Histopathological morphometric study of cochleosaccular dysplasia in Dalmatian dogs.
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.


CONCLUSIONS
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.
Sampaio
Andre L L
AL
Brasilia University Medical School - Capes, Fulbright Scholarship, Brasilia, Brazil. andremarjy@uol.com.br
Paine
Elizabeth
E
Schachern
Patricia A
PA
Sutherland
Carolyn
C
Cureoglu
Sebahattin
S
Olivieira
Carlos A C P
CA
Paparella
Michael M
MM
D000818:Animals; D003051:Cochlea / Q000473:pathology*; D003638:Deafness / Q000151:congenital*; D004285:Dogs; D006199:Hair Cells, Auditory, Inner / Q000473:pathology; D009925:Organ of Corti / Q000473:pathology; D012444:Saccule and Utricle / Q000473:pathology; D013136:Spiral Ganglion / Q000473:pathology; D013316:Stria Vascularis / Q000473:pathology; D013701:Temporal Bone / Q000473:pathology*