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100%, but 74% (3). In one study, which included various

patients with an indication for middle ear surgery, the

prevalence was very low (19).

Diagnostic Test Measures

The sensitivity ranged between 60 and 95% in seven

studies (2,3,6,16–20). The two studies (2,3) that com-

pared CT to histopathology found lower sensitivities of

60 and 66% than the studies comparing CT to middle ear

surgery (6,16–20). Specificity was 100% in two studies

(3,18). In another two studies specificity was lower: 83%

and 75% (16,17,19). Specificity could be calculated for

one of the studies that compared CT to histopathology

and was not different from the specificities found in the

studies that compared CT to middle-ear surgery (100%

(3) compared with 75 to 100% [16–19]).

Post-test Probability of Otosclerosis

In patients with a clinical suspicion of otosclerosis, the

positive post-test probability of otosclerosis with an

abnormal CT was 99% (16,17). The positive post-test

probability was much lower in a study population of

patients with an indication for middle-ear surgery (23%)

(19). The negative post-test probabilities were reported in

three studies: 51%, 53%, and 67% (3,16–18). In patients

with an indication for middle-ear surgery, the negative

post-test probability of otosclerosis with a normal CT

was only 3% (19).

DISCUSSION

Summary of Main Results

The pretest probability or prevalence of otosclerosis

was very high in the majority of the included studies

(2,3,6,16–18,20). In those studies with a high prevalence

of disease (74–97%), both positive and negative post-test

probabilities were (relatively) high (3,16–18). Positive

post-test probability was 99% in one of these studies

(16,17) and negative post-test probabilities ranged

between 51% and 67% (3,16–18). In one study (19)

with a low prevalence of disease (9%), both positive and

negative post-test probabilities were much lower com-

pared with studies with a high prevalence of disease (23%

and 3%, respectively). Overall, reported sensitivities

ranged between 60% and 95% (2,3,6,16–20). The sen-

sitivity and specificity for operatively confirmed otoscle-

rosis were 61 to 95% (6,16–20) and 75 to 100% (16–19),

respectively. The sensitivity and specificity for histopa-

thologically confirmed otosclerosis were 60 to 66% (2,3)

and 100% (3), respectively.

The diagnostic measures and post-test probabilities

were generally higher in newer studies. The advance-

ments in scanners and techniques may have attributed to

higher detection rates in newer studies. Prevalence influ-

ences post-test probabilities; post-test probabilities are

generally higher in studies with a high prevalence of

disease and lower in studies with a low prevalence

of disease. Indeed, the studies with a high prevalence

of otosclerosis reported higher post-test probabilities than

those studies with a low prevalence of otosclerosis.

Prevalence of disease in these studies may have been

influenced by the choice of inclusion and exclusion

criteria (suspected otosclerosis based on clinical history

and physical examination versus intraoperatively con-

firmed otosclerosis) and the choice of reference test

(middle-ear surgery versus histopathology).

Previous studies and reviews highlight the importance

of CT in the diagnostic evaluation of otosclerosis

(2,3,28). However, the prevalence of otosclerosis in

patients with a clinical suspicion of otosclerosis and/or

an indication for surgery is generally high. As a result,

preoperative CT has little to add in establishing otoscle-

rosis and may not be necessary to confirm the diagnosis.

Although its role in diagnosing otosclerosis is limited, CT

imaging may still be useful in establishing the extent of

disease and cochlear involvement, and in detecting

concomitant abnormalities.

CT scans were qualitatively analyzed in the included

studies. Otosclerosis is usually confirmed on CT on the

basis of visual confirmation of double ring signs, hypo-

densities around the otic capsule, and/or thickening of the

footplate. Several authors adopted a more quantitative

approach and measured bone densities in the area

immediately anterior to the oval window: the fissula ante

fenestram (20,21,29,30). These studies did find statisti-

cally significant differences in Hounsfield units measured

over the fissula ante fenestram region between patients

with otosclerosis and control patients, but not in several

other regions surrounding the otic capsule. Tringali et al.

(20) performed analyses in a subgroup of patients with

otosclerosis and normal-appearing CT scan and found no

significant differences for densitometric measurements in

this subgroup compared with control subjects without

otologic disease and control patients with cholesteatoma.

Unfortunately, none of these studies defined a cut-off

value that can be used to create two-by-two contingency

tables and calculate diagnostic test measures.

Quality of Evidence

The majority of the included studies were character-

ized by a moderate relevance and moderate risk of bias.

Most studies only included patients with surgically con-

firmed otosclerosis. As a result, prevalence of otoscle-

rosis is 100% in these studies and only sensitivities can be

calculated. Second, only studies using surgery or

histology as a reference test were included, since this

is the only adequate reference test for confirming oto-

sclerosis. This implies that all included patients had an

indication for surgery. This causes a substantial risk of

selection bias, because the study populations probably

will not include patients with very mild disease or

patients with a severe sensorineural component. There-

fore, these results will not translate into the general group

of patients with otosclerosis.

Potential Biases in Review

To our knowledge, this is the first review to not only

systematically evaluate, but also critically appraise the

I. WEGNER ET AL.

Otology & Neurotology, Vol. 37, No. 1, 2016

146