Characterization of newborn hearing screening failures in multigestational births.

OBJECTIVE: To define the rate and characterize the type of newborn hearing screening failures in multigestational births. METHODS: Retrospective chart review of all multigestational births that occurred in a 10-year period (2002-2012) in which at least one newborn failed newborn hearing screening at two tertiary care hospitals in the Northwell Health System. RESULTS: Out of 125,405 total births, we identified 2961 multigestational births, of which 59 (2.0%) newborns failed newborn hearing screening. None of their 66 twin/triplet siblings failed their newborn hearing screens. Of 43 newborns that returned for follow-up, 56.0% (24/43) had confirmed hearing loss, resulting in an overall rate of 0.81% in all multigestational newborns with hearing loss. Of 19 infants that passed repeat testing, two were judged to need myringotomy tube placement. Twenty-four infants had a confirmed hearing loss, 11 of which had sensorineural hearing loss (0.37%), and 13 with a conductive or mixed hearing loss (0.44%). CONCLUSIONS: We identified a greater than expected risk of conductive hearing loss, not attributable to otitis media, than sensorineural hearing loss in this population. These observations are consistent with the increased risk of birth defects in multigestational births.

The experience of bilateral cochlear implantation in a child with LEOPARD syndrome.

We present a 3-year old boy with Leopard syndrome. His clinical manifestations included a congenital bilateral sensorineural hearing loss. He underwent cochlear implantation on the right side at age 1 year and on the left side at age 1.5 years. The patient is doing very well and mainstreamed in a regular pre-school program with a teacher of the deaf and home based speech therapy. Bilateral cochlear implantation in the case of a child with Leopard syndrome can be successful.

Electrical compound action potentials recorded with automated neural response telemetry: threshold changes as a function of time and electrode position.

OBJECTIVE: Since the introduction of neural response telemetry (NRT) for the Nucleus 24 cochlear implant (CI24), researchers and clinicians have investigated the feasibility of using the electrically evoked compound action potential (ECAP) threshold to objectively predict psychophysical measurements that are used in the programming of the speech processor. The ability to substitute objective for behavioral measurements, particularly measurements made at the time of surgery, would greatly facilitate programming the MAP for young children and other individuals who are not able to provide reliable behavioral data required for MAP programming. There have been a number of studies that have examined characteristics of the ECAP measured at the time of surgery and postoperatively; however, all the available published data are based on the CI24. With the introduction of the Nucleus Freedom device, an automated NRT (AutoNRT) program became available, which was capable of measuring ECAP thresholds at lower levels than was previously possible with NRT software associated with the CI24 device. It was hypothesized that the enhancements to the NRT program may improve the predictability of postoperative measurements from intraoperatively recorded ECAP thresholds. The purpose of this study was to track ECAP thresholds obtained using AutoNRT as a function of time and electrode position. DESIGN: ECAP thresholds were recorded from 71 children and adults implanted with the Nucleus Freedom device using the AutoNRT test protocol. ECAP thresholds were obtained at the time of surgery, at initial stimulation, and 3 mos poststimulation. Five electrodes located at basal, middle, and apical positions in the cochlea were tested at each time interval and thresholds were compared. RESULTS: Significant differences were found in ECAP thresholds measured with AutoNRT as a function of both time and electrode position. Basal electrodes had higher ECAP thresholds than apical electrodes and that relationship was consistent for each time period. Thresholds for all electrodes decreased between surgery and initial stimulation and remained relatively stable at 3 mos poststimulation. ECAP thresholds were consistently lower for children compared with adults at each time point. Mid-array electrodes (11 and 16) showed the least amount of change over time. CONCLUSIONS: AutoNRT thresholds demonstrated significant change over time, limiting the ability to use intraoperatively recorded ECAP thresholds to predict postoperative measurements. In this study, electrodes 11 and 16 showed the least amount of change in ECAP threshold over time and therefore would be the best choices for estimating postoperative ECAP thresholds. Although not an ideal solution, mid-array ECAP thresholds obtained intraoperatively may prove to be helpful in creating a first MAP when no other behavioral or electrophysiological data are available.