Cortical potentials evoked by tone frequency changes compared to frequency discrimination and speech perception: Thresholds in normal-hearing and hearing-impaired subjects.

Frequency discrimination ability varies within the normal hearing population, partially explained by factors such as musical training and age, and it deteriorates with hearing loss. Frequency discrimination, while essential for several auditory tasks, is not routinely measured in clinical setting. This study investigates cortical auditory evoked potentials in response to frequency changes, known as acoustic change complexes (ACCs), and explores their value as a clinically applicable objective measurement of frequency discrimination. In 12 normal-hearing and 13 age-matched hearing-impaired subjects, ACC thresholds were recorded at 4 base frequencies (0.5, 1, 2, 4 kHz) and compared to psychophysically assessed frequency discrimination thresholds. ACC thresholds had a moderate to strong correlation to psychophysical frequency discrimination thresholds. In addition, ACC thresholds increased with hearing loss and higher ACC thresholds were associated with poorer speech perception in noise. The ACC threshold in response to a frequency change therefore holds promise as an objective clinical measurement in hearing impairment, indicative of frequency discrimination ability and related to speech perception. However, recordings as conducted in the current study are relatively time consuming. The current clinical application would be most relevant in cases where behavioral testing is unreliable.

Assessing auditory nerve condition by tone decay in deaf subjects with a cochlear implant.

The condition of the auditory nerve is a factor determining hearing performance of cochlear implant (CI) recipients. Abnormal loudness adaptation is associated with poor auditory nerve survival. We examined which stimulus conditions are suitable for tone decay measurements to differentiate between CI recipients with respect to their speech perception. Tone decay was defined here as occurring when the percept disappears before the stimulus stops. We measured the duration of the percept of a 60-s pulse train. Current levels ranged from below threshold up to maximum acceptable loudness, pulse rates from 250 to 5000 pulses/s, and duty cycles (percentages of time the burst of pulses is on) from 10% to 100%. Ten adult CI recipients were included: seven with good and three with poor speech perception. Largest differences among the subjects were found at 5000 pulses/s and 100% duty cycle. The well performing subjects had a continuous percept of the 60-s stimulus within 3 dB above threshold. Two poorly performing subjects showed abnormal loudness adaptation, that is, no continuous percept even at levels greater than 6 dB above threshold. We conclude that abnormal loudness adaptation can be detected via an electric tone decay test using a high pulse rate and 100% duty cycle.

Hearing loss and speech perception in noise difficulties in Fanconi anemia.

OBJECTIVES/HYPOTHESIS: Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients. STUDY DESIGN: Retrospective chart review. METHODS: A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties. RESULTS: In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%). CONCLUSIONS: Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2358-2361, 2017.