Mike's Story

Up until recently, I was completely unaware of Waardenburg Syndrome (WS). I went through most of my life basically deaf in one ear. It had never occurred to me that a hearing loss, in combination with other non-impairing characteristics, could be the manifestations of a genetic syndrome. I am now a 60-year-old retired man, and a father of twin boys who are now adults. Neither of them exhibit characteristics of Waardenburg Syndrome at this time. My characteristics include hearing loss in my right ear, blue eyes, prematurely gray hair, and vertigo (dizzy spells).

I have a profound hearing loss in my right ear. I do not recall any difficulty hearing until about age 20. I had been sitting next to someone. They were continuously talking, and I soon realized that although that person had been speaking, I had heard nothing. I soon made the connection between someone sitting on my right side and the possibility of hearing loss in my right ear. This instantly became a problem that needed medical attention. I saw a doctor who was able to confirm that I had a loss of over 90% in my right ear. Thereafter, I did not seek any further testing or treatment for my hearing loss. I merely decided to carry on with life using my left ear as my hearing source. To this day, I still turn my head to listen with my “good ear.”

The greatest difficulty that I have living with a unilateral profound hearing loss includes listening in noisy situations, or situations where there is a lot of background noise. I also have difficulty localizing sound, or being able to identify which direction sound is coming from. Finally, hearing loss especially affects me when others have attempted to communicate with me, but I simply did not hear them. Then, I am often taken as being rude or ignorant for not responding. This is quite frustrating to me. On the other hand, my sons understood my hearing loss (and often took advantage of it as you might imagine sons would do to their father).

My WS-characteristic hair had prematurely turned gray while I was in my mid-30's. Currently, my gray hair is darker in some areas than others, which is typical in WS. My mother’s hair had also turned gray prematurely. I have my mother’s gray-blue eyes, but my father had bright blue eyes. My sister has multicolored eyes, or heterochromia iridis. This is also indicative of WS. Another characteristic of WS is the occurence of moles, or multiple nevi. I have some moles and my sister has over 50.

Vertigo, or dizzy spells, is another problem that occurs for me. Sometimes the dizziness gets so intense that I feel as though I am going to fall down. These spells have been occurring for a long time, and often in an irregular pattern. Sometimes I will have dizzy spells for several days, and then none for several months. My sister experiences the same dizzy spells, but of greater intensity.

I met with Dr. Kahn at Miami University’s Speech and Hearing Clinic. She helped me make the connections between my and my family’s characteristics that may be indicative of Waardenburg Syndrome. Now that I am aware of Waardenburg Syndrome, I am interested in looking more and more into the genetic link between WS and my family. Dr. Kahn also informed me of the genetic testing available to confirm the type of WS that I may have. I am also now choosing to finally follow up on my hearing loss and have an audiological evaluation. In the end, having a specific name for what I have lived with my entire life is much more interesting than anything negative. I especially realize the importance of informing my sons and family members so that they can be aware of the possibly occurring characteristics of WS, including hearing loss.

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