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O To Sclerosis
O To Sclerosis
Hearing loss affects the educational, psychological, and physical well-being of 360
million people worldwide. Otosclerosis,aprocessofprogressive 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.
PATHOPHYSIOLOGY
Normal bone remodeling occurs at a rate of 10% per year throughout skeletal regions;
however, a normal otic capsule has very little bone remodeling—only 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
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
Researchers found estrogen receptors on otosclerotic cells, although the specific regulatory
exacerbation of hearing 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 developing 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 otosclerosis. 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 of measles in the development or progression of otosclerosis is still
unknown.
Inflammation secondary to inflammatory and regulatory cytokines has been implicated in the
development of otosclerosis. Inflammatory cytokine and cytotoxic mediators are released from
spongy bone deposits during the early stages of the disease.4 Tumor necrosis factor alfa, an
inflammatory cytokine, has been found in otosclerotic bone.9 Research on this topic is in the
nascent phase and no specific inflammatory or autoimmune condition has been found to directly
cause otosclerosis.
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 confirm diagnosis. 19
FIGURE 1. Audiogram of bilateral low-frequency conductive hearing loss in a patient with
otosclerosis
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 implants are
indicated for sensorineural hearing loss and provide hearing improvement similar to
traditional hearing 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 transmission of acoustic energy provides
greater amplification in patients with sensorineural hearing loss. 26 Cochlear implants pose
some challenges in patients with otosclerosis. 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 combined with hearing aids results in good outcomes in
patients with severe mixed hearing loss.30 This approach is recommended as first-line
treatment, before considering 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 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
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.