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Although studies demonstrate 4 to 20% of patients with pulsatile tinnitus (PT) have associated sigmoid sinus anomalies, no consensus exists regarding optimal management. Our objective was to perform a systematic review exploring surgical and endovascular intervention of PT caused by sigmoid sinus anomalies.A systematic review was performed using the Preferred Reporting Systems for Systematic Reviews and Meta-Analysis guidelines for reporting of results, with a target population encompassing patients with PT and either sigmoid sinus diverticulum or sigmoid wall dehiscence. From an initial search yielding 74 articles, 21 manuscripts met inclusion criteria.Of 139 patients, 90.4% were female. Mean age was 39.0 years. Diagnosis was sigmoid sinus diverticulum/aneurysm in 47.5% of patients, sigmoid sinus dehiscence in 35.3% of patients, and both in 17.3%. Sigmoid sinus wall reconstruction/resurfacing (SSW R/R) was used in 91.4% and endovascular procedures in 7.9% of patients. Postoperative recurrence was 3.5% (mean follow-up 21.1 m). Although there was no association between resolution rate and age or sex, right-sided PT resolved at a higher rate. For every increase in body mass index by 1 kg/m2, the odds of PT resolution increased 9.2%.PT as a result of sigmoid sinus diverticula, aneurysms, and dehiscence is a rare, but largely treatable condition. Available interventions include SSW R/R, endovascular intervention, and cardiac U-clip techniques. In SSW R/R, bone pate, unspecified soft-tissue graft, and bone cement had the highest rates of PT resolution. While temporalis fascia and autologous bone chips were the materials most commonly used, they had significantly lower rates of PT resolution compared with the other materials, with the exception of auricular cartilage and bone cement. Most episodes of recurrence are resolved with medical management or a revision procedure. This study serves to summarize the current state of knowledge on the treatment of pulsatile tinnitus across disciplines.