Otosclerosis An Update On Diagnosis and Treatment.3
Otosclerosis An Update On Diagnosis and Treatment.3
Otosclerosis An Update On Diagnosis and Treatment.3
Otosclerosis:
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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
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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 tests—two techniques, Rinne and Weber,
use of assistive devices, for patients with otosclerosis. used to measure air and bone conduction at 512 Hz. Both
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40
early stages of the disease.4 Tumor
necrosis factor alfa, an inflammatory
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,
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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
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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 patient’s 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 patient’s 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 years—they 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
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
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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
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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