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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

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.

ICD-10
H80

Author
Tanja Schub, BS

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.

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

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

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

44 Laboratory Tests That May Be Ordered 44 Other Diagnostic Tests/Studies


There are no laboratory tests used in the diagnosis of otosclerosis 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

May 11, 2012

Published by Cinahl Information Systems. Copyright2012, Cinahl Information Systems. All rights reserved. No part of this may be reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, or by any information storage and retrieval system, without permission in writing from the publisher. Cinahl Information Systems accepts no liability for advice or information given herein or errors/omissions in the text. It is merely intended as a 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
Babighian, G. G., & Albu, S. (2009). Failures in stapedotomy for otosclerosis. OtolaryngologyHead and Neck Surgery, 141(3), 395-400. 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., Vol. 2, p. 1817). Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. 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. Ferri, F. F. (2012). Otosclerosis (otospongiosis). In F. F. Ferri, 2012 Ferris clinical advisor: 5 books in 1 (p. 731). Philadelphia: Mosby Elsevier. 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.