Pediatric Otosclerosis Differential Diagnoses

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

Diagnostic Considerations

Differential diagnosis of otosclerosis should include other causes of conductive hearing loss.

  • In the context of a normal CT scan result, the only way to make a definitive diagnosis of otosclerosis is by exploratory tympanotomy and palpation of the stapes.
  • A history of recurrent otitis media suggests an ossicular discontinuity due to incus necrosis or ossicular chain fixation due to tympanosclerosis.
  • Congenital stapedial footplate fixation is present at an earlier age than juvenile otosclerosis. Congenital footplate fixation is generally detectable at age 3 years, whereas juvenile otosclerosis is rarely if ever detected before age 10 years.
  • Paget disease can be diagnosed based on its manifestation in areas other than the otic capsule. Osteogenesis imperfecta, similarly, is diagnosed on the basis of associated symptoms (eg, blue sclerae, multiple fractures).
  • A newly diagnosed cause of conductive hearing loss is dehiscent semicircular canal syndrome. This disorder is classically associated with Tullio phenomenon (vertigo and/or nystagmus associated with loud noises). Diagnosis depends on CT scans that reveal a dehiscent superior semicircular canal when the appropriate symptomatology is present. Tullio phenomenon should be objectively documented. Vestibular evoked myogenic responses are frequently altered in this disease.
  • Other problems to be considered include the following:
    • Ossicular discontinuity
    • Fixation of the head of the malleus or incus
    • Congenital stapes fixation
    • Paget disease
    • Superior semicircular canal dehiscence syndrome

Differential Diagnoses

Proceed to Workup
 
 
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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  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].

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

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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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