The woman who heard too much

Trees blowing, birds singing, children laughing, all things many people enjoy hearing. For one woman just about every sound was painful to hear. She had a big hearing problem caused by something very small.

Imagine hearing your own heartbeat pounding inside your head, your voice echoing. Every sound you make, every sound you hear amplified. That was the reality for Kerrie Aitken.

"My whole world just changed,” says Kerrie Aitken. “I had no control over myself anymore."

For nine-months, Kerrie suffered from dizziness, nausea, and depression. Doctors didn't know why. She was told to see a psychiatrist.

"It was way too much for her to handle," says John Aitken Kerrie’s Husband.

"She was pretty beaten down,” explains Dr. Quinton Gopen, MD, Head & Neck Surgeon at UCLA Medical Center. “She had lost a lot of weight."

UCLA Head and Neck Surgeon Quinton Gopen pinpointed the problem inside Kerrie's ear. It was smaller than a pinhead.

"The problem that Kerrie had was at the top of this balance chamber, the superior canal, a little opening in the bone formed," explains Dr. Gopen. "In the normal situation, there is still this white cap of bone there now a little tube is now opening up."

Doctor Gopen opened Kerrie's skull, moved the brain to get to her ear and filled the two millimeter hole with a bone wax, lined the area with muscle, and covered it with a tiny piece of bone from her skull.

"People wake up in tears, not from pain, but from joy because the noise is gone immediately after surgery," says Dr. Gopen.

"The first thing I did not hear was that wonderful heart beating,” describes Kerrie. “I was so happy."

Kerrie's problem is called superior canal dehiscence. The condition wasn't discovered until 1998 and it's just beginning to be taught to students in medical schools.

TOPIC: The woman who heard too much
REPORT: MB # 3547

BACKGROUND: Superhero hearing may not be such a good thing. The condition is called Superior Canal Dehiscence Syndrome (SCD). SCD (discovered in 1998) is where the vestibular and auditory symptoms and signs can result from a dehiscence (opening) in the bone overlaying the superior semicircular canal of the inner ear. Patients with SCD will experience vertigo and oscillopsia (the motion of stationary objects) with heightened loud noises and maneuvers that change middle-ear pressure (like coughing, sneezing, and straining). Auditory consequences of this syndrome include the increased resonance of one's own voice, hypersensitivity to bone-conducted sounds, and hearing loss. (Source: vestibular.org)

RISK FACTORS: With an opening in the bone that is usually supposed to cover the semicircular canal, the fluid in the membranous superior canal can be displaced by pressure and sound. Usually there are only two points of increased pressure in the inner ear: the oval window (through which sound energy is transmitted into the inner ear) and the round window (through which sound energy is dissipated from the inner ear). SCD creates a third mobile window. Symptoms of this syndrome result from the physiological consequences. Those who are diagnosed with SCD are around 45 years old. Patients can experience it unilaterally or bilaterally. About one-third have the condition bilaterally. A common notion of this SCD is that it is due to a developmental abnormality. Studies show that approximately 1-2% of the population has abnormally thin bone overlying the superior canal. A thin layer of the canal suggests that trauma or perhaps over time the pressure of the overlying temporal lobe of the brain that leads to the onset of symptoms. (Source: vestibular.org)

SYMPTOMS: Most of the time patients seek medical attention as soon as they notice the debilitating effects. Specifically, patients note that loud noises cause them to see things move and when they cough, sneeze, or strain. Sometimes patients perceive objects to be moving in the same time with their pulse. Also patients can experience a feeling of constant imbalance. (Source: vestibular.org)

NEW TECHNOLOGY: Treatment options include surgical correction for those who are severely affected by SCD. The middle cranial fossa approach has been used mostly. Plugging of the canal with fibrous tissue has been shown to be more effective than canal resurfacing. Most surgeries involve going through the cranial fossa craniotomy on the affected side (surgery through the brain). The temporal lobe is retracted. The opening in the superior canal can be covered with bone wax, bone cement, or fascia; a small procedure that can fix a life altering condition. (Source: emedicine.medscape.com)

FOR MORE INFORMATION, PLEASE CONTACT:

Quinton Gopen, MD
UCLA Medical Center
qgopen@mednet.ucla.edu


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