Although pulmonary infections caused by Pseudomonas aeruginosa can hardly be eradicated in patients with cystic fibrosis (CF, the most common genetic disease among Caucasians), these patients are mainly treated with intravenous and nebulized tobramycin. Long-term treatment with tobramycin, however, may induce ototoxic effects. We assessed the clinical histories and postmortem temporal bones of six patients with CF for signs of this ototoxicity. Four bones showed typical manifestations of ototoxicity induced by aminoglycosides (AGs): loss of hair cells in the lower turns, and degeneration of ganglion cells. Six bones revealed no loss or scattered loss of hair cells, however, degeneration of the spiral ganglion cells was observed. This suggests that degeneration of the spiral ganglion may occur as a primary manifestation in some cases of ototoxicity due to aminoglycosides. Recent reports have shown that trophic factors (neurotrophins and acidic fibroblast growth factor) interacting with hair cells and the spiral ganglion protect the inner ear from damage. It may be that disturbances in supply of such trophic factors caused degeneration of ganglion cells without loss of hair cells in the cases we studied.