Binaural Unmasking with Bilateral Cochlear Implants

Nearly 100,000 deaf patients worldwide have had their hearing restored by a cochlear implant (CI) fitted to one ear. However, although many patients understand speech well in quiet, even the most successful experience difficulty in noisy situations. In contrast, normal-hearing (NH) listeners achieve improved speech understanding in noise by processing the differences between the waveforms reaching the two ears. Here we show that a form of binaural processing can be achieved by patients fitted with an implant in each ear, leading to substantial improvements in signal detection in the presence of competing sounds. The stimulus in each ear consisted of a narrowband noise masker, to which a tonal signal was sometimes added; this mixture was half-wave rectified, lowpass-filtered, and then used to modulate a 1000-pps biphasic pulse train. All four CI users tested showed significantly better signal detection when the signal was presented out of phase at the two ears than when it was in phase. This advantage occurred even though subjects only received information about the slowly varying sound envelope to be presented, contrary to previous reports that waveform fine structure dominates binaural processing. If this advantage generalizes to multichannel situations, it would demonstrate that envelope-based CI speech-processing strategies may allow patients to exploit binaural unmasking in order to improve speech understanding in noise. Furthermore, because the tested patients had been deprived of binaural hearing for eight or more years, our results show that some sensitivity to time-varying interaural cues can persist over extended periods of binaural deprivation.     

Sensitivity to Interaural Time Differences with Combined Cochlear Implant and Acoustic Stimulation

The interaural time difference (ITD) is an important cue to localize sound sources. Sensitivity to ITD was measured in eight users of a cochlear implant (CI) in the one ear and a hearing aid (HA) in the other severely impaired ear. The stimulus consisted of an electric pulse train of 100 pps and an acoustic filtered click train. Just-noticeable differences (JNDs) in ITD were measured using a lateralization paradigm. Four subjects exhibited median JNDs in ITD of 156, 341, 254, and 91 μs; the other subjects could not lateralize the stimuli consistently. Only the subjects who could lateralize had average acoustic hearing thresholds at 1,000 and 2,000 Hz better than 100-dB SPL. The electric signal had to be delayed by 1.5 ms to achieve synchronous stimulation at the auditory nerves.

Interaural Time-Delay Sensitivity in Bilateral Cochlear Implant Users: Effects of Pulse Rate, Modulation Rate, and Place of Stimulation

Electrical interaural time delay (ITD) discrimination was measured using 300-ms bursts applied to binaural pitch matched electrodes at basal, mid, and apical locations in each ear. Six bilateral implant users, who had previously shown good ITD sensitivity at a pulse rate of 100 pulses per second (pps), were assessed. Thresholds were measured as a function of pulse rate between 100 and 1,000 Hz, as well as modulation rate over that same range for high-rate pulse trains at 6,000 pps. Results were similar for all three places of stimulation and showed decreasing ITD sensitivity as either pulse rate or modulation rate increased, although the extent of that effect varied across subjects. The results support a model comprising a common ITD mechanism for high- and low-frequency places of stimulation, which, for electrical stimulation, is rate-limited in the same way across electrodes because peripheral temporal responses are largely place invariant. Overall, ITD sensitivity was somewhat better with unmodulated pulse trains than with high-rate pulse trains modulated at matched rates, although comparisons at individual rates showed that difference to be significant only at 300 Hz. Electrodes presenting with the lowest thresholds at 600 Hz were further assessed using bursts with a ramped onset of 10 ms. The slower rise time resulted in decreased performance in four of the listeners, but not in the two best performers, indicating that those two could use ongoing cues at 600 Hz. Performance at each place was also measured using single-pulse stimuli. Comparison of those data with the unmodulated 300-ms burst thresholds showed that on average, the addition of ongoing cues beyond the onset enhanced overall ITD sensitivity at 100 and 300 Hz, but not at 600 Hz. At 1,000 Hz, the added ongoing cues actually decreased performance. That result is attributed to the introduction of ambiguous cues within the physiologically relevant range and increased dichotic firing.     

Neural and Behavioral Sensitivity to Interaural Time Differences Using Amplitude Modulated Tones with Mismatched Carrier Frequencies

Bilateral cochlear implantation is intended to provide the advantages of binaural hearing, including sound localization and better speech recognition in noise. In most modern implants, temporal information is carried by the envelope of pulsatile stimulation, and thresholds to interaural time differences (ITDs) are generally high compared to those obtained in normal hearing observers. One factor thought to influence ITD sensitivity is the overlap of neural populations stimulated on each side. The present study investigated the effects of acoustically stimulating bilaterally mismatched neural populations in two related paradigms: rabbit neural recordings and human psychophysical testing. The neural coding of interaural envelope timing information was measured in recordings from neurons in the inferior colliculus of the unanesthetized rabbit. Binaural beat stimuli with a 1-Hz difference in modulation frequency were presented at the best modulation frequency and intensity as the carrier frequencies at each ear were varied. Some neurons encoded envelope ITDs with carrier frequency mismatches as great as several octaves. The synchronization strength was typically nonmonotonically related to intensity. Psychophysical data showed that human listeners could also make use of binaural envelope cues for carrier mismatches of up to 2–3 octaves. Thus, the physiological and psychophysical data were broadly consistent, and suggest that bilateral cochlear implants should provide information sufficient to detect envelope ITDs even in the face of bilateral mismatch in the neural populations responding to stimulation. However, the strongly nonmonotonic synchronization to envelope ITDs suggests that the limited dynamic range with electrical stimulation may be an important consideration for ITD encoding.     

Models of Brainstem Responses to Bilateral Electrical Stimulation

A simple, biophysically specified cell model is used to predict responses of binaurally sensitive neurons to patterns of input spikes that represent stimulation by acoustic and electric waveforms. Specifically, the effects of changes in parameters of input spike trains on model responses to interaural time difference (ITD) were studied for low-frequency periodic stimuli, with or without amplitude modulation. Simulations were limited to purely excitatory, bilaterally driven cell models with basic ionic currents and multiple input fibers. Parameters explored include average firing rate, synchrony index, modulation frequency, and latency dispersion of the input trains as well as the excitatory conductance and time constant of individual synapses in the cell model. Results are compared to physiological recordings from the inferior colliculus (IC) and discussed in terms of ITD-discrimination abilities of listeners with cochlear implants. Several empirically observed aspects of ITD sensitivity were simulated without evoking complex neural processing. Specifically, our results show saturation effects in rate–ITD curves, the absence of sustained responses to high-rate unmodulated pulse trains, the renewal of sensitivity to ITD in high-rate trains when inputs are amplitude-modulated, and interactions between envelope and fine-structure delays for some modulation frequencies.     

Barriers to Cochlear Implant Access: Acknowledging the Challenges,Changing the Future: The Importance of Access to Bilateral Hearing through Cochlear Implants in Children

Children with hearing loss require early access to sound in both ears to support their development. In this article, we describe barriers to providing bilateral hearing and developmental consequences of delays during early sensitive periods. Barriers include late identification of hearing loss in one or both ears and delayed access to intervention with hearing devices such as cochlear implants. Effects of delayed bilateral input on the auditory pathways and brain are discussed as well as behavioral effects on speech perception and other developmental outcomes including language and academics. Evidence for these effects has supported an evolution in cochlear implant candidacy in children that was started with unilateral implantation in children with profound deafness bilaterally to bilateral implantation to implantation of children with asymmetric hearing loss including children with single-side deafness. Opportunities to enhance the developmental benefits of bilateral hearing in children with hearing loss are also discussed including efforts to improve binaural/spatial hearing and consideration of concurrent vestibular deficits which are common in children with hearing loss.     

Audio-Visual Speech Intelligibility Benefits with Bilateral Cochlear Implants when Talker Location Varies

One of the key benefits of using cochlear implants (CIs) in both ears rather than just one is improved localization. It is likely that in complex listening scenes, improved localization allows bilateral CI users to orient toward talkers to improve signal-to-noise ratios and gain access to visual cues, but to date, that conjecture has not been tested. To obtain an objective measure of that benefit, seven bilateral CI users were assessed for both auditory-only and audio-visual speech intelligibility in noise using a novel dynamic spatial audio-visual test paradigm. For each trial conducted in spatially distributed noise, first, an auditory-only cueing phrase that was spoken by one of four talkers was selected and presented from one of four locations. Shortly afterward, a target sentence was presented that was either audio-visual or, in another test configuration, audio-only and was spoken by the same talker and from the same location as the cueing phrase. During the target presentation, visual distractors were added at other spatial locations. Results showed that in terms of speech reception thresholds (SRTs), the average improvement for bilateral listening over the better performing ear alone was 9 dB for the audio-visual mode, and 3 dB for audition-alone. Comparison of bilateral performance for audio-visual and audition-alone showed that inclusion of visual cues led to an average SRT improvement of 5 dB. For unilateral device use, no such benefit arose, presumably due to the greatly reduced ability to localize the target talker to acquire visual information. The bilateral CI speech intelligibility advantage over the better ear in the present study is much larger than that previously reported for static talker locations and indicates greater everyday speech benefits and improved cost-benefit than estimated to date.     

Brainstem Auditory Evoked Potentials Suggest a Role for the Ventral Cochlear Nucleus in Tinnitus

Numerous studies have demonstrated elevated spontaneous and sound-evoked brainstem activity in animal models of tinnitus, but data on brainstem function in people with this common clinical condition are sparse. Here, auditory nerve and brainstem function in response to sound was assessed via auditory brainstem responses (ABR) in humans with tinnitus and without. Tinnitus subjects showed reduced wave I amplitude (indicating reduced auditory nerve activity) but enhanced wave V (reflecting elevated input to the inferior colliculi) compared with non-tinnitus subjects matched in age, sex, and pure-tone threshold. The transformation from reduced peripheral activity to central hyperactivity in the tinnitus group was especially apparent in the V/I and III/I amplitude ratios. Compared with a third cohort of younger, non-tinnitus subjects, both tinnitus, and matched, non-tinnitus groups showed elevated thresholds above 4 kHz and reduced wave I amplitude, indicating that the differences between tinnitus and matched non-tinnitus subjects occurred against a backdrop of shared peripheral dysfunction that, while not tinnitus specific, cannot be discounted as a factor in tinnitus development. Animal lesion and human neuroanatomical data combine to indicate that waves III and V in humans reflect activity in a pathway originating in the ventral cochlear nucleus (VCN) and with spherical bushy cells (SBC) in particular. We conclude that the elevated III/I and V/I amplitude ratios in tinnitus subjects reflect disproportionately high activity in the SBC pathway for a given amount of peripheral input. The results imply a role for the VCN in tinnitus and suggest the SBC pathway as a target for tinnitus treatment.     

Modulation Enhancement in the Electrical Signal Improves Perception of Interaural Time Differences with Bimodal Stimulation

Interaural timing cues are important for sound source localization and for binaural unmasking of speech that is spatially separated from interfering sounds. Users of a cochlear implant (CI) with residual hearing in the non-implanted ear (bimodal listeners) can only make very limited use of interaural timing cues with their clinical devices. Previous studies showed that bimodal listeners can be sensitive to interaural time differences (ITDs) for simple single- and three-channel stimuli. The modulation enhancement strategy (MEnS) was developed to improve the ITD perception of bimodal listeners. It enhances temporal modulations on all stimulated electrodes, synchronously with modulations in the acoustic signal presented to the non-implanted ear, based on measurement of the amplitude peaks occurring at the rate of the fundamental frequency in voiced phonemes. In the first experiment, ITD detection thresholds were measured using the method of constant stimuli for five bimodal listeners for an artificial vowel, processed with either the advanced combination encoder (ACE) strategy or with MEnS. With MEnS, detection thresholds were significantly lower, and for four subjects well within the physically relevant range. In the second experiment, the extent of lateralization was measured in three subjects with both strategies, and ITD sensitivity was determined using an adaptive procedure. All subjects could lateralize sounds based on ITD and sensitivity was significantly better with MEnS than with ACE. The current results indicate that ITD cues can be provided to bimodal listeners with modified sound processing.     

Binaural advantages in using a cochlear implant for adults with profound unilateral hearing loss

Background: Recent studies of cochlear implants (CIs) in profound unilateral hearing loss (UHL) patients have demonstrated a restoration of some binaural hearing.

Aims/Objectives: The objective was to evaluate three possible advantages of binaural hearing in CIs adult users with UHL including single-side deafness (SSD) and asymmetric hearing loss (AHL) subgroups.

Material and methods: A prospective study was conducted that included 70 sequentially implanted patients. Subgroups of these subjects included 64 with a postlingual onset of a profound hearing loss on the implanted side and 6 with a prelingual onset of that loss. Three binaural effects – redundancy, head shadow, and squelch – were evaluated.

Results: Significant differences between the ‘CI on’ and ‘CI off’ conditions were found for all three binaural effects for the study group as a whole and for the postlingual subgroup. However, results for the subjects in the prelingual subgroup did not demonstrate any of the binaural advantages.

Conclusion and significance: Patients with a postlingual onset of a profound hearing loss in one ear and normal hearing or only a moderate loss in the other ear are able to make the effective use of a CI in the profound-loss ear in conjunction with acoustic stimulation of the other ear.     

Auditory Prosthesis with a Penetrating Nerve Array

Contemporary auditory prostheses (“cochlear implants”) employ arrays of stimulating electrodes implanted in the scala tympani of the cochlea. Such arrays have been implanted in some 100,000 profoundly or severely deaf people worldwide and arguably are the most successful of present-day neural prostheses. Nevertheless, most implant users show poor understanding of speech in noisy backgrounds, poor pitch recognition, and poor spatial hearing, even when using bilateral implants. Many of these limitations can be attributed to the remote location of stimulating electrodes relative to excitable cochlear neural elements. That is, a scala tympani electrode array lies within a bony compartment filled with electrically conductive fluid. Moreover, scala tympani arrays typically do not extend to the apical turn of the cochlea in which low frequencies are represented. In the present study, we have tested in an animal model an alternative to the conventional cochlear implant: a multielectrode array implanted directly into the auditory nerve. We monitored the specificity of stimulation of the auditory pathway by recording extracellular unit activity at 32 sites along the tonotopic axis of the inferior colliculus. The results demonstrate the activation of specific auditory nerve populations throughout essentially the entire frequency range that is represented by characteristic frequencies in the inferior colliculus. Compared to conventional scala tympani stimulation, thresholds for neural excitation are as much as 50-fold lower and interference between electrodes stimulated simultaneously is markedly reduced. The results suggest that if an intraneural stimulating array were incorporated into an auditory prosthesis system for humans, it could offer substantial improvement in hearing replacement compared to contemporary cochlear implants.     

Across-Site Variation in Detection Thresholds and Maximum Comfortable Loudness Levels for Cochlear Implants

In cochlear implants, variation across stimulation sites in psychophysical detection thresholds (T levels) and maximum comfortable loudness levels (C levels) can be large when narrow-bipolar (BP) stimulation is used. This across-site variation is typically smaller when monopolar (MP) stimulation is used. At least two models can account for across-site variation and the effects of electrode configuration on the magnitude of the variation. According to one model, across-site variation reflects site-to-site differences in the distances between the stimulating electrodes and the sites of action-potential initiation. Under this model, the lower across-site variation with MP stimulation is due to shallower current versus distance gradients. An alternative model assumes that T and C levels depend on integration of activity across the whole population of neurons and that MP stimulation activates neurons over a larger spatial extent than does BP stimulation. If T and C levels are determined by integration of activity across large overlapping populations of neurons, then their values at adjacent sites should be more similar than if these levels result from integration across smaller, more independent populations. We tested the models by examining the effects on across-site variation of three variables believed to affect the spatial extent of activation: electrode configuration, stimulus level within the dynamic range, and electrode-array design. T levels and C levels were measured in 13 subjects with Nucleus^® CI24M (straight array) and 9 subjects with Nucleus^® CI24R(CS) (Contour) cochlear implants using bipolar (BP) and monopolar (MP) electrode configurations. Site-to-site variation in T and C levels for BP stimulation was 2.1–3.3 times larger than that for MP stimulation. Contrary to the across-neuron integration hypothesis, no significant differences were found between across-site variation for T levels and that for C levels for the BP configuration. There was considerable overlap in site-to-site variation values for the two types of implants but mean site-to-site variation in C levels for CI24M implants was significantly lower than that for CI24R(CS) implants. Control studies suggested that these results were not an artifact of the scale, and not due to differences in inherent variability of the psychophysical measures, or to the method of quantifying across-site variation.     

Sensitivity to Interaural Correlation of Single Neurons in the Inferior Colliculusof Guinea Pigs

Sensitivity to changes in the interaural correlation of 50-ms bursts of narrowband or broadband noise was measured in single neurons in the inferior colliculus of urethane-anaesthetized guinea pigs. Rate vs. interaural correlation functions (rICFs) were measured using two methods. These methods compensated in different ways for the inherent variance in interaural correlation between tokens with the same expected correlation. The shape of all rICFs could be best described by power functions allowing them to be summarized by two parameters. Most rICFs were best fit by a power below 2, indicating that they were only slightly nonlinear. However, there were a few fitted functions that had a power of 3–6, indicating marked curvature. Modeling results indicate that the nonlinearity of the majority of rICFs was explicable in terms of the monaural transduction stages; however, some of the rICFs with power greater than 2 require either multiple inputs to the coincidence detector or additional nonlinearities to be included in the model. Discrimination thresholds were estimated at reference correlations of −1, 0, and +1 using receiver operating characteristic (ROC) analysis of the spike-count distribution at each correlation. Thresholds spanned the full possible range, from a minimum of 0.1 to the maximum possible of 2. Thresholds were generally highest with a reference correlation of −1, intermediate with a reference of 0, and lowest with a reference correlation of +1. Thresholds were lowest for the most steeply sloped rICFs, but thresholds were not strongly correlated to the spike rate variance. The lowest thresholds occurred using narrowband noise that was compensated for internal delays, but they were still about three times larger than human psychophysical thresholds measured using similar stimuli. The data suggest that, unlike pure tone interaural time difference, discrimination of a population measure is required to account for behavioral interaural correlation discrimination performance.     

Perceptual Interactions Between Electrodes Using Focused and Monopolar Cochlear Stimulation

In today’s cochlear implant (CI) systems, the monopolar (MP) electrode configuration is the most commonly used stimulation mode, requiring only a single current source. However, with an implant that will allow simultaneous activation of multiple independent current sources, it is possible to implement an all-polar (AP) stimulation mode designed to create a focused electrical field. The goal of this experiment was to study the potential benefits of this all-polar mode for reducing uncontrolled electrode interactions compared with the monopolar mode. The five participants who took part in the study were implanted with a research device that was connected via a percutaneous connector to a benchtop stimulator providing 22 independent current sources. The perceptual effects of the AP mode were tested in three experiments. In Experiment 1, the current level difference between loudness-matched sequential and simultaneous stimuli composed of 2 spatially separated pulse trains was measured as function of the electrode separation. Results indicated a strong current-summation interaction for simultaneous stimuli in the MP mode for separations up to at least 4.8 mm. No significant interaction was found in the AP mode beyond a separation of 2.4 mm. In Experiment 2, a forward-masking paradigm was used with fixed equally loud probes in AP and MP modes, and AP maskers presented on different electrode positions. Results indicated a similar spatial masking pattern between modes. In Experiment 3, subjects were asked to discriminate between across-electrode temporal delays. It was hypothesized that discrimination would decrease with electrode separation faster in AP compared to MP modes. However, results showed no difference between the two modes. Overall, the results indicated that the AP mode produced less current spread than MP mode but did not lead to a significant advantage in terms of spread of neuronal excitation at equally loud levels.     

Effects of Electrical Stimulation of Olivocochlear Fibers in Cochlear Potentials in the Chinchilla

The mammalian cochlea has two types of sensory cells; inner hair cells, which receive auditory-nerve afferent innervation, and outer hair cells, innervated by efferent axons of the medial olivocochlear (MOC) system. The role of the MOC system in hearing is still controversial. Recently, by recording cochlear potentials in behaving chinchillas, we suggested that one of the possible functions of the efferent system is to reduce cochlear sensitivity during attention to other sensory modalities (Delano et al. in J Neurosci 27:4146–4153, 2007). However, in spite of these compelling results, the physiological effects of electrical MOC activation on cochlear potentials have not been described in detail in chinchillas. The main objective of the present work was to describe these efferent effects in the chinchilla, comparing them with those in other species and in behavioral experiments. We activated the MOC efferent axons in chinchillas with sectioned middle-ear muscles by applying current pulses at the fourth-ventricle floor. Auditory-nerve compound action potentials (CAP) and cochlear microphonics (CM) were acquired in response to clicks and tones of several frequencies, using a round-window electrode. Electrical efferent stimulation produced CAP amplitude suppressions reaching up to 11 dB. They were higher for low to moderate sound levels. Additionally, CM amplitude increments were found, the largest (≤ 2.5 dB) for low intensity tones. CAP suppression was present at all stimulus frequencies, but was greatest for 2 kHz. CM increments were highest for low-frequency tones, and almost absent at high frequencies. We conclude that the effect obtained in chinchilla is similar to but smaller than that observed in cats, and that the effects seen in awake chinchillas, albeit different in magnitude, are consistent with the activation of efferent fibers.     

Electrode Spanning with Partial Tripolar Stimulation Mode in Cochlear Implants

The perceptual effects of electrode spanning (i.e., the use of nonadjacent return electrodes) in partial tripolar (pTP) mode were tested on a main electrode EL8 in five cochlear implant (CI) users. Current focusing was controlled by σ (the ratio of current returned within the cochlea), and current steering was controlled by α (the ratio of current returned to the basal electrode). Experiment 1 tested whether asymmetric spanning with α = 0.5 can create additional channels around standard pTP stimuli. It was found that in general, apical spanning (i.e., returning current to EL6 rather than EL7) elicited a pitch between those of standard pTP stimuli on main electrodes EL8 and EL9, while basal spanning (i.e., returning current to EL10 rather than EL9) elicited a pitch between those of standard pTP stimuli on main electrodes EL7 and EL8. The pitch increase caused by apical spanning was more salient than the pitch decrease caused by basal spanning. To replace the standard pTP channel on the main electrode EL8 when EL7 or EL9 is defective, experiment 2 tested asymmetrically spanned pTP stimuli with various α, and experiment 3 tested symmetrically spanned pTP stimuli with various σ. The results showed that pitch increased with decreasing α in asymmetric spanning, or with increasing σ in symmetric spanning. Apical spanning with α around 0.69 and basal spanning with α around 0.38 may both elicit a similar pitch as the standard pTP stimulus. With the same σ, the symmetrically spanned pTP stimulus was higher in pitch than the standard pTP stimulus. A smaller σ was thus required for symmetric spanning to match the pitch of the standard pTP stimulus. In summary, electrode spanning is an effective field-shaping technique that is useful for adding spectral channels and handling defective electrodes with CIs.     

Improving Interaural Time Difference Sensitivity Using Short Inter-pulse Intervals with Amplitude-Modulated Pulse Trains in Bilateral Cochlear Implants

Journal of the Association for Research in Otolaryngology - Interaural time differences (ITDs) at low frequencies are important for sound localization and spatial speech unmasking. These ITD cues...     

Cochlear implantation in a patient with Paget's disease

Hearing loss in Paget's disease, a metabolic bone disease, has been reported in as many as 60% of cases, and is presumably related to changes in mineral density of the otic capsule. We describe a 59-year-old man with Paget's disease who had severe to profound hearing loss and was referred to us for cochlear implantation. Preoperative evaluation revealed poor communication skills despite significant residual hearing, raising concerns about postimplantation outcome. Nevertheless, implantation was successful. As our literature search yielded only one report of cochlear implantation in Paget's disease, we record our clinical experience and discuss the hearing rehabilitation dilemmas in this case.     

Cochlear implantation for children with GJB2-related deafness

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.