The temporal bone pathology of a 74-year-old female affected by vestibular schwannoma was compared with findings of auditory brainstem response and electrocochleography. At age 71, she complained of hearing loss in the left ear in which pure tone audiometry revealed threshold elevation in the middle- and high-frequency range. Temporal bone CT scanning revealed a medium-sized cerebellopontine angle tumour in the left ear. ABR showed no response in the left ear, but the electrocochleography showed clear compound action potentials. Three years later, at age 74, she died of metastatic lung cancer and sepsis. The left temporal bone pathology consisted primarily of a large vestibular schwannoma occupying the internal auditory meatus. The organ of Corti was well preserved in each turn. In the modiolus, the numbers of spiral ganglion cells and cochlear nerve fibres in each turn were decreased. These histological findings suggest that clear compound action potentials were recorded from the distal portion of the cochlear nerve in spite of the presence of the vestibular schwannoma, but ABR could not be detected because of the blockade of the proximal portion of the cochlear nerve by the vestibular schwannoma.