Quantitative study of the vestibular sensory epithelium in cochleosaccular dysplasia.
BACKGROUND
Cochleosaccular dysplasia is the most common pathologic finding seen in children with profound congenital sensorineural hearing loss. There has been no quantitative study on the peripheral vestibular system in cochleosaccular dysplasia.


OBJECTIVE
To investigate quantitatively the extent of pathologic changes of the vestibular sensory epithelium in cochleosaccular dysplasia.


METHODS
Thirteen temporal bones with congenital deafness from 10 individuals were selected for this study from the temporal bone collection of University of Minnesota that showed suitable pathologic findings for the histopathologic criteria of cochleosaccular dysplasia. Age-matched normal control temporal bones were also selected. The vestibular hair cells including types I and II hair cells were counted separately in the saccular macula, utricular macula, and three cristae of the semicircular canals using Nomarski microscopy.


RESULTS
The hair cell densities of types I and II hair cells in the macula of the saccule in cochleosaccular dysplasia were significantly decreased compared with the data of normal subjects. Both types I and II hair cells in the utricular macula and the cristae of the three semicircular canals in cochleosaccular dysplasia were well preserved, and no significant difference was observed between findings of cochleosaccular dysplasia and normal controls in the utricle and the three semicircular canals.


CONCLUSIONS
In cases with cochleosaccular dysplasia, the neurosensorial hair cells of the saccule were affected; however, the osseous labyrinth, the membranous utricle, and the semicircular canals were normal. Further studies should be performed to establish the pathogenesis of cochleosaccular dysplasia in humans.
Kariya
Shin
S
Department of Otolaryngology, University of Minnesota, Minneapolis, Minnesota, USA.
Cureoglu
Sebahattin
S
Schachern
Patricia A
PA
Sampaio
Andre L L
AL
Paparella
Michael M
MM
Kusunoki
Takeshi
T
Oktay
Mehmet F
MF
Nishizaki
Kazunori
K
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016022:Case-Control Studies; D002452:Cell Count; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities*; D000013:Congenital Abnormalities / Q000473:pathology; D003638:Deafness / Q000151:congenital* / Q000473:pathology*; D005260:Female; D018069:Hair Cells, Vestibular / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D012444:Saccule and Utricle / Q000002:abnormalities*; D013701:Temporal Bone / Q000473:pathology