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Otosclerosis
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Otosclerosis
Classification and external
resources

ICD-10 H80.

ICD-9 387

Otosclerosis is an abnormal growth of bone of the middle ear which can result in hearing
loss.

Contents
[hide]

• 1 Clinical Description
• 2 Pathophysiology
• 3 Treatment
• 4 Famous patients
• 5 References in popular culture
• 6 Notes

• 7 External links

[edit] Clinical Description

Chronic conductive hearing loss (CHL) is the finding in almost all cases of otosclerosis
(in fact should a person present with sensorineural hearing loss they would likely never
be diagnosed with otosclerosis). This usually will begin in one ear but will eventually
affect both ears with a variable course. On audiometry, the hearing loss is
characteristically low-frequency, with higher frequencies being affected later.
Sensorineural hearing loss (SNHL) has also been noted in patients with otosclerosis; this
is usually a high-frequency loss, and usually manifests late in the disease.

Approximately 0.5% of the population will eventually be diagnosed with otosclerosis.

Post mortem studies show that as many as 10% of people may have otosclerotic lesions
of their temporal bone, but apparently never had symptoms warranting a diagnosis.
Whites are the most affected race, with the prevalence in the Black and Asian populations
being much lower. Females are twice as likely as males to be affected. Usually noticeable
hearing loss begins at middle-age, but can start much sooner. The hearing loss often
grows worse during pregnancy.[1]

The disease can be considered to be heritable, but its penetrance and the degree of
expression is so highly variable that it may be difficult to detect an inheritance pattern.
Most of the implicated genes are transmitted in an autosomal dominant fashion.

[edit] Pathophysiology
The pathophysiology of otosclerosis is complex. The key lesions of otosclerosis are
multifocal areas of sclerosis within the endochondral temporal bone. These lesions share
some characteristics with Paget’s Disease, but they are not thought to be otherwise
related. Histopathologic studies have all been done on cadaveric temporal bones, so only
inferences can be made about progression of the disease histologically. This being said, it
seems that the lesions go through an active “spongiotic” / hypervascular phase before
developing into “sclerotic” phase lesions. There have been many genes and proteins
identified that, when mutated, may lead to these lesions. Also there is mounting evidence
that measles virus is present within the otosclerotic foci, implicating an infectious
etiology (this has also been noted in Paget’s Disease).

CHL in otosclerosis is caused by two main sites of involvement of the sclerotic (or scar-
like) lesions. The best understood mechanism is fixation of the stapes footplate to the
oval window of the cochlea. This greatly impairs movement of the stapes and therefore
transmission of sound into the inner ear (“ossicular coupling”). Additionally the cochlea’s
round window can also become sclerotic, and in a similar way impair movement of sound
pressure waves through the inner ear (“acoustic coupling”).

SNHL in otosclerosis is controversial. Over the past century, leading otologists and
neurotologic researchers have argued whether the finding of SNHL late in the course of
otosclerosis is due to otosclerosis or simply to typical presbycusis. There are certainly a
few well documented instances of sclerotic lesions directly obliterating sensory structures
within the cochlea and spiral ligament, which have been photographed and reported post-
mortem. Other supporting data includes a consistent loss of cochlear hair cells in patients
with otosclerosis; these cells being the chief sensory organs of sound reception. A
suggested mechanism for this is the release of hydrolytic enzymes into the inner ear
structures by the spongiotic lesions.

[edit] Treatment
Treatment of otosclerosis relies on two primary options: hearing aids (more recently
including bone-conduction hearing aids) and a surgery called a stapedectomy. Hearing
aids are usually very effective early in the course of the disease, but eventually a
stapedectomy may be required for definitive treatment. Early attempts at hearing
restoration via the simple freeing the stapes from its sclerotic attachments to the oval
window were met with temporary improvement in hearing, but the conductive hearing
loss would almost always recur. A stapedectomy consists of removing a portion of the
sclerotic stapes footplate and replacing it with an implant that is secured to the incus. This
procedure restores continuity of ossicular movement and allows transmission of sound
waves from the eardrum to the inner ear. A modern variant of this surgery called a
stapedotomy, is performed by drilling a small hole in the stapes footplate with a micro-
drill or a laser, and the insertion of a piston-like prothesis. The success rate of either a
stapedotomy or a stapedectomy depends greatly on the skill and the familiarity with the
procedure of the surgeon.[2]

Other less successful treatment includes fluoride administration, which theoretically

becomes incorporated into bone and inhibits otosclerotic progression. This treatment
cannot reverse conductive hearing loss, but may slow the progression of both the
conductive and sensorineural components of the disease process. Recently, some success
has been reported with bisphosphonate medications, which stimulate bone-deposition
without stimulating bony destruction.

[edit] Famous patients

The renowned German composer Beethoven was theorized to suffer from otosclerosis,
although this is controversial.[3] Victorian journalist Harriet Martineau gradually lost her
hearing during her young life, and later medical historians have diagnosed her with
probably suffering from otosclerosis as well.[4] Howard Hughes the pioneering American
aviator, engineer, industrialist, and film producer also suffered from otosclerosis.[5]
Frankie Valli, lead singer of The Four Seasons, suffered from it in the 1970s, forcing him
to "sing from memory" in the latter part of the decade (surgery restored most of his
hearing by 1980).[6]

[edit] References in popular culture

During the first three seasons of the CBS TV series CSI: Crime Scene Investigation, Gil
Grissom suffered from otosclerosis, which he inherited from his mother. At the end of the
show's third season, Grissom underwent a stapedectomy to correct it.

New player for the Philadelphia Flyers in the 07-08 season, Steve Downie, suffers from
otosclerosis.

Dwayne Schneider, the building superintendent on "One Day at a Time", undergoes a

stapedectomy to correct otosclerosis in one episode.

[edit] Notes
 1. ^ De Souza, Christopher. Otosclerosis and Stapedectomy. New York: Thieme
Medical Publishers, 2004.
2. ^ de Souza; Glassock (2004), Otosclerosis and Stapedectomy, ISBN 1588901696
3. ^ The Ludwig van Beethoven biography,
http://www.kunstderfuge.com/bios/beethoven.html
4. ^ Mary Jo Deegan, "Making Lemonade: Harriet Martineau on Being Deaf, p. 41-
58 in Harriet Martineau: Theoretical and Methodological Perspectives, NY, NY:
Routledge 2001
5. ^ Charles Higham, Howard Hughes: The Secret Life
6. ^ Fred Bronson, The Billboard Book of Number One Hits (3rd edition), Billboard
Books 1992. big foot also had otosclerosis ISBN 0-8230-8298-0

[edit] External links

• NIH/Medline
• NIH/NIDCD
• eMedicine
• DDB 29289
• Online 'Mendelian Inheritance in Man' (OMIM) 166800 - OTSC1
• Online 'Mendelian Inheritance in Man' (OMIM) 605727 - OTSC2
• Otosclerosis Patient Accounts and Support - Patient Support

[hide]
v•d•e
Diseases of the ear and mastoid process (H60-H99, 380-389)

External ear Otitis externa

Otitis media - Mastoiditis (Bezold's abscess, Gradenigo's

Middle ear and mastoid
syndrome) - Cholesteatoma - Perforated eardrum

Otosclerosis - Balance disorder - Ménière's disease -

Benign paroxysmal positional vertigo - Vestibular
Inner ear
neuronitis - Vertigo - Labyrinthitis - Perilymph fistula -
Superior canal dehiscence syndrome (SCDS)

Conductive hearing loss - Sensorineural hearing loss

Hearing impairment
(Central hearing loss, Presbycusis)

Other Tinnitus - Hyperacusis

See also congenital

Retrieved from "http://en.wikipedia.org/wiki/Otosclerosis"
Categories: Otology
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• This page was last modified on 10 October 2008, at 02:42.

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