Pediatric Otosclerosis 

  • Author: Peter S Roland, MD; Chief Editor: Glenn C Isaacson, MD, FACS, FAAP   more...
 
Updated: Dec 6, 2011
 

Background

Otosclerosis is a genetically mediated metabolic bone disease that affects only the human otic capsule and ossicles.[1, 2] Its mode of inheritance is autosomal dominant, but penetrance and expressivity both vary. Women are affected twice as often as are men.

Usually, symptomatic hearing loss from otosclerosis first develops early in the third decade of life, although onset in the teenage years does occur. Otosclerosis is well recognized as a cause for conductive hearing loss due to fixation of the stapedial footplate in the oval window niche. Less well recognized is the fact that otosclerosis can involve other portions of the cochlea and produce sensorineural hearing loss (SNHL). The incidence of cochlear otosclerosis and that of cochlear otosclerosis resulting in clinically significant SNHL are unclear. The disease occurs in 8-10% of the white population; however, of those individuals affected, only 10-15% have clinical symptoms. Consequently, conductive hearing loss that requires treatment eventually develops in about 1% of the white population. Clinical disease occurs in about 0.5% of Asians and South Americans and in about 0.1% of Africans. The disease is bilateral in most cases.

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Pathophysiology

Two separate pathologic phases of the disease process can be identified: an early otospongiotic phase and a later otosclerotic phase.

The otosclerotic phase begins when osteoclasts are slowly replaced by osteoblasts and dense sclerotic bone is deposited in the areas of previous bone resorption. When this process involves the oval window in the area of the footplate, the footplate becomes fixed, resulting in conductive hearing loss.[3]

See Histologic Findings below.

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Epidemiology

Frequency

United States

Histologically, otosclerosis has a prevalence of about 10%. However, only about 1 in 10 persons with histologic disease are clinically affected, thus placing the prevalence of clinically significant disease at about 1%.

Mortality/Morbidity

The only known morbidity from otosclerosis is hearing loss. Although conductive hearing loss is considered the hallmark of the disease, involvement of portions of the otic capsule other than the stapedial footplate can result in SNHL.

Race

Otosclerosis is much more common in whites than in persons of other races. Histologic otosclerosis occurs in 10-20% of whites but only about 1% of blacks.

Sex

More women seek medical attention for hearing loss due to otosclerosis than do men. However, the disease is not sex-linked, and a histologic study of a large series of temporal bones shows no difference in prevalence between men and women.

Age

Clinical otosclerosis can manifest as early as age 7-8 years but most commonly appears in persons aged 15-35 years.

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Contributor Information and Disclosures
Author

Peter S Roland, MD  Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, University of Texas School of Human Development

Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Alcon Labs Honoraria Speaking and teaching; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear Corp Honoraria Board membership; Med El Corp travel grants Consulting

Specialty Editor Board

Orval Brown, MD  Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas

Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John E McClay, MD  Associate Professor of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Dallas, University of Texas Southwestern Medical School

John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Glenn C Isaacson, MD, FACS, FAAP  Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Temple University School of Medicine

Glenn C Isaacson, MD, FACS, FAAP is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Society of Pediatric Otolaryngology, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Covidien Honoraria Consulting

References

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  3. Michaels L, Soucek S. Origin and growth of otosclerosis. Acta Otolaryngol. May 2011;131(5):460-8. [Medline].

  4. Shambaugh GE Jr, Causse J. Ten years experience with fluoride in otosclerotic (otospongiotic) patients. Ann Otol Rhinol Laryngol. Sep-Oct 1974;83(5):635-42. [Medline].

  5. Shambaugh GE Jr, Petrovic A. Effects of sodium fluoride on bone. Application to otosclerosis and other decalcifying bone diseases. JAMA. Jun 10 1968;204(11):969-73. [Medline].

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  8. Causse JR, Causse JB, Bretlau P, et al. Etiology of otospongiotic sensorineural losses. Am J Otol. Mar 1989;10(2):99-107. [Medline].

  9. Dornhoffer JL, Bailey HA Jr, Graham SS. Long-term hearing results following stapedotomy. Am J Otol. Sep 1994;15(5):674-8. [Medline].

  10. Hannley MT. Audiologic characteristics of the patient with otosclerosis. Otolaryngol Clin North Am. Jun 1993;26(3):373-87. [Medline].

  11. Hinojosa R, Marion M. Otosclerosis and sensorineural hearing loss: a histopathologic study. Am J Otolaryngol. Sep-Oct 1987;8(5):296-307. [Medline].

  12. Hough JV, Dyer RK Jr. Stapedectomy. Causes of failure and revision surgery in otosclerosis. Otolaryngol Clin North Am. Jun 1993;26(3):453-70. [Medline].

  13. Iurato S, Ettorre GC, Onofri M, Davidson C. Very far-advanced otosclerosis. Am J Otol. Sep 1992;13(5):482-7. [Medline].

  14. Linthicum FH Jr. Histopathology of otosclerosis. Otolaryngol Clin North Am. Jun 1993;26(3):335-52. [Medline].

  15. Rama-Lopez J, Cervera-Paz FJ, Manrique M. Cochlear implantation of patients with far-advanced otosclerosis. Otol Neurotol. Feb 2006;27(2):153-8. [Medline].

  16. Rizer FM, Lippy WH. Evolution of techniques of stapedectomy from the total stapedectomy to the small fenestra stapedectomy. Otolaryngol Clin North Am. Jun 1993;26(3):443-51. [Medline].

  17. Roland PS, Meyerhof WL. Otosclerosis. In: Head and neck Surgery-Otolaryngology. Vol 2. 1998:2083-97.

  18. Willis R. Stapedectomy--past and present. Ann Acad Med Singapore. Sep 1991;20(5):680-5. [Medline].

  19. Zehnder AF, Kristiansen AG, Adams JC, et al. Osteoprotegrin knockout mice demonstrate abnormal remodeling of the otic capsule and progressive hearing loss. Laryngoscope. Feb 2006;116(2):201-6. [Medline].

 
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