Objective: To describe an atypical waveform, termed an abnormal positive po
tential (APP), on round window electrocochleograms (RW ECochG) of children
and to relate its occurrence to clinical history.
Study Design: APPs were identified prospectively, and a retrospective analy
sis was made of these patients' clinical histories, audiograms, and auditor
y outcomes (hearing aid, cochlear implant, or nonauditory communication)
Setting: Tertiary referral teaching hospital, day surgery and clinics.
Patients: All 431 children <110 months of age suspected of a severe to prof
ound hearing loss who underwent RW ECochG From January 1993 to August 1997.
Intervention: Diagnostic RW ECochG for auditory threshold estimation.
Main Outcome Measure: The presence on the RW ECochG of the APP: an early po
sitive potential in the absence of a compound action potential (CAP).
Results: An APP was observed in 34 children. The APP was most marked in res
ponse to clicks and 8-kHz tones. The APP click threshold averaged 70 dB hea
ring loss. The brainstem evoked potential of these children showed an absen
ce of waves, or a broad positive wave with no subsequent waves. Twenty-nine
of 30 behavioral audiograms obtained were indicative of severe to profound
hearing loss. Auditory outcomes were available from 26 children; 45% of th
em derived no help from a hearing aid, and 8 children received a cochlear i
mplant. Clinical factors frequently associated with APP were prematurity in
combination with kernicterus or hypoxia.
Conclusions: APP thresholds were lower than neural thresholds or behavioral
thresholds. Children with APP need close follow-up, because half of those
studied needed nonauditory strategies to develop effective communication.