Explore the unique world of deafblindness. Learn about challenges, adaptive strategies, and inspiring stories. Meri Sehat sheds light.

Deafblindness

Overview

Deafblindness is a rare sensory disability in which an individual experiences a combination of sight and hearing loss or impairment. 
&nbsp;

Deafblindness is also called dual sensory impairment and dual sensory loss. A deafblind individual may have their sense of hearing and sight missing entirely. Others may have these senses reduced or distorted to a great degree, such that they face significant challenges in terms of communication, mobility, daily living functioning, and social interactions.<a href="#accordion">[1]</a>

The prevalence of deafblindness is relatively low, with approximately 1 in 27,000 newborns worldwide being affected, with the condition being part of several genetic disorders.<a href="#accordion">[2][3]</a>&nbsp;According to a study, 36% of individuals aged 85 and above are deafblind.<a href="#accordion">[6]</a>

According to statistics, 10% of cases occur as complications from prematurity,12% of cases result from postnatal complications (e.g., severe head injury, <a href="https://merisehat.pk/disease/viral-meningitis">meningitis</a>), and 14% are related to prenatal complications (e.g., hydrocephalus). 47% of cases, however, are because of hereditary disorders/syndromes, while the etiology of 17% of cases remains unknown.<a href="#accordion">[8]</a> 
&nbsp;

Deafblindness can be categorized into the following two types based on the age of onset and the degree of impairment.<a href="#accordion">[6]</a>

<ul>
	<li>Congenital Deafblindness: It refers to individuals who either present with deafblindness at the time of birth or experience combined hearing and vision loss before they can develop signed, spoken, or other visual forms of communication.</li>
	<li>Acquired Deafblindness: It occurs when an individual experiences a gradual or sudden loss of both senses later in life. 
	&nbsp;</li>
</ul>

Sign and Symptoms

The characteristic symptoms and signs associated with deafblindness vary depending on the underlying cause and the severity of the impairment. 87% of deafblind people may have one or more additional disabilities.<a href="#accordion">[8]</a>Common signs include:<a href="#accordion">[7]</a>

<ul>
	<li>Difficulty moving around unfamiliar places</li>
	<li>Asking others to speak slowly, loudly, and more clearly and having difficulty following a conversation</li>
	<li>Needing to turn up the volume of the radio or television</li>
	<li>Problems seeing in bright or low light</li>
	<li>Holding reading material very close or sitting close to screens to see properly 
	&nbsp;</li>
</ul>

Causes

Deafblindness can have various causes, depending on the type.

<ul>
	<li>Congenital Deafblindness is usually due to infection contracted by the mother during pregnancy, hereditary conditions, or any disease or injury that affects early childhood development.</li>
	<li>Acquired Deafblindness occurs due to age-related degenerative diseases, such as cataracts or age-related hearing loss. This deterioration can also be due to an accident or disease at a later stage in life. 
	&nbsp;</li>
</ul>

The condition is usually detected during newborn screening if the baby is deafblind by birth.

However, routine checks are important to detect deafblindness as there might be some crucial indicators, e.g., babies who are deafblind may not focus on faces or things by 4-5 weeks of age. They may not make much eye contact. Likewise, at 4 months, babies react to sounds, but one who is deafblind may not do so. 
&nbsp;

Moreover, these babies tend to sleep a lot, bang their heads, cry only a little bit, poke their eyes, and rock back and forth.

Even if that is not the case, adults should have their little ones eyes tested every two years to check for any problems. 
&nbsp;

Diagnosis

The diagnosis of deafblindness involves a multidisciplinary approach, including assessments by audiologists, ophthalmologists, and other <a href="https://merisehat.pk/article/the-virtual-healthcare-revolution-bridging-gaps-with-online-doctor-consultations">healthcare</a> professionals.

<ul>
	<li>Audiological Evaluations are conducted to assess hearing function. They include pure-tone audiometry and auditory brainstem response testing.</li>
</ul>

<ul>
	<li>Visual Assessments include visual acuity tests and visual field tests.</li>
	<li>Functional Assessments are conducted to determine the impact of the sensory impairments on daily living activities, communication, and mobility. 
	&nbsp;</li>
</ul>

Treatment

There is no cure for deafblindness, but various interventions and strategies can help individuals with deafblindness lead fulfilling lives. Treatment plans are based on the severity of the problems patients face. 
&nbsp;

These may include:

<ul>
	<li>Assistive Devices such as cochlear implantation</li>
	<li>Alternative Methods of communication such as hands-on signing, braille, and augmentative and alternative communication systems</li>
	<li>Rehabilitation Services, including training to use a guide dog or long cane and counseling</li>
	<li>Treating Underlying Conditions using medication or surgery, including eye drops or laser surgery</li>
</ul>

Depending on the individual situation and their preferences, the patient may be offered one-to-one support, such as an interpreter, a communicator guide, or an intervenor to communicate. 
&nbsp;

Deafblindness can lead to several complications, including social isolation, limited educational and employment opportunities, and increased dependence on others for daily living tasks.

Deafblindness affects development, which means the child may experience difficulty or delay in understanding and communicating what&rsquo;s going on around them. Consequently, they don&rsquo;t see someone smiling at them, cannot identify the difference between day and night, may not point or move toward objects of interest, and are afraid to touch or explore certain textures and areas.

Individuals with deafblindness may also experience mental health issues such as depression, anxiety, and low self-esteem. For example, continually relying on tactile or alternative communication methods can be frustrating. 
&nbsp;

These include:

<ul>
	<li>Premature birth (birth before 37 weeks of pregnancy)</li>
	<li>Viral infections during pregnancy like toxoplasmosis, rubella, and cytomegalovirus</li>
	<li>Cerebral palsy</li>
	<li>Fetal alcohol syndrome</li>
	<li>Genetic conditions like Down&rsquo;s syndrome, Usher syndrome, or CHARGE syndrome</li>
	<li>Underlying health issues like diabetic retinopathy, glaucoma, and stroke 
	&nbsp;</li>
</ul>

Some causes of deafblindness are not preventable. However, the following measures can help others on the spectrum:

<ul>
	<li>Vaccination against congenital infections</li>
	<li>Prenatal Care and screening for maternal infections during pregnancy</li>
	<li>Taking Precautions to prevent traumatic injuries such as wearing protective gear 
	&nbsp;</li>
</ul>

Key Points

<ul>
	<li>Deafblindness is a rare sensory disability in which an individual experiences a combination of sight and hearing loss or impairment.</li>
	<li>There are two types of deafblindness &ndash; congenital and acquired.</li>
	<li>Common signs and symptoms of deafblindness include difficulty moving around unfamiliar places and asking others to speak slowly, loudly, and more clearly.</li>
	<li>The causes of deafblindness can include genetic conditions and age-related degenerative diseases.</li>
	<li>Treatment options for deafblindness focus on maximizing functional abilities and promoting independence.</li>
	<li>Complications of deafblindness can include social isolation, limited educational and employment opportunities, and mental health issues.</li>
	<li>Risk factors for deafblindness include genetic conditions, viral infections during pregnancy, prematurity, and trauma.</li>
	<li>Preventive measures for deafblindness include vaccination, prenatal care, and safety precautions to prevent injuries.</li>
</ul>

[1] Jaiswal, A., Aldersey, H. M., Wittich, W., Mirza, M., & Finlayson, M. (2018). Participation experiences of people with deafblindness or dual sensory loss: a scoping review of global deafblind literature. Plos One, 13(9), e0203772.

[2] Sundqvist, A., Wahlqvist, M., Anderzén‐Carlsson, A., & Olsson, E. (2022). Interventions for children with deafblindness—an integrative review. Child: Care, Health and Development, 49(3), 407-430.

[3] Zaki, M., Heller, R., Thoenes, M., Nürnberg, G., Stern-Schneider, G., Nürnberg, P., … & Bolz, H. (2015). Pex6 is expressed in photoreceptor cilia and mutated in deafblindness with enamel dysplasia and microcephaly. Human Mutation, 37(2), 170-174.

[4] Jaiswal, A., Aldersey, H., Wittich, W., Mirza, M., & Finlayson, M. (2018). Participation experiences of people with deafblindness or dual sensory loss: a scoping review of global deafblind literature. Plos One, 13(9), e0203772.

[5] Bourne, R., Jonas, J., Flaxman, S., Keeffe, J., Leasher, J., Naidoo, K., … & Taylor, H. (2014). Prevalence and causes of vision loss in high-income countries and in eastern and central europe: 1990–2010. British Journal of Ophthalmology, 98(5), 629-638.