A Maple Grove, Minnesota woman Stephanie Schmitz, has been diagnosed with Superior Canal Dehiscence Syndrome (SCDS), a rare condition of the inner ear in which an abnormal opening in the bone covering part of the balance system causes unusual hearing and balance symptoms. In her case, the condition has led her to hear internal body sounds, including the movement of her own eyeballs, muscles creaking, a phenomenon that has significantly affected daily life and sensory experience.
Schmitz was diagnosed with Superior Canal Dehiscence Syndrome (SCDS) in early 2021. Unlike most cases that involve the superior canal, Stephanie’s condition affects the posterior canal, an even rarer and more complex presentation. She experiences symptoms in both ears, more severe on the right including dizziness, sound-induced vertigo, extreme sound sensitivity, brain fog, fatigue, tinnitus, and hearing internal body sounds, at times leaving her nearly immobile. The condition is progressive, has no effective medical treatment, and surgery is the only option, though it carries significant risks and uncertain outcomes, along with a long recovery period and need for rehabilitation.
SCDS has profoundly affected every aspect of Stephanie’s life, particularly her ability to work, participate in daily activities, and care for her 9-year-old son, as even routine sounds or movements can worsen her symptoms hours or days later. The unpredictability of flare-ups limits planning and social engagement, despite precautions such as ear protection and avoiding noisy environments. Accessing specialized care is another major challenge, as experienced surgeons are largely out of state (Minnesota) and often out of network, leaving most costs, including consultations, travel, surgery, recovery, and therapy uncovered by insurance.
Superior Canal Dehiscence Syndrome is a rare inner ear disorder resulting from a hole between the brain cavity and the superior semicircular canal, one of three fluid-filled structures in the inner ear. The hole is normally covered with dura (lining of the brain cavity). Normally, sound waves travel through the outer and middle ear into the cochlea for hearing, while the semicircular canals sense head motion and contribute to balance. An opening in the bone disrupts normal fluid and pressure dynamics, allowing sounds and pressure changes to enter the inner ear in the wrong direction.
This abnormal communication can alter the way the brain processes sound and balance signals, leading to the unique symptoms associated with SCDS.
Individuals with SCDS may experience a range of auditory and vestibular symptoms, including:
Autophony: Hearing internal sounds, such as one’s own voice, heartbeat, breathing, or eyeball movements, unusually loudly or clearly.
Hyperacusis: Heightened sensitivity to external sound.
Dizziness or vertigo: Triggered by loud noises or changes in pressure (e.g., coughing, sneezing).
Feeling of ear fullness or pressure.
Balance instability and motion sensations.
In Stephanie's case, the patient described everyday sounds, including internal bodily sounds in a much more pronounced way than typical hearing experiences.
Diagnosing SCDS generally involves a combination of clinical history, hearing and balance testing, and advanced imaging:
A hearing test (audiogram) to assess auditory function.
Vestibular evoked myogenic potential (VEMP) testing for balance system response.
High-resolution CT scan of the temporal bone to identify the bony dehiscence.
Because the condition is uncommon and symptoms overlap with other ear disorders, careful evaluation by ear-nose-throat (ENT) specialists or neurotologists is often necessary to confirm the diagnosis.
Not all individuals with SCDS require surgery. Options include:
Lifestyle and behavioral modifications: Avoiding activities that trigger symptoms (e.g., loud noises, heavy lifting).
Vestibular therapy: Physical therapy aimed at improving balance and reducing dizziness.
Surgical repair: In severe cases, surgeons may plug or resurface the abnormal opening in the bone to reduce symptoms and restore more normal inner ear function. Studies show that 70 % to 90 % of patients report symptom improvement after surgery.
Although the exact cause of SCDS remains unclear, research suggests it may be congenital or develop due to bone thinning or trauma, and is more likely to be diagnosed as imaging techniques improve.
Superior Canal Dehiscence Syndrome is a rare and complex condition of the inner ear that can cause unusual auditory experiences and balance disturbances. In cases such as the Maple Grove woman’s, symptoms like hearing one’s own eyeballs move reflect the unique ways SCDS can interact with the body’s sensory systems. Accurate diagnosis and specialist care are crucial for SCDS.