Life strategies of people with deafblindness due to Usher syndrome type 2a - a qualitative study

Figures & data

Table I. Demographic data.

	Demographics	Range
N	14
Women, n (%)	4 (29%)
Age mean (SD)	41 y (13 y)	20–64 y
Hearing loss diagnosis age mean (SD)	4 y (2 y)	0,5–10 y
Vision loss diagnosis age mean (SD)	22 y (9 y)	7–42 y
Hearing loss PTA4* mean (SD)	65 dB (9 dB)	48–80 dB
Visual field category** (median)	4 (<10 degrees)	2–5
Best corrected visual acuity*** mean (SD)	0,4 (0,3)	0,05–1,0

*Hearing was assessed by Pure-tone audiometry with calculation of the pure tone average for the frequencies 0.5, 1, 2, and 4 kHz (PTA4). Thresholds were classified from mild to profound hearing loss.

**Visual field tests were performed by Goldmann perimetry and categorized into five phenotypes (1–5): category 1 was a normal visual field; category 2 the presence of a partial or complete ring scotoma (the latter either extending or not extending into the periphery); category 3 concentric central field loss with a remaining peripheral island less than one-half of the field circumference; category 4 marked concentric loss <10 degrees; and category 5, no visual field at all (blind) (Grover, Fishman, Anderson, Alexander, & Derlacki, 1997).

***Best corrected visual acuity (VA) (the ability to discriminate details) was measured by Snellen chart-based standard tests, given in decimals, 1.0 indicating normal VA whereas 0.05 is a severely reduced VA (in the US VA ≤ 0.1 is defined as legal blindness).

Table II. Theme, sub-themes categories and sub-categories.

Theme	Sub-themes	Categories	Sub-categories
Being at the helm	Resolving or preventing challenges	Remaining active	Business as usual’
			Adapting activities
			Using memory and attention
		Using devices	Accessibility aids
			Everyday tools
		Using support	Formal support
			Informal support
		Sharing knowledge	Informing in everyday situations
			Educating professionals
	Comforting oneself	Appreciating the present	Seize the moment
			Enjoy meaningful activities
		Maintaining a positive image	Negotiate who I am
			Stand up for myself
			Boost self-confidence
		Alleviating emotional pain	Self-distance
			Escapism
			Hope

Grover, S., Fishman, G. A., Anderson, R. J., Alexander, K. R., & Derlacki, D. J. (1997). Rate of visual field loss in retinitis pigmentosa. Ophthalmology, 104(3), 460–465.

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