Superior canal dehiscence syndrome (SCDS) is a rare condition affecting the ear that causes people afflicted with it to hear sounds inside their body. Some people report hearing their heart, pulse, or the very movement of eyes within their eye sockets.
The medical case of ESPN sportscaster Sean McDonough is drawing attention to a rare ear condition that is often misdiagnosed. Sean was hit on the head while telecasting a golf tournament and immediately experienced a loud squealing noise in his left ear that soon subsided to a dull roar. He was later diagnosed with superior canal dehiscence syndrome (SCDS), a rare condition affecting the ear that causes people afflicted with it to hear sounds inside their body. Some people report hearing their heart, pulse, or the very movement of eyes within their eye sockets. It was first described in 1998 by physicians at Johns Hopkins University.
SCDS is caused by a small hole in the bone covering part of the inner ear causing the inner ear to come into contact with the lining of the brain. It can result in distortion of hearing and impaired balance. In Sean’s case, it appears the tap to his head by the putter caused the small hole that led to his condition. In other patients, the genesis of the condition may not be so apparent. Some patients are told they were crazy when they reported hearing weird sounds in their head or were diagnosed with unexplained vertigo or tinnitus. The popular show “Grey’s Anatomy” even featured an episode where a patient with SCDS was regarded as schizophrenic until she was correctly diagnosed.
The cause of SCDS is unclear, but one theory posits that about 1 or 2 percent of the population fails to develop a normal thickness of bone overlying the superior canal. That bone can be eroded over time due to pressure from the brain sitting on top of it or changes in intracranial pressure. Trauma, even slight in the case of Sean, may also cause a perforation in the bone. The median age of onset is in the early 40s, and it does tend to affect men and women equally. Surgery, required to plug the hole, is done by Dr. Doherty and myself and can significantly relieve and possibly cure the symptoms.
To read more about Sean’s case, click here.