Usher syndrome is the most common cause of deafblindness in people under 65 years of age, accounting for around fifty percent. In most cases, Usher syndrome is the combination of congenital deafness and acquired low vision that degenerates over time. It may be associated with balance disorders and cataracts. The hearing loss is usually stable, but subtypes have been identified where this, too, degenerates. The loss of vision over time, usually starting in the first or second decade of life, is caused by retinitis pigmentosa.
There is very little awareness among health and social care professionals and practitioners, so I and two colleagues have decided to write a book about Usher but by people with usher (or parents/partners/children /siblings of). What better way to showcase how capable people with dual sensory impairment or deafblindness are than by writing a book for health and social care students at universities and colleges, and writing it ourselves: Nothing about us without us.
Emma Boswell and I (both with usher syndrome) are meeting in West Sussex to plan out the guts and direction of the usher syndrome book. We are meeting by Zoom with Emily Shepherd, our third author, from Melbourne in a few days to get her feedback and finalise who will do what and by when. One of the interesting aspects of working with people with dual sensory impairments is how we match up our communication methods and preferences.
This meeting with Emma and myself is a good example of that. Emma uses British sign language and can read large print if not too fatigued. I on the other hand use hearing aids (and they just aren’t enough) plus speech-to-text systems of varying quality and temperament (some won’t work in noisy environments and some have really crazy transcriptions, such as puppy for coffee or pizza for Peter or killer for carer). This can lead to much confusion and or much laughter.
So today we have an interpreter (for Emma), an accessibility assistant for Annmaree and assorted devices. My assistant Suzanne gets inspiration, the room is reasonably quiet, so we use a speech-to-text app called AVA. It turns out that Emma can read this easily so we have it on the iPad on a stand between the two of us. The interpreter backs up what is being transcribed on the iPad by AVA for Emma, and Suzanne keeps an eye on what the transcription is doing and saying to ensure it remains meaningful and sensible. We have a few laughs about some of the misinterpretations but mostly it goes well.
Such negotiations of communication are common when two or more people with dual sensory impairment-deafblindness get together. And it underscores an important point that we all need to be flexible and creative when it comes to accessibility. What works for one person may not work for another. We also have to have patience in negotiating how a communication encounter will work, especially because assistive technology doesn’t always work. At a recent meeting, Haben Girma, the first deafblind law graduate from Harvard, also made this point about the importance of being patient. Relaying communication via interpreter or communication guide or using assistive methods may take longer and involve the occasional breakdown.
The usher syndrome book is due for release at the end of 2025 with Lived Places Publishing. We shall keep you posted on its progress.