Otologic Disease in Polyostotic Fibrous Dysplasia and McCune-Albright Syndrome

Abstract

Objective: 1) Characterize the spectrum of otologic disease in patients with polyostotic fibrous dysplasia (PFD) and McCune-Albright Syndrome (MAS). 2) Correlate audiometric analysis with temporal bone computed tomography (CT) findings in this cohort.

Method: A retrospective review was performed in a cohort of PFD/MAS (n = 123) followed in a longitudinal natural history protocol. Patients with craniofacial involvement and those who underwent comprehensive otologic evaluation, audiologic analysis, and cranial CT imaging evaluation were included.

Results: Complete evaluations were available for 107 patients. Fibrous dysplasia (FD) of the temporal bones was identified in the majority (71%). Clinically significant hearing loss (.5/1/2/4 kHz PTA >20 dB HL) was identified in 30 ears (20%). A conductive loss (70%) was more frequent than a sensorineural loss (30%) and overall was primarily mild (70%). External auditory canal stenosis, epi-tympanic involvement, and internal auditory canal involvement based on CT were found to be rare potential contributors to overall hearing loss (n = 10 ears). While canal cholesteatoma can be a major complication, it was only identified in one patient.

Conclusion: FD of the temporal bones in patients with PFD/MAS was common. Clinical manifestations depend on the location and degree of skeletal involvement. Otologic problems and hearing loss were present in a minority and were generally mild. Serious complications were rare. Conservative management may often be the best plan of care.