Pediatric Otosclerosis Differential Diagnoses

Updated: Mar 08, 2021
  • Author: Joe Walter Kutz, Jr, MD, FACS; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
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Diagnostic Considerations

Differential diagnosis of otosclerosis should include other causes of conductive hearing loss.

In the context of a normal computed tomography (CT) scan, the only way to make a definitive diagnosis of otosclerosis is by means of exploratory tympanotomy and palpation of the stapes.

A history of recurrent otitis media suggests an ossicular discontinuity due to incus necrosis or ossicular chain fixation due to tympanosclerosis.

Congenital stapedial footplate fixation is present at an earlier age than juvenile otosclerosis. Congenital footplate fixation is generally detectable at age 3 years, whereas juvenile otosclerosis is rarely if ever detected before age 10 years.

Paget disease can be diagnosed on the basis of its manifestation in areas other than the otic capsule. Osteogenesis imperfecta, similarly, is diagnosed on the basis of associated symptoms (eg, blue sclerae or multiple fractures).

Another cause of conductive hearing loss is dehiscent semicircular canal syndrome. This disorder is classically associated with Tullio phenomenon (vertigo and/or nystagmus associated with loud noises). Diagnosis depends on CT scans that reveal a dehiscent superior semicircular canal when the appropriate symptomatology is present. Tullio phenomenon should be objectively documented. Vestibular evoked myogenic responses frequently show decreased thresholds in this disease. Electrocochleography usually yields abnormal findings.

Differential Diagnoses