An otoscopic examination typically is normal, with the exception of an increased

redness along the promontory of the tympanic membrane (Schwartz sign). The
Schwartz sign is inconsistently found in patients with otosclerosis and is not
necessary for diagnosis.12 Audiometric screenings are general assessments of
hear- ing loss and can be performed quickly in any quiet clinical setting.
Clinicians can perform a number of screenings that may aid in audiometric
referral for patients with otoscle- rosis, including questionnaires, tuning fork tests,
whisper- voice test, and audioscope (Table 1). A systematic review examined the
accuracy of these commonly used screening tools in identifying hearing loss
within a clinical setting. Researchers found the Hearing Handicap Inventory for
the Elderly Screening Version, a commonly used question- naire that quanti es
hearing handicap, to accurately cor- relate to hearing loss veri ed on audiometric
ndings.13 Although the meta-analysis was limited in quality studies regarding
tuning fork accuracies, researchers concluded tuning fork tests to be inaccurate
screening tools in iden- tifying hearing loss of any cause due to Rinnes inability
to distinguish sensorineural hearing loss from normal hearing and Webers
inability to identify bilateral hearing loss.13 The whisper test and audioscope were
found to have appropriate and similar diagnostic accuracy in identifying hearing
loss.13 Recent studies also have evaluated a new screening tool, the uHear iPhone
app by Unitron, and have found this app to be a useful screening tool for
identifying hearing loss across a variety of age groups.14-16 Hearing screening
should not take the place of formal audiometric testing in patients with suspected
otosclerosis or other audiologic pathologies. Audiograms, in addition to medical
history and physi- cal examination, have traditionally been used for diag- nosis of
otosclerosis.17 An audiogram measures air and bone conductions and interactions
throughout various frequencies (Hz) at various loudness levels (dB). An
audiogram that results in hearing thresholds greater than 25 dB is abnormal.
Otosclerosis typically presents with low frequency conductive hearing loss
(Figure 1).18 A loss of bone conduction at the frequency regions around 2,000 Hz
(Carhart notch) historically has been considered an indicator of otosclerosis;
however, recent research has found the Carhart notch cannot be used to con rm
diagnosis.19 Otosclerosis progression can be monitored by an audio- gram
because the progression of the disease directly correlates to hearing loss. When
the ossicles stiffen and the connection between the stapes and oval window begins
to change, a low-frequency mild conductive loss (small air-bone gap) will occur
(Figure 1).18 The air-bone gap is the difference between air and bone conduction;
a value greater than 10 dB is considered abnormal. As the stapes footplate
becomes xed to the oval window, the conduc- tive loss worsens (indicated by a
widening air-bone gap) and begins to involve all frequencies.18 If cochlear
lesions develop, as is the case in 10% of patients, high-frequency sensory loss
results in a mixed sensorineural and conduc- tive hearing loss pattern on the
audiogram.2,18 Extensive cochlear progression will result in mixed hearing loss
in all frequencies. Tympanometry is the measure of acoustic energy trans-
mission. Tympanograms often are normal in patients with otosclerosis. Only in
extensive cases of otosclerosis may the patients tympanogram demonstrate some
attening secondary to severe ossicular chain xation.18 High-resolution CT is
beginning to be used in diagnosis and surgical planning of otosclerosis due to
improvements in technology allowing for identi cation of smaller bony
lesions.17 High-resolution CT has high diagnostic sensitiv- ity and speci city,
and reveals variants in patient anatomy and severity of disease.17 Common
ndings of otosclerosis on a high-resolution CT include areas of increased bony
radiolucency in the otic capsule around the anterior foot- plate, thickening of the
stapes, and widening of the oval window.17 High-resolution CT also can reveal
cochlear involvement by demonstrating a demineralized area outlin- ing the
cochlea (double-ring sign).17 The main disadvantage to the use of this test is its
high cost.