Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tried cochlear implants. Approximately 15% reported some benefit from hearing aids for language learning, while improvement in speech comprehension and language acquisition was reported in 85% of patients who were implanted. Approximately 5% (13/260) of the total population developed normal speech and language without intervention. Patients were diagnosed at our laboratory (n=66) or referred from other sites (n=194), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often 'ringing' cochlear microphonics, and the presence or history of otoacoustic emissions. Etiologies and co-existing conditions included genetic (n=41), peripheral neuropathies (n=20), perinatal jaundice and/or anoxia and/or prematurity (n=74). These patients comprise 10% or more of hearing impaired patients; their language acquisition trajectories are generally unpredictable from their audiograms.

Cochlear implant recipients have demonstrated remarkable increases in speech perception since US FDA approval was granted in 1984. Improved performance is due to a number of factors including improved cochlear implant technology, evolving speech coding strategies, and individuals with increasingly more residual hearing receiving implants. Despite this evolution, the same recommendations for pre- and postimplant speech recognition testing have been in place for over 10 years in the United States. To determine whether new recommendations are warranted, speech perception performance was assessed for 156 adult, postlingually deafened implant recipients as well as 50 hearing aid users on monosyllabic word recognition (CNC) and sentence recognition in quiet (HINT and AzBio sentences) and in noise (BKB-SIN). Results demonstrated that for HINT sentences in quiet, 28% of the subjects tested achieved maximum performance of 100% correct and that scores did not agree well with monosyllables (CNC) or sentence recognition in noise (BKB-SIN). For a more difficult sentence recognition material (AzBio), only 0.7% of the subjects achieved 100% performance and scores were in much better agreement with monosyllables and sentence recognition in noise. These results suggest that more difficult materials are needed to assess speech perception performance of postimplant patients – and perhaps also for determining implant candidacy.

Using the protocol outlined in this study, it is possible to reliably record EAP responses from most subjects and for most electrodes in Nucleus CI24M cochlear implant users. These responses are robust and recording these responses does not require that the subject sleep or remain still. Based on these results, a specific protocol is proposed for measurement of the EAP using the NRT system of the CI24M cochlear implant. Potential clinical implications of these results are discussed.

Objectives The primary objective of this study was to determine whether a revision and/or expansion of current audiologic cochlear implant candidacy criteria is warranted. Design The study design was a retrospective review of postoperative speech perception performance for 22 adult cochlear implant recipients who demonstrated preoperative Consonant Nucleus Consonant word recognition scores of 30% or higher in the best-aided condition. This criterion was chosen to exceed that specified by the North American clinical trial of the Nucleus Freedom cochlear implant system. Results The mean preoperative best-aided monosyllabic word score for the 22 patients was 41% correct. The degree of postoperative benefit for the best postoperative condition (electric only or bimodal) ranged from 10 to 68 percentage points with a mean benefit of 27 percentage points for the electric-only condition and 40 percentage points for the bimodal condition. Statistical analyses revealed highly significant differences between preoperative-aided, implant-only, and bimodal performance on Consonant Nucleus Consonant monosyllabic word recognition performance. That is, both postoperative scores— electric only and bimodal—were significantly different from one another and from the preoperative best-aided performance. Conclusions The current results suggest that a large-scale reassessment of manufacturer and Medicare preoperative audiologic candidacy criteria for adults is warranted to allow more hearing-impaired individuals to take advantage of the benefits offered by cochlear implantation.

Our experiences with cochlear implantation for children diagnosed with auditory neuropathy have been very positive. The five children we have implanted have not had any complications postoperatively, and each child has shown improved listening and communication skills that have enabled each child to take advantage of different communication and educational options.

No abstract

AutoNRT provides comparable accuracy to an average clinician but with the added benefit of significant time savings over manual recordings. This makes it a valuable tool for clinical measurement of ECAP threshold in cochlear implant recipients.

The statement is based on 1) preliminary research and consultation by a steering committee selected for their relevant experience representing the professions of individuals working with cochlear implants; 2) presentations by representatives of this working group on issues relevant to the consensus questions during an open session at the Consensus Conference on Cochlear Implant Soft Failures held in conjunction with the 10 th Symposium on Cochlear Implantation in Children (Dallas, Texas, March 15-19, 2005); 3) closed deliberations by members of the steering committee; and 4) input from attendees during the open session of the Consensus Conference. This statement is an independent report and is not a policy statement of any organization.The statement reflects the panel's assessment of relevant information available at the time it was written. It is to be expected that new information and data constantly are being generated that may alter the recommendations of this panel. The list of references accompanying this report includes the primary sources used by the panelists in developing their preliminary statements (1-19). OBJECTIVESTo provide professionals working in all areas of cochlear implantation with a current consensus on the terminology, definition, diagnosis, and management of suspected cochlear implant malfunctions widely referred to as soft failures. PARTICIPANTSThe panels consisted of 18 individuals directly involved in development, manufacturing, and clinical application of cochlear implants. Representatives from the specialties of otolaryngology, audiology, bioengineering, speech and language pathology, communication sciences, and industry participated. Interactive presentations were made during an open session in the Consensus Conference to an audience of approximately 190 professionals.