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Otosclerosis

Description/Etiology
Otosclerosis (also called otospongiosis) refers to an abnormal bone growth in the middle ear that results in gradual
hearing loss. The disorder is the most frequent cause of middle ear hearing loss in young adults.
In otosclerosis, new, abnormal spongy bone forms around the oval window of the middle ear, resulting in fixation of
the stapes (i.e., the small, stirrup-shaped bone in the middle ears). Bone growth prevents the stapes from vibrating
in response to sound waves, which leads to progressive conductive hearing loss. Sensorineural hearing loss occurs
when the bone growth extends to the cochlear endosteum (i.e., the lining of the cochlea, the auditory portion of the
inner ear) and cochlear hair cells. Although otosclerosis can be inherited, its degree of expression and penetrance are
variable, such that inheritance of the causative gene does not guarantee the development of otosclerosis.
Diagnosis of otosclerosis is clinical, based on patient history, physical examination, and audiometric evaluation.
Because hearing loss in otosclerosis is gradual, patients may not require treatment until they develop severe hearing
impairment. Medications (e.g., oral fluoride and calcium) can help control hearing loss by reducing abnormal bone
formation. Hearing aids are used for hearing loss, but do not prevent progression of the condition. Otosclerosis can
be cured with stapedectomy (i.e., surgical removal of stapes and replacement with prosthesis). Surgery is performed
on one ear at time with the more severely affected ear treated first; the two surgeries are generally separated by 312
months. If surgery is unsuccessful, total hearing loss may occur.

Facts and Figures

The incidence of otosclerosis varies by racial/ethnic group. The condition affects 810% of Whites; however, only
1015% of those affected have clinical manifestations, such that conductive hearing loss due to otosclerosis affects
~ 1% of the White population. Clinical otosclerosis is reported in 0.5% of Asians and South Americans, and 0.1% of
Africans. Women are affected twice as often as men and ~ 60% of patients have positive family history. Symptom
onset usually occurs between the ages of 1530 years, but clinical otosclerosis has been reported in patients as young
as 78 years of age. Most cases are bilateral, with just 15% affecting only one ear. Stapedectomy results in complete
elimination of conductive hearing loss in > 90% of cases; however, in 12% of surgeries, all hearing is lost in the
affected ear.
ICD-9
387

ICD-10
H80

Author
Tanja Schub, BS

Reviewers
Kathleen Walsh, RN, MSN, CCRN
Cinahl Information Systems
Glendale, California
Darlene A. Strayer, RN, MBA
Cinahl Information Systems
Glendale, California
Nursing Practice Council
Glendale Adventist Medical Center
Glendale, California

Editor
Diane Pravikoff, RN, PhD, FAAN
Cinahl Information Systems

May 11, 2012

Risk Factors
Family history is the primary risk factor for otosclerosis, with 60% of patients with the condition reporting a positive
family history. Some experts believe that pregnancy or oral contraception can trigger the onset of otosclerosis. Measles
virus has been implicated as a possible cause of otosclerosis; measles virus RNA is found in the stapes footplate in
most patients with otosclerosis, and vaccination against measles is associated with reduced rates of otosclerosis.

Signs and Symptoms/Clinical Presentation

Otosclerosis typically manifests with slowly progressive hearing loss and tinnitus (i.e., ringing in the ears). In some
cases, dizziness can result.

Assessment

44 Patient History
Ask about family history of hearing loss, onset and duration of hearing loss, associated manifestations,
subjective severity, and the social impact of the condition

44 Physical Findings of Particular Interest

Otoscopic examination reveals Schwartze sign (i.e., reddish hue on promontory of middle ear), but other
physical examination findings are normal; abnormalities of the tympanic membrane, external ear canal, or
middle ear suggest other causes for hearing loss

44 Laboratory Tests That May Be Ordered

There are no laboratory tests used in the diagnosis of otosclerosis

44 Other Diagnostic Tests/Studies

Audiometric testing will identify extent of hearing loss

Tuning fork tests (e.g., Rinne test, Weber test) will identify conductive hearing loss
Temporal-bone CT scan will distinguish otosclerosis from other causes of hearing loss

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general informational overview of the subject for the healthcare professional. Cinahl Information Systems, 1509 Wilson Terrace, Glendale, CA 91206

Treatment Goals

44 Promote Optimum Auditory Function and Reduce Risk of Complications

Assess level of hearing loss and devise alternate means of communication, if appropriate
Administer sodium fluoride and calcium, as ordered, to slow progression of disease; administer vitamin D, as ordered, to promote calcium and phosphate
absorption from small intestine

Follow facility pre- and postsurgical protocols if patient becomes a candidate for surgery (e.g., stapedectomy)
Reinforce pre- and postsurgical education and ensure completion of facility informed consent documents
Monitor for postsurgical pain and complications (e.g., hearing loss, facial nerve injury, disequilibrium, vertigo with nausea and vomiting); administer
symptomatic relief, as ordered

44 Provide Emotional Support and Educate

Assess patient/family member anxiety level and coping ability; provide emotional support, educate, and encourage discussion about otosclerosis
pathophysiology, the diagnostic process, potential complications, treatment risks and benefits, and individualized prognosis
Request referral, if appropriate, to a
social worker for identification of local resources for support groups and assistive devices
mental health clinician for counseling on strategies for coping with hearing loss

Food for Thought

44 No trial has investigated how long fluoride treatment should be continued in patients with otosclerosis

Red Flags
44 Excretion of sodium fluoride is impaired in patients with renal failure; toxic levels may result in these patients, and careful monitoring is essential

What Do I Need to Tell the Patient/Patients Family?

44 Following surgery, advise patient to avoid
blowing his or her nose for 1 week after surgery
people with respiratory or other infections
bending, lifting, or straining
loud noises or sudden pressure changes (e.g., associated with scuba diving, flying, driving in the mountains)
44 Educate regarding proper care and maintenance of hearing aids, including weekly cleaning of ear molds; storage in a cool, dry place; and routine removal of
the battery when not in use to extend battery life

Note
44 Recent review of the literature has found no updated research evidence on this topic since previous publication on August 12, 2011

References
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Cash, J. (2010). Assessment and management of patients with hearing and balance disorders. In S. C. Smeltzer, B. G. Bare, J. L. Hinkle, & K. H. Cheever (Eds.), Brunner & Suddarths textbook of medical-surgical nursing (12th ed.,
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Caylakli, F., Yavuz, H., Yilmazer, C., Yilmaz, I., & Ozluoglu, L. N. (2009). Effect of preoperative hearing level on success of stapes surgery. OtolaryngologyHead and Neck Surgery, 141(1), 12-15.
Cruise, A. S., Singh, A., & Quiney, R. E. (2010). Sodium fluoride in otosclerosis treatment: review. Journal of Laryngology and Otology, 124(6), 583-586.
Cureoglu, S., Baylan, M. Y., & Paparella, M. M. (2010). Cochlear otosclerosis. Current Opinion in Otolaryngology & Head and Neck Surgery, 18(5), 357-362.
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Niedermeyer, H. P., & Arnold, W. (2008). Otosclerosis and measles virus - association or causation? ORL, Journal for Oto-rhino-laryngology and its related specialties, 70(1), 63-70.
Roland, P. S. (2011). Pediatric otosclerosis. Medscape Reference. Retrieved May 9, 2012, from http://emedicine.medscape.com/article/994891-overview
Schrauwen, I., & Van Camp, G. (2010). The etiology of otosclerosis: a combination of genes and environment. Laryngoscope, 120(6), 1195-1202.
Uppal, S., Bajaj, Y., & Coatesworth, A. P. (2010). Otosclerosis 2: The medical management of otosclerosis. International Journal of Clinical Practice, 64(2), 256-265.
Uppal, S., Bajaj, Y., Rustom, I., & Coatesworth, A. P. (2009). Otosclerosis 1: The aetiopathogenesis of otosclerosis. International Journal of Clinical Practice, 63(10), 1526-1530.
Wolfrey, J. D. (2012). Otosclerosis (otospongiosis). In F. J. Domino (Ed.), The 5-minute clinical consult 2012 (20th ed., pp. 934-935). Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins.