TY - JOUR

T1 - Pink hearing aids and purple shampoo: biographical implications of Waardenburg syndrome type-1

AU - Wheeler, Sara

PY - 2017/7/17

Y1 - 2017/7/17

N2 - I have Waardenburg syndrome type 1 (WST1), a rare genetic disorder caused by mutations in the PAX3 gene. It is characterised by varying degrees of sensorineural hearing loss, unusual facial features, and distinctive eye, hair and skin colour – sometimes described as pigmentary abnormalities; these may be present at birth or else manifest during the early life-course. I was born hearing into a Deafhearing family, gradually developing low frequency hearing loss, in itself unusual, made more so by age of onset. My once dark hair has turned prematurely silver and my eye and skin colour have also altered. I exhibit facial characteristics of WST1, notably dystopia canthorum. I received genetic counselling and initially experienced onset of changes as biographical disruption, inkeeping with the predominantly negative framing of WST1. In recent years, however, I have embraced the changes and have begun following their progress with interest. I have incorporated the characteristics into my persona, choosing pretty hearing aids, and using purple shampoo to emphasise my silver hair. In this way, I am treating WST1-related changes as a process of biographical emergence and re-invention, rather than disruption. I present my experiences as an autopathographic case study and narrative of the associated biographical implications.

AB - I have Waardenburg syndrome type 1 (WST1), a rare genetic disorder caused by mutations in the PAX3 gene. It is characterised by varying degrees of sensorineural hearing loss, unusual facial features, and distinctive eye, hair and skin colour – sometimes described as pigmentary abnormalities; these may be present at birth or else manifest during the early life-course. I was born hearing into a Deafhearing family, gradually developing low frequency hearing loss, in itself unusual, made more so by age of onset. My once dark hair has turned prematurely silver and my eye and skin colour have also altered. I exhibit facial characteristics of WST1, notably dystopia canthorum. I received genetic counselling and initially experienced onset of changes as biographical disruption, inkeeping with the predominantly negative framing of WST1. In recent years, however, I have embraced the changes and have begun following their progress with interest. I have incorporated the characteristics into my persona, choosing pretty hearing aids, and using purple shampoo to emphasise my silver hair. In this way, I am treating WST1-related changes as a process of biographical emergence and re-invention, rather than disruption. I present my experiences as an autopathographic case study and narrative of the associated biographical implications.

KW - Waardenburg syndrome type 1; hearing loss; Deaf; biographical disruption; autoethnography; autppathography; hearing aids; Pigment;

M3 - Article

JO - Auto/Biography Yearbook: The Annual Journal of the British Sociological Assocation Study Group on Auto/Biography

JF - Auto/Biography Yearbook: The Annual Journal of the British Sociological Assocation Study Group on Auto/Biography

SN - 2040-2996

ER -