Rob's Story

My name is Rob. I was born with unilateral deafness. I am now 36 years old and only discovered recently that my hearing loss is caused by the Waardenburg syndrome.

I was in fact not diagnosed as profoundly deaf in one ear until the age of 8. I remember as a child telling my mother that when I was in the bath it sounded different when I immersed my right ear compared to when I immersed my left ear, but she didn’t pay much attention. During a medical exam at school when I was 8 years old the nurse discovered that I really couldn’t hear her watch ticking with my right ear. After that I had several medical exams in the hospital. All they could conclude then (in 1980) was that the cause of the profound deafness in my right ear was not due to middle ear problems, but had a sensorineural origin and was probably hereditary. And that was it for the following 27 years of my life. I was born in the Netherlands, but grew up in France far away from my uncles, aunts, and cousins. We migrated back to the Netherlands in 1986.

In 2001 my nephew was diagnosed with a hearing loss (in both ears), which we all thought was peculiar since my brother and his wife had no hearing loss at all. A few years later the diagnosis was that my nephew was profoundly deaf. By that time I had two daughters with not any sign of hearing loss.

In January 2007 our third daughter Ella was born. Five days after her birth her hearing was tested and she didn’t respond. She is profoundly deaf in both ears. The otologist who examined her at the institute for the deaf also knew about my nephew. When we asked him how it was possible  that the hearing loss was getting worse from one generation to the next, he started asking questions that to us didn’t seem to have any relation with the subject. Did we have family members with a white forelock? Or family members with two differently coloured eyes? He was the first person to mention the Waardenburg syndrome as a possible cause of her hearing loss. After reading more about the syndrome and thinking it through, it was like the pieces of a big puzzle finally coming together.

The pattern of hearing loss in my family from my mothers side concords with what can be expected with Waardenburg syndrome type 2. My mother has 13 brothers and sisters, of whom 11 are married and have children and grandchildren. My mother and two of her sisters have always had a significant hearing loss. Several of her sisters had prematurely grey hair (before the age of 30). I have a cousin with unilateral deafness and eyes with two different colours. As mentioned above my brother’s son is profoundly deaf in both ears. Ella my daughter has beautiful bicolour eyes (blue and brown). A cousin, who has himself prematurely grey hair, has one child with significant hearing loss and another child that is profoundly deaf in both ears.

I understand that the fact that this condition is called a syndrome might be frightening to some people. I have also been advised to be careful in sharing this “revelation” with other family members that don’t know about it yet. Personally I was relieved when I finally (after 27 years) was given a probable cause for my hearing loss. I also think this will be very important for Ella and my nephew, when they will be old enough to start asking why they are different.  Knowing what is really causing it demystifies the condition.

Ella is now 1 year old and is doing really well. Soon she will undergo surgery for a cochlear implant.

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