BackgroundSuperior canal dehiscence syndrome (SCDS) can be treated surgically in patients with incapacitating symptoms. However, the ideal treatment has not been determined.ObjectivesThis systematic literature review aims to assess available evidence on the comparative effectiveness and risks of different surgical treatments regarding: (1) symptom improvement; (2) objectively measurable auditory and vestibular function; (3) adverse effects, and (4) length of hospitalization.Search method and data sourcesA systematic database search according to PRISMA statement was conducted on Pubmed, Embase, and Cochrane library. In addition, reference lists were searched. No correspondence with the authors was established. The last search was conducted on June 9, 2017.Study eligibility criteriaRetrospective and prospective cohort studies were held applicable under the condition that they investigated the association between a surgical treatment method and the relief of vestibular and/or auditory symptoms. Only studies including quantitative assessment of the pre- to postoperative success rate of a surgical treatment method were included. Case reports, reviews, meta-analysis, and studies not published in English, Dutch, or German were excluded.Data collection and analysisThe first author searched literature and extracted data; the first and last analyzed the data.Main resultsSeventeen studies (354 participants, 367 dehiscences) met the eligibility criteria and were grouped according to surgical approach. Seven combinations of surgical approaches and methods for addressing the dehiscence were identified: plugging, resurfacing, or a combination of both through the middle fossa (middle fossa approach); plugging, resurfacing, or a combination of both through the mastoid (transmastoid approach); round window reinforcement through the ear canal (transcanal approach). Several studies showed high internal validity, but quality was often downgraded due to study design (1). Outcome measures and timing of postsurgical assessment varied among studies, making it unfeasible to pool data to perform a meta-analysis.ConclusionA standardized protocol including outcome measures and timeframes is needed to compare the effectiveness and safety SCDS treatments. It should include symptom severity assessments and changes in vestibular and auditory function before and after treatment.