CME

Otosclerosis:
An update on diagnosis and treatment
Lora Batson, MPAS, PA-C; Denise Rizzolo, PA-C, PhD

JOS ANTONIO PEAS / SCIENCE SOURCE

H
ABSTRACT earing loss affects the educational, psychological,
Otosclerosis is a complex and progressive disease of patho- and physical well-being of 360 million people
logical bone remodeling that affects the otic capsule of the worldwide.1 Otosclerosis, a process of progressive
temporal bone, resulting in hearing loss. Although tradi- pathologic bone remodeling, is one of the more complex
tional diagnostic methods are still used, improvements in diseases that leads to hearing loss.2 In patients with oto-
technology and research have paved the way for additional sclerosis, aberrant bone deposits surround and adhere to
diagnostic techniques and advancements. The traditional the ossicles, impairing the mechanical transmission of
treatment of otosclerosis, stapes surgery, is now being aug-
sound and leading to conductive hearing loss. In some
mented or replaced by innovations in hearing aid technology
and cochlear implants. Earlier diagnosis of otosclerosis can
patients with advanced disease, the lesions may extend
occur through understanding of the cause, risk factors, and into the bony labyrinth of the inner ear, affecting the cochlea
current diagnostic testing. and resulting in a mixed conductive and sensorineural
Keywords: otosclerosis, hearing loss, bone remodeling, audi- hearing loss.
ometry, cochlear implant, otic capsule 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,
Lora Batson is a research assistant in the rheumatology department at Asians, and Native Americans.3 The average age of onset
Massachusetts General Hospital in Boston, Mass. Denise Rizzolo is a
research coordinator in the PA program at Kean University in Union,
is 30.2 Clinically, the ratio of occurrence is 1.5 to 2 females
N.J., and a faculty member at the Pace Completion Program in New to 1 male.2 Otosclerosis is a progressive, insidious disease
York City, N.Y. The authors have disclosed no potential conflicts of not routinely seen in general practice. Clinicians can ben-
interest, financial or otherwise. efit from a better understanding and awareness of the
DOI:10.1097/01.JAA.0000511784.21936.1b presentation, methods of diagnosis, and treatment options;
Copyright 2017 American Academy of Physician Assistants so patients can be referred and educated appropriately.

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CME

Learning objectives TABLE 1. Audiometric screening tools13,35

Explain the basic histopathology, phases, causes, and risk Hearing Handicap Inventory for the Elderly Screening
factors of otosclerosis. Version (HHIE-S)a 10-question self-administered ques-
tionnaire developed to measure the social and emotional
Discuss the relevant history, physical examination, and effects of hearing loss. Scoring is from 0 (no handicap) to
diagnostic findings consistent with otosclerosis. 40 (maximum handicap).
Suggest appropriate management options, including the Tuning fork teststwo techniques, Rinne and Weber,
use of assistive devices, for patients with otosclerosis. 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
Key points behind the patient and whispers three random letter and
Otosclerosis causes hearing loss through pathological bone number combinations while also occluding and rubbing
remodeling that affects the otic capsule of the temporal the external auditory canal of the nontest ear. The patient
bone. then repeats the whispered combinations. The test is to be
Screening tests include questionnaires, tuning fork tests, conducted twice, each time with different letter-number
whisper-voice test, and audioscope. combinations. The patient passes if he or she can cor-
rectly repeat three out of the six combinations.
Innovations in hearing aid technology and cochlear
implants may replace stapes surgery, the traditional treat- Audioscopea rechargeable, handheld audiometer and
ment for otosclerosis. 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.
PATHOPHYSIOLOGY uHeariPhone application with three components: an
Normal bone remodeling occurs at a rate of 10% per year audiometric hearing screening to be performed in a quiet
throughout skeletal regions; however, a normal otic capsule noise environment (about 5 minutes); evaluation of ability
has very little bone remodelingonly 0.13% per year.2 In to understand speech in noise (1 minute); and 12 multi-
patients with otosclerosis, bone remodeling within the otic ple-choice questions from the Hearing-Dependent Daily
capsule is increased, leading to accumulation of bone Activities Scale to Evaluate Impact of Hearing Loss in Older
deposits that damage audiologic structures and worsen People. All information can easily be accessed using
normal sound transmission. The extent of aberrant bone iPhone, iPad, or iTouch and requires only the use of
remodeling in the otic capsule directly correlates to the iPhone earbuds.
abnormal audiologic findings.
Abnormal bone remodeling in otosclerosis occurs in Clinicians should consider otosclerosis as a cause of hear-
three phases: ing loss in patients who report a family history of the disease.
The otospongiosis phase, which represents an increase Hormonal conditions such as puberty, pregnancy, and
in both osteoclast activity and microvascularity.4 menopause may be associated with exacerbation of hear-
The transitional phase, which begins with deposits of ing loss in patients with preexisting otosclerosis.4 Research-
spongy bone by osteoblasts in areas of previous bone ers found estrogen receptors on otosclerotic cells, although
reabsorption.4 the specific regulatory mechanism of these receptors is
The otosclerotic phase, characterized by spongy bone unknown.7 Lippy and colleagues compared pregnant to
deposits developing into dense bone that narrows the micro- nonpregnant patients with otosclerosis and found no direct
circulation previously developed in the otospongiosis phase.4 association between pregnancy and exacerbation of hear-
These aberrant lesions can occur in many regions in the ing loss.8 Although additional research is needed to identify
following areas: anterior to oval window and stapes footplate the specific influence hormones may exhibit on hearing
(80%), round window (30%), pericochlear region (21%), loss, clinicians should suspect preexisting otosclerosis in
and anterior segment of the internal auditory canal (19%).5 patients who develop hearing loss during times of increased
hormonal production.
CAUSES AND RISK FACTORS Measles exposure is considered a risk factor for develop-
Genetic influences can contribute to otosclerosis; 60% of ing otosclerosis. Recent studies found viral materials in the
patients report a family history of the disease.4 Most nucleic acid of the stapes footplates and antibodies to the
researchers consider otosclerosis to be a condition of auto- measles virus in the inner ears of patients with otosclero-
somal dominant inheritance with an incomplete penetration; sis.9,10 Paradoxically, Komune and colleagues found that
although in 40% to 50% of patients, otosclerosis occurred a complete mRNA sequence of measles had not been
spontaneously or with variable patterns of inheritance.4,6 isolated from any otic sample.11 The exact etiologic function

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 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

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CME

correlates to hearing loss. When the ossicles stiffen and Vincent and colleagues reviewed 3,050 stapedotomies
the connection between the stapes and oval window begins and found the surgical procedure to be safe and successful
to change, a low-frequency mild conductive loss (small in treating conductive hearing loss in 94.2% of patients.20
air-bone gap) will occur (Figure 1).18 The air-bone gap is Surgical complications are rare but can include deafness,
the difference between air and bone conduction; a value necrosis of the incus, tympanic membrane perforation,
greater than 10 dB is considered abnormal. As the stapes facial nerve injury, disturbance of taste, perilymph gusher,
footplate becomes fixed to the oval window, the conduc- floating or subluxed stapes footplate, and vertigo. The
tive loss worsens (indicated by a widening air-bone gap) surgical failure rate commonly results from prosthesis
and begins to involve all frequencies.18 If cochlear lesions malposition or inappropriate prosthesis length.18
develop, as is the case in 10% of patients, high-frequency Due to the progressive nature of the disease, 10% to
sensory loss results in a mixed sensorineural and conduc- 20% of patients will require surgical revision.21 Who will
tive hearing loss pattern on the audiogram.2,18 Extensive develop disease progression or cochlear involvement can-
cochlear progression will result in mixed hearing loss in not be predicted. Following stapes surgery, hearing loss
all frequencies. can progress at variable and unpredictable rates.22 Redfors
Tympanometry is the measure of acoustic energy trans- and colleagues looked at 30 years poststapedectomy data
mission. Tympanograms often are normal in patients with and found that 88% of patients had bilateral involvement
otosclerosis. Only in extensive cases of otosclerosis may and 66% of patients showed moderate to profound loss
the patients tympanogram demonstrate some flattening secondary to progressive development of sensorineural
secondary to severe ossicular chain fixation.18 involvement.23
High-resolution CT is beginning to be used in diagnosis Hearing aids are an alternative for patients who are not
and surgical planning of otosclerosis due to improvements candidates for stapes surgery or are in need of sensorineu-
in technology allowing for identification of smaller bony ral hearing loss correction. Hearing aids amplify sound,
lesions.17 High-resolution CT has high diagnostic sensitiv- transmitting greater energy through the stiffened ossicles
ity and specificity, and reveals variants in patient anatomy and improving sound transmission into the inner ear.
and severity of disease.17 Common findings of otosclerosis Patients with a hearing loss greater than 25 dB are candi-
on a high-resolution CT include areas of increased bony dates for hearing aids.24 Hearing aids can be customized
radiolucency in the otic capsule around the anterior foot- to amplify only the frequencies that are needed based on
plate, thickening of the stapes, and widening of the oval findings from the patients audiometry. As otosclerosis
window.17 High-resolution CT also can reveal cochlear progresses, additional adjustments in amplification may
involvement by demonstrating a demineralized area outlin- be required. Hearing aid technology has improved greatly
ing the cochlea (double-ring sign).17 The main disadvantage over the last few yearsthey can be used more easily with
to the use of this test is its high cost. telephones, and some interact directly with smartphones
and tablets. Federal Communications Commission rules
TREATMENT require cell phone companies to make phones that are
Stapes surgery restores the mechanical transmission of compatible with hearing aids and cochlear implants.25
sound through the middle ear, correcting conductive hear- Hearing aids can be very expensive and may require mul-
ing loss. It does not correct sensorineural hearing loss tiple visits to an audiologist for sizing and adjustment.
secondary to otosclerotic extension into the cochlea. Patients also may have increased irritation and infection
Stapes surgery is a minimally invasive one-day procedure of the ear canal.
performed under general anesthesia; more recently, some Implantable hearing aids, such as middle ear implants
surgeons have begun to perform stapes surgery under local and bone conduction implants, are now being used in
anesthesia.6 The two variations of the surgery are: patients with otosclerosis who do not tolerate traditional
Stapedectomy, in which the stapes footplate and the crura hearing aids.26 These implantable hearing aids, like tra-
are removed and replaced with a prosthesis. ditional hearing aids, enhance the acoustic signal trans-
Stapedotomy, in which a small hole is made in the central mitted to the cochlea; however, the devices are technically
aspect of the stapes footplate for the prosthesis without very different (Table 2).
the removal of the structure. Middle ear implants amplify sound by mechanically
Indications for stapes surgery include conductive hearing vibrating the ossicles in which they are surgically affixed.
loss, air-bone gap of at least 20 dB, speech discrimination These devices require ossicular chain motion, which is
score of 60% or greater, and good patient health.12 Con- often limited in patients with otosclerosis due to bony
traindications include poor patient physical condition, deposits; therefore, middle ear implants should only be
fluctuating hearing loss with vertigo, tympanic membrane implanted at the time of stapes surgery or after stapes
perforation, infection, and hearing loss of 70 dB or worse surgery.27 Research found similar improvements in hearing
unless the patient has a speech discrimination score of 80% regardless of whether implantation occurred at the time
or better.12 of stapes surgery or after stapes surgery.27 Middle ear

20 www.JAAPA.com Volume 30 Number 2 February 2017

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 Otosclerosis: An update on diagnosis and treatment

implants are indicated for sensorineural hearing loss and

TABLE 2. Differences between hearing aids and cochlear
provide hearing improvement similar to traditional hear-
ing aids.28 implants27,36,37
Bone conduction implants are indicated for patients Hearing aids
with conductive losses or mixed hearing loss with minor Function: amplify acoustic signal from the outer ear, creat-
sensorineural involvement. These devices bypass the outer ing a greater acoustic energy and improving the mechani-
and middle ear, are attached to the temporal bone, and cal transmission of sound through the middle ear into the
transmit vibrational energy directly to the cochlea. Bone inner ear. Perception is of normal sounds but louder.
conduction implants can be implanted bilaterally but are Indications: patients with conductive and sensorineural
typically implanted unilaterally because the vibration is hearing loss greater than 25 dB
often strong enough to stimulate the contralateral Disadvantages: ear canal irritation and infection. Expen-
cochlea.29 Research conflicts on whether bone conduction sive and may require multiple adjustments.
implants are better than traditional hearing aids in cor- Middle ear implants
recting conductive losses.29 Bone conduction implants are Function: surgically attached to the ossicles to amplify
expensive and patients should try a traditional hearing acoustic signals received in the middle ear by increasing
aid first.29 vibration of the ossicles. The whole device is surgically
Cochlear implants do not amplify acoustic signals like implanted but some models require an external receiver to
hearing aids. These devices convert acoustic signals to be attached magnetically behind the ear. Perception is of
electric signals that are transmitted via electrodes to the normal sounds but louder.
auditory nerve (Table 2). Bypassing the natural transmis- Indications: patients with mixed and sensorineural hear-
ing loss who do not tolerate traditional hearing aids
sion of acoustic energy provides greater amplification in
Disadvantages: chorda tympani and facial nerve damage
patients with sensorineural hearing loss.26 Cochlear
from surgery, residual hearing loss from weight of implant
implants pose some challenges in patients with otoscle- on ossicles.
rosis. They may be more difficult to position surgically
and patients may have an increased risk postoperatively Bone conduction implants
of cochlear ossification and facial nerve stimulation.30,31 Function: convert acoustic signal to vibration and use
transmitted vibration signal to stimulate inner ear. Requires
These factors may result in reduced functioning of the
a functioning cochlea. Device is partially implanted: exter-
implant itself or require more frequent implant revisions nal receiver worn behind ear in temporal bone area and is
or reimplantations.32 Lenarz and colleagues found that attached magnetically or percutaneous to surgical implant
patients with otosclerosis and moderate-to-severe mixed coupled to temporal bone beneath the skin. Perception is
hearing loss benefitted from cochlear implants; improved of normal sound but louder.
hearing was measured by audiometric testing.26 Indications: patients with conductive, unilateral deafness;
Although cochlear implants are beneficial for some can be used in patients with mixed hearing loss if the sen-
patients, other research suggests that stapedotomy com- sorineural hearing loss is very minor
bined with hearing aids results in good outcomes in Disadvantages: expensive, surgical infections risk
patients with severe mixed hearing loss.30 This approach Cochlear implants
is recommended as first-line treatment, before consider- Function: transform acoustic signals into electrical signals
ing a cochlear implant because of the permanent nature that are transmitted to the auditory nerve. Device is partially
of the implant surgery.30 In 2014, the FDA approved the implanted: external (microphone, speech processor, trans-
first hybrid cochlear implant/hearing aid system for mitter) and surgically implanted (receiver and electrodes).
patients age 18 years and older.33 The hybrid system Surgery modifies the normal auditory structure. Patient is
reduces the risk of intracochlear trauma due to implanta- not able to revert back to hearing aid if unsatisfied. Percep-
tion and increases the chances of preserving some resid- tion of sounds is distorted. Patient requires extensive aural
ual hearing. Because of the built-in hearing aid, the hybrid rehabilitation.
system also can amplify low-frequency hearing. More Indications: patients with severe to profound sensorineu-
ral loss
research is needed to identify whether hybrid systems
Disadvantages: increased risk of Streptococcus pneu-
should be used as treatment before traditional cochlear
moniae meningitis, electrode migration, tinnitus, facial
implant surgery. nerve stimulation.
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 sodium fluoride required to arrest bone remodeling in the
commonly prescribed medication, evidence to support its otic capsule has yet to be determined.34 Bisphosphonates
use is limited and conflicting.34 Sodium fluoride acts as an and vitamin D also are being considered as possible future
antagonist to bone remodeling and osteoclast activation treatments for patients with otosclerosis; however, research
throughout the skeletal system.34 The adequate dosage of is in an early phase.34

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CME

CONCLUSION 18. Goodhill V, Haris I, Canalis R. Otosclerosis. In: Canalis R,

Otosclerosis is a progressive yet treatable form of hear- Lambert PR, eds. The Ear: Comprehensive Otology. Philadel-
phia, PA: Lippincott Williams and Wilkens; 2000:467-487.
ing loss. Improvements in technology and research have
19. Wegner I, Bittermann AJ, Hentschel MA, et al. Pure-tone
paved the way for additional diagnostic techniques and audiometry in otosclerosis: insufficient evidence for the
advancement in treatments. Understanding of this diagnostic value of the Carhart notch. Otolaryngol Head Neck
complex disease leads to earlier diagnosis, referral, Surg. 2013;149(4):528-532.
treatment, and improved patient education for those 20. Vincent R, Sperling NM, Oates J, Jindal M. Surgical findings
with otosclerosis. JAAPA and long-term hearing results in 3,050 stapedotomies for
primary otosclerosis: a prospective study with the otology-
neurotology database. Otol Neurotol. 2006;27(8 suppl 2):
Earn Category I CME Credit by reading both CME articles in this issue, S25-S47.
reviewing the post-test, then taking the online test at http://cme.aapa.
21. Meyer TA, Lambert PR. Primary and revision stapedectomy in
org. Successful completion is defined as a cumulative score of at least elderly patients. Curr Opin Otolaryngol Head Neck Surg. 2004;
70% correct. This material has been reviewed and is approved for 1 hour 12(5):387-392.
of clinical Category I (Preapproved) CME credit by the AAPA. The term of
22. Sakihara Y, Parving A. Clinical otosclerosis, prevalence esti-
approval is for 1 year from the publication date of February 2017.
mates and spontaneous progress. Acta Otolaryngol. 1999;119(4):
468-472.
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