Add like
Add dislike
Add to saved papers

Paget's Disease of the Temporal Bone: A Single-Institution Contemporary Review of 27 Patients.

OBJECTIVES: To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB).

STUDY DESIGN: Retrospective chart review of patients evaluated from 1998 to 2016.

SETTING: Quaternary referral center.

PATIENTS: Patients with radiographically confirmed PDTB.

MAIN OUTCOME MEASURES: Clinical, audiological, and radiological features and management strategies of PDTB.

RESULTS: A total of 50 temporal bones in 27 patients (15 men) were diagnosed with PDTB. Symptoms at presentation included hearing loss (n = 23, 85%), headache (n = 18, 67%), dizziness (n = 14, 52%), tinnitus (n = 5, 19%), chronic otitis media (n = 2, 7%), hemifacial spasm without facial paralysis (n = 1, 4%), multiple cranial neuropathies (n = 1, 4%), and neoplastic transformation (n = 1, 4%). Of the 23 ears with audiometric data available for review, 65% exhibited sensorineural hearing loss, and 35% mixed hearing loss. Long-term audiometric follow-up was available on two patients, both of whom demonstrated hearing loss at a rate greater than would be expected for normal aging. Two patients underwent successful cochlear implantation, achieving open-set speech recognition. Radiographic features of temporal bone involvement are reviewed and illustrated.

CONCLUSION: This is the largest single-institution clinical series examining patients with PDTB in the English literature. Variable patterns of temporal bone involvement by Paget's disease are observed leading to a diverse set of clinical symptoms, including slowly progressive hearing loss, tinnitus, compressive cranial neuropathies, and benign or malignant tumorigenesis. Involvement typically begins in the petrous apex and progresses laterally. Otic capsule bone demineralization occurs late in the disease process. Cochlear implantation appears to be an effective management strategy for patients with severe-to-profound hearing loss.

Full text links

We have located links that may give you full text access.
Can't access the paper?
Try logging in through your university/institutional subscription. For a smoother one-click institutional access experience, please use our mobile app.

Related Resources

For the best experience, use the Read mobile app

Mobile app image

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

By using this service, you agree to our terms of use and privacy policy.

Your Privacy Choices Toggle icon

You can now claim free CME credits for this literature searchClaim now

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app