This paper presents a longitudinal evaluation of electrocochleographic asse
ssment in severely hearing-impaired infants. Electrophysiological data were
obtained by transtympanic electrocochleography to tone-burst stimuli at oc
tave frequencies of 500 to 8000 Hz at the age of 0-6 years in a group of 12
6 subjects. The results are compared with auditory thresholds determined at
school age in the same children by means of purl-tone audiometry. Cochlear
microphonics could be recorded in virtually all ears: although the majorit
y of subjects had hearing losses of 90 dB and more. Compound action potenti
als (CAPs) showed waveforms varying from normal to a wide range of abnormal
ities. Audiometric thresholds correlated generally well with the compound a
ction potential (CAP) thresholds obtained in infancy. The error in the pred
icted audiometric thresholds is between 15 and 20 dB, as compared with 11 d
B reported for more moderate hearing losses. It is shown that, in spite of
the high stimulus levels used, substantial frequency-specific threshold inf
ormation is retained. Occasional large discrepancies in thresholds were oft
en associated with markedly abnormal response waveforms. Among the many cas
es in which no ABR could be elicited, 68 per cent produced detectable elect
rocochleographic responses in the 1000-4000 Hz range. It is concluded that
electrocochleography is a valuable method for the assessment of residual he
aring in infants suspected of having a severe hearing impairment.