History

Patients with superior canal dehiscence syndrome (SCDS) usually present with symptoms of sound- or pressure-induced dizziness. Chronic imbalance is another symptom of SCDS. The patient's symptoms may be re-created when sound or pressure is presented to the affected ear. Patients often control these symptoms with strict avoidance of noisy environments. In addition, some patients may hear a swishing noise when they move their eyes in a certain direction. This gaze-evoked tinnitus can be found in almost 25% of patients with SCDS. Hyperacusis is defined as an unusual sensitivity to normal everyday sounds. Although not specific, this condition is found in a high percentage of patients with SCDS.

Physical

After a general head and neck examination is performed, a detailed neurootological examination should be performed in all patients with vertigo. Other, more common, causes of vertigo and imbalance must be eliminated before SCDS can be diagnosed. Note the following tests and examinations:

Gait test: Determine whether the patient staggers or is off-balance with gait. Typically, patients with SCDS demonstrate a normal gait pattern.
Oculomotor examination: All patients must be assessed for an intranuclear ophthalmoplegia and other signs of multiple sclerosis. In addition, gaze-dependent nystagmus must be eliminated as a cause of the imbalance. Nystagmus of peripheral (ie, labyrinthine) origin is usually unidirectional. Nystagmus of central origin (ie, brainstem) is usually bidirectional. Patients with SCDS do not typically demonstrate nystagmus upon routine examination. Nystagmus can be induced with loud sounds or with a pressure fistula test.
Romberg test: This test is used to evaluate peripheral sensation, dorsal column function, and midline cerebellar function. The results of this test are usually abnormal in patients with a central pathologic condition. Patients with SCDS demonstrate normal Romberg test results.
Fukuda test: Patients who undergo this test are asked to step in place for 20-30 seconds with their eyes closed. Rotation of the patient may indicate a unilateral loss of vestibular function. The results of this nonspecific test are typically normal in patients with SCDS.
Dix-Hallpike maneuver: The Dix-Hallpike maneuver is performed by laying a patient back suddenly with the patient's head turned to one side. The test results are considered abnormal (or positive) if the examiner sees geotropic or ageotropic rotatory nystagmus that typically lasts less than 60 seconds. An abnormal or positive Dix-Hallpike examination result is most likely due to benign paroxysmal positional vertigo (see the Medscape Reference article Benign Paroxysmal Positional Vertigo). Patients with SCDS do not typically have positive Dix-Hallpike test results.
Head-shake test: The patient wears Frenzel lenses, and the examiner shakes the patient's head at approximately 1 Hz in the horizontal plane for 20 seconds. After the shaking is stopped, the eyes are observed for nystagmus. This test can reveal latent nystagmus and indicate which labyrinth is malfunctioning. In this test, the fast phase of nystagmus is directed toward the normal (or better-performing) labyrinth. The results of this nonspecific test may be abnormal in patients with SCDS.
Head-thrust test: The patient is asked to gaze steadily at a target in the room while the examiner briskly moves the patient's head from side to side. If the patient's eyes remain fixed on the target, the test result is normal. When the eyes make a compensatory movement after the head is stopped to reacquire the target (a refixation saccade), the test results are abnormal. This test can indicate if the output of one or both labyrinths is depressed. This is usually not a typical finding in SCDS.
Visual dynamic acuity test: Before and during vigorous vertical shaking, followed by horizontal head shaking, the patient is asked to read the smallest visible line on the Snellen eye chart. A normal result is the ability to maintain acuity within 2 lines of the acuity at rest. An abnormal visual acuity test suggests bilateral vestibulopathy, which is most commonly observed in ototoxicity. The dynamic visual acuity test results are normal in patients with SCDS.
Fistula test: The fistula test is designed to elicit symptoms and signs of an abnormal connection between the labyrinth and the surrounding structures. This is usually performed while the patient wears Frenzel lenses. Pressure can be applied to the patient's ear by pushing the tragus over the ear canal or with the use of a Bruening otoscope. If vertiginous symptoms are elicited or if nystagmus is seen the patient has positive fistula test results. In SCDS, the superior canal can be thought of as a fistula with connection to the middle cranial fossa. With SCDS, the direction of the nystagmus, as a result of pressure applied to the ear, results in vertical-torsional nystagmus with slow waves directed away from the labyrinth suspected of being dehiscent. Other tests, such as the pinched nostril test, can demonstrate similar findings, whereas a Valsalva maneuver produces nystagmus in the opposite direction.
Eye movements evoked by sound are found in most patients with SCDS. These sound-induced eye movements are typically found at frequencies of 500-2000 Hz, with an intensity of 100-110 dB. The eye movements are typically vertical and torsional, away from the side of the stimulus.
A Barany noise box can also be used to help elicit the noise-induced vertigo (Tullio phenomenon). This commercially available box simply makes a loud (100 dB) noise. When the box is slowly moved towards the patient's symptomatic ear, the vertiginous symptoms may be re-created.

Causes

An embryological etiology of SCDS has been proposed; this theory involves a postnatal failure of bone formation over the superior semicircular canal. Tsunoda and Terasaki, with the use of a computer simulation model, determined that the cause of bony dehiscence of the superior semicircular canal was due to a malpositioned primitive otocyst.^[5]When this otocyst lies too close to the developing brain, the migratory patterns of the loose reticular cells are altered. These mesenchymal cells are thought to be necessary for completion of the bony development of the labyrinthine structures. This region may be left with incomplete or thin bony development over the superior semicircular canal.

Differential Diagnoses

Chilvers G, McKay-Davies I. Recent advances in superior semicircular canal dehiscence syndrome. J Laryngol Otol. 2015 Mar. 129 (3):217-25. [QxMD MEDLINE Link].
Minor LB. Superior canal dehiscence syndrome. Am J Otol. 2000 Jan. 21(1):9-19. [QxMD MEDLINE Link].
Kartush JK. Superior Canal Dehiscence Syndrome symptoms resolved by reinforcement of the oval and round windows. Unpublished data. 2002.
Castellucci A, Piras G, Del Vecchio V, et al. The effect of superior canal dehiscence size and location on audiometric measurements, vestibular-evoked myogenic potentials and video-head impulse testing. Eur Arch Otorhinolaryngol. 2020 Jun 26. [QxMD MEDLINE Link].
Tsunoda A, Terasaki O. Dehiscence of the bony roof of the superior semicircular canal in the middle cranial fossa. J Laryngol Otol. 2002 Jul. 116(7):514-8. [QxMD MEDLINE Link].
Brantberg K, Verrecchia L. Testing vestibular-evoked myogenic potentials with 90-dB clicks is effective in the diagnosis of superior canal dehiscence syndrome. Audiol Neurootol. Sep 2009. 14(1):54-8. [QxMD MEDLINE Link].
Verrecchia L, Westin M, Duan M, Brantberg K. Ocular vestibular evoked myogenic potentials to vertex low frequency vibration as a diagnostic test for superior canal dehiscence. Clin Neurophysiol. 2016 Jan 12. [QxMD MEDLINE Link].
White JA, Hughes GB, Ruggieri PN. Vibration-induced nystagmus as an office procedure for the diagnosis of superior semicircular canal dehiscence. Otol Neurotol. 2007 Oct. 28(7):911-6. [QxMD MEDLINE Link].
Lee GS, Zhou G, Poe D, Kenna M, Amin M, Ohlms L, et al. Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children. Laryngoscope. 2011 Oct. 121(10):2256-61. [QxMD MEDLINE Link].
Brantberg K, Bergenius J, Mendel L, Witt H, Tribukait A, Ygge J. Symptoms, findings and treatment in patients with dehiscence of the superior semicircular canal. Acta Otolaryngol. 2001 Jan. 121(1):68-75. [QxMD MEDLINE Link].
Banakis Hartl RM, Cass SP. Effectiveness of Transmastoid Plugging for Semicircular Canal Dehiscence Syndrome. Otolaryngol Head Neck Surg. 2018 Mar. 158 (3):534-40. [QxMD MEDLINE Link].
Silverstein et al. Round Window Reinforcement for Superior Canal Dehiscence. Oto-HNS. Sept 2012. 147:93.
Silverstein H, Kartush JM, Parnes LS, Poe DS, Babu SC, Levenson MJ. Round window reinforcement for superior semicircular canal dehiscence: A retrospective multi-center case series. Am J Otolaryngol. 2014 May-Jun. 35(3):286-93. [QxMD MEDLINE Link].
Barber SR, Cheng YS, Owoc M, et al. Benign paroxysmal positional vertigo commonly occurs following repair of superior canal dehiscence. Laryngoscope. 2015 Dec 15. [QxMD MEDLINE Link].
Gianoli GJ, Soileau JS. The dehiscent middle fossa: prevalence, manifestations, associated findings and results of 24 surgical explorations for superior semicircular canal dehiscence. Publication pending: presented as triologic thesis, 2005.

Media Gallery

Coronal high-resolution computed tomography scan (1-mm sections) that demonstrates the presence of the superior semicircular canal (figure A, black arrow). As the scan is followed posteriorly (figures B, C, D), the bony dehiscence over the superior canal (black arrow) becomes more apparent.

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Author

Wayne T Shaia, MD Director, The Balance and Ear Center

Disclosure: Nothing to disclose.

Coauthor(s)

Jack M Kartush, MD Clinical Professor, Department of Otolaryngology, Wayne State University School of Medicine; Professor Emeritus, Michigan Ear Institute at Providence Hospital; Clinical Professor, Oakland University William Beaumont School of Medicine

Jack M Kartush, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Otological Society, American Neurotology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Gerard J Gianoli, MD Clinical Associate Professor, Departments of Otolaryngology-Head and Neck Surgery and Pediatrics, Tulane University School of Medicine; President, The Ear and Balance Institute; Board of Directors, Ponchartrain Surgery Center

Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, Triological Society

Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan; Ryte; Neosoma; MI10<br/>Received income in an amount equal to or greater than $250 from: Neosoma; Cyberionix (CYBX)<br/>Received ownership interest from Cerescan for consulting for: Neosoma, MI10.

Additional Contributors

Robert A Battista, MD, FACS Assistant Professor of Otolaryngology, Northwestern University, The Feinberg School of Medicine; Physician, Ear Institute of Chicago, LLC

Robert A Battista, MD, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, Illinois State Medical Society, American Neurotology Society, American College of Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

Angela Shu-Yuen Peng, MD Resident Physician, Department of Otolaryngology/Head and Neck Surgery, Virginia Commonwealth University Medical Center

Angela Shu-Yuen Peng, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, and Virginia Society of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.