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Cochlear degeneration in leigh disease: histopathologic features.

Seckin O Ulualp1, Charles G Wright, Karen Pawlowski

  • 1Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas, 5323 Harry Hines Boulevard, Dallas, TX 75390-9035, U.S.A.

The Laryngoscope
|November 27, 2004
PubMed
Summary

Leigh disease can cause inner ear degeneration, affecting hearing. Pathologic findings reveal sensorineural damage in the cochlea, suggesting potential hearing loss in affected infants.

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Area of Science:

  • Neuropathology
  • Otopathology
  • Pediatric Pathology

Background:

  • Leigh disease is a severe neurological disorder affecting infants.
  • Auditory system involvement in Leigh disease is not well-documented.

Observation:

  • Autopsy examination of temporal bones from an infant with Leigh disease.
  • Microdissection and light microscopy of middle and inner ear structures.
  • Histological analysis of cochlear components and stria vascularis.

Findings:

  • Normal middle ear structures bilaterally, with evidence of otitis media.
  • Sensorineural degeneration in the cochlea, including hair cell loss and nerve fiber degeneration.
  • Basophilic deposits observed in the stria vascularis of the left cochlea.

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Implications:

  • Inner ear degeneration is a potential pathologic finding in Leigh disease.
  • Cochlear dysfunction may contribute to hearing impairment in patients with Leigh disease.
  • Hearing assessment should consider potential sensorineural hearing loss in infants with Leigh disease.