Mental and behavioral disorders among people with congenital deafblindness
Research highlights
▶ Prevalence of mental and behavioral disorders among 95 congenitally deafblind adults. ▶ Mental retardation was found among 34%, psychosis among 13%. ▶ Interaction of dual sensory loss and mental and behavioral disorders are discussed.
Introduction
People with congenital deafblindness either are born deaf and blind or become deaf and blind early in life before the development of language (pre-lingual deafblindness). Post-lingual deafblindness refers to people who become deaf and blind after the development of language (acquired deafblindness).
The prevalence of congenital deafblindness has been reported to be extremely rare, with about one in 27,000 newborn babies affected (Dammeyer, 2010b). The prevalence of congenitally deafblind people is around 1/5 of the total population of deafblind people (congenitally and acquired) (Rødbroe & Janssen, 2008).
There is a wide range of causes for congenital deafblindness. Among the more common are CHARGE syndrome, prematurity, meningitis, cytomegalovirus and, formerly, Rubella syndrome (Dammeyer, 2010b). There are also many causes for acquired deafblindness. The most common is Usher syndrome which accounts for approximately half of the people with acquired deafblindness (Møller, 2003).
Evaluation of mental and behavioral disorders (ICD-10, Block F) among people who are congenitally deafblind has not been reported. One reason is that the group of people who are congenitally deafblind is small and heterogeneous, another that only little attention had been paid to this group in existing research. But among people with syndromes associated to deafblindness, few studies had reported mental and behavioral disorders. Among individuals with Usher syndrome psychosis has been reported in case studies (Hess-Röver et al., 1999, Jumaian and Fergusson, 2003, Mangotich and Misiaszek, 1983, McDonald et al., 1998, Rijavec and Grubic, 2009), but a higher prevalence in general was not found (Grøndahl and Mjøen, 1986, Mangotich and Misiaszek, 1983). Different mental and behavioral disorders had been discussed among people with Rubella syndrome from case studies (Sever, South, & Shaver, 1985), but no overall characteristics or prevalence has been reported. Finally, behavioral features of autism among individuals with CHARGE syndrome has been reported (Graham et al., 2005, Hartshorne and Cypher, 2004, Smith et al., 2005), but the evaluated behavior were found to be associated to the dual sensory impairment (Graham et al., 2005, Vervloed et al., 2006).
Next to prevalence, the etiology of mental and behavioral symptoms among people with deafblindness is an important issue of concern. To what degree does dual sensory loss cause mental and behavioral symptoms? Some argue that the appearance of mental and behavioral symptoms can be caused by the loss of hearing and vision, e.g. psychosis among people with Usher syndrome (Mangotich & Misiaszek, 1983). In another study McDonnell (2009) found that communication and social support were important to avoid the experience of depression among people with acquired deafblindness, i.e. the communicative difficulties caused by the dual sensory loss, increase the risk of depression.
Cognitive decline among people with acquired deafblindness may illustrate some of the interaction between dual sensory loss and mental and behavioral disorders. Laforge, Spector, and Sternberg (1992) found, after adjusting for age, sex and cognitive status, that persons who had vision impairment or both vision and hearing impairments were 2.5 and 3.5 times more likely to experience functional decline than were unimpaired older adults, respectively. In a large study of 6112 women aged 69 and older, Lin et al. (2004) found similar odds for reduced cognitive function (2.19) for women with combined visual and hearing impairment as compared to women without impairments.
Pervasive developmental disabilities are also of interest with regard to people who are deafblind and the etiology of mental and behavioral disorders. Autism or autism-related conditions are common among blind children (Cass, 1998). Similarly though, to a lesser degree, there have been found more occurrences of autism within the group of children with hearing loss (Carvill, 2001, Jure et al., 1991). With regard to children with deafblindness, there are no accounts of the occurrence, but one must assume that the occurrence of autism-related symptoms is at least the same. Similarities of communicative, social and cognitive symptoms of autism may be similar to symptoms of dual sensory loss (Hoevenaars-van den Boom, Antonissen, Knoors, & Vervloed, 2009).
Mental retardation may also be a common occurrence among individuals who are deafblind due to common underlying etiology, such as genetically based syndromes, prenatal infections etc. No report on prevalence could be found. It is difficult to differentiate between behavioral symptoms of deafblindness and mental retardation. Deafblindness among people with mental retardation has been reported to be undiagnosed (Fellinger et al., 2009, Meuwese-Jongejeugd et al., 2008).
The complexities of mental and behavioral issues associated with people who are deafblind do often complicate and limit the foundation for appropriate clinical treatment. More knowledge of the prevalence and etiology of mental and behavioral disorders among people who are congenitally deafblind are needed. The aim of this study is to explore the prevalence and etiology of mental and behavioral symptoms among people with congenital deafblindness.
Section snippets
Participants
All people in Denmark above the age of 18 known to be congenitally deafblind were included in the study. This amounted to 123 individuals. The Scandinavian definition of deafblindness was used: “Deafblindness is a combined vision and hearing disability. It limits activities of a person and restricts full participation in society to a degree which requires that society compensates by means of specific services, environmental alterations and/or technology.”(Nordisk Lederforum, 2007). The people
Population
Fifty-nine percent (n = 56) were men and mean age for both men and women were 41 years (SD = 12.6). Etiology distributed to 27% (n = 26) with Rubella syndrome, 8% (n = 8) Downs syndrome, 6% (n = 6) prematurity, 4% (n = 4) CHARGE syndrome, 4% (n = 4) CMV. For 22% (n = 21), etiology was unknown, and among the rest 27% (n = 26), etiology was rare, with 3 or less individuals of each syndrome.
Thirty-three percent lived at an institution for intellectual disabilities not specialized for individuals with sensory loss,
Discussion
This study found a high prevalence of mental and behavioral disorders among individuals with congenital deafblindness, in special mental retardation and psychotic symptoms. Only one out of four did not have any mental or behavior disorder.
As introduced, etiology of mental and behavioral symptoms among people with deafblindness is an important issue to discuss to explain the high prevalence and to be able to offer appropriate intervention. To what degree does dual sensory loss cause mental and
Conclusion
People who are congenital deafblind face a high risk of mental and behavioral symptoms. Mental retardation and psychosis are common conditions. In most cases there is an interaction of dual sensory loss and symptoms of mental and behavioral disorders. Focus must be on communicative development in a multiannual and cross-disciplinary approach.
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