When Bone Forms Beyond Bone: An Otologic Diagnostic Dilemma in a 28-Year-Old Woman with Fibrodysplasia Ossificans Progressiva (Stone Man Syndrome)
Keywords:
fibrodysplasia ossificans progressiva, stoneman syndrome, hearing lossAbstract
Objective: To describe the audiologic findings and diagnostic challenges of hearing loss in a patient with fibrodysplasia ossificans progressiva (FOP).
Design: Case Report.
Setting: Tertiary Private University Training Hospital
Patient: One.
Results: A 28-year-old Filipino woman with a known diagnosis of FOP presented with a 12-year duration of bilateral hearing loss. Otoscopic examination demonstrated patent external auditory canals and intact, non-hyperemic tympanic membranes bilaterally. Pure-tone audiometry revealed moderate sensorineural hearing loss in the right ear and severe mixed hearing loss in the left ear. Tympanometry showed a Type As tracing on the right and a Type B tracing on the left. High-resolution computed tomography of the temporal bones showed no ossicular chain fixation, or middle ear mass, failing to demonstrate a structural correlate for the degree of hearing loss observed. The use of hearing aids for amplification provided benefit.
Conclusion: This case highlights the complexity of evaluating hearing loss in FOP, where audiometric and immittance findings may not correlate with otoscopic or radiologic features. Recognition of this variability is essential to avoid unnecessary invasive surgical interventions and to emphasize conservative management, including hearing rehabilitation. FOP-related hearing loss likely represents a multifactorial process involving ossicular stiffness, altered middle ear compliance, and potential cochlear biomechanical dysfunction. High-resolution imaging may underestimate middle ear pathology, necessitating reliance on comprehensive audiologic assessment. Surgical intervention should be approached with extreme caution due to the risk of heterotopic ossification flare-ups.
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