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Objectives/Hypothesis: Speech perception scores using cochlear implants have ranged widely in all published series. The underlying determinants of success in word recognition are incompletely defined. Although it has been assumed that residual spiral ganglion cell population in the deaf ear may play a critical role, published data from temporal bone specimens from patients have not supported this hypothesis. The depth of insertion of a multichannel cochlear implant has also been suggested as a clinical variable that may be correlated with word recognition. In the current study these correlations were evaluated in 15 human subjects. Study Design: Retrospective review of temporal bone histopathology. Methods: Temporal bones were fixed and prepared for histological study by standard techniques. Specimens were then serially sectioned and reconstructed by two-dimensional methods. The spiral ganglion cells were counted, and the depth of insertion of the cochlear implant as measured from the round window was determined. Correlation analyses were then performed between the NU6 word scores and spiral ganglion cell counts and the depth of insertion. Results: The segmental and total spiral ganglion cell counts were not significantly correlated ( P > .50) with NU6 word scores for the 15 subjects. Statistically significant correlations were not achieved by separate analysis of implant types. Similarly, no significant correlation between the depth of insertion of the electrode array and postoperative NU6 word score was identified for the group. Conclusion: Although it is unlikely that the number of residual spiral ganglion cell counts is irrelevant to the determination of word recognition following cochlear implantation, there are, clearly, other clinical variables not yet identified that play an important role in determining success with cochlear implantation. [ABSTRACT FROM AUTHOR]

Objective: To determine the histopathologic abnormalities within the cochlea in Alport syndrome. Background: Alport syndrome, which manifests as hereditary nephritis and sensorineural hearing loss (SNHL), is caused by mutations in genes that code for the ∝3, ∝4, and ∝5 chains of type IV collagen. The ∝3, ∝4, and ∝5 chains of type IV collagen are present in the basement membrane of the organ of Corti. Previous temporal bone studies have failed to identify histopathologic correlates for the SNHL. Methods: We examined temporal bones from nine individuals with a clinical diagnosis of Alport syndrome. One of our cases also had genetic testing that showed a mutation in the type IV collagen ∝5 chain gene. Results: By light microscopy, eight of nine cases demonstrated two unique pathologic changes: 1) a 'zone of separation' between the basilar membrane and overlying cells of the organ of Corti and 2) presence of cells filling the tunnel of Corti and extracellular spaces of Nuel. The cytologic losses of hair cells, stria vascularis, and cochlear neuronal cells were insufficient to account for the observed SNHL in our cases. Electron microscopy was performed in four cases; all four demonstrated the following: 1) the zone of separation that was observed at light microscopy occurred between the basement membrane and the basilar membrane, 2) the cells within the tunnel of Corti and spaces of Nuel were morphologically similar to supporting cells, and 3) the basement membrane of strial capillaries and the spiral vessel (under the basilar membrane) were normal. Conclusions: The histopathologic correlates of cochlear involvement in Alport syndrome are abnormalities of the basement membrane of cells of the organ of Corti and dysmorphogenesis (cellular infilling of the tunnel and extracellular spaces) of the organ of Corti. We hypothesize that these abnormalities result in SNHL by altering cochlear micromechanics. [ABSTRACT FROM AUTHOR]

The histopathology of the temporal bones of a patient who died of unrelated causes 10 weeks following cochlear implantation using a Richards Ineraid® device is presented. Deafness was caused by a prolonged course of intravenous gentamycin therapy 5 years prior to implantation. The electrode array of the cochlear implant was left in situ throughout histologic preparation and sectioning. Despite displacement and disruption of supporting structures of the inner ear, particularly in the 6-to-15-mm range as measured from the round window, there was no significant difference in the mean densities of spiral ganglion cells in the implanted and unimplanted sides. This case is presented as evidence that despite significant disruption of supporting elements of the inner ear, which is common during cochlear implantation, there appears to be little effect on the residual spiral ganglion cell count, at least in the short term. [ABSTRACT FROM AUTHOR]