Fibrous dysplasia of the temporal bone: systematic review of management and hearing outcomes.

Abstract

The management options for otologic symptoms (i.e., hearing loss, otorrhea) in patients with fibrous dysplasia of the temporal bone (FDTB) include either observation or otologic surgery. The objective of this review is to describe the hearing outcomes in patients with FDTB to compare surgical intervention and conservative management to determine an evidence-based approach to patient management. Cochrane Central Register of Controlled Trials (1995-April 5, 2013), MEDLINE (January 1948-April 5, 2013), EMBASE (January 1974-April 5, 2013), conference proceedings, and the reference lists of articles. Studies describing interventions or no intervention in patients with FDTB were included. Studies failing to report hearing outcomes were excluded. Two authors independently reviewed titles and abstracts, read full-text articles, assessed quality, and extracted data. The Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines were followed. Seventy-two studies involving 215 patients were included. Thirty-one studies (n = 49 patients), including three case series and 28 case reports, had sufficient information for analysis of hearing outcomes (Level 4 evidence). The surgical intervention showing the best hearing outcomes was canaloplasty, with improvement in 84% of patients (n = 16 patients) and stable hearing thresholds in 18% (n = 3 patients). The literature lacks consistent reporting of hearing outcomes after otologic surgery in FDTB. Level 4 evidence suggests that an improvement in hearing thresholds is achievable in most patients when the disease involves the external ear canal. A management algorithm is proposed using the available evidence.