Acoustic component programming in children with cochlear implants using electrocochleography.

OBJECTIVE: Objective measurements improve reliability and effectiveness of hearing assessment and cochlear implant (CI) programming in young children. In CI recipients with acoustic hearing in the implanted ear, electrocochleography (ECochG) can be conducted using intracochlear electrodes. The cochlear microphonic (CM) portion of ECochG has been shown to correlate with pure-tone audiometric thresholds in adult and paediatric CI recipients. Our goal was to determine if ECochG thresholds can be used to appropriately fit the acoustic component to the implanted ear in children. DESIGN: Prospective. STUDY SAMPLE: Eight children (aged 3.5-15.5 years, 10 ears) implanted with Advanced Bionics HiFocus Mid-Scala electrode array were recruited. CI ear acoustic thresholds were measured behaviourally and via ECochG. Two acoustic component enabled CI programs were created using the two sets of thresholds. Age and language appropriate speech outcomes and subjective feedback were obtained. RESULTS: Speech scores were equivalent with the behavioural and ECochG thresholds programs. Subjectively, the ECochG thresholds program was preferred by 7/8 subjects. One subject preferred to use an electric only program. CONCLUSION: Our data suggest that ECochG thresholds can be used to supplement the behavioural clinical methods and aid the reliable fitting of the acoustic component in paediatric CI recipients.

Comparison of Pure-Tone Thresholds and Cochlear Microphonics Thresholds in Pediatric Cochlear Implant Patients.

OBJECTIVES: In adult cochlear implant patients, conventional audiometry is used to measure postoperative residual hearing which requires active listening and patient feedback. However, audiological measurements in pediatric cochlear implant patients are both challenging as well as time consuming. Intracochlear electrocochleography (ECOG) offers an objective and a time-efficient method to measure frequency-specific cochlear microphonic or difference thresholds (CM/DIF) thresholds that closely approximate auditory thresholds in adult cochlear implant patients. The correlation between CM/DIF and behavioral thresholds has not been established in pediatric cochlear implant patients. In the present study, CM/DIF thresholds were compared with audiometric thresholds in pediatric cochlear implant patients with postoperative residual hearing. DESIGN: Thirteen (11 unilateral and 2 bilateral) pediatric cochlear implant patients (mean age = 9.2 years ± 5.1) participated in this study. Audiometric thresholds were estimated using conventional, condition play, or visual reinforcement audiometry. A warble tone stimulus was used to measure audiometric thresholds at 125, 250, 500, 1000, and 2000 Hz. ECOG waveforms were elicited using 50-msec acoustic tone-bursts. The most apical intracochlear electrode was used as the recording electrode with an extra-cochlear ground electrode. The ECOG waveforms were analyzed to determine CM/DIF thresholds that were compared with pediatric cochlear implant patients' audiometric thresholds. RESULTS: The results show a significant correlation (r = 0.77, p < 0.01) between audiometric and CM/DIF thresholds over a frequency range of 125 to 2000 Hz in pediatric cochlear implant patients. Frequency-specific comparisons revealed a correlation of 0.82, 0.74, 0.69, 0.41, and 0.32 between the audiometric thresholds and CM/DIF thresholds measured at 125, 250, 500, 1000, and 2000 Hz, respectively. An average difference of 0.4 dB (±14 dB) was measured between the audiometric and CM/DIF thresholds. CONCLUSIONS: Intracochlear ECOG can be used to measure CM/DIF thresholds in pediatric cochlear implant patients with residual hearing in the implanted ear. The CM/DIF thresholds are similar to the audiometric thresholds at lower test frequencies and offer an objective method to monitor residual hearing in difficult-to-test pediatric cochlear implant patients.

Retrocochlear hearing loss in infants: a case study of juvenile pilocytic astrocytoma.

OBJECTIVE: The purpose of this paper was to describe a child with brainstem juvenile pilocytic astrocytoma (JPA) and associated auditory symptoms. DESIGN: Case study of a 5-year-old female who presented with a complaint of headaches, accompanied with a history of symptoms that could be associated with a left unilateral congenital auditory neuropathy. She was later diagnosed with a large, left-sided posterior fossa mass determined to be a JPA. STUDY SAMPLE: Case study of a child identified at birth with a constellation of symptoms consistent with unilateral auditory neuropathy spectrum disorder (ANSD). RESULTS: The early onset symptoms, that can be the same for ANSD or for a brainstem tumor that involves the auditory nerve, suggests that the JPA was congenital. This child, if initially followed by neurology may not have been identified earlier with this tumor, however a baseline MRI or monitoring neuroimaging may have been available for reference or led to earlier detection of the lesion. It may not have changed her management, however, this case stresses the importance of the recommendations in the 'Guidelines for Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder' that include a referral to a neurologist for a baseline assessment and neuroimaging. CONCLUSION: An undetected JPA can have the same audiological clinical presentation as a unilateral ANSD. This case description highlights the need to heighten awareness for neurological and radiological monitoring in cases of ANSD, especially when the presentation is unilateral.