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Conditions We Treat: Superior Canal Dehiscence Syndrome

Superior canal dehiscence syndrome (SCDS) is caused by an abnormal opening in the uppermost canal of the vestibule of the inner ear. The problem occurs during fetal development if the bony surface of the ear canal fails to grow to its normal thickness. In some cases, a head trauma may occur before symptoms appear.

Symptoms of SCDS include vertigo triggered by loud noises, coughing, sneezing or straining, and loss or distortion of hearing external noises. Often, people with SCDS are hypersensitive to sounds within their own bodies and can hear their own heartbeat or the sound of their eyes moving

Superior Canal Dehiscence Syndrome: Q&A with a Johns Hopkins Expert

Learn how superior canal dehiscence syndrome is diagnosed and treated from Johns Hopkins neurotologist John Carey.

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SCDS: Why Choose Johns Hopkins

  • Researchers at Johns Hopkins discovered SCDS in 1995 and pioneered its treatment.
  • Johns Hopkins is one of the few medical centers in the world treating SCDS.
  • Advanced diagnostic and treatment technologies and a multidisciplinary approach ensure that you get the best possible treatment for SCDS and other problems affecting the inner ear. 

SCDS: Diagnosis

Diagnosing SCDS starts with a careful medical history, examination of a patient for characteristic eye movements evoked by sound, pressure hearing tests and certain vestibular tests.

A CT scan of the inner ears (temporal bones) is essential to the diagnosis. Not all openings apparent on CT are sufficient to cause symptoms or indicate a need for surgery.

Expertise in Diagnosis and Treatment

Learn more as Dr. John Carey explains how SCDS is diagnosed and treated.

SCDS: Treatment

SCDS is a rare disorder, and only a few centers have extensive experience with its diagnosis and treatment. As with most surgeries, patients should look to such centers to get the best results.

SCDS Surgery: What to Expect

The primary treatment for SCDS is surgery to close the hole in the superior canal in a way that prevents further pressure transmission between the inner ear and brain cavity. The team uses general anesthesia so you will be asleep during the procedure.

After Surgery

After your surgery you will be transported to the postanesthesia care unit.

When you wake up, you will have a bulky dressing over the ear that was operated on, and a catheter will remain in your bladder.

You can have a family member or care partner come and stay with you in the postanesthesia care unit once you are alert. Dr. Carey and the medical care team will observe you in the unit for a few hours before you go to the neurosurgical ward for recovery. There are pullout beds for your care partner to stay in the room with you overnight.

For the most up-to-date family visitation and nursing policies, please refer to the Johns Hopkins Hospital Patient and Family Handbook.

Moving around will make you feel dizzy for the first night after surgery, so it is important that you minimize movement. To reduce the risk of nausea, you will have IV fluids instead of regular food and drink for the first night.

Medications

Most patients are given two doses of IV antibiotics after surgery; longer courses have not shown to prevent infection.

Immediately after surgery, your doctor will prescribe IV narcotics given by patient-controlled push-button. This is usually only necessary for the first day after surgery. After that you will get acetaminophen, with or without a low-dose oral narcotic as needed for pain.

In addition to pain medication, you may get the following:

  • Stool softeners to prevent constipation
  • Subcutaneous heparin to prevent blood clots in the legs while you are in the hospital
  • High doses of steroids, typically dexamethasone, during and after surgery to prevent brain swelling and inner ear inflammation. Most patients take 6 milligrams to 8 milligrams, three to four times a day in the beginning, and taper off over the course of five to 10 days.

The Day After Surgery

The day after surgery, a nurse will remove the urinary catheter and you can begin eating and carefully walking with assistance. You will also begin vestibular physical therapy to train you how to move your head while stabilizing a visual target. This helps the brain to compensate for the small loss of vestibular function caused by plugging the superior canal.

Your medications will be changed from IV to oral. Most patients can eat a regular diet by the end of the day.

Day Two After Surgery

Most patients are able to walk more independently two days after surgery. You may have another vestibular physical therapy session. Based on your ability to walk and maintain balance, the medical team will assess whether or not you are ready to be discharged from the hospital. About half of patients are ready for discharge on the second day after surgery, while most of the rest are ready on the third day after surgery.

Maintaining close contact with experts in neurotology is advised for the first week to ensure you get proper care in the rare event of a complication. With that in mind, if you live locally, you can return home. If you live in the Mid-Atlantic region, your team will ensure you are close enough to return to The Johns Hopkins Hospital or another major hospital with neurotology or neurosurgery coverage before releasing you. If you live outside the region, you will be advised to stay local for one week.

You will also need to continue working with a qualified vestibular physical therapist after you are discharged from the hospital. If you live in an area where you do not have access to this specialized care, let your Johns Hopkins team know. The team can help you schedule an outpatient vestibular physical therapy visit while you are in Baltimore.

One Week After Surgery

Between five and seven days after surgery, you will return to The Johns Hopkins Hospital for a follow-up appointment with Dr. Carey and undergo a hearing test. If you have sutures that need to be removed, they will be taken out at this appointment.

Tests will check the status of your hearing and vestibular function and determine whether you will get a longer course of steroids to support your recovery.  

Your care team will also go over expectations for the following six weeks. Recommendations include:

  • Do your prescribed vestibular physical therapy exercises 30 minutes a day, several times a day, as much as you can tolerate (some fatigue is normal).
  • Avoid bending below the waist. This will prevent pressure from building up inside the head or ear and encourage proper healing.
  • Consult your vestibular physical therapist for safety clearance to drive (usually within two weeks) or return to work (after six weeks). Do not drive while taking narcotics.
  • Arrange to have a friend or family member stay with you for at least two weeks.

Six Weeks After Surgery

Approximately six weeks after surgery you will return for a follow-up visit for another hearing test. Dr. Carey will also check to be sure you are healing well and to see if there is any tightness in your jaw muscles. Most people are ready to go back to work full time by now.

If you live outside the Mid-Atlantic region, you may be able to forego the in-person visit at six weeks if you submit an audiogram. Your care team will update you through MyChart or email.

Three Months After Surgery

Approximately three months after surgery, you will return for your final follow-up visit to check your hearing one more time and assess the success of the operation.

Our Physicians

Dr. John Carey with model of the ear

Yuri Agrawal
John Carey, division director
Wade Chien
Charles Della Santina
Matthew Stewart

Sensory overload: How superpower hearing turned into a sonic nightmare

ear exam

Learn how surgeon John Carey, MD, rescued one woman from an unbearable condition

"Those sounds were unshakable. There wasn't any way to turn them down, they never went away. I could hear the bones in my neck cracking, kind of like sandpaper. At one point I could hear my eyes move."

Read more.

Patient Resources

  • Read the research papers on SCDS conducted by Johns Hopkins faculty. 
  • Ask the expert: John Carey, M.D., answers SCDS questions. Learn more