Psychological aspects related to Usher Syndrome. How low vision and hearing loss transform the personality


Writing an article about psychology in relation to disability, and in particular, to Usher syndrome is a challenge not to be underestimated. This topic has many facets that make it impossible to comprehensively deal with in one article. Also, it is a delicate issue that requires certain expertise and training.
However, my personal experience as a person with bilateral congenital hearing loss and retinitis pigmentosa, gives me enough ideas to propose some considerations.

Psychosocial dysfunction related to visual impairment develops long before blindness. When sight begins to deteriorate, problems arise such as: difficulty in reading, driving, mobility, employment, and risk of accidental injury. The worsening or unstable vision requires frequent adjustments and changes in social roles.
Loss of friends, self-esteem, independence and responsibility may intervene long before you reach legal blindness.

As described in a previous article, low vision has intrinsic versatility.
When referring to visual disturbances, most people consider only the acuity, but may not realize that perception of light, color, and depth perception can also be affected. Technology can allow you to make up for some problems, with magnifying systems and aids which assist the human capacity to generate the missing visual inputs.
Although we might have 4% visual field remaining, we might feel or think that we see more, especially when we are familiar with the environment. This happens on a cognitive level because the brain supplements our vision with the information we need, but we actually do not really see the complete picture. It takes awhile for the brain to catch up to our vision loss, which makes it harder to emotionally adjust to our decreasing sight
Hearing loss can be more or less serious, but the use of hearing aids and lip reading, also make it an invisible disability.

How to present yourself to others?
Usher Syndrome, including the two conditions that characterize it, is a relatively unknown disability, and often not easily recognizable based on physical appearance. Therefore, theoretically it would be better to let others know from the beginning its limitations and difficulties. But having to explain every time what the disease is and exposing yourself means being immediately identified as ’different’. Moreover in our culture, it is difficult to differentiate yourself from the predominant myth of the strong and efficient man, dynamic, integrated and ”perfect”.What is certain is that the benefits that a person with low vision / hearing loss may experience from hiding their condition often ends up being a lot less than the disadvantages arising from fatigue, misunderstanding, and frustration.
In any case, everyone will wonder why we crash into chairs, why we stumble, and why we do not hear. Our desire to remain anonymous means practical and psychological problems do not get solved.
It is common to try to appear normal and to feel a sense of shame and inadequacy, but I can say that I have never been faced with denigration, devaluation, or derision when I tell people about Usher Syndrome.
The reaction is rather surprise, inevitable disorientation and compassion. Only the most intelligent understand that being visually impaired / hearing impaired does not mean being inferior, but different just as two ordinary people cannot properly judge their differences because they think, see or feel in different ways.
It is obvious that acceptance must come first on a personal level to be able to find it on a social level. Anything else is merely a reflection of our attitude and our position towards the disease.

Learning of the degenerative character of retinitis pigmentosa (or other diseases of sight) is definitely a shock, and in most cases begins a search for cures, treatments, alternative medicines, solutions, hope. Unfortunately, despite some sporadic cases of improvement too subjective to be reliable, the reality is that hearing loss and retinitis pigmentosa are currently incurable, with the possibility of ending up more or less fast and sure in the dark, or worse, in a silent dark.
 Anger, depression, disinterest in life are common steps if not inevitable, but with a slow maturation process many can come to a peaceful acceptance.

Acceptance is the hardest thing in all aspects of life but with a noticeable disability, it’s harder. Years ago I fully accepted my retinitis pigmentosa; you can too, if u haven’t already. Holding on to that usable vision that’s really not usable will only prolong the inevitable. Let go of the anger, and the bitterness and be real with yourself, let yourself out of your prison, be free. (Comment by D.F. on Retinitis Pigmentosa Support Group)

Fitzgerald, R.G. suggested a theory that is one of the most comprehensive on the subject. It consists of 6 stages

a) Shock and denial: the phase following diagnosis.
We want help but we cannot ask. Start the process of denial or searching for a solution.

b) Feelings of guilt: we impute to ourselves the cause of the disease and try to identify the behaviors (or situations) triggering the disease.

c) unrealistic and magical thoughts: manifestation of the conviction that certain behaviors can resolve or reverse the damage.

d) Anger: towards ourselves and towards others.
The “cruel” fate, family members considered ”insufficiently solicitous”, the doctor ”incompetent” are the people to which we most frequently direct aggression.

e) Depression: complete awareness of the irreversibility.
This is the most delicate stage, because it can develop into a chronic phase and make way for regression to one of the previous stages. If depression does not continue, then it contributes to the acceptance.

f) Adjustment/Acceptance: acceptance is when the subject expresses a renewed interest in life and their future. We are able to talk about the condition without excessive disturbance and are willing to come into contact with others.

If and when blindness comes, it is a new beginning, made of new strategies, seeking a new identity and a new self-image.
Giving up your own habits, daily routine, changing your choices is a difficult and slow process, where sounds, contacts, smells, tastes and colors (yes, even the colors) takes on new meaning, different values . The aesthetic experience of a world heavily focused on the image, means we have to change our values or scenarios to make room for a new imagination.
On the one hand we must resign ourselves to the loss of the pleasure of seeing, on the other, we must learn to see in new ways. The vision does not end in sight, the vision is not in the eye but in the mind and heart.

This process is not necessarily about building a new ’me’, but depends on the ability to integrate past and present experiences into a single existential journey. In this way the individual will be able to keep old interests alive for the future without denying the past.



The social sources of adjustment to blindness.
Adaptation to Visual Handicap: Short-Term Group Approach
The Struggle of Blind People for Self-Determination: The Dependency-Rehabilitation Conflict : Empowerment in the Blindness Community
The social psychology of blindness
Blindness in later life: myths, attitudes and reality
Psychosocial Correlates of Mild Visual Loss
Coping With the Problems of Declining Vision Dorothy H. Stiefel


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