Journal Reading

Otosklerosis

Oleh : Nor Azmina Binti Abd Basir

11 2017 074
 ABSTRACT

Otosclerosis is a complex and progressive disease of pathological bone

remodeling that affects the otic capsule of the temporal bone, resulting in
hearing loss. Although traditional 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 augmented 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, audiometry, cochlear

implant, otic capsule
 INTRODUCTION

 Otosclerosis, a process of progressive pathologic bone remodeling, is one of the more

complex diseases that leads to hearing loss.
 In patients with otosclerosis, 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.
EPIDEMIOLOGY

 Otosclerosis is found in 12% of whites, with 0.3% to 0.4% of these

patients presenting with clinical symptoms
 The prevalence is lower in blacks, Asians, and Native Americans.
 The average age of onset is 30.
 Clinically, the ratio of occurrence is 1.5 to 2 females to 1 male.
 PATHOPHYSIOLOGY
Extent of aberrant
Accumulation of bone
bone remodeling in
Bone remodeling deposits that damage
the otic capsule
within the otic capsule audiologic structures
directly correlates to
is increased and worsen normal
the abnormal
sound transmission
audiologic findings
Aberrant lesions
PATHOPHYSIOLOGY
can occur in many
regions in the
following areas:
 Anterior to oval
window and
Abnormal bone remodeling in otosclerosis occurs in
stapes footplate three phases:
(80%),
 Round window • The otosclerotic
(30%) phase, characterized by
The transitional phase,
 Pericochlear The otospongiosis spongy bone
which begins with
region (21%), phase, which represents deposits of deposits developing into
an increase dense bone that
 Anterior spongy bone by
segment of the in both osteoclast narrows the
osteoblasts in areas of
activity and microcirculation
internal auditory previous bone
canal (19%). microvascularity. previously developed in
reabsorption.
the otospongiosis
phase.
 CAUSES AND RISK FACTORS

– Genetic influences ( 60% ) -> a condition of autosomal dominant inheritance with an incomplete
penetration.
– ( 40% to 50% )-> occurred spontaneously or with variable patterns of inheritance
– Hormonal conditions such as puberty, pregnancy, and menopause may be associated with
exacerbation of hearing loss in patients with preexisting otosclerosis -> found estrogen receptors
on otosclerotic cells although the specific regulatory mechanism of these receptors is unknown.
– Lippy and colleagues compared pregnant to nonpregnant patients with otosclerosis and found no
direct association between pregnancy and exacerbation of hearing loss.
 CAUSES AND RISK FACTORS

– Measles exposure -> 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. The
exact etiologic function of measles in the development or progression of otosclerosis is still
unknown.
– Inflammation secondary to inflammatory and regulatory cytokines -> Inflammatory cytokine
and cytotoxic mediators are released from spongy bone deposits during the early stages of the
disease. Tumor necrosis factor alfa, an inflammatory cytokine, has been found in otosclerotic
bone.
HISTORY AND DIAGNOSTIC
EXAMINATION
– Patients with otosclerosis present with progressive hearing loss
that is worse in lower tones and/or frequencies.
– 50% of patients also have tinnitus.
– 10% of patients report vertigo - otosclerosis has extended to the
inner ear, affecting the semicircular canals responsible for
balance.
– found bilaterally in 80% of patients however, patients often present
with unilateral involvement early in the disease.
 HISTORY AND DIAGNOSTIC
EXAMINATION
– Otoscopic examination typically is normal, with the exception of an increased
redness along the promontory of the tympanic membrane (Schwartz sign) -> is
inconsistently found in patients with otosclerosis and is not necessary for diagnosis.
– Audiometric screenings : questionnaires, tuning fork tests, whisper voice test, and
audioscope
– Researchers found the Hearing Handicap Inventory for the Elderly Screening
Version -> commonly used questionnaire that quantifies hearing handicap, to
accurately correlate to hearing loss verified on audiometric findings.
– Researchers concluded tuning fork tests to be inaccurate screening tools in
identifying hearing loss of any cause due to Rinne’s inability to distinguish
sensorineural hearing loss from normal hearing and Weber’s inability to identify
bilateral hearing loss.
 HISTORY AND
DIAGNOSTIC
EXAMINATION
– The whisper test and audioscope were found to have appropriate and similar
diagnostic accuracy in identifying hearing loss.
– Recent studies also have evaluated a new screening tool, the uHear iPhone app
by Unitron, and have found this app to be a useful screening tool for identifying
hearing loss across a variety of age groups.
– Audiograms -> 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 . A loss of bone conduction at
the frequency regions around 2,000 Hz (Carhart notch) historically has been
considered an indicator of otosclerosis.
HISTORY AND DIAGNOSTIC
EXAMINATION
– Tympanometry is the measure of acoustic energy transmission. Only in
extensive cases of otosclerosis may the patient’s tympanogram
demonstrate some flattening secondary to severe ossicular chain fixation.
– High-resolution CT -> allowing for identification of smaller bony lesions
(high diagnostic sensitivity and specifi city, and reveals variants in patient
anatomy and severity of disease) - areas of increased bony radiolucency
in the otic capsule around the anterior footplate, thickening of the stapes,
and widening of the oval window.
TREATMENTS

Stapes surgery restores the mechanical transmission

of sound through the middle ear, correcting
conductive hearing loss.

Does not correct sensorineural hearing loss

secondary to otosclerotic extension into the cochlea
variations of Indications for Contraindications

the surgery stapes surgery

poor patient
physical condition

• Stapedectomy, in which the conductive hearing

loss, air-bone gap of fluctuating hearing
stapes footplate and the crura at least 20 dB loss with vertigo
are removed and replaced with a
prosthesis.
tympanic membrane
perforation
speech
discrimination hearing loss of 70 dB or
• Stapedotomy, in which a small
hole is made in the central worse unless the patient
aspect of the stapes footplate for has a speech
the prosthesis without the discrimination score of
removal of the structure. 80% or better
score of 60% or
greater, and good
patient health infection
 • deafness,
• necrosis of the incus
• tympanic membrane perforation,
• facial nerve injury,
• disturbance of taste
Surgical • perilymph gusher,
Complications • floating or subluxed stapes footplate
• vertigo.

• Hearing aids -> need of sensorineural hearing loss correction

(hearing loss greater than 25 dB)
• Middle ear implant and bone conduction implants. Middle ear
implants -> sensorineural hearing loss and provide hearing
improvement similar to traditional hearing aids. Bone conduction
implants -> patients with conductive losses or mixed hearing loss
Alternatives with minor sensorineural involvement. Bone conduction implants can
be implanted bilaterally but are ypically implanted unilaterally
because the vibration is often strong enough to stimulate the
contralateral cochlea.
TREATMENTS

– Other research suggests that stapedotomy combined with hearing aids

results in good outcomes in patients with severe mixed hearing loss ->
first-line treatment.
– FDA approved the first hybrid cochlear implant/hearing aid system for
patients age 18 years and older.
– Pharmacological options are not considered mainstream treatment for
otosclerosis.
 Sodium fluoride - antagonist to bone remodeling and osteoclast activation
throughout the skeletal system.
 Bisphosphonates and vitamin D
CONCLUSION

– Otosclerosis is a progressive yet treatable form of hearing

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.