present. Other congenital infections may occasionally produce combined sensory impairment.

Prematurity and perinatal causes

Extreme premature birth can lead to combined visual and hearing impairment through several pathways. Visual loss may result from severe retinopathy of prematurity, cerebral visual impairment or other neurological injury, while hearing loss may be associated with extreme prematurity, neonatal intensive-care complications, ototoxic exposure, severe infection or neurological injury. Deafblindness in children born very preterm therefore often reflects multiple interacting causes rather than a single disorder.

Hypoxic-ischaemic brain injury, meningitis, encephalitis, severe neonatal infection and other neurological conditions can likewise affect both auditory and visual pathways. In some individuals the sensory impairment is partly peripheral and partly central.

Acquired and progressive causes

Deafblindness can develop when a person with an existing hearing or visual impairment later develops impairment of the other sense. Progressive genetic disorders such as Usher syndrome are a characteristic example, but combined sensory impairment may also follow trauma, infection, inflammatory or neurological disease, ototoxicity, retinal or optic nerve disease, or other acquired conditions.

In adulthood and particularly in older age groups, deafblindness frequently results from the coexistence of common acquired causes of hearing and vision loss rather than from a single syndrome. Age-related hearing loss may coexist with age-related macular degeneration, cataract, glaucoma, diabetic retinopathy or other ocular disease. Consequently, the etiological profile of late-life deafblindness differs substantially from that of congenital or early-onset deafblindness.

In some people no single cause can be identified, and more than one condition may contribute to the combined sensory impairment. Etiological classification therefore does not always correspond directly to functional severity or support needs.

Communication

Deafblind people communicate in many different ways as determined by the nature of their condition, the age of onset, and what resources are available to them. For example, someone who grew up deaf and experienced vision loss later in life is likely to use a sign language (in a visually modified or tactile form). Others who grew up blind and later became deaf are more likely to use a tactile mode of spoken/written language. Methods of communication include:

• Use of residual hearing (speaking clearly, hearing aids, or cochlear implants) or sight (signing within a restricted visual field, writing with large print)
• Tactile signing, sign language, or a manual alphabet such as the American Manual Alphabet or Deaf-blind Alphabet (also known as "two-hand manual") with tactile or visual modifications
• Interpreting services (such as sign-language interpreters or communication aides)
• Communication devices such as Tellatouch or its computerized versions known as the TeleBraille and Screen Braille Communicator.
• Tadoma, a tactile modality
• Square script, a method of writing along tactile guidelines
• Protactile, a tactile language related to American Sign Language in the Francosign language family

Multisensory methods have been used to help deafblind people enhance their communication skills. These can be taught to very young children with developmental delays (to help with pre-intentional communication), young people with learning difficulties, and older people, including those with dementia. One such process is Tacpac.

Deafblind people often use the assistance of people known as support-service providers (SSPs), who help the deafblind with tasks such as routine errands, guiding the deafblind through unfamiliar environments, and facilitating communication between the deafblind person and another person.

Technology

Assistive technology can support communication, access to information, environmental awareness, orientation and mobility, and independent living for people with deafblindness. Because the degree and combination of hearing and vision impairment vary widely, technologies that are effective for people with only visual or only hearing impairment may be less usable when both senses are affected.

Access to information and communication

People with usable residual vision may use enlarged text, high-contrast interfaces, screen magnification or other visual adaptations, while those who primarily access information through touch may use refreshable Braille displays, Braille keyboards and other tactile interfaces. Screen readers can also be useful when sufficient hearing remains or when their output is routed to a Braille display. Smartphones, tablets and computers increasingly combine accessibility functions such as magnification, text-to-speech, Braille input and output, speech recognition and vibration-based notifications.

The suitability of a particular technology depends on sensory function, communication preferences, previous experience, cognitive and motor abilities, environmental conditions and the accessibility of the software or service being used. Assistive products for communication are among the environmental factors most frequently identified as relevant to functioning in research underlying the International Classification of Functioning, Disability and Health Core Sets for deafblindness.

Hearing and visual technologies

Depending on residual sensory function, conventional visual and hearing technologies may remain useful. These include hearing aids, cochlear implants and assistive listening systems, as well as optical magnification, electronic video magnifiers and other low-vision aids. Their effectiveness in deafblindness depends on whether the remaining auditory or visual information is sufficient to compensate for limitations in the other sensory channel.

Cochlear implantation has been used in both children and adults with deafblindness. Outcomes vary with etiology, age of implantation, duration of auditory deprivation, additional disabilities and rehabilitation, and evidence specific to deafblind populations remains less extensive than for hearing loss alone.

Tactile and haptic technology

Touch can provide an additional or alternative channel for communication and environmental information when auditory and visual access is limited. Tactile technologies include vibration-based alerts, tactile displays and experimental systems that convey language, spatial or contextual information through patterns applied to the hand or body.

Research into technology-mediated tactile communication is developing, but remains substantially less mature than technology designed separately for visual or hearing impairment. Experimental systems have investigated tactile fingerspelling and wearable haptic communication, although studies show that technologically generated tactile language may be less easily recognized than communication produced directly by another person.

Recent research has also examined wearable haptic systems developed with input from DeafBlind users and the Protactile community. These approaches attempt to convey communicative or contextual information through structured touch rather than relying exclusively on sound or vision.

Orientation, mobility and environmental information

Assistive technologies may also support orientation and mobility by providing information about location, obstacles or the surrounding environment. Depending on residual hearing and vision, systems may use visual enlargement, audio cues, vibration or other haptic signals. However, solutions that rely mainly on auditory feedback can be unsuitable for people with substantial hearing impairment, illustrating the need for multimodal or tactile alternatives.

Technologies used for environmental alerts may translate sounds such as doorbells, alarms or telephone calls into visual, vibrating or tactile signals. Connected devices and smart-home systems can extend these functions, but their usefulness depends on accessibility, reliability and the ability to configure output to the individual's available sensory channels.

Emerging technologies

Research is exploring technologies designed specifically for combined sensory impairment rather than adapting products developed for a single sensory disability. Areas of investigation include wearable haptic interfaces, tactile communication systems, object and environmental recognition, navigation systems and customized tactile materials.

3D printing has been explored as a means of producing tactile educational materials, models and individualized adaptations for communication, orientation, learning and independent living. Evidence remains preliminary and implementation in routine deafblind rehabilitation is limited.

Haptic technologies are also being investigated as a means of conveying spatial, social or contextual information that is normally obtained visually or auditorily. Much of this work remains experimental, and researchers have emphasized the importance of developing such technologies together with people who are deafblind rather than simply transferring technologies designed for other disability groups.

Adoption and accessibility

Access to technology does not necessarily result in sustained use. Factors influencing adoption include usability, training, cost, maintenance, compatibility with other devices, changes in sensory function and whether the technology fits the person's everyday activities and communication strategies. Research on deafblindness has found that many assistive devices studied were originally designed for either visual or hearing impairment rather than for combined sensory loss, and that evidence on technologies specifically designed for deafblind users remains limited.

History

The history of deafblindness is closely connected with the development of special education, tactile communication, rehabilitation services and, more recently, disability rights and self-advocacy. During the nineteenth century, people with combined hearing and vision impairment were often regarded as incapable of formal education. Early educational experiments helped challenge this assumption and contributed to the emergence of specialized approaches to communication and learning.

Early education

One of the best-documented early cases was Laura Bridgman (1829–1889), who became deafblind in early childhood and entered the Perkins School for the Blind in Massachusetts in 1837. Under the direction of Samuel Gridley Howe, she was taught using raised-letter labels and later tactile fingerspelling. Bridgman's education attracted international attention and became an influential demonstration that a person with deafblindness could acquire language and receive formal education.

Bridgman's education subsequently influenced the teaching of Helen Keller (1880–1968). Keller became deafblind in infancy and in 1887 began working with Anne Sullivan, a graduate of Perkins who had studied Howe's accounts of Bridgman's education and had communicated with Bridgman herself. Sullivan used fingerspelling into Keller's hand and developed an individualized approach based on objects, experiences and language. Keller later studied at Radcliffe College, graduating in 1904, and became an internationally known author and advocate for people with disabilities.

Comparable educational developments occurred in Europe during the nineteenth and early twentieth centuries. Institutions for children who were deaf, blind or deafblind experimented with tactile alphabets, sign communication and other methods. These developments contributed to the gradual institutionalization of deafblind education as a distinct field rather than treating combined sensory impairment solely within services for blindness or deafness.

Development of specialized education and rehabilitation

During the first half of the twentieth century, educational approaches for deafblind students expanded beyond raised print and fingerspelling. Methods included Braille, sign language and the Tadoma method, in which speech is perceived through touch on the face and neck of a speaker. By the mid-twentieth century, specialized deafblind programs and professional training for teachers were becoming established in several countries.

In the United States, Perkins and Boston University established a graduate training program in deafblind education in 1956. Similar specialist schools and services developed in Europe, including programs in the United Kingdom, Germany, the Netherlands and the Nordic countries. Cooperation between these centers contributed to the development of an international professional field concerned specifically with deafblind education.

Rehabilitation services for adults also developed during the twentieth century. In the United States, the Industrial Home for the Blind, later part of Helen Keller Services, began serving people with deafblindness in the early twentieth century and developed a formal deafblind program in 1945. The Helen Keller National Center for DeafBlind Youths and Adults was authorized by the United States Congress in 1967 and subsequently became a national center for rehabilitation, training and technical assistance.

Rubella and the expansion of services

The rubella epidemics of the 1960s had a major effect on the development of deafblind services. Congenital rubella syndrome caused hearing and visual impairment, often together with cardiac, neurological and developmental conditions, and resulted in a substantial increase in the number of children requiring specialized educational support.

The increase in children with congenital deafblindness accelerated the development of specialized schools, teacher training and regional support programs. At Perkins, for example, the deafblind program expanded substantially during the 1960s in response to the rubella epidemic.

The rubella epidemic also altered the population served by deafblind programs. Earlier educational models had often focused on children whose primary disabilities were sensory. Many children affected by congenital rubella had additional medical or developmental conditions, contributing to a broader understanding of deafblindness as a complex and heterogeneous disability.

International cooperation

International professional cooperation in deafblindness expanded after the Second World War. Schools and organizations in Europe and North America began exchanging educational experience during the 1950s, and the first international conference devoted to teaching children with deafblindness was held at Condover Hall in the United Kingdom in 1962.

At a conference in Sydney in 1976, the International Association for the Education of the Deafblind (IAEDB) was formally established. Its original emphasis was largely educational, but its scope later expanded to include rehabilitation, family support, research and services across the lifespan. In 1999, the organization adopted the name Deafblind International (DbI).

Parallel to the development of professional organizations, people with deafblindness increasingly established their own representative organizations. The decision to create the World Federation of the Deafblind (WFDB) was taken at the sixth Helen Keller World Conference in Paipa, Colombia, in 1997, and its founding General Assembly was held in Auckland, New Zealand, in 2001.

This development marked a broader shift from models centred primarily on professional care and education toward participation, self-representation and the recognition of people with deafblindness as rights-holders and contributors to policy and service development.

Legal recognition and organizations

Legal recognition and service provision for people with deafblindness vary considerably between countries. In some jurisdictions deafblindness is explicitly recognized as a distinct disability or is given a functional legal definition, while elsewhere support is provided through broader legislation relating to sensory impairment, disability, education or social care.

At the international level, the World Federation of the Deafblind (WFDB) represents organizations of people with deafblindness and advocates for their participation and rights. Deafblind International (DbI) is an international network of people with deafblindness, families, professionals, researchers and service providers concerned with education, rehabilitation, communication, research and support.

In the European Union, the European Parliament adopted a declaration on the rights of deafblind people in 2004. It described deafblindness as a distinct disability resulting from combined sight and hearing impairments and called on European institutions and member states to recognize the rights of deafblind people, including access to appropriate health and social care, education, employment and one-to-one support from communicator-guides, deafblind interpreters or intervenors where required.

The Nordic countries have developed a shared functional approach to deafblindness. The Nordic definition, first developed through regional cooperation and revised most recently in 2024, defines deafblindness as a combined vision and hearing impairment in which the impaired senses have difficulty compensating for one another. The Nordic Welfare Centre coordinates cross-border knowledge development and professional cooperation in the deafblind field.

In England, statutory social-care guidance uses a functional definition of deafblindness based on combined sight and hearing impairment causing difficulties with communication, access to information and mobility. Under the Care Act 2014, local authorities must involve an appropriately trained specialist in assessments of adults who are deafblind, including cases where each sensory impairment considered separately may appear relatively mild. National organizations include Sense and Deafblind UK, which provide support, information and advocacy for people with combined sensory impairment and their families.

In the United States, federal legislation established the Helen Keller National Center for DeafBlind Youths and Adults as a national resource for rehabilitation, training and technical assistance. The Helen Keller National Center Act identifies a continuing national need for specialist services for people who are deafblind.

In Canada, deafblind services differ between provinces. Ontario, for example, publicly funds intervenor services for adults with deafblindness. Intervenors facilitate communication and provide visual and auditory information to support access to services, community participation, decision-making, orientation and independent living.

In Spain, deafblindness is explicitly addressed in Law 27/2007, which recognizes Spanish sign languages and regulates communication support for deaf, hard-of-hearing and deafblind people. The law describes the particular communication difficulties associated with combined visual and hearing impairment, recognizes the heterogeneity of the deafblind population and establishes a right to choose between available forms of communication support. Specialist national organizations include the Federation of Associations of Deafblind People of Spain (FASOCIDE), the Spanish Federation of Deafblindness (FESOCE), and the Spanish Association of Families of Deafblind Persons (APASCIDE). The Spanish National Organization of the Blind also provides specialized support through its deafblindness services and the Fundación ONCE para la Atención de Personas con Sordoceguera (FOAPS), established in 2007 to develop programs addressing the specific needs of people with deafblindness, particularly in communication, education, employment and socio-educational mediation.

In popular culture

The play The Miracle Worker (1959), which was adapted into the film The Miracle Worker (1962), recounts Anne Sullivan's efforts to draw Helen Keller from her world of blindness and deafness.

The Who’s album Tommy (1969) tells one continuous life story about a deafblind mute boy named Tommy through songs.

The Bollywood film Black (2005) featured Rani Mukerji as a deafblind character named Michelle McNally.

The film Marie's Story (2014) relates the childhood and education of Marie Heurtin (1885–1921), a deafblind woman.

Haben Girma, the first deafblind individual to graduate from Harvard Law School, released an autobiography entitled Haben: The Deafblind Woman Who Conquered Harvard Law (2019).

Feeling Through (2019) is an American short drama film directed by Doug Roland that was the first film ever to star a deafblind actor (Robert Tarango) in a lead role; it is about a teenager and a deafblind man. It was nominated for the 2021 Academy Award for Best Live Action Short Film.

The Persistence of Vision is a 1978 novella by John Varley describing the life of a blind-and-deaf community.

Beginning in 1922, Helen May Martin (1893-1947) performed across the United States as a concert pianist.

Irene Ransburg was an Austrian poet of Jewish descent who was later baptized a Catholic and lived in a Catholic home for the blind. In 1944, she was deported to Theresienstadt and murdered. A memorial stone was placed near her home, the first such Stolperstein to be written in Braille.