Danesh 2018
Danesh 2018
Danesh 2018
Otosclerosis
Ali A. Danesh, MS, PhDa,b,*, Navid Shahnaz, c
PhD ,
James W. Hall III, PhDd,e
KEYWORDS
Carhart notch Immittance measurement and otosclerosis Reflectance
Wideband acoustic immittance and otosclerosis Middle ear muscle reflex
Power absorbance and otosclerosis Hearing aids and otosclerosis
Tinnitus and otosclerosis
KEY POINTS
For most patients with otosclerosis, audiologic biomarkers include reduced middle ear
compliance as revealed by tympanometry, and a 10- to 15-dB reduction in sound trans-
mission via bone conduction most often in the vicinity of 2000 Hz (known as Carhart
notch).
Wideband acoustic immittance is an effective technique in identifying middle ear pathol-
ogies, such as otosclerosis; it can provide all the useful information that could be obtained
from conventional and multifrequency tympanometry and additional information on the
transfer of energy into the middle ear system across much wider range of frequencies.
Middle ear resonance frequency shifts to higher frequency regions in most of the otoscler-
otic ears.
In addition to middle ear ossicular surgery, hearing aids and implantable hearing devices are
alternative approaches for the management of hearing loss in patients with otosclerosis.
Tinnitus sound therapy and cognitive behavioral therapy are successfully used for the
management of tinnitus in the otosclerotic population.
INTRODUCTION
For many otologists and audiologists, otosclerosis is not a puzzling condition anymore.
Advances in diagnostic and therapeutic procedures have provided a vast number of pa-
tients with otosclerosis with proper management. This article is designed in a fashion
that enables otologists in better diagnosis and management of otosclerosis with the
use of audiologic procedures. The accuracy of audiometric air-bone gap, appropriate
use of masking techniques, and immittance measurements can completely influence
the decisions made by otologists for the surgical management of otosclerosis. Otolo-
gists rely on the precision of the audiologic results and determination of the degree of
the conductive component. Therefore, a precise audiologic work-up is a crucial part
of the diagnostic protocol for otosclerosis. This article reviews the audiologic diagnostic
test battery and the audiologic management of auditory effects of otosclerosis.
AUDIOMETRIC PATTERNS
As with other middle ear disorders, otosclerosis reduces sound-related energy pass-
ing from the tympanic membrane to the inner ear. Fixation and resultant stiffening of
the ossicular chain almost always produces a hearing loss, particularly for lower-
frequency sounds. The characteristic pattern of hearing loss in otosclerosis is useful
in diagnosing the disease.1–3 The diagnostic value of hearing assessment is enhanced
when such test procedures as pure tone audiometry, tympanometry, and acoustic re-
flexes are combined into a test battery. Indeed, for most patients with otosclerosis, a
unique pattern of findings for an appropriate collection of auditory tests almost always
contributes to early and accurate diagnosis. Basic hearing test findings in patients with
otosclerosis are summarized in Table 1.
Table 1
Patterns of basic auditory findings in patients with the diagnosis of otosclerosis
Procedure Findings
Pure tone audiometry
Air conduction Hearing loss greater for low frequencies.
Bone conduction Apparent decrease in bone conduction thresholds sometimes with a
notching deficit at 2000 Hz (Carhart notch). Actual bone
conduction hearing is typically normal.
Audiometric Weber Perception of low-frequency pure tone stimuli in the ear with
test conductive hearing loss.
Sensorineural acuity Presence of an air-bone gap and confirmation of normal bone
level test conduction hearing.
Acoustic immittance measures
Tympanometry Shallow type A tympanogram reflecting increased stiffness of the
ossicular chain (see immittance measurement section for further
discussion).
Acoustic reflexes Absence of stapedial acoustic reflex activity even in patients with
minimal air-bone gap and conductive hearing loss. Atypical
acoustic reflex pattern in patients with very early subclinical
otosclerosis.
Otoacoustic emissions Otoacoustic emissions cannot be detected in patients with
otosclerosis and conductive hearing loss. Recovery of detectable
otoacoustic emissions is possible in patients following
microtraumatic stapedotomy.
The Audiology of Otosclerosis 3
Representative pure tone audiometry findings for one ear of a patient with otoscle-
rosis are shown in Fig. 1. There is a conductive hearing loss with considerably poorer
hearing sensitivity for air versus bone conduction hearing. Bone conduction hearing is
generally normal with the exception of a distinct notch-like decrease in bone conduc-
tion thresholds in the audiogram region of the 2000 Hz. The horizontal dotted line
indicates actual bone conduction hearing whereas the bone conduction thresholds
reflect an apparent deficit in sensory function. “Mechanical modifications” and effects
of middle ear resonant frequency in bone conduction hearing associated with stapes
fixation in patients with otosclerosis have been appreciated since the 1940s (dis-
cussed later). Indeed, normal bone conduction hearing sensitivity is unusual for pa-
tients with otosclerosis. Multiple theories have been offered for the negative effect
of middle ear abnormalities on the response to bone conduction stimulation. However,
evidence and agreement in support of a single mechanism are lacking.
Named after the well-known audiologist who first described it in detail,1,4 Carhart
notch has for more than 60 years been one of the most recognizable audiometric fea-
tures of otosclerosis. Carhart1 described an average decrease in bone conduction
thresholds of 5 dB at 500 Hz, 10 dB at 1000 Hz, 15 dB at 2000 Hz, and 5 dB at
4000 Hz, as illustrated in Fig. 1.
More recent studies raise three general questions about the diagnostic value and
specificity of a notching deficit in bone conduction thresholds at 2000 Hz.5–8 First,
bone conduction hearing thresholds are often decreased also at other test frequencies
in patients with the diagnosis of otosclerosis. Researchers have observed for patients
with the diagnosis of otosclerosis the possibility of a notching deficit in bone conduc-
tion thresholds in the low-, mid-, and high-frequency region, not just at 2000 Hz.9,10
Second, Carhart notch at 2000 Hz is not invariably observed in patients with the
diagnosis of otosclerosis or fixation of the ossicular chain. A group of scientists re-
ported a 2000-Hz notch in bone conduction thresholds for only 31% of 102 patients
Fig. 1. Typical air and bone conduction hearing threshold patterns for a patient with
otosclerosis. Notice the appearance of Carhart notch in bone conduction hearing at
2000 Hz. The dotted line indicates true bone conduction hearing or “cochlear reserve.”
(Courtesy of James W. Hall III, PhD, Salus University, Elkins Park, PA.)
4 Danesh et al
with stapes fixation.7 Finally, related to this second point, patients with etiologies for
conductive hearing loss other than otosclerosis may show a notching deficit in bone
conduction thresholds at 2000 Hz. Studies have reported the presence of Carhart
notch in one-third of a series of 75 patients with congenital aural atresia.8 Carhart
notch pattern was shown only for 30% of patients with malleus or incus fixation and
for 26% of 19 patients with detachment or discontinuity at the malleus and incus joint.7
The resonant frequencies of the middle ear and particularly the ossicular chain seem
to be in the vicinity of 2000 Hz, which is potentially why a reduction at 2000 Hz is seen
in a good number of patients with stapes fixation as seen in otosclerosis.4,7,8 In
advanced cases of otosclerosis, conductive hearing loss develops into mixed hearing
loss. Additionally, in cases with cochlear otosclerosis, moderate to profound sensori-
neural hearing losses are commonly observed in clinical practice.
Two additional pure tone hearing tests deserve mention because they sometimes
contribute to the accurate assessment of auditory status in patients with otosclerosis.
One is the audiometric Weber test and the other procedure is the sensorineural acuity
level or sensorineural acuity level test.2,3,11,12 The sensorineural acuity level technique
provides valuable clinical information and plays a unique role in clinical audiology
when performed with insert earphones and used as a supplement to conventional
bone-conduction measurements for confirming ear-specific information on sensory
hearing thresholds (see Table 1).
As summarized in Table 1, three other auditory findings are consistent with fixation
of the ossicular chain and typical of patients with otosclerosis, in addition to the
conductive hearing loss and Carhart notch. One is a shallow type A tympanogram,
referred to as type As, which reflects abnormal restriction of the ossicular chain (dis-
cussed later).3,13 A second typical finding is the absence of normal acoustic reflex ac-
tivity, even for patients who have little evidence of conductive hearing loss with pure
tone audiometry.2,3 Indeed, the presence of acoustic reflex activity at expected inten-
sity levels, that is, about 85 dB for pure tone stimuli, essentially rules out fixation of the
ossicular chain and otosclerosis. Third, word recognition scores in quiet are good or
excellent in most patients with otosclerosis, even in those with some apparent deficit
in bone conduction hearing thresholds.
We conclude this discussion of auditory findings in otosclerosis with a few com-
ments about the possible application of otoacoustic emissions (OAEs). There is a gen-
eral consensus that OAEs are not recordable in patients with middle ear dysfunction
including those with fixation of the ossicular chain and otosclerosis. However, several
recent published papers describe a potential role for OAEs in the evaluation of auditory
function following “microtraumatic stapedotomy.”14,15 Although results are inconsis-
tent among studies and variable among patients, there are reports of the emergence
of detectable OAEs in the frequency region of 1000 to 1500 Hz perhaps associated
with normalization of the resonance frequency of the middle ear following microtrau-
matic stapedotomy.
For clinicians, middle ear analysis is the most important diagnostic component of
otosclerosis. Many have encountered cases with a conductive pathology and normally
appearing tympanograms where the nature of underlying pathology is not clear. Sim-
ply put, not all of the ears with otosclerosis show a reduced tympanometric compli-
ance and not all of the tympanograms with reduced compliance are caused by
otosclerosis. The following section describes the science behind differentiation of
the underlying middle ear pathologies with the use of immittance measurements.
The Audiology of Otosclerosis 5
Immittance Measurements
Immittance measurement has been used for several decades in the assessment of
middle ear disorders. Immittance measurement consists of tympanometry and middle
ear muscle reflex (MMR). Individuals with otosclerosis typically present a conductive
hearing loss, sometimes type As or normal type A tympanograms,16,17 absent
MMRs, and normal otoscopic results. The normal otoscopy with conductive hearing
loss is not distinctive to otosclerosis.18 Similar patterns have been observed in cases
of superior canal dehiscence and ossicular discontinuity.19 However, MMRs are pre-
sent in superior canal dehiscence and a type Ad tympanogram is observed in ossicular
chain discontinuity. Differentiation of middle ear pathologies with the use of immit-
tance measurements can sometimes be paradoxic.
Tympanometry
Tympanometry is a safe and quick method for assessing middle ear function. In this
technique, a pliable probe is sealed in the external ear canal. Then a sound is presented
while the air pressure is changed within the ear canal. The sound pressure level moni-
tored at the probe tip provides an index of the ease with which acoustic energy flows
into the middle ear system, which is referred to as acoustic admittance (Ya). Currently,
tympanometry is mainly conducted at a conventional low probe tone frequency. Tym-
panometry performed at conventional low probe tone frequency (226 Hz) cannot iden-
tify most of the lesions that specifically affect the ossicular chain. For example,
information provided by a conventional 226-Hz tympanogram is typically inadequate
for distinguishing a normal middle ear from otosclerotic (stapes fixation) ears.20–26
Different parameters can be obtained from a conventional low probe tone frequency
tympanogram. Two absolute parameters, static admittance (Ytm – admittance at the
level of the tympanic membrane) and tympanometric width in daPa, are most often
derived from conventional low probe tone frequency tympanometry. Several studies
have compared Ytm in healthy and otosclerotic ears.16,25–30 These studies have
consistently shown that, on average, Ytm tends to be lower in otosclerotic ears. How-
ever, the extensive overlap in the distributions of Ytm for these two groups at conven-
tional low probe tone frequency severely limits the diagnostic utility of this measure.
It has been shown that an abnormality is most obvious when the probe tone fre-
quency approaches the frequency at which middle ear vibrates most readily.26,31–33
This frequency is called the resonant frequency. Middle ear pathologies, such as
otosclerosis, affect the resonant frequency of the middle ear system. The greatest
impact of middle ear pathology on the Ytm is at frequencies close to the resonant fre-
quency.26,34 Therefore, Ytm measured in the vicinity of the resonant frequency may
provide the most useful information regarding the differential diagnosis of middle
ear pathologies. Several clinical and laboratory studies have reported prominent dif-
ferences between healthy and otosclerotic ears25,26,33,35–37 when Ytm recorded using
higher probe tone frequencies or resonant frequency were compared between healthy
ears and otosclerotic ears.
Tympanometric shape has also been reported to be affected by otosclerosis. A
measure that is, most commonly used to index the sharpness of the tympanometric
peak at conventional low probe tone frequency is the tympanometric width. Some
studies have reported narrower tympanometric peaks in otosclerotic ears than healthy
ears.25,38–40
The appearance of multifrequency devices has made it possible to derive immit-
tance subcomponents, susceptance (B) and conductance (G), and to perform tym-
panometry across a wide range of probe tone frequencies. Recent studies suggest
6 Danesh et al
does not depend on the distance between the probe tip and the eardrum and so the
location of the probe in the ear canal is not as critical as it is in tympanometry in chil-
dren and adults.51 Finally, WAI can be run at ambient pressure and does not require
pressurization of the ear canal.52 It is, however, possible to run a pressurized WAI
measurement by varying the pressure in a manner identical to tympanometry.53 At
ambient pressures, healthy adults show a pattern of low absorption in the low fre-
quencies, which increases to a maximum between 1000 Hz and 4000 Hz before
decreasing again at high frequencies.54 Fig. 3 demonstrates this pattern in a
normal-hearing individual.
Growing body of the literature suggests that WAI is a good indicator of middle ear
pathologies in neonates, children, and adults.55–63 Compared with conventional
226-Hz tympanometry, WAI may provide for a more sensitive test in evaluating middle
ear disorders and conductive hearing loss.52,64,65 In contrast to conventional 226-Hz
tympanometry, WAI is significantly more sensitive to ossicular pathologies.18 More-
over, the patterns of absorbance vary depending on the status of the middle ear
and thus different pathologies result in different patterns of absorbance. Generally,
a stiffening pathology results in decreased absorbance over a specific frequency
range. For example, otosclerotic ears demonstrate significantly increased reflectance
between 400 Hz and 1000 Hz.18 Researchers found that PA was the most effective
way of identifying ears with otosclerosis compared with 226-Hz tympanometry and
multifrequency tympanometry.18 PA was able to identify otosclerosis in 82% of their
sample and had a false-positive rate of 17.2%. The research suggests that the use
of PA in conjunction with other tools for assessment of middle ear function will improve
the identification of otosclerotic ears in a clinical setting.18 Fig. 4 demonstrates an
example of PA in surgically confirmed otosclerotic ears. The PA was obtained before
the surgery and fixation of the stapes was confirmed during the surgery.
Auditory complications of otosclerosis include hearing loss and tinnitus. Involved pa-
tients rarely complain about sound sensitivity disorders, such as hyperacusis, and the
8 Danesh et al
Fig. 3. Power absorbance in a normal-hearing adult. The y-axis is absorbance in % and the
x-axis is the frequency in Hz. The shaded areas represent 80% (dark gray) and 90% range
(light gray) of the normative data. (Courtesy of Navid Shahnaz, PhD, University of British
Columbia, Vancouver, BC, Canada.)
Fig. 4. Power absorbance in a surgically confirmed otosclerotic ear. Note that the absorption
less than 1500 Hz is significantly reduced compared with the 90% range for normal individuals
(shaded area). The y-axis is absorbance in % and the x-axis is the frequency in Hz. (Courtesy of
Navid Shahnaz, PhD, University of British Columbia, Vancouver, BC, Canada.)
The Audiology of Otosclerosis 9
reports of vertigo and balance disorders are not common in presurgical otosclerotic
ears. Many patients with otosclerosis are treated with otologic surgery; however,
occasionally patients may choose amplification instead of surgery for medical compli-
cations, such as stapes gusher or dehiscence of anterior semicircular canals, which
may be revealed by high-resolution computed tomography scans.66 A survey of 184
otologists indicated that hearing aids are advised before surgery.67
Fig. 5. Modern hearing aids. These hearing aids can stream acoustic signals from electronic
devices, such as cell phones and tablets, directly to the hearing aids. (Courtesy of Starkey
Laboratories, Eden Prairie, MN; with permission.)
10 Danesh et al
REFERENCES
55. Hunter LL, Feeney MP, Lapsley Miller JA, et al. Wideband reflectance in new-
borns: normative regions and relationship to hearing screening results. Ear
Hear 2010;31:599–610.
56. Keefe DH, Abdala C. Theory of forward and reverse middle-ear transmission
applied to optoacoustic emissions in infant and adult ears. J Acoust Soc Am
2007;121:978–93.
57. Keefe DH, Folsom R, Gorga MP, et al. Identification of neonatal hearing impair-
ment: ear-canal measurements of acoustic admittance and reflectance in neo-
nates. Ear Hear 2000;21:443–61.
58. Merchant GR, Horton NJ, Voss SE. Normative reflectance and transmittance
measurements on healthy newborn and 1-month-old infants. Ear Hear 2010;
31:746–54.
59. Sanford CA, Keefe DH, Liu YW, et al. Sound-conduction effects on distortion-
product optoacoustic emission screening outcomes in newborn infants: test per-
formance of wideband acoustic transfer functions and 1-kHz tympanometry. Ear
Hear 2009;30:635–52.
60. Hunter LL, Tubaugh L, Jackson JA, et al. Wideband middle ear power measure-
ment in infants and children. J Am Acad Audiol 2008;19:309–24.
61. Keefe DH, Bulen JC, Arehart KH, et al. Ear-canal impedance and reflection co-
efficient in human infants and adults. J Acoust Soc Am 1993;94:2617–38.
62. Sanford C, Feeney MP. Effects of maturation on tympanometric wideband
acoustic transfer functions in human infants. J Acoust Soc Am 2008;124:
2106–22.
63. Van der Werff KR, Prieve BA, Georgantas LM. Test–retest reliability of wideband
reflectance measures in infants under screening and diagnostic test conditions.
Ear Hear 2007;28:669–81.
64. Beers AN, Shahnaz N, Westerberg BD, et al. Wideband reflectance in normal
Caucasian and Chinese school-aged children and in children with otitis media
with effusion. Ear Hear 2010;31:221–33.
65. Feeney MP, Grant IL, Marryott LP. Wideband energy reflectance measurements
in adults with middle-ear disorders. J Speech Lang Hear Res 2003;46:901–11.
66. Nguyen DQ, Morel N, Dumas G, et al. Dehiscence of the anterior semicircular
canal and otosclerosis: a case report. Rev Laryngol Otol Rhinol (Bord) 2006;
127:151–5.
67. Lancer H, Manickavasagam J, Zaman A, et al. Stapes surgery: a National Sur-
vey of British Otologists. Eur Arch Otorhinolaryngol 2016;273:371–9.
68. Justicz N, Strickland KF, Motamedi KK, et al. Review of a single surgeon’s sta-
pedotomy cases performed with a nickel titanium prosthesis over a 14-year
period. Acta Otolaryngol 2017;137:442–6.
69. Johnson EW. Hearing aids and otosclerosis. Otolaryngol Clin North Am 1993;26:
491–502.
70. Redfors YD, Möller C. Otosclerosis: thirty-year follow-up after surgery. Ann Otol
Rhinol Laryngol 2011;120:608–14.
71. Redfors YD, Hellgren J, Möller C. Hearing-aid use and benefit: a long-term
follow-up in patients undergoing surgery for otosclerosis. Int J Audiol 2013;52:
194–9.
72. Ishai R, Halpin CF, Shin JJ, et al. Long-term incidence and degree of sensori-
neural hearing loss in otosclerosis. Otol Neurotol 2016;37:1489–96.
73. Saul RS, Danesh AA, Williams DF. The auditory system. In: Williams DF, editor.
Communication sciences and disorders: an introduction to the professions.
New York: Psychology Press, Taylor & Francis Group; 2012. p. 241–73.
The Audiology of Otosclerosis 15
95. de Vilhena D, Gambôa I, Duarte D, et al. Vestibular disorders after stapedial sur-
gery in patients with otosclerosis. Int J Otolaryngol 2016;2016:6830648.
96. Grayeli AB, Sterkers O, Toupet M. Audiovestibular function in patients with
otosclerosis and balance disorders. Otol Neurotol 2009;30:1085–91.
97. Hirvonen TP, Aalto H. Immediate postoperative nystagmus and vestibular symp-
toms after stapes surgery. Acta Otolaryngol 2013;133:842–5.
98. Ferster APO, Cureoglu S, Keskin N, et al. Secondary endolymphatic hydrops.
Otol Neurotol 2017;38:774–9.
99. Lin KY, Young YH. Role of ocular VEMP test in assessing the occurrence of ver-
tigo in otosclerosis patients. Clin Neurophysiol 2015;126:187–93.
100. Tramontani O, Gkoritsa E, Ferekidis E, et al. Contribution of vestibular-evoked
myogenic potential (VEMP) testing in the assessment and the differential diag-
nosis of otosclerosis. Med Sci Monit 2014;20:205–13.
101. Hope A, Fagan P. Latent superior canal dehiscence syndrome unmasked by
stapedotomy for otosclerosis. J Laryngol Otol 2010;124:428–30.
102. Van Rompaey V, Potvin J, van den Hauwe L, et al. Third mobile window associ-
ated with suspected otosclerotic foci in two patients with an air-bone gap.
J Laryngol Otol 2011;125:89–92.
103. Hunter JB, Patel NS, O’Connell BP, et al. Cervical and ocular VEMP testing in
diagnosing superior semicircular canal dehiscence. Otolaryngol Head Neck
Surg 2017;156:917–23.
104. Morozova SV, Dobrotin VE, Kulakova LA, et al. Vestibular disorders in patients
with otosclerosis: prevalence, diagnostic and therapeutic options. Vestn Otori-
nolaringol 2009;2:20–2.