Pediatric Otosclerosis Clinical Presentation

Updated: Mar 08, 2021
  • Author: Joe Walter Kutz, Jr, MD, FACS; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
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Hearing loss and tinnitus are the principal symptoms of otosclerosis. Hearing loss is progressive, but the rate of progression varies, and loss of hearing may proceed by fits and starts, even within the same individual. Tinnitus widely varies but generally tends to become more severe as the degree of hearing loss worsens.

Occasionally, dizziness can result. The patient may describe only vague disequilibrium or may experience paroxysms of severe rotatory vertigo. Dizziness due to otosclerosis alone is sometimes termed otosclerotic inner ear syndrome. [4] Dizziness secondary to otosclerosis can be difficult to distinguish from dizziness related to other causes, especially secondary endolymphatic hydrops (ie, Ménière syndrome).

In individuals with a significant history of otitis media, ossicular pathology, especially partial or complete necrosis of the long process of the incus, should be seriously considered. In such circumstances, tympanography may reveal very high compliance (ie, the opposite of otosclerosis). A type AD tympanogram suggests ossicular discontinuity. Often, as incus necrosis progresses, the union between the incus and the stapes is replaced by a dense fibrous band. Such a fibrous union may result in an air-bone gap that is wider in high frequencies than in low frequencies.

Congenital stapes fixation is nonprogressive and is detected in the first decade of life. Fixation of the head of the malleus can occur congenitally, often in association with other stigmata of aural atresia, or it can be acquired, usually from an infectious process that resulted in tympanosclerosis. Tympanosclerosis can also result in stapes immobility by filling the oval window niche with tympanosclerotic plaques. Such a process usually occurs in individuals with a long history of otitis media and is more commonly unilateral, in contrast to the bilateral disease more often seen in otosclerosis.

Paget disease produces a clinical picture indistinguishable from that of otosclerosis. Histopathologically, Paget disease begins in the periosteal layer and not within the otic capsule.

Osteogenesis imperfecta also results in stapes fixation. Osteogenesis imperfecta is readily identified in most individuals who have other stigmata of the disease (eg, multiple flexures or blue sclera), but subtle cases may be indistinguishable from typical otosclerosis. At the time of operation, surgical findings are identical.


Physical Examination

Tuning fork tests reveal a conductive hearing loss in individuals with footplate fixation. Results of tuning fork tests may be difficult to interpret in patients with mixed losses.

The Rinne test should demonstrate bone conduction to be better than air conduction (negative Rinne) in patients contemplating a stapedectomy procedure.

The Weber test should lateralize to the ear with a greater degree of conductive hearing loss.

The remainder of the physical examination findings should be normal.

Abnormalities of the tympanic membrane, external ear canal, or middle ear suggest other causes for conductive hearing loss, though they do not rule out the possibility of stapes fixation due to otosclerosis. The exception is the presence of a Schwartze sign, with which increased vascularity is seen over the cochlear promontory. A positive Schwartze sign is a finding characteristic of otosclerosis.

A thorough audiometric evalution is essential before a diagnosis is established or a treatment plan determined.