CC BY-NC-ND 4.0 · Laryngorhinootologie 2020; 99(S 02): S323
DOI: 10.1055/s-0040-1711304
Abstracts
Otology

Osteopetrosis of the Temporal Bone as differential diagnosis in case of hearing loss

M Ahmed
1   Klinikum Bielefeld, Hno, Bielefeld
,
I Todt
1   Klinikum Bielefeld, Hno, Bielefeld
,
Lars-Uwe Scholtz
1   Klinikum Bielefeld, Hno, Bielefeld
,
H Sudhoff
1   Klinikum Bielefeld, Hno, Bielefeld
› Author Affiliations
 

Background Osteopetrosis describes a heterogenous group of heritable skeletal disorders characterized by increased bone formation. There are different forms: an autosomal recessive form and two forms of autosomal dominant forms, ADOI and ADOII.

The incidence varies between 1: 250,000 in malignant (autosomal recessive) and 1: 20,000 in non-malignant (autosomal dominant). Neurootological complications of osteopetrosis of the temporal bone include hearing loss, ossicular fixation, otitis media, facial palsy and vertigo. Temporal bone CT may show thickening and sclerosis of the calvarium, poor mastoid pneumatization and narrowing of the external and internal auditory canals, tympanic cavities, Eustachian tube and petrous carotid canal.

Case presentation We report a 44-year-old caucasian female patient with recurrent bilateral hearing loss with tinnitus. Otoscopy revealed no pathological findings. Sound and speech audiometry as well as the measurement of OAE and BERA were unremarkable. (HR CT) revealed bone sclerosis with poorly pneumatized mastoid cavities, small middle ear clefts bilaterally and narrow inner auditory canals.

Conclusion Osteopetrosis should be considered as a possible pathology in recurrent bilateral falls and unremarkable audiological diagnosis. To confirm the diagnosis, a high-resolution CT is the method of choice. Differential diagnoses include hypervitaminosis D, hypoparathroidism, Paget's disease and osteoblastic bone metastases.

Poster-PDF A-1945.PDF



Publication History

Article published online:
10 June 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

© Georg Thieme Verlag KG
Stuttgart · New York