CME
Otosclerosis:
An update on diagnosis and treatment
Lora Batson, MPAS, PA-C; Denise Rizzolo, PA-C, PhD
ABSTRACT
Otosclerosis is a complex and progressive disease of patho-
logical bone remodeling that affects the otic capsule of the
temporal bone, resulting in hearing loss. Although tradi-
tional diagnostic methods are still used, improvements in
technology and research have paved the way for additional
diagnostic techniques and advancements. The traditional
treatment of otosclerosis, stapes surgery, is now being aug-
mented or replaced by innovations in hearing aid technology
and cochlear implants. Earlier diagnosis of otosclerosis can
occur through understanding of the cause, risk factors, and
current diagnostic testing.
Keywords: otosclerosis, hearing loss, bone remodeling, audi-
ometry, cochlear implant, otic capsule
Lora Batson is a research assistant in the rheumatology department at
Massachusetts General Hospital in Boston, Mass. Denise Rizzolo is a
research coordinator in the PA program at Kean University in Union,
N.J., and a faculty member at the Pace Completion Program in New
York City, N.Y. The authors have disclosed no potential conflicts of
interest, financial or otherwise.
DOI:10.1097/01.JAA.0000511784.21936.1b
Copyright 2017 American Academy of Physician Assistants
JAAPA Journal of the American Academy of Physician Assistants
Copyright 2017 American Academy of Physician Assistants
JOS ANTONIO PEAS / SCIENCE SOURCE
H
earing loss affects the educational, psychological,
and physical well-being of 360 million people
worldwide.1 Otosclerosis, a process of progressive
pathologic bone remodeling, is one of the more complex
diseases that leads to hearing loss.2 In patients with oto-
sclerosis, aberrant bone deposits surround and adhere to
the ossicles, impairing the mechanical transmission of
sound and leading to conductive hearing loss. In some
patients with advanced disease, the lesions may extend
into the bony labyrinth of the inner ear, affecting the cochlea
and resulting in a mixed conductive and sensorineural
hearing loss.
Histologically, otosclerosis is found in 12% of whites,
with 0.3% to 0.4% of these patients presenting with
clinical symptoms.2 The prevalence is lower in blacks,
Asians, and Native Americans.3 The average age of onset
is 30.2 Clinically, the ratio of occurrence is 1.5 to 2 females
to 1 male.2 Otosclerosis is a progressive, insidious disease
not routinely seen in general practice. Clinicians can ben-
efit from a better understanding and awareness of the
presentation, methods of diagnosis, and treatment options;
so patients can be referred and educated appropriately.
www.JAAPA.com 17
CME
Learning objectives
Explain the basic histopathology, phases, causes, and risk
factors of otosclerosis.
Discuss the relevant history, physical examination, and
diagnostic findings consistent with otosclerosis.
Suggest appropriate management options, including the
use of assistive devices, for patients with otosclerosis.
Key points
Otosclerosis causes hearing loss through pathological bone
remodeling that affects the otic capsule of the temporal
bone.
Screening tests include questionnaires, tuning fork tests,
whisper-voice test, and audioscope.
Innovations in hearing aid technology and cochlear
implants may replace stapes surgery, the traditional treat-
ment for otosclerosis.
PATHOPHYSIOLOGY
Normal bone remodeling occurs at a rate of 10% per year
throughout skeletal regions; however, a normal otic capsule
has very little bone remodelingonly 0.13% per year.2 In
patients with otosclerosis, bone remodeling within the otic
capsule is increased, leading to accumulation of bone
deposits that damage audiologic structures and worsen
normal sound transmission. The extent of aberrant bone
remodeling in the otic capsule directly correlates to the
abnormal audiologic findings.
Abnormal bone remodeling in otosclerosis occurs in
three phases:
The otospongiosis phase, which represents an increase
in both osteoclast activity and microvascularity.4
The transitional phase, which begins with deposits of
spongy bone by osteoblasts in areas of previous bone
reabsorption.4
The otosclerotic phase, characterized by spongy bone
deposits developing into dense bone that narrows the micro-
circulation previously developed in the otospongiosis phase.4
These aberrant lesions can occur in many regions in the
following areas: anterior to oval window and stapes footplate
(80%), round window (30%), pericochlear region (21%),
and anterior segment of the internal auditory canal (19%).5
CAUSES AND RISK FACTORS
Genetic influences can contribute to otosclerosis; 60% of
patients report a family history of the disease.4 Most
researchers consider otosclerosis to be a condition of auto-
somal dominant inheritance with an incomplete penetration;
although in 40% to 50% of patients, otosclerosis occurred
spontaneously or with variable patterns of inheritance.4,6
18 www.JAAPA.com
Copyright 2017 American Academy of Physician Assistants
TABLE 1. Audiometric screening tools13,35
Hearing Handicap Inventory for the Elderly Screening
Version (HHIE-S)a 10-question self-administered ques-
tionnaire developed to measure the social and emotional
effects of hearing loss. Scoring is from 0 (no handicap) to
40 (maximum handicap).
Tuning fork teststwo techniques, Rinne and Weber,
used to measure air and bone conduction at 512 Hz. Both
tuning fork tests can be influenced by user experience
and materials.
Whisper-voice testthe examiner stands an arms length
behind the patient and whispers three random letter and
number combinations while also occluding and rubbing
the external auditory canal of the nontest ear. The patient
then repeats the whispered combinations. The test is to be
conducted twice, each time with different letter-number
combinations. The patient passes if he or she can cor-
rectly repeat three out of the six combinations.
Audioscopea rechargeable, handheld audiometer and
otoscope combination that measures hearing thresholds of
500; 1,000; 2,000; and 4,000 Hz at 20, 25, and 40 dB.
Audioscope screenings can be performed in a clinical set-
ting in less than 90 seconds.
uHeariPhone application with three components: an
audiometric hearing screening to be performed in a quiet
noise environment (about 5 minutes); evaluation of ability
to understand speech in noise (1 minute); and 12 multi-
ple-choice questions from the Hearing-Dependent Daily
Activities Scale to Evaluate Impact of Hearing Loss in Older
People. All information can easily be accessed using
iPhone, iPad, or iTouch and requires only the use of
iPhone earbuds.
Clinicians should consider otosclerosis as a cause of hear-
ing loss in patients who report a family history of the disease.
Hormonal conditions such as puberty, pregnancy, and
menopause may be associated with exacerbation of hear-
ing loss in patients with preexisting otosclerosis.4 Research-
ers found estrogen receptors on otosclerotic cells, although
the specific regulatory mechanism of these receptors is
unknown.7 Lippy and colleagues compared pregnant to
nonpregnant patients with otosclerosis and found no direct
association between pregnancy and exacerbation of hear-
ing loss.8 Although additional research is needed to identify
the specific influence hormones may exhibit on hearing
loss, clinicians should suspect preexisting otosclerosis in
patients who develop hearing loss during times of increased
hormonal production.
Measles exposure is considered a risk factor for develop-
ing otosclerosis. Recent studies found viral materials in the
nucleic acid of the stapes footplates and antibodies to the
measles virus in the inner ears of patients with otosclero-
sis.9,10 Paradoxically, Komune and colleagues found that
a complete mRNA sequence of measles had not been
isolated from any otic sample.11 The exact etiologic function
Volume 30 Number 2 February 2017
 Otosclerosis: An update on diagnosis and treatment

of measles in the development or 10

progression of otosclerosis is still
unknown. 0

Inflammation secondary to inflam-

10
matory and regulatory cytokines has
been implicated in the development 20
of otosclerosis. Inflammatory cytokine
and cytotoxic mediators are released 30

from spongy bone deposits during the

40
early stages of the disease.4 Tumor
necrosis factor alfa, an inflammatory

Hearing level (dBHL)

50
cytokine, has been found in otoscle-
rotic bone.9 Research on this topic is 60
in the nascent phase and no specific
inflammatory or autoimmune condi- 70

tion has been found to directly cause

80
otosclerosis. Left ear (Blue) Right ear (Red)

90 Air Conduction unMasked

HISTORY AND DIAGNOSTIC
EXAMINATION 100
Bone Conduction Masked (noise
opposite ear while assessing test ear
Patients with otosclerosis present with
progressive hearing loss that is worse 110

in lower tones and/or frequencies. For

120
example, patients often report diffi-
culty hearing male voices or vowel 130
sounds in words. About 50% of 125 250 500 1000 2000 4000 8000
patients also have tinnitus.4 Only 10% Frequency (Hz)
of patients report vertigo, which is not FIGURE 1. Audiogram of bilateral low-frequency conductive hearing loss in a patient
present unless otosclerosis has with otosclerosis
extended to the inner ear, affecting the
semicircular canals responsible for balance.2 Otosclerosis is hearing and Webers inability to identify bilateral hearing
found bilaterally in 80% of patients; however, patients often loss.13 The whisper test and audioscope were found to have
present with unilateral involvement early in the disease.6 appropriate and similar diagnostic accuracy in identifying
An otoscopic examination typically is normal, with the hearing loss.13 Recent studies also have evaluated a new
exception of an increased redness along the promontory screening tool, the uHear iPhone app by Unitron, and have
of the tympanic membrane (Schwartz sign). The Schwartz found this app to be a useful screening tool for identifying
sign is inconsistently found in patients with otosclerosis hearing loss across a variety of age groups.14-16 Hearing
12
and is not necessary for diagnosis. screening should not take the place of formal audiometric
Audiometric screenings are general assessments of hear- testing in patients with suspected otosclerosis or other
ing loss and can be performed quickly in any quiet clinical audiologic pathologies.
setting. Clinicians can perform a number of screenings that Audiograms, in addition to medical history and physi-
may aid in audiometric referral for patients with otoscle- cal examination, have traditionally been used for diag-
rosis, including questionnaires, tuning fork tests, whisper- nosis of otosclerosis.17 An audiogram measures air and
voice test, and audioscope (Table 1). A systematic review bone conductions and interactions throughout various
examined the accuracy of these commonly used screening frequencies (Hz) at various loudness levels (dB). An
tools in identifying hearing loss within a clinical setting. audiogram that results in hearing thresholds greater than
Researchers found the Hearing Handicap Inventory for 25 dB is abnormal. Otosclerosis typically presents with
the Elderly Screening Version, a commonly used question- low frequency conductive hearing loss (Figure 1).18 A loss
naire that quantifies hearing handicap, to accurately cor- of bone conduction at the frequency regions around 2,000
relate to hearing loss verified on audiometric findings.13 Hz (Carhart notch) historically has been considered
Although the meta-analysis was limited in quality studies an indicator of otosclerosis; however, recent research
regarding tuning fork accuracies, researchers concluded has found the Carhart notch cannot be used to confirm
tuning fork tests to be inaccurate screening tools in iden- diagnosis.19
tifying hearing loss of any cause due to Rinnes inability Otosclerosis progression can be monitored by an audio-
to distinguish sensorineural hearing loss from normal gram because the progression of the disease directly

JAAPA Journal of the American Academy of Physician Assistants www.JAAPA.com 19

Copyright 2017 American Academy of Physician Assistants

CME
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 fixed 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 flattening
secondary to severe ossicular chain fixation.18
High-resolution CT is beginning to be used in diagnosis
and surgical planning of otosclerosis due to improvements
in technology allowing for identification of smaller bony
lesions.17 High-resolution CT has high diagnostic sensitiv-
ity and specificity, and reveals variants in patient anatomy
and severity of disease.17 Common findings 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.
TREATMENT
Stapes surgery restores the mechanical transmission of
sound through the middle ear, correcting conductive hear-
ing loss. It does not correct sensorineural hearing loss
secondary to otosclerotic extension into the cochlea.
Stapes surgery is a minimally invasive one-day procedure
performed under general anesthesia; more recently, some
surgeons have begun to perform stapes surgery under local
anesthesia.6 The two variations of the surgery are:
Stapedectomy, in which the stapes footplate and the crura
are removed and replaced with a prosthesis.
Stapedotomy, in which a small hole is made in the central
aspect of the stapes footplate for the prosthesis without
the removal of the structure.
Indications for stapes surgery include conductive hearing
loss, air-bone gap of at least 20 dB, speech discrimination
score of 60% or greater, and good patient health.12 Con-
traindications include poor patient physical condition,
fluctuating hearing loss with vertigo, tympanic membrane
perforation, infection, and hearing loss of 70 dB or worse
unless the patient has a speech discrimination score of 80%
or better.12
20 www.JAAPA.com
Copyright 2017 American Academy of Physician Assistants
Vincent and colleagues reviewed 3,050 stapedotomies
and found the surgical procedure to be safe and successful
in treating conductive hearing loss in 94.2% of patients.20
Surgical complications are rare but can include deafness,
necrosis of the incus, tympanic membrane perforation,
facial nerve injury, disturbance of taste, perilymph gusher,
floating or subluxed stapes footplate, and vertigo. The
surgical failure rate commonly results from prosthesis
malposition or inappropriate prosthesis length.18
Due to the progressive nature of the disease, 10% to
20% of patients will require surgical revision.21 Who will
develop disease progression or cochlear involvement can-
not be predicted. Following stapes surgery, hearing loss
can progress at variable and unpredictable rates.22 Redfors
and colleagues looked at 30 years poststapedectomy data
and found that 88% of patients had bilateral involvement
and 66% of patients showed moderate to profound loss
secondary to progressive development of sensorineural
involvement.23
Hearing aids are an alternative for patients who are not
candidates for stapes surgery or are in need of sensorineu-
ral hearing loss correction. Hearing aids amplify sound,
transmitting greater energy through the stiffened ossicles
and improving sound transmission into the inner ear.
Patients with a hearing loss greater than 25 dB are candi-
dates for hearing aids.24 Hearing aids can be customized
to amplify only the frequencies that are needed based on
findings from the patients audiometry. As otosclerosis
progresses, additional adjustments in amplification may
be required. Hearing aid technology has improved greatly
over the last few yearsthey can be used more easily with
telephones, and some interact directly with smartphones
and tablets. Federal Communications Commission rules
require cell phone companies to make phones that are
compatible with hearing aids and cochlear implants.25
Hearing aids can be very expensive and may require mul-
tiple visits to an audiologist for sizing and adjustment.
Patients also may have increased irritation and infection
of the ear canal.
Implantable hearing aids, such as middle ear implants
and bone conduction implants, are now being used in
patients with otosclerosis who do not tolerate traditional
hearing aids.26 These implantable hearing aids, like tra-
ditional hearing aids, enhance the acoustic signal trans-
mitted to the cochlea; however, the devices are technically
very different (Table 2).
Middle ear implants amplify sound by mechanically
vibrating the ossicles in which they are surgically affixed.
These devices require ossicular chain motion, which is
often limited in patients with otosclerosis due to bony
deposits; therefore, middle ear implants should only be
implanted at the time of stapes surgery or after stapes
surgery.27 Research found similar improvements in hearing
regardless of whether implantation occurred at the time
of stapes surgery or after stapes surgery.27 Middle ear
Volume 30 Number 2 February 2017

implants are indicated for sensorineural hearing loss and
provide hearing improvement similar to traditional hear-
ing aids.28
Bone conduction implants are indicated for patients
with conductive losses or mixed hearing loss with minor
sensorineural involvement. These devices bypass the outer
and middle ear, are attached to the temporal bone, and
transmit vibrational energy directly to the cochlea. Bone
conduction implants can be implanted bilaterally but are
typically implanted unilaterally because the vibration is
often strong enough to stimulate the contralateral
cochlea.29 Research conflicts on whether bone conduction
implants are better than traditional hearing aids in cor-
recting conductive losses.29 Bone conduction implants are
expensive and patients should try a traditional hearing
aid first.29
Cochlear implants do not amplify acoustic signals like
hearing aids. These devices convert acoustic signals to
electric signals that are transmitted via electrodes to the
auditory nerve (Table 2). Bypassing the natural transmis-
sion of acoustic energy provides greater amplification in
patients with sensorineural hearing loss.26 Cochlear
implants pose some challenges in patients with otoscle-
rosis. They may be more difficult to position surgically
and patients may have an increased risk postoperatively
of cochlear ossification and facial nerve stimulation.30,31
These factors may result in reduced functioning of the
implant itself or require more frequent implant revisions
or reimplantations.32 Lenarz and colleagues found that
patients with otosclerosis and moderate-to-severe mixed
hearing loss benefitted from cochlear implants; improved
hearing was measured by audiometric testing.26
Although cochlear implants are beneficial for some
patients, other research suggests that stapedotomy com-
bined with hearing aids results in good outcomes in
patients with severe mixed hearing loss.30 This approach
is recommended as first-line treatment, before consider-
ing a cochlear implant because of the permanent nature
of the implant surgery.30 In 2014, the FDA approved the
first hybrid cochlear implant/hearing aid system for
patients age 18 years and older.33 The hybrid system
reduces the risk of intracochlear trauma due to implanta-
tion and increases the chances of preserving some resid-
ual hearing. Because of the built-in hearing aid, the hybrid
system also can amplify low-frequency hearing. More
research is needed to identify whether hybrid systems
should be used as treatment before traditional cochlear
implant surgery.
Pharmacological options are not considered mainstream
treatment for otosclerosis; the efficacy of various treatments
is still in question.34 Although sodium fluoride is the most
commonly prescribed medication, evidence to support its
use is limited and conflicting.34 Sodium fluoride acts as an
antagonist to bone remodeling and osteoclast activation
throughout the skeletal system.34 The adequate dosage of
JAAPA Journal of the American Academy of Physician Assistants
Copyright 2017 American Academy of Physician Assistants
Otosclerosis: An update on diagnosis and treatment
TABLE 2. Differences between hearing aids and cochlear
implants27,36,37
Hearing aids
Function: amplify acoustic signal from the outer ear, creat-
ing a greater acoustic energy and improving the mechani-
cal transmission of sound through the middle ear into the
inner ear. Perception is of normal sounds but louder.
Indications: patients with conductive and sensorineural
hearing loss greater than 25 dB
Disadvantages: ear canal irritation and infection. Expen-
sive and may require multiple adjustments.
Middle ear implants
Function: surgically attached to the ossicles to amplify
acoustic signals received in the middle ear by increasing
vibration of the ossicles. The whole device is surgically
implanted but some models require an external receiver to
be attached magnetically behind the ear. Perception is of
normal sounds but louder.
Indications: patients with mixed and sensorineural hear-
ing loss who do not tolerate traditional hearing aids
Disadvantages: chorda tympani and facial nerve damage
from surgery, residual hearing loss from weight of implant
on ossicles.
Bone conduction implants
Function: convert acoustic signal to vibration and use
transmitted vibration signal to stimulate inner ear. Requires
a functioning cochlea. Device is partially implanted: exter-
nal receiver worn behind ear in temporal bone area and is
attached magnetically or percutaneous to surgical implant
coupled to temporal bone beneath the skin. Perception is
of normal sound but louder.
Indications: patients with conductive, unilateral deafness;
can be used in patients with mixed hearing loss if the sen-
sorineural hearing loss is very minor
Disadvantages: expensive, surgical infections risk
Cochlear implants
Function: transform acoustic signals into electrical signals
that are transmitted to the auditory nerve. Device is partially
implanted: external (microphone, speech processor, trans-
mitter) and surgically implanted (receiver and electrodes).
Surgery modifies the normal auditory structure. Patient is
not able to revert back to hearing aid if unsatisfied. Percep-
tion of sounds is distorted. Patient requires extensive aural
rehabilitation.
Indications: patients with severe to profound sensorineu-
ral loss
Disadvantages: increased risk of Streptococcus pneu-
moniae meningitis, electrode migration, tinnitus, facial
nerve stimulation.
sodium fluoride required to arrest bone remodeling in the
otic capsule has yet to be determined.34 Bisphosphonates
and vitamin D also are being considered as possible future
treatments for patients with otosclerosis; however, research
is in an early phase.34
www.JAAPA.com 21
CME
CONCLUSION
Otosclerosis is a progressive yet treatable form of hear-
ing loss. Improvements in technology and research have
paved the way for additional diagnostic techniques and
advancement in treatments. Understanding of this
complex disease leads to earlier diagnosis, referral,
treatment, and improved patient education for those
with otosclerosis. JAAPA
Earn Category I CME Credit by reading both CME articles in this issue,
reviewing the post-test, then taking the online test at http://cme.aapa.
org. Successful completion is defined as a cumulative score of at least
70% correct. This material has been reviewed and is approved for 1 hour
of clinical Category I (Preapproved) CME credit by the AAPA. The term of
approval is for 1 year from the publication date of February 2017.
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Volume 30 Number 2 February 2017