Pediatric Otosclerosis Clinical Presentation

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

History

Hearing loss and tinnitus are the principal symptoms of otosclerosis. Hearing loss is progressive but the rate of progression varies and may proceed by fits and starts, even within the same individual. Tinnitus widely varies but generally tends to become more severe as the degree of hearing loss worsens. Occasionally, dizziness can result. The patient may describe only vague disequilibrium or may experience paroxysms of severe rotatory vertigo. Dizziness due to otosclerosis alone is sometimes termed otosclerotic inner ear syndrome. Dizziness secondary to otosclerosis can be difficult to distinguish from other causes, especially secondary endolymphatic hydrops (ie, Ménière syndrome).

  • In individuals with a significant history of otitis media, ossicular pathology, especially partial or complete necrosis of the long process of the incus, should be seriously considered. In such circumstances, tympanography may reveal very high compliance (ie, the opposite of otosclerosis). A type AD tympanogram suggests ossicular discontinuity. Often, as incus necrosis proceeds, the union between the incus and the stapes is replaced by a dense fibrous band. Such a fibrous union may result in an air-bone gap that is wider in high frequencies than in low frequencies.
  • Congenital stapes fixation is nonprogressive and is detected in the first decade of life. Fixation of the malleal head can occur congenitally, often in association with other stigmata of aural atresia, or it can be acquired, usually due to an infectious process that resulted in tympanosclerosis. Tympanosclerosis can also result in stapes immobility by filling the oval window niche with tympanosclerotic plaques. Such a process usually occurs in individuals with a long history of otitis media and is more commonly unilateral than is otosclerosis.
  • Paget disease produces a clinical picture indistinguishable from otosclerosis. Histopathologically, Paget disease begins in the periosteal layer and not within the otic capsule.
  • Osteogenesis imperfecta also results in stapes fixation. Osteogenesis imperfecta is readily identified in most individuals who have other stigmata of the disease (eg, multiple flexures, blue sclera) but subtle cases may be indistinguishable from typical otosclerosis. At the time of operation, surgical findings are identical.
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Physical

  • Tuning fork tests reveal a conductive hearing loss in individuals with footplate fixation. Results of tuning fork tests may be difficult to interpret in patients with mixed losses.
  • The Rinne test should demonstrate bone conduction to be better than air conduction (negative Rinne) in patients contemplating a stapes procedure.
  • The Weber test should lateralize to the ear with a greater degree of conducting hearing loss.
  • The remainder of the physical examination findings should be normal.
  • Abnormalities of the tympanic membrane, external ear canal, or middle ear suggest other causes for conductive hearing loss, although they do not rule out the possibility of stapes fixation due to otosclerosis. The exception is the presence of a Schwartze sign. Upon physical examination, this is the finding characteristic of otosclerosis.
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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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  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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