Characteristics of individuals with congenital and acquired deaf-blindness.

Abstract: Using a standardized assessment instrument, the authors compared 182 adults with congenital deaf-blindness and those with acquired deaf-blindness. They found that those with congenital deaf-blindness were more likely to have impairments in cognition, activities of daily living, and social interactions and were less likely to use speech for communication.

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People who are deaf-blind differ in their degree of vision and hearing loss and in the age of onset of deaf-blindness, language development, communication mode, and level of independence. With this functional diversity among persons who are deaf-blind, it is important to identify the needs of this population at the individual level.

People who are deaf-blind can be classified into at least two groups: those who are congenitally deaf-blind, who experienced the onset of both hearing and visual impairment from birth to age 2 (Munroe, 2001), and those with acquired deaf-blindness, whose onset was later in life. Common causes of congenital deaf-blindness include intrauterine infections (like congenital rubella); congenital brain damage; and chromosomal abnormalities, such as CHARGE syndrome (Munroe, 2001; Watters, Owen, & Munroe, 2004). Acquired deaf-blindness can also be caused by genetically inherited disorders (such as Usher syndrome), as well as aging, postnatal or early childhood infections, and acquired brain injury (Munroe, 2001).

In Canada, approximately 55% to 70% of persons who are deaf-blind become deaf-blind after age 2 (Munroe, 2001; Watters et al., 2004). A survey by the Canadian Deafblind and Rubella Association resulted in the development of a National Volunteer Registry of Persons with Deafblindness in Canada (Munroe, 2001). Of the 1,725 individuals with deaf-blindness who were identified as eligible to participate in the registry, 777 (45.0%) completed the survey. The survey included questions related to the participants' demographic characteristics, modes of communication, diagnoses associated with vision and hearing loss, education and training, and the receipt of intervenor services for persons with congenital and acquired deaf-blindness. These two populations differed in many respects. A higher proportion of persons with acquired deaf-blindness lived independently, had a significant other, and used speech or writing or printing as modes of communication than did those with congenital deaf-blindness. The majority of individuals in the congenital group (69.6%) received at least 21 hours per week of intervenor services compared with only 12.3% among the acquired group.

In Canada, little is known about the cognitive, functional, and psychosocial characteristics of persons with deaf-blindness. The Ontario provincial government is moving toward more equitable and individual-level funding for services to individuals who are deaf-blind. Information on the characteristics of consumers is necessary to gain a better understanding of these individuals and to put policies in place that will provide for an equitable provision of services. This article is the second of two that examines a sample of the deaf-blind population in Ontario. The first article, "Development and Psychometric Properties of a Standardized Assessment for Adults Who Are Deaf-Blind" (Dalby, Hirdes, Stolee, Strong, Poss, Tjam, Bowman, & Ashworth, 2009) described the development of the newly created Deafblind Supplement to the interRAI Community Health Assessment (interRAI CHA) (Morris et al., 2006) and reported its psychometric properties. In this article, we compare participants with congenital and acquired deaf-blindness.

Method

MEASURES

The 182 participants with congenital or acquired deaf-blindness who were eligible for intervenor services from the provincial government were assessed with interRAI CHA (Morris et al., 2006) and the accompanying Deafblind Supplement. The instrument and its psychometric properties were described in our previous article (Dalby et al., 2009). In brief, interRAI CHA is a modular instrument that is intended to assess individuals living in the community who may be at risk of a functional decline. It is based on a common core assessment that can be supplemented by additional modules to address the needs of specialized subpopulations.

Health Index measures

Embedded in the assessment are a number of scales to help professionals evaluate a person's current health and functional status. These scales were used to describe the cognitive, functional, psychosocial, and health characteristics of individuals with deaf-blindness. A full description of these scales was presented in the first article (Dalby et al., 2009).

Clinical Assessment Protocols (CAPs)

CAPs provide guidance to service providers and clinicians on the need for further assessments or the provision of services to help individuals who are deaf-blind maintain their health and independence (Morris et al., 2006). They were derived from specific combinations of items within the instrument and help to identify individuals who are at risk of certain health problems or adverse outcomes. Once CAPs are identified for a specific individual, this trigger leads to a more in-depth review of the causes of the person's identified problems or presented risk.

Specific items in interRAI CHA will trigger the fact that CAP has been flagged for the individual. For example, the "pain" CAP is triggered if the individual complains or shows evidence of pain, as documented on interRAI CHA. A total of 12 CAPs are available, including those related to psychosocial functioning (such as social function and depression), physical health (including cognitive decline, physical activity, vision, dehydration, falls, pain, and preventive health), and the risk of harm to self or others (like alcohol abuse).

ANALYSES

We compared the congenital and acquired groups on select demographic and functional characteristics that were chosen on the basis of the previous literature (Munroe, 2001) and knowledge of factors that may differentiate between the two groups (such as cognitive impairment). We used chi-square tests to evaluate the significant differences between the categorical variables and an independent samples t-test for the continuous variables. An alpha level of .05 was used in all the analyses, which were performed with SAS software 9.1. (SAS Institute, 2001). The study was approved by the Wilfrid Laurier University Research Ethics Board, and all the participants were provided with written informed consent.

Results

CHARACTERISTICS OF THE SAMPLE

The average age of the sample was 42.7 years (SD = 17.8), and 51.6% (n = 94) of the participants had acquired deafblindness. In the acquired group, most participants were never married, were more likely to be females, lived in private homes or apartments, and had an average age of 52.8 years. In comparison, those in the congenital group were approximately 20 years younger on average and were more likely to be male, to have never been married, and to live with nonrelatives or parents or guardians (see Table 1).

COGNITIVE AND FUNCTIONAL STATUS

Of the participants in the acquired group, 48.9% had no cognitive impairment, compared with 12.5% in the congenital group. Among the participants with congenital deaf-blindness, a significantly lower proportion (25.0%) were considered to be able to perform activities of daily living independently, compared with 78.5% among the acquired group. Only 9.2% of the congenital group had no difficulty performing instrumental activities of daily living (see Table 2).

HEALTH STATUS

Many participants reported their health to be good or excellent (46.2%), although 68.2% in the congenital group did not respond to this question. A small number of participants had a diagnosis of diabetes mellitus (n = 18, 10.0%), coronary heart disease (n = 4, 2.2%), or cancer (n = 3, 1.6%), and these proportions were similar across the two groups. There was no significant difference between the two groups in terms of pain, health instability, and signs and symptoms of depression (see Table 3).

HEARING AND VISION

On the basis of the Deafblind Severity Index (DbSI), a new scale developed as part of this project (see Dalby et al., 2009), a large proportion of participants had a severe impairment in one sense and a mild or moderate impairment in the other sense (48.3%), and 30.2% had severe impairments in both senses (see Table 4). The most common conditions related to vision loss were cataracts (41.2%), retinitis pigmentosa (31.7%), and myopia (21.5%). The prevalence of retinitis pigmentosa was significantly higher among the acquired group (52.2% versus 10.2%). Congenital syndromes were the most common diagnoses associated with hearing loss (56.7%).

COMMUNICATION

The participants reported a number of communication modes that they routinely used. The items on the assessment are not mutually exclusive, so the assessors recorded all communication modes that an individual participant used.

The five most common communication systems reported were idiosyncratic signs, gestures, or behaviors (57.1%); any adapted or manually coded language (48.9%); speech (43.6%); a tactile method for expressive communication (42.3%); and any form of sign language (40.7%) (see Table 4). Individuals with congenital deafblindness were more likely to use idiosyncratic signs, gestures, or behaviors (78.4% versus 37.6%) or any adapted or manually coded language (68.1% versus 30.9%). The opposite was true for speech, which was more prevalent among those with acquired deafblindness (62.4% versus 23.9%).

The congenital group had greater difficulty with expressive communication than did the acquired group. For example, 15.9% of those in the congenital group and 57.5% in the acquired group were able to express ideas without difficulty. In terms of comprehension, a similar pattern was seen, with 5.7% of those in the congenital group but 39.4% of those in the acquired group able to understand others clearly.

ORIENTATION AND MOBILITY

Overall, 76.4% of the sample was independent or received minimal help with orientation and mobility in a familiar indoor environment. This proportion fell to 39.8% in a familiar outdoor environment. A small proportion of participants were independent in moving within an unfamiliar indoor environment (14.9%), with the acquired group being much more likely to be independent than the congenital group (23.7% versus 5.7%). Most participants (81.9%) were able to differentiate among floor and wall textures while walking or trailing, but those in the congenital group were less able to do so (51.1% versus 74.5%).

PSYCHOSOCIAL WELL-BEING

Most participants had a positive outlook (68.5%) and found meaning in day-to-day life (77.4%), and these proportions were similar between the two groups (see Table 5). Over half the participants showed moderate to high levels of social engagement (58.7%), although many experienced severe to very severe difficulty interacting with others (67.8%), which was particularly prevalent in the congenital group (58.6%).

CAPs

On average, the participants triggered 4.7 (SD = 1.6) CAPs. As expected, nearly all the participants (94.5%) triggered the CAP for visual function. However, a high proportion of participants triggered the CAP for preventive health (90.1%), which identifies participants who did not receive public health procedures--such as blood pressure monitoring, influenza vaccinations, and hearing and eye examinations--within the previous one to two years. The acquired group was significantly more likely to trigger the CAPs for physical activity, social function, and alcohol abuse (see Table 6).

Discussion

The interRAI CHA and Deafblind Supplement can be useful in describing persons who are deaf-blind because it captures in formation on their characteristics and level of functioning in a valid and reliable way. On the basis of feedback from the assessors, as well as from interviews with individuals who are deaf-blind or their family members, this assessment appears to be appropriate for use with both people with congenital and acquired deaf-blindness (Dalby et al., 2009). Thus, this instrument can form part of an integrated information system for persons with deaf-blindness. Through standardized assessment, interRAI CHA and Deafblind Supplement can help service providers and staff to implement supportive services that address the needs of individual participants.

The CAPs provide the foundation on which to build a participant-centered set of supportive services and referrals. The vast majority of participants were not receiving important preventive health measures, and nearly 50% were at risk of not being involved in physical activity, depression, and declines in their level of social participation. The results suggest that there may be unmet health-related needs in this population. Future research needs to address how community service providers may use CAPs in developing a service plan and monitoring changes in participants over time.

Previous research has suggested that services for persons with deaf-blindness should be planned around how a person functions in motor, linguistic, leisure, and social activities, rather than being based solely on auditory and visual deficits (Ronnberg & Borg, 2001). That is, service providers need to take into account the social and adaptive aspects of persons who are deaf-blind, rather than just vision and hearing loss. The interRAI CHA and Deafblind Supplement is comprehensive and multidimensional and meets this need. The information in the assessment itself, as well as the CAPs and health index measures embedded within the tool, provide a broad perspective of a person and allow for the assessment and monitoring of change over time.

These results show that there are significant differences between persons with congenital and acquired deaf-blindness. Differences in living arrangements, marital status, communication modes, and employment status between the groups are consistent with previous research (Munroe, 2001). Persons with congenital deaf-blindness appear to have multiple challenges, including the functions of communication, social interaction, orientation and mobility, and activities of daily living and instrumental activities of daily living. Those with acquired deafblindness also have a unique set of issues, such as adjusting to their acquired dual sensory impairment (they are more likely to experience lost roles) and feelings of loneliness. The findings indicate that although the acquired group has the capacity to function more independently in the community, given their functional and social skills, they are more socially isolated and more likely to report feeling lonely. These results may reflect a lower level of supportive services in this group; however, they may also reflect, at least in part, the difficulty of assessing constructs like loneliness and depression.

A potential limitation of the findings was the assessors' ability to capture signs and symptoms of depression. Although fairly large proportions of participants reported limitations in their activities of daily living, instrumental, and issues related to pain and loneliness, the prevalence of signs and symptoms of depression was 16% in the acquired group and 24% in the congenital group. On the basis of anecdotal information provided by the assessors, we suspect that these percentages may reflect the assessors' lack of experience in assessing this construct. The assessors noted that they would appreciate further training in the area of assessment in relation to mental health issues.

Implications for practice

Together with our previous article (Dalby et al., 2009), this article provides preliminary evidence for the reliability and validity of a standardized assessment for individuals with deaf-blindness. Service providers who work in the community, as well as governmental agencies that provide funding for services, are continually called on to make decisions about levels of services for their consumers who have dual sensory impairments. This instrument, together with professional expertise, can provide practical information for service providers and policy makers as they aim to learn more about persons with deaf-blindness, their preferences, their abilities, and their need for programs and services. The interRAI CHA and Deafblind Supplement give both providers and policy makers the necessary information to make informed decisions that are based on the needs of individuals who are deaf-blind and that are captured in a standardized and systematic way.

Conclusion

This analysis has revealed that persons with acquired deaf-blindness have different needs from those with congenital deaf-blindness. Many of these individuals have acquired both hearing and vision loss as a result of aging. With the aging of the Canadian population, there will be an associated increase in the prevalence of acquired deaf-blindness. Therefore, although the incidence of deaf-blindness is low in the population as a whole, the number of persons with dual sensory impairments is expected to increase over time. Future studies need to examine the rate of clinical change among persons with acquired and congenital deafblindness to gain a better understanding of how their needs change over time so as to ensure that both groups are provided with adequate services.

We thank the community service providers, assessors, and individuals with deaf-blindness who assisted with this project. We also acknowledge the financial support provided by the Ontario Ministry of Community and Social Services.

References

Dalby, D. M., Hirdes, J. P., Stolee, P., Strong, J. G., Poss, J., Tjam, E. Y., Bowman, L., & Ashworth, M. (2009). Development and psychometric properties of a standardized assessment for adults who are deaf-blind. Journal of Visual Impairment & Blindness, 103, 7-16.

Morris, J. N., Berg, K., Bjorkgren, M. A., Frijters, D., Fries, B. E., Gilgen, R., Hawes, C., Henrard, J., Hirdes, J. P., Ljunggren, G., Nonemaker, S., Phillips, C. D., & Steel, K. (2006). interRAI-Community Health Assessment (CHA): Guide for use of the interRAI CHA assessment form, Canadian version 07. Boston: interRAI.

Munroe, S. (2001). Developing a national volunteer registry of persons with deafblindness in Canada: Results from the study, 1999-2001. Port Morien, Nova Scotia: Canadian Deafblind and Rubella Association.

Ronnberg, J., & Borg, E. (2001). A review and evaluation of research on the deaf-blind from perceptual, communicative, social and rehabilitative perspectives. Scandinavian Audiology, 30, 67-77.

SAS Institute. (2001). The SAS system for Windows. Cary, NC: Author.

Watters, C., Owen, M., & Munroe, S. (2004). A study of deaf-blind demographics and services in Canada. North York, ON: Canadian National Society of the Deaf-Blind.

Dawn M. Dalby, Ph.D., assistant professor, Department of Kinesiology and Physical Education, BA 521, Wilfrid Laurier University, 75 University Avenue West, Waterloo, ON, Canada, N2L 3C5; e-mail: <[e-mail]>. John P. Hirdes, Ph.D., professor, Department of Health Studies and Gerontology, University of Waterloo, 200 University Avenue West, Waterloo, ON, Canada, N2L 3G1, and director, Homewood Research Institute, Guelph, ON, Canada; e-mail: <[e-mail]>. Paul Stolee, Ph.D., associate professor, Department of Health Studies and Gerontology, University of Waterloo; e-mail: <[e-mail]>. J. Graham Strong, O.D., M.Sc., director, Centre for Sight Enhancement and School of Optometry, University of Waterloo; e-mail: <[e-mail]>. Jeff Poss, Ph.D., research assistant professor, Department of Health Studies and Gerontology, University of Waterloo; e-mail: <[e-mail]>. Erin Y. Tiara, Ph.D., director of research, St. Mary's General Hospital, 911 Queen's Boulevard, Kitchener. ON, N2M 1B2, Canada; e-mail: <[e-mail]>. Lindsay Bowman, M.P.H., public health planner, Family and Community Resources, Region of Waterloo Public Health, 99 Regina Street South, Waterloo, Ontario, N2J 4V3, Canada; e-mail: <[e-mail]>. Melody Ashworth, M.Sc., Ph.D. candidate, Ontario Institute for Studies in Education, Department of Human Development and Applied Psychology, University of Toronto, 252 Bloor Street West, Toronto, ON, Canada, M5S 1V6; e-mail: <[e-mail]>.

Table 1
Personal characteristics of the participants, by onset of
deaf-blindness (percentages; numbers in parentheses).

                                                         Congenital
                                            All             group
Characteristic                           (n = 182)        (n = 88)

Age (mean, SD)                           42.7 (17.8)      32.1 (9.8)
Male                                     51.9 (94)        60.9 (53)
Marital status
  Never married                          72.6 (130)       93.2 (82)
  Married or significant other           14.5 (26)         4.5 (4)
  Widowed, separated,
   or divorced                           12.9 (23)         2.3 (2)
Residential status
  Private home, apartment,
    or rented room                       53.9 (98)        42.1 (37)
  Board and care or
    assisted living                       6.6 (12)         8.0 (7)
  Group home                              3.3 (6)          4.6 (4)
  Setting for persons
    with intellectual
    disabilities                          1.7 (3)          2.3 (2)
  Long-term care facility
   (nursing home)                         5.5 (10)         1.1 (1)
  Other                                  29.1 (53)        42.1 (37)
Living arrangement
  Alone                                  26.4 (48)        10.2 (9)
  With spouse or partner only             8.8 (16)         2.3 (2)
  With family                             9.3 (17)         3.4 (3)
  With parent or guardian                19.2 (35)        29.6 (26)
  With nonrelatives                      36.3 (66)        54.5 (48)

                                      Acquired group
Characteristic                            (n = 94)          p-value

Age (mean, SD)                           52.8 (18.0)       <.0001
Male                                     43.6 (41)         0.02
Marital status
  Never married                          52.8 (48)         <.0001
  Married or significant other           24.2 (22)
  Widowed, separated,
   or divorced                           23.1 (21)
Residential status
  Private home, apartment,
    or rented room                       64.9 (61)          .001
  Board and care or
    assisted living                        5.3 (5)
  Group home                               2.1 (2)
  Setting for persons
    with intellectual
    disabilities                           1.1 (1)
  Long-term care facility
   (nursing home)                          9.8 (9)
  Other                                   30.2 (17.0)
Living arrangement
  Alone                                   41.5 (39)        <.0001
  With spouse or partner only             14.9 (14)
  With family                             14.9 (14)
  With parent or guardian                  9.6 (9)
  With nonrelatives                       19.2 (18)

Table 2
Cognitive and functional status of the participants, by onset
of deafblindness (percentages; numbers in parentheses).

                                                     Congenital
Cognitive and                            All           group
functional status                     (n = 182)       (n = 88)

Cognitive Performance Scale
  Intact (0)                          31.3 (57)      12.5 (11)
  Borderline to
   moderate impairment (1-3)          34.6 (63)      25.0 (22)
  Moderately severe
    to very severe
    impairment (4-6)                  34.1 (62)      62.5 (55)
  Average score (SD)                   2.2 (1.9)      3.4 (1.6)
ADL Hierarchy Scale
  Independent (0)                     52.5 (95)      25.0 (22)
   Supervised to
   extensive assistance (1-3)         29.3 (53)      45.4 (40)
  Extensive assistance (4-6)          18.2 (33)      29.5 (26)
  Average score (SD)                   1.6 (1.9)      2.5 (2.0)
IADL Difficulty Scale
  No difficulty on any
   of the three areas (0)             24.2 (43)       9.2 (8)
  Some difficulty in one
   to three areas (1-3)               13.5 (24)       8.1 (7)
  Great difficulty in one
   to three areas (4-6)               62.4 (111)     82.8 (72)
  Average score (SD)                   3.5 (2.5)      4.8 (2.0)

                                       Acquired
Cognitive and                           group
functional status                      (n = 94)       p-value

Cognitive Performance Scale
  Intact (0)                          48.9 (46)       <.0001
  Borderline to
   moderate impairment (1-3)          43.6 (41)
  Moderately severe
    to very severe
    impairment (4-6)                   7.4 (7)
  Average score (SD)                   1.1 (1.4)
ADL Hierarchy Scale
  Independent (0)                     78.5 (73)       <.0001
   Supervised to
   extensive assistance (1-3)         14.0 (13)
  Extensive assistance (4-6)           7.5 (7)
  Average score (SD)                   0.7 (1.4)
IADL Difficulty Scale
  No difficulty on any
   of the three areas (0)             38.5 (35)       <.0001
  Some difficulty in one
   to three areas (1-3)               18.7 (17)
  Great difficulty in one
   to three areas (4-6)               42.9 (39)
  Average score (SD)                   2.3 (2.3)

Note: ADL = activities of daily living; IADL = instrumental ADLs.

Table 3
Health status of the participants, by onset of deaf-blindness
(percentages; numbers in parentheses).

                                                Congenital
                                   All            group
Health status                   (n = 182)        (n = 88)

Self-reported health
  Excellent                     12.1 (22)       12.5 (11)
  Good                          34.1 (62)       13.6 (12)
  Fair                          13.2 (24)        4.5 (4)
  Poor                           3.8 (7)         1.1 (1)
  Did not respond               36.8 (67)       68.2 (60)
Disease diagnoses
  Diabetes mellitus             10.0 (18)        8.1 (7)
  Coronary heart disease         2.2 (4)         1.1 (1)
  Cancer                         1.6 (3)         0.0 (0)
Falls
  No fall in the past
   90 days                      69.9 (123)      70.6 (60)
  None in the past
    30 days; fell 31-90
    days ago                     8.5 (15)        4.7 (4)
  One or more falls in
   the past 30 days             21.6 (38)       24.7 (21)
Depression Rating Scale
  No signs of
   depression (0-2)             80.1 (145)      76.1 (67)
  Signs or symptoms
   of depression (3+)           19.9 (36)       23.9 (21)
Pain Scale
  No pain (0)                   51.7 (94)       47.7 (42)
  Any pain (1-3)                48.3 (88)       52.3 (46)
CHESS Scale
  No health
   instability (0)              68.2 (122)      65.5 (57)
  Any health
   instability (1-4)            31.8 (57)       34.5 (30)

                                 Acquired
                                   group
Health status                    (n = 94)        p-value

Self-reported health
  Excellent                     11.7 (11)        <.0001
  Good                          53.2 (50)
  Fair                          21.3 (20)
  Poor                           6.4 (6)
  Did not respond                7.4 (7)
Disease diagnoses
  Diabetes mellitus             11.7 (11)         .43
  Coronary heart disease         3.2 (3)          .62
  Cancer                         3.2 (3)          .25
Falls
  No fall in the past
   90 days                      69.2 (63)         .17
  None in the past
    30 days; fell 31-90
    days ago                    12.1 (11)
  One or more falls in
   the past 30 days             18.7 (17)
Depression Rating Scale
  No signs of
   depression (0-2)             83.9 (78)         .19
  Signs or symptoms
   of depression (3+)           16.1 (15)
Pain Scale
  No pain (0)                   55.3 (52)         .31
  Any pain (1-3)                44.7 (42)
CHESS Scale
  No health
   instability (0)              70.7 (65)         .46
  Any health
   instability (1-4)            29.3 (27)

Table 4
The participants' hearing, vision, and communication, by onset
of deaf-blindness (percentages; numbers in parentheses).

                                                    Congenital
Hearing, vision,                       All            group
and communication                   (n = 182)        (n = 88)

Deafblind Severity Index
  No hearing or
    vision impairment                 0.5 (1)         0.0 (0)
  Mild or moderate
    impairment in one sense           2.2 (4)         2.3 (2)
  Mild or moderate
   impairment in both senses         14.8 (27)       10.2 (9)
  Severe impairment
   in one sense                       3.8 (7)         3.4 (3)
  Severe impairment in
   one sense, mild or
  moderate in the other              48.3 (88)       46.6 (41)
  Severe impairment
   in both senses                    30.2 (55)       37.5 (33)
Diagnoses related
 to vision loss
  Cataracts                          41.2 (73)       45.5 (40)
  Retinitis pigmentosa               31.7 (57)       10.2 (9)
  Myopia                             21.5 (38)       29.6 (26)
Diagnoses related
 to hearing loss
  Congenital syndromes               56.7 (97)       63.2 (55)
  Neurological damage
   due to trauma                     10.5 (18)       12.8 (11)
  Tinnitus                           10.3 (18)        4.6 (4)
Most commonly
 reported modes of
 communication
  Idiosyncratic signs,
   gestures, behaviors               57.1 (104)      78.4 (69)
  Any adapted or
   manually coded
   language (such as
   signed English
   or French, print
    on palm)                         48.9 (89)       68.1 (60)
  Speech, including
   speech reading                    43.6 (79)       23.9 (21)
  Tactile method
    for expressive
    communication (such
    as two-hand
    manual, finger
    spelling)                        42.3 (77)       43.2 (38)
  Any sign language (such
    as American Sign
    Language or Langue
    des Signes
    Quebecoise)                      40.7 (74)       34.1 (30)
Making self understood
  Understood                         37.4 (68)       15.9 (14)
  Usually understood                 14.8 (27)        5.7 (5)
  Often understood                    9.9 (18)       12.5 (11)
  Sometimes understood               25.8 (47)       44.3 (39)
  Rarely or never
   understood                        12.1 (22)       21.6 (19)
Ability to understand others
  Understands                        23.1 (42)        5.7 (5)
  Usually understands                17.0 (31)       10.2 (9)
  Often understands                  22.5 (41)       28.4 (25)
  Sometimes understands              25.3 (46)       39.8 (35)
  Rarely or never
   understands                       12.1 (22)       15.9 (14)

                                     Acquired
Hearing, vision,                      group
and communication                    (n = 94)         p-value

Deafblind Severity Index
  No hearing or
    vision impairment                 1.1 (1)          .26
  Mild or moderate
    impairment in one sense           2.1 (2)
  Mild or moderate
   impairment in both senses         19.1 (18)
  Severe impairment
   in one sense                       4.3 (4)
  Severe impairment in
   one sense, mild or
  moderate in the other              50.0 (47)
  Severe impairment
   in both senses                    23.4 (22)
Diagnoses related
 to vision loss
  Cataracts                          37.1 (33)         .26
  Retinitis pigmentosa               52.2 (48)        <.0001
  Myopia                             13.5 (12)         .009
Diagnoses related
 to hearing loss
  Congenital syndromes               50.0 (42)         .08
  Neurological damage
   due to trauma                      8.1 (7)          .32
  Tinnitus                           16.1 (14)         .01
Most commonly
 reported modes of
 communication
  Idiosyncratic signs,
   gestures, behaviors               37.6 (35)        <.0001
  Any adapted or
   manually coded
   language (such as
   signed English
   or French, print
    on palm)                         30.9 (29)        <.0001
  Speech, including
   speech reading                    62.4 (58)        <.0001
  Tactile method
    for expressive
    communication (such
    as two-hand
    manual, finger
    spelling)                        41.5 (39)         .82
  Any sign language (such
    as American Sign
    Language or Langue
    des Signes
    Quebecoise)                      46.8 (44)         .08
Making self understood
  Understood                         57.5 (54)        <.0001
  Usually understood                 23.4 (22)
  Often understood                    7.5 (7)
  Sometimes understood                8.5 (8)
  Rarely or never
   understood                         3.2 (3)
Ability to understand others
  Understands                        39.4 (37)        <.0001
  Usually understands                23.4 (22)
  Often understands                  17.0 (16)
  Sometimes understands              11.7 (11)
  Rarely or never
   understands                        8.5 (8)

Table 5
Social characteristics of the participants, by onset of deaf-blindness
(percentages; numbers in parentheses).

                                                        Congenital
                                          All             group
Social characteristics                 (n = 182)         (n = 88)

Past roles
  Strongly identifies with
    past roles and life
    status                             39.6 (72)        25.0 (22)
  Expresses sadness,
    anger, or empty feelings
    over lost roles                    32.0 (58)        18.4 (16)
Personal strengths
  Has a confidant                      45.0 (81)        27.3 (24)
  Has a consistent
   positive outlook                    68.5 (124)       60.9 (53)
  Finds meaning in
   day-to-day life                     77.4 (140)       65.5 (57)
Level of difficulty in
 social interactions
  None                                  4.6 (8)          1.2 (1)
  Minimal                              14.4 (25)         5.8 (5)
  Moderate                             13.2 (23)         5.8 (5)
  Severe                               29.9 (52)        28.7 (25)
  Very severe                          37.9 (66)        58.6 (51)
  Reports feeling lonely               41.1 (74)        24.1 (21)
Index of Social Engagement
  Not socially engaged (0)              4.5 (8)          6.9 (6)
  Mild or moderate
   social engagement (1-2)             36.9 (66)        46.0 (40)
  Moderate or high
   social engagement (3-6)             58.7 (105)       47.1 (41)

                                        Acquired
                                         group
Social characteristics                  (n = 94)          p-value

Past roles
  Strongly identifies with
    past roles and life
    status                             53.2 (50)          .0001
  Expresses sadness,
    anger, or empty feelings
    over lost roles                    44.7 (42)          .0002
Personal strengths
  Has a confidant                      62.0 (57)         <.0001
  Has a consistent
   positive outlook                    75.5 (71)          .04
  Finds meaning in
   day-to-day life                     88.3 (83)          .0003
Level of difficulty in
 social interactions
  None                                  8.1 (7)          <.0001
  Minimal                              23.0 (20)
  Moderate                             20.7 (18)
  Severe                               31.0 (27)
  Very severe                          17.2 (15)
  Reports feeling lonely               57.0 (53)         <.0001
Index of Social Engagement
  Not socially engaged (0)              2.2 (2)          0.0007
  Mild or moderate
   social engagement (1-2)             28.3 (26)
  Moderate or high
   social engagement (3-6)             69.6 (64)

Table 6
Percentage triggered on Clinical Assessment Protocols (OAPs), by
onset of deaf-blindness (percentages; numbers in parentheses).

CAPs                  All (n= 182)      Congenital group
                                            (n = 88)

Visual function        94.5 (172)          96.6 (85)
Preventive health      90.1 (164)          96.6 (85)
Cognitive decline       7.1 (13)            8.0 (7)
Physical activity      48.9 (89)           35.2 (31)
Depression             46.2 (84)           56.8 (50)
Social function        41.2 (75)           25.0 (22)
Falls                  29.1 (53)           28.4 (25)
Pain                   20.3 (37)           18.2 (16)
Abuse                  11.0 (20)            5.7 (5)
Alcohol abuse          11.0 (20)            3.4 (3)
Dehydration             9.9 (18)           13.6 (12)
Smoking                 7.1 (13)            3.4 (3)

CAPs                  Acquired group       p-value
                          (n=94)

Visual function        92.5 (87)            0.23
Preventive health      84.0 (79)            0.005
Cognitive decline       6.4 (6)             0.68
Physical activity      61.7 (58)            0.0004
Depression             36.2 (34)            0.005
Social function        56.4 (53)            <.0001
Falls                  29.8 (28)            0.84
Pain                   22.3 (21)            0.49
Abuse                  16.0 (15)            0.03
Alcohol abuse          18.1 (17)            0.002
Dehydration             6.4 (6)             0.10
Smoking                10.6 (10)            0.06