Pediatric Otosclerosis Workup

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

Imaging Studies

  • In otosclerosis, fine-cut CT scanning can often reveal deposition of new bone in the area of the round or oval window and/or otosclerotic foci within other portions of the labyrinthine capsule and cochlea. The outlining of the resultant labyrinth is sometimes referred to as a "halo sign."
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Other Tests

  • Diagnosis depends on a combination of audiometric testing and historical features. An audiometric evaluation reveals conductive hearing loss with absent stapedius reflex. Historically, patients report a relatively slowly progressive hearing loss in the absence of significant infectious ear disease or otologic trauma. Several other indications that can support the diagnosis include the following:
    • The Carhart notch is a frequent audiometric to otosclerosis but is also seen in other types of conductive hearing loss. It consists of the appearance of a sensorineural hearing loss (SNHL) of 20-30 decibels (dB) at approximately 2000 cycles per second. The Carhart notch does not represent a genuine SNHL because it disappears after successful stapedectomy.
    • Speech discrimination is usually excellent.
    • A type A tympanogram with very low compliance may be noted. Sometimes termed a stiffness curve, this is often referred to as an As pattern. This configuration is occasionally present but is frequently absent.
    • Fine-cut CT scanning can often show deposition of new bone in the area of the round or oval window and/or otosclerotic foci within other portions of the labyrinthine capsule and cochlea.
    • Vestibular testing should be included when dizziness is present. Although no findings are characteristic for otosclerotic inner ear syndrome, findings suggestive of either superior semicircular canal dehiscence or of Ménière disease alter treatment plans.
  • Audiometric testing is the principal study required for the management of otosclerosis. Both air and bone conduction must be evaluated. If the clinical manifestations are all due to footplate fixation, audiography reveals a purely conductive loss. If involvement of the otic capsule in other areas causes hair cell injury, SNHL is also detected. No explicit criteria have been developed that allow the clinician to definitively distinguish SNHL caused by otosclerosis from hearing loss due to other causes.
  • Diagnosis of cochlear otosclerosis (ie, SNHL due to otosclerosis) generally requires a halo sign on fine-cut CT scanning or SNHL that is more advanced than expected from presbycusis, in the presence of some sign of stapedial involvement (eg, conductive hearing loss from footplate fixation, abnormalities of stapedius reflex testing).
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Histologic Findings

  • The otospongiotic phase of the disorder is characterized by bone resorption. Several cell types, such as histiocytes, osteocytes, and osteoblasts, participate in this process.
  • Bone resorption begins around existing vessels with consequent enlargement of vascular channels. Resultant hypervascularization, or a red blush, seen on the medial wall of the inner ear through the tympanic membrane is the Schwartze sign. This hypervascularization can sometimes be visualized during clinical otoscopy.
  • As the otospongiotic phase continues, a ground substance is deposited as a replacement for resorbed bone. The result is new spongy bone that has a blue appearance on histologic staining. These blue areas seen on hematoxylin and eosin (H and E) preparations are referred to as blue mantles of Manasse.
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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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  11. Hinojosa R, Marion M. Otosclerosis and sensorineural hearing loss: a histopathologic study. Am J Otolaryngol. Sep-Oct 1987;8(5):296-307. [Medline].

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  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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