HOUT-22. TWO CASE REPORTS OF PATIENTS WITH SUPERIOR SEMICIRCULAR CANAL DEHISCENCE AND EHLERS-DANLOS SYNDROME

AbstractObjective:This study aimed to review the current advances in superior semicircular canal dehiscence syndrome and to ascertain its aetiology, whether dehiscence size correlates with symptoms, signs and investigation results, the best investigations, and its surgical management.Methods:A literature search using the key words ‘superior semicircular canal dehiscence’ was performed using the Allied and Complementary Medicine Database and the Embase, Health Management Information Consortium, Medline, PsycINFO, British Nursing Index, Cinahl and Health Business Elite databases for the period January 2009 to May 2014. Systematic reviews, meta-analyses, randomised controlled trials, prospective and retrospective case series, case reports, and observational studies were included.Results:Of the 205 papers identified, 35 were considered relevant.Conclusion:The aetiology of superior semicircular canal dehiscence syndrome is unclear. Dehiscence size significantly affects the air–bone gap and ocular vestibular evoked myogenic potential thresholds. Computed tomography evaluation has a high false positive rate. The middle cranial fossa approach is the surgical standard for treating this syndrome; however, the transmastoid approach is gaining popularity.

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‘Honeycomb’ tegmen: multiple tegmen defects associated with superior semicircular canal dehiscence

The Journal of Laryngology & Otology ◽

10.1017/s0022215109991411 ◽

2009 ◽

Vol 124 (5) ◽

pp. 560-563 ◽

Cited By ~ 32

Author(s):

R Suryanarayanan ◽

T H Lesser

Keyword(s):

Semicircular Canal ◽

Case Reports ◽

Relevant Literature ◽

Developmental Defect ◽

Middle Fossa ◽

Superior Semicircular Canal Dehiscence ◽

Superior Semicircular Canal ◽

Temporal Bones ◽

Honeycomb Appearance ◽

Canal Dehiscence

AbstractObjective:To report the coexistence of multiple tegmen defects, forming a ‘honeycomb’ pattern, together with dehiscence of the superior semicircular canal.Case reports:We describe three cases in which the above findings were noted, and we review the relevant literature.Conclusion:Superior semicircular canal dehiscence is defined as the absence of portions of bone over the canal along the floor of the middle fossa. Most published articles describe the defect as an isolated finding which is either unilateral or bilateral. Studies on temporal bones show either a defect over the superior semicircular canal or isolated defects over the tegmen. We describe three cases in which we found multiple tegmen defects, giving a characteristic honeycomb appearance, coexisting with dehiscence over the superior semicircular canal. This finding, which supports the theory of a developmental defect as the origin of the condition, has not previously been reported. A literature review is presented, with discussion of the aetiology and management of superior semicircular canal dehiscence.

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