International Journal of Pediatric Otorhinolaryngology
Volume 74, Issue 8 , Pages 934-938, August 2010

Histopathological morphometric study of cochleosaccular dysplasia in Dalmatian dogs☆☆

  • Andre L.L. Sampaio

      Affiliations

    • Brasilia University Medical School - Capes, Fulbright Scholarship, Brasilia, Brazil
    • International Hearing Foundation, Minneapolis, MN, United States
    • Corresponding Author InformationCorresponding author at: SQN 205 Bloco B Apartamento 506, 70843020 - Asa Norte, Brasília, Distrito Federal, Brazil. Tel.: +55 61 30375484; fax: +55 61 34433397.
  • ,
  • Elizabeth Paine

      Affiliations

    • International Hearing Foundation, Minneapolis, MN, United States
  • ,
  • Patricia A. Schachern

      Affiliations

    • Department of Otolaryngology, Otitis Media Research Center, University of Minnesota, Minneapolis, MN, United States
    • International Hearing Foundation, Minneapolis, MN, United States
  • ,
  • Carolyn Sutherland

      Affiliations

    • Department of Otolaryngology, Otitis Media Research Center, University of Minnesota, Minneapolis, MN, United States
  • ,
  • Sebahattin Cureoglu

      Affiliations

    • Department of Otolaryngology, Otitis Media Research Center, University of Minnesota, Minneapolis, MN, United States
    • International Hearing Foundation, Minneapolis, MN, United States
  • ,
  • Carlos A.C.P. Olivieira

      Affiliations

    • Brasilia University Medical School - Capes, Fulbright Scholarship, Brasilia, Brazil
  • ,
  • Michael M. Paparella

      Affiliations

    • Department of Otolaryngology, Otitis Media Research Center, University of Minnesota, Minneapolis, MN, United States
    • International Hearing Foundation, Minneapolis, MN, United States
    • Paparella Ear Head and Neck Institute, Minneapolis, MN, United States

Received 14 February 2010; received in revised form 17 May 2010; accepted 18 May 2010.

Abstract 

Objective

To analyze temporal bones of deaf Dalmatian dogs from 5 days after birth to adulthood to better understand the pathogenesis of cochleosaccular dysplasia.

Methods

This is an experimental animal histopathological temporal bone study that included two groups of temporal bones. Group I consisted of 41 temporal bones from deaf Dalmatian dogs and group II of 25 temporal bones from 15 “normal” aged-matched, hearing Black Labradors. Morphometric analysis included: stria vascularis and spiral ligament area measurements, and cell counts of spiral ganglion, Scarpa's ganglion, and hair cells of saccular macula.

Results

The following findings were significantly less in deaf Dalmatian group compared to hearing Labradors: (1) cellular area of the stria vascularis in all cochlear turns; (2) cellular area of spiral ligament in the inferior part of the basal turn; (3) cellular density of spiral ganglion cells within segments III and IV; (4) number of Scarpa's ganglion cells; and (5) density of saccular hair cells types I and II. A borderline negative correlation was found between average density of spiral ganglion cells of segments III and IV and age in group I. Young deaf animals showed some cochlear hair cells, however in adult dogs all hair cells were replaced by supporting cells.

Conclusion

General pattern of cochleosaccular dysplasia is variable, even when only one etiology, the genetic one, is involved. The gradual degeneration of inner ear elements in the cochleosaccular degeneration might indicate that early intervention might be crucial to stop the progression of cochleosaccular dysplasia.

Keywords: Cochleosaccular dysplasia, Dalmatian dog, Histopathology

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 This work was supported in part by the International Hearing Foundation, The Starkey Hearing Foundation and Hubbard Broadcasting Foundation, MN, USA.

☆☆ Presented as a poster in the Annual Meeting of the AAO-HNS, NY, September, 2004 and as an oral presentation in the 8th Conference on Cholesteatoma and Ear Surgery, Turkey, July 2008.

PII: S0165-5876(10)00251-X

doi:10.1016/j.ijporl.2010.05.020

International Journal of Pediatric Otorhinolaryngology
Volume 74, Issue 8 , Pages 934-938, August 2010