A Prospective Study of Genetic Variants in Infants with Congenital Unilateral Sensorineural Hearing Loss.

Children with unilateral sensorineural hearing loss (uSNHL) have a high risk of speech-language delays and academic difficulties. Still, challenges remain in the diagnosis of uSNHL. With a prospective cross-sectional design, 20 infants were consecutively recruited from a universal newborn hearing screening program and invited to genetic testing. Eighteen of the subjects agreed to genetic testing, 15 subjects with OtoSCOPE® v.9 screening 224 genes, and four subjects underwent targeted testing, screening for chromosomal abnormalities or 105-137 gene mutations. The genetic results were described together with the 20 infants' previously published auditory profiles and imaging results. Genetic causes for the uSNHL were found in 28% of subjects (5/18) including CHARGE syndrome (CHD7), autosomal recessive non-syndromic hearing loss (GJB2), Townes-Brocks syndrome (SALL1), Pendred Syndrome (SLC26A4) and Chromosome 8P inverted duplication and deletion syndrome. In subjects with comorbidities (malformation of fingers, anus, brain, and heart), 100% were diagnosed with a genetic cause for uSNHL (3/3 subjects), while 13% (2/15 subjects) were diagnosed without comorbidities observed at birth (p = 0.002). Genetic testing for congenital uSNHL is currently efficient for alleged syndromes, whereas genetic variants for non-syndromic congenital uSNHL need further research.

Development of Sound Localization in Infants and Young Children with Cochlear Implants.

Cochlear implantation as a treatment for severe-to-profound hearing loss allows children to develop hearing, speech, and language in many cases. However, cochlear implants are generally provided beyond the infant period and outcomes are assessed after years of implant use, making comparison with normal development difficult. The aim was to study whether the rate of improvement of horizontal localization accuracy in children with bilateral implants is similar to children with normal hearing. A convenience sample of 20 children with a median age at simultaneous bilateral implantation = 0.58 years (0.42−2.3 years) participated in this cohort study. Longitudinal follow-up of sound localization accuracy for an average of ≈1 year generated 42 observations at a mean age = 1.5 years (0.58−3.6 years). The rate of development was compared to historical control groups including children with normal hearing and with relatively late bilateral implantation (≈4 years of age). There was a significant main effect of time with bilateral implants on localization accuracy (slope = 0.21/year, R2 = 0.25, F = 13.6, p < 0.001, n = 42). No differences between slopes (F = 0.30, p = 0.58) or correlation coefficients (Cohen's q = 0.28, p = 0.45) existed when comparing children with implants and normal hearing (slope = 0.16/year since birth, p = 0.015, n = 12). The rate of development was identical to children implanted late. Results suggest that early bilateral implantation in children with severe-to-profound hearing loss allows development of sound localization at a similar age to children with normal hearing. Similar rates in children with early and late implantation and normal hearing suggest an intrinsic mechanism for the development of horizontal sound localization abilities.

Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children: Comprehensive Characterization of Auditory Function and Hearing Aid Benefit.

A prospective cross-sectional design was used to characterize congenital bilateral sensorineural hearing loss (SNHL). The underlying material of >30,000 consecutively screened newborns comprised 11 subjects with nonprofound, alleged nonsyndromic, SNHL. Comprehensive audiological testing was performed at ≈11 years of age. Results showed symmetrical sigmoid-like median pure-tone thresholds (PTTs) reaching 50−60 dB HL. The congenital SNHL revealed recruitment, increased upward spread of masking, distortion product otoacoustic emission (DPOAE) dependent on PTT (≤60 dB HL), reduced auditory brainstem response (ABR) amplitude, and normal magnetic resonance imaging. Unaided recognition of speech in spatially separate competing speech (SCS) deteriorated with increasing uncomfortable loudness level (UCL), plausibly linked to reduced afferent signals. Most subjects demonstrated hearing aid (HA) benefit in a demanding laboratory listening situation. Questionnaires revealed HA benefit in real-world listening situations. This functional characterization should be important for the outline of clinical guidelines. The distinct relationship between DPOAE and PTT, up to the theoretical limit of cochlear amplification, and the low ABR amplitude remain to be elucidated. The significant relation between UCL and SCS has implications for HA-fitting. The fitting of HAs based on causes, mechanisms, and functional characterization of the SNHL may be an individualized intervention approach and deserves future research.

A Prospective Study of Etiology and Auditory Profiles in Infants with Congenital Unilateral Sensorineural Hearing Loss.

Congenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with congenital uSNHL consecutively recruited from a universal neonatal hearing-screening program. All normal-hearing ears showed ≤20 dB nHL auditory brainstem response (ABR) thresholds (ABRthrs). The impaired ear median ABRthr was 55 dB nHL, where 40% had no recordable ABRthr. None of the subjects tested positive for congenital cytomegalovirus (CMV) infection. Fourteen subjects agreed to participate in magnetic resonance imaging (MRI). Malformations were common for all degrees of uSNHL and found in 64% of all scans. Half of the MRIs demonstrated cochlear nerve aplasia or severe hypoplasia and 29% showed inner ear malformations. Impaired ear and normal-hearing ear ABR input/output functions on a group level for subjects with ABRthrs < 90 dB nHL were parallel shifted. A significant difference in interaural acoustic reflex thresholds (ARTs) existed. In congenital uSNHL, MRI is powerful in finding a possible hearing loss cause, while congenital CMV infection may be relatively uncommon. ABRs and ARTs indicated an absence of loudness recruitment, with implications for further research on hearing devices.

The Development of Sound Localization Latency in Infants and Young Children with Normal Hearing.

With the advances in eye tracking, saccadic reflexes towards auditory stimuli have become an easily accessible behavioral response. The present study investigated the development of horizontal sound localization latency quantified by saccadic reflexes in infants and young children with normal hearing (0.55 to 5.6 years, n = 22). The subject was seated in front of an array of 12 loudspeaker/display-pairs arranged equidistantly in an arc from -55 to + 55° azimuth. An ongoing auditory-visual stimulus was presented at 63 dB SPL and shifted to another randomly selected pair at 24 occasions. At each shift, the visual part of the stimulus was blanked for 1.6 s providing auditory-only localization cues. A sigmoid model was fitted to the gaze samples following the azimuthal sound shifts. The overall sound localization latency (SLL) for a subject was defined as the mean of the latencies for all trials included by objective criteria. The SLL was assessed in 21 of 22 children with a mean of 6.1 valid trials. The SLL ranged 400 to 1400 ms (mean = 860 ms). An inverse model demonstrated a significant relationship between SLL and age (R2 = 0.79, p < 0.001), reflecting a distinct reduction of latency with increasing age. No partial correlation between SLL and sound localization accuracy was found when controlling for age (p = 0.5), suggesting that localization latency may provide diagnostic value beyond accuracy.

Twin study of neonatal transient-evoked otoacoustic emissions.

Our knowledge of which physiological mechanisms shape transient evoked otoacoustic emissions (TEOAEs) is incomplete, although thousands of TEOAEs are recorded each day as part of universal newborn hearing-screening (UNHS). TEOAE heritability may explain some of the large TEOAE variability observed in neonates, and give insights into the TEOAE generators and modulators, and why TEOAEs are generally larger in females and right ears. The aim was to estimate TEOAE heritability and describe ear and sex effects in a consecutive subset of all twins that passed UNHS at the same occasion at two hospitals during a six-year period (more than 30 000 neonates screened in total). TEOAEs were studied and TEOAE level correlations compared in twin sets of same-sex (SS, 302 individual twins, 151 twin pairs) and opposite-sex (OS, 152 individual twins, 76 twin pairs). A mathematical model was used to estimate and compare monozygotic (MZ) and dizygotic (DZ) intra-twin pair TEOAE level correlations, based on the data from the SS and OS twin sets. For both SS and OS twin pairs TEOAE levels were significantly higher in right ears and females, compared to left ears and males, as previously demonstrated in young adult twins and large groups of neonates. Neonatal females in OS twin pairs did not demonstrate masculinized TEOAEs, as has been demonstrated for OAEs in young adult females in OS twin pairs. The within-twin pair TEOAE level correlations were higher for SS twin pairs than for OS twin pairs, whereas the within-pair correlation coefficients could not be distinguished from zero when twins were randomly paired. These results reflect heredity as a key factor in TEOAE level variability. Additionally, the estimated MZ within-twin pair TEOAE level correlations were higher than those for DZ twin pairs. The heritability estimates reached up to 100% TEOAE heritability, which is numerically larger than previous estimates of about 75% in young adult twins.

Sound localization latency in normal hearing and simulated unilateral hearing loss.

Directing gaze towards auditory events is a natural behavior. In addition to the well-known accuracy of auditory elicited gaze responses for normal binaural listening, their latency is a measure of possible clinical interest and methodological importance. The aim was to develop a clinically feasible method to assess sound localization latency (SLL), and to study SLL as a function of simulated unilateral hearing loss (SUHL) and the relationship with accuracy. Eight healthy and normal-hearing adults (18-40 years) participated in this study. Horizontal gaze responses, recorded by non-invasive corneal reflection eye-tracking, were obtained during azimuthal shifts (24 trials) of a 3-min continuous auditory stimulus. In each trial, a sigmoid function was fitted to gaze samples. Latency was estimated by the abscissa corresponding to 50% of the arctangent amplitude. SLL was defined as the mean latency across trials. SLL was measured in normal-hearing and simulated SUHL conditions (SUHL30 and SUHL43: mean threshold of 30 dB HL and 43 dB HL across 0.5, 1, 2, and 4 kHz). In the normal-hearing condition, the mean ± SD SLL was 280 ± 40 ms (n = 8) with a test-retest SD = 20 ms. A linear mixed model showed a statistically significant effect of listening condition on SLL. The SUHL30 and SUHL43 conditions revealed a mean SLL of 370 ± 49 ms and 540 ± 120 ms, respectively. Repeated measures correlation analysis showed a clear relationship between SLL and the average sound localization accuracy (R2 = 0.94). The rapid and reliable method to obtain SLL may be an important clinical tool for evaluation of binaural processing. Future studies in clinical cohorts are needed to assess whether SLL may reveal information about binaural processing abilities beyond that afforded by sound localization accuracy.

Children With Congenital Unilateral Sensorineural Hearing Loss: Effects of Late Hearing Aid Amplification-A Pilot Study.

OBJECTIVES: Although children with unilateral hearing loss (uHL) have high risk of experiencing academic difficulties, speech-language delays, poor sound localization, and speech recognition in noise, studies on hearing aid (HA) outcomes are few. Consequently, it is unknown when and how amplification is optimally provided. The aim was to study whether children with mild-to-moderate congenital unilateral sensorineural hearing loss (uSNHL) benefit from HAs. DESIGN: All 6- to 11-year-old children with nonsyndromic congenital uSNHL and at least 6 months of HA use were invited (born in Stockholm county council, n = 7). Participants were 6 children (9.7- to 10.8-years-old) with late HA fittings (>4.8 years of age). Unaided and aided hearing was studied with a comprehensive test battery in a within-subject design. Questionnaires were used to study overall hearing performance and disability. Sound localization accuracy (SLA) and speech recognition thresholds (SRTs) in competing speech were measured in sound field to study hearing under demanding listening conditions. SLA was measured by recording eye-gaze in response to auditory-visual stimuli presented from 12 loudspeaker-video display pairs arranged equidistantly within ±55° in the frontal horizontal plane. The SRTs were measured for target sentences at 0° in spatially separated (±30° and ±150°) continuous speech. Auditory brainstem responses (ABRs) were obtained in both ears separately to study auditory nerve function at the brainstem level. RESULTS: The mean ± SD pure-tone average (0.5, 1, 2, and 4 kHz) was 45 ± 8 dB HL and 6 ± 4 dB HL in the impaired and normal hearing ear, respectively (n = 6). Horizontal SLA was significantly poorer in the aided compared with unaided condition. A significant relationship was found between aided SLA (quantified by an error index) and the impaired ear's ABR I to V interval, suggesting a relationship between the two. Results from questionnaires revealed aided benefit in one-to-one communication, whereas no significant benefit was found for communication in background noise or reverberation. No aided benefit was found for the SRTs in competing speech. CONCLUSIONS: Children with congenital uSNHL benefit from late HA intervention in one-to-one communication but not in demanding listening situations, and there is a risk of degraded SLA. The results indicate that neural transmission time from the impaired cochlea to the upper brainstem may have an important role in unilaterally aided spatial hearing, warranting further study in children with uHL receiving early HA intervention.

The effect of simulated unilateral hearing loss on horizontal sound localization accuracy and recognition of speech in spatially separate competing speech.

Unilateral hearing loss (UHL) occurs in 25% of cases of congenital sensorineural hearing loss. Due to the unilaterally reduced audibility associated with UHL, everyday demanding listening situations may be disrupted despite normal hearing in one ear. The aim of this study was to quantify acute changes in recognition of speech in spatially separate competing speech and sound localization accuracy, and relate those changes to two levels of temporary induced UHL (UHL30 and UHL43; suffixes denote the average hearing threshold across 0.5, 1, 2, and 4 kHz) for 8 normal-hearing adults. A within-subject repeated-measures design was used (normal binaural conditions, UHL30 and UHL43). The main outcome measures were the threshold for 40% correct speech recognition and the overall variance in sound localization accuracy quantified by an Error Index (0 = perfect performance, 1.0 = random performance). Distinct and statistically significant deterioration in speech recognition (2.0 dB increase in threshold, p < 0.01) and sound localization (Error Index increase of 0.16, p < 0.001) occurred in the UHL30 condition. Speech recognition did not significantly deteriorate further in the UHL43 condition (1.0 dB increase in speech recognition threshold, p > 0.05), while sound localization was additionally impaired (Error Index increase of 0.33, p < 0.01) with an associated large increase in individual variability. Qualitative analyses on a subject-by-subject basis showed that high-frequency audibility was important for speech recognition, while low-frequency audibility was important for horizontal sound localization accuracy. While the data might not be entirely applicable to individuals with long-standing UHL, the results suggest a need for intervention for mild-to-moderate UHL.

Concentration-Response Relationship of Hearing Impairment Caused by Quinine and Salicylate: Pharmacological Similarities but Different Molecular Mechanisms.

This review has the purpose to summarize concentration-effect studies made with quinine and to compare the effects on hearing between quinine and salicylate. Quinine and salicylate have roles in experimental hearing research and may induce pronounced and reversible hearing impairment when administered in sizeable doses. The quinine-induced increase in hearing threshold and its recovery can be analysed according to 'the psychophysical power function'. The power function is a special case of the Hill equation when the stimulus (e.g. a drug concentration) is exceedingly small compared with the concentration that would elicit a half-maximum response. Quinine and salicylate induce sensorineural hearing impairment and tinnitus when given in higher dose ranges in man. The drugs influence the presence, magnitude, and quality of audiological responses, such as spontaneous and evoked otoacoustic emissions. Quinine reversibly reduces frequency selectivity and hearing sensitivity, whereas the self-attained most comfortable speech level and the acoustic stapedius reflex are not affected, that is the dynamic range of hearing is reversibly reduced. This observation supports the view that quinine acts on the outer hair cell of the cochlea. Both drugs share a protective effect against the permanent hearing damages caused by gentamicin. This action is interpreted as a request for functioning mechanoelectric transducer (MET) channels to elicit the ill effect of aminoglycosides. Both drugs may interfere with the cochlear amplifier through blocking MET channels and the motor protein prestin. This review finds considerable overlap between type and extent of pharmacological actions of quinine and salicylate, supposedly caused by partly shared mechanisms of action but performed with different molecular mechanisms.

Corneal-Reflection Eye-Tracking Technique for the Assessment of Horizontal Sound Localization Accuracy from 6 Months of Age.

OBJECTIVES: The evaluation of sound localization accuracy (SLA) requires precise behavioral responses from the listener. Such responses are not always possible to elicit in infants and young children, and procedures for the assessment of SLA are time consuming. The aim of this study was to develop a fast, valid, and objective method for the assessment of SLA from 6 months of age. To this end, pupil positions toward spatially distributed continuous auditory and visual stimuli were recorded. DESIGN: Twelve children (29 to 157 weeks of age) who passed the universal newborn hearing screening and eight adults (18 to 40 years of age) who had pure-tone thresholds ≤20 dB HL in both ears participated in this study. Horizontal SLA was measured in a sound field with 12 loudspeaker/display (LD)-pairs placed in an audiological test room at 10 degrees intervals in the frontal horizontal plane (±55 degrees azimuth). An ongoing auditory-visual stimulus was presented at 63 dB SPL(A) and shifted to randomized loudspeakers simultaneously with pauses of the visual stimulus. The visual stimulus was automatically reintroduced at the azimuth of the sounding loudspeaker after a sound-only period of 1.6 sec. A corneal-reflection eye-tracking technique allowed the acquisition of the subjects' pupil positions relative to the LD-pairs. The perceived azimuth was defined as the median of the intersections between gaze and LD-pairs during the final 500 msec of the sound-only period. Overall SLA was quantified by an Error Index (EI), where EI = 0 corresponded to perfect match between perceived and presented azimuths, whereas EI = 1 corresponded to chance. RESULTS: SLA was rapidly measured in children (mean = 168 sec, n = 12) and adults (mean = 162 sec, n = 8). Visual inspection of gaze data indicated that gaze shifts occurred in sound-only periods. The medians of the perceived sound-source azimuths either coincided with the presenting sound-source azimuth or were offset by a maximum of 20 degrees in children. In contrast, adults revealed a perfect match from -55 to 55 degrees, except at 15 degrees azimuth (median = 20 degrees), with 9/12 of the quartile ranges = 0 degrees. Children showed a mean (SD) EI of 0.42 (0.17), which was significantly higher than that in adults (p < 0.0001). However, children revealed a distinct age-related EI improvement of 16 percentage points per year (r = -0.68, p = 0.015, n = 12), suggesting an ongoing maturation of SLA in the studied age range (29 to 157 weeks). The eight adults showed high SLA and high reliability as demonstrated by the low mean (SD) EI (0.054 [0.021]) and the low variability in test-retest differences (95% confidence interval = -0.020 to 0.046). CONCLUSIONS: Corneal-reflection eye-tracking provides an objective and fast assessment of horizontal SLA from about 6 months of age and may enable gaze to be used as an objective measure for sound localization in this age group. Infant SLA is immature and improvements are related to increasing age. Adults show high overall SLA and low intra- and intersubject variability in SLA. The technique may be used as a clinical tool for the evaluation of very early intervention in a young, preverbal population and throughout the life span.

Reliability in Hearing Threshold Prediction in Normal-Hearing and Hearing-Impaired Participants Using Mixed Multiple ASSR.

BACKGROUND AND PURPOSE: The rapidly evolving field of hearing aid fitting in infants requires rapid, objective, and highly reliable methods for diagnosing hearing impairment. The aim was to determine test-retest reliability in hearing thresholds predicted by multiple auditory steady-state response (ASSRthr) among normal-hearing (NH) and hearing-impaired (HI) adults, and to study differences between ASSRthr and pure-tone threshold (PTT) as a function of frequency in each participant. ASSR amplitude versus stimulus level was analyzed to study ASSR growth rate in NH and HI participants, especially at ASSRthr. RESEARCH DESIGN AND STUDY SAMPLE: Mixed multiple ASSR (100% AM, 20% FM), using long-time averaging at a wide range of stimulus levels, and PTT were recorded in 10 NH and 14 HI adults. ASSRthr was obtained in 10 dB steps simultaneously in both ears using a test-retest protocol (center frequencies = 500, 1000, 2000, and 4000 Hz; modulation frequencies = 80-96 Hz). The growth rate at ASSRthr was calculated as the slope (nV/dB) of the ASSR amplitudes obtained at, and 10 dB above, ASSRthr. PTT was obtained in both ears in 1 dB steps using a fixed-frequency Békésy technique. All of the NH participants showed PTTs better than 20 dB HL (125-8000 Hz), and mean pure-tone average (PTA; 500-4000 Hz) was 1.8 dB HL. The HI participants exhibited quite symmetrical sensorineural hearing losses, as revealed by a mean interaural PTA difference of 6.5 dB. Their mean PTA in the better ear was 38.7 dB HL. RESULTS: High ASSRthr reproducibility (independent of PTT) was found in both NH and HI participants (test-retest interquartile range = 10 dB). The prediction error was numerically higher in NH participants (f ≥1000 Hz), although only a significant difference existed at 1000 Hz. The median difference between ASSRthr (dB HL) and PTT (dB HL) was approximately 10 dB in the HI group at frequencies of 1000 Hz or greater, and 20 dB at 500 Hz. In general, the prediction error decreased (p < 0.001) with increasing hearing threshold, although large intersubject variability existed. Regression analysis (PTT versus ASSRthr) in HI participants revealed correlation coefficients between 0.72-0.88 (500-4000 Hz) and slopes at approximately 1.0. Large variability in ASSRthr-PTT versus frequency was demonstrated across HI participants (interquartile range approximately 20 dB). The maximum across-frequency difference (ASSRthr-PTT) in an individual participant was 50 dB. HI participants showed overall significantly higher amplitudes and slopes at ASSRthr than did NH participants (p < 0.02). The amplitude-intensity function revealed monotonically increasing ASSRs in NH participants (slope 2 nV/dB), whereas HI participants exhibited heterogeneous and mostly nonmonotonically increasing ASSRs. CONCLUSIONS: Long-time averaging of ASSR revealed high ASSRthr reproducibility and systematic decrease in prediction error with increasing hearing threshold, albeit large intersubject variability in prediction error existed. A plausible explanation for the systematic difference in ASSRthr between NH and HI adults might be significantly higher ASSR amplitudes and higher overall growth rates at ASSRthr among HI participants. Across-frequency comparison of PTT and ASSRthr in an individual HI participant demonstrated large variation; thus, ASSR may not be optimal for, e.g., reliable threshold prediction in infants and subsequent fine-tuning of hearing aids.

Analysis of click-evoked auditory brainstem responses using time domain cross-correlations between interleaved responses.

OBJECTIVES: The rapidly evolving field of early diagnostics after the introduction of newborn hearing screening requires rapid, valid, and objective methods, which have to be thoroughly evaluated in adults before use in infants. The aim was to study cross-correlation analysis of interleaved auditory brainstem responses (ABRs) in a wide dynamic range in normal-hearing adults. Off-line analysis allowed for comparison with psychoacoustical click threshold (PCT), pure-tone threshold, and determination of ABR input/output function. Specifically, nonfiltered and band-pass filtered ABRs were studied in various time segments along with time elapsed for ensemble of sweeps reaching a specific detection criterion. DESIGN: Fourteen healthy normal-hearing subjects (18 to 35 years of age, 50% females) without any history of noise exposure participated. They all had pure-tone thresholds better than 20 dB HL (125 to 8000 Hz). ABRs were recorded in both ears using 100 µsec clicks, from 71.5 dB nHL down to -18.5 dB nHL, in 10 dB steps (repetition rate, 39 Hz; time window, 15 msec; filter, 30 to 8000 Hz). The number of sweeps increased from 2000 at 71.5 dB nHL, up to 30000 at -18.5 dB nHL. Each sweep was stored in a data base for off-line analysis. Cross-correlation analysis between two subaverages of interleaved responses was performed in the time domain for nonfiltered and digitally band-pass filtered (300 to 1500 Hz) entire and time-windowed (1 to 11 and 5 to 11 msec) responses. PCTs were measured using a Bekesy technique with the same insert phone and stimulus as used for the ABR (repetition rate, 20 Hz). Time elapsed (≈ number of accepted sweeps/repetition rate) for the ensemble of sweeps needed to reach a cross-correlation coefficient (ρ) of 0.70 (=3.7 dB signal-to-noise ratio [SNR]) was analyzed. RESULTS: Mean cross-correlation coefficients exceeded 0.90 in both ears at stimulus levels ≥11.5 dB nHL for the entire nonfiltered ABR. At 1.5 dB nHL, mean(SD) ρ was 0.53(0.32) and 0.44(0.40) for left and right ears, respectively (n = 14) (=0 dB SNR). In comparison, mean(SD) PCT was -1.9(2.9) and -2.5(3.2) dB nHL for left and right ears, respectively (n = 14), while mean pure-tone average (500 to 2000 Hz) was 2.5 dB HL (n = 28). Almost no effect of band-pass filtering or reduced analysis time window existed. Average time elapsed needed to reach ρ = 0.70 was approximately 20 seconds or less at stimulus levels ≥41.5 dB nHL, and ≈30 seconds at 31.5 dB nHL. The average (interpolated) stimulus level corresponding to ρ=0.70 for the entire nonfiltered ABR was 6.5 dB nHL (n = 28), which coincided with the estimated psychoacoustical threshold for single clicks. CONCLUSIONS: ABR could be identified in a short period of time using cross-correlation analysis between interleaved responses. The average stimulus level corresponding to 0 dB SNR in the entire nonfiltered ABR occurred at 1.5 dB nHL, 4 dB above the average PCT. The mean input/output function for the ensemble of sweeps required to reach ρ = 0.70 increased monotonically with increasing stimulus level, in parallel with the ABR based on all sweeps (≥1.5 dB nHL). Time domain cross-correlation analysis of ABR might form the basis for automatic response identification and future threshold-seeking procedures.

Horizontal sound localization in children with bilateral cochlear implants: effects of auditory experience and age at implantation.

OBJECTIVE: To study sound localization performance in relation to auditory experience and age at implantation in children with bilateral cochlear implants. STUDY DESIGN: Clinical study. SETTING: University hospital. PATIENTS: Sixty-two sequentially and 4 simultaneously bilaterally implanted children participated in this clinical sound localization study, at a median age of 5.6 years. They underwent sequential implantations at median ages of 1.9 and 4.2 years, respectively. Simultaneous implantations were performed at a median age of 2.0 years. METHODS: Localization performance was measured with pink noise presented in random order from 5 loudspeakers in the frontal horizontal plane. Twenty-one subjects participated in repeated testing. MAIN OUTCOME MEASURE: Sound localization performance as quantified with an Error Index. RESULTS: On group level, bilaterally implanted children pinpointed the sounding loudspeaker in the frontal horizontal plane. Sound localization performance improved significantly with increasing bilateral cochlear implant experience, as observed in the entire study group and intraindividually, albeit large interindividual variability existed. Regression analyses in the entire study group and in the sequentially implanted children did not reveal any relationship between sound localization performance and ages at first and second implantation, interimplant interval, or age. However, second implantation before 4 years of age contributed to faster sound localization improvement with increasing bilateral cochlear implant experience. CONCLUSION: Median perceived azimuths coincided with presented azimuths. We suggest that listening experience after bilateral cochlear implantation affects sound localization performance, possibly owing to the ongoing stimulus-driven maturation of the central auditory system. The amount of time listening with bilateral implants has methodologic implications on sound localization assessment in bilaterally implanted children.

Outcome of a universal newborn hearing-screening programme based on multiple transient-evoked otoacoustic emissions and clinical brainstem response audiometry.

CONCLUSION: This universal newborn hearing-screening (UNHS) programme revealed high efficacy. The proportion of congenital sensorineural hearing loss was higher in left ears and in males than in right ears and females, which was in line with the systematic ear asymmetries and sex differences in transient-evoked otoacoustic emission (TEOAE) pass percentage. OBJECTIVES: To study the long-term outcome of a UNHS programme based on multiple TEOAEs and clinical click-evoked auditory brainstem response (ABR). METHOD: The study included all the newborns that were screened during a 6-year period (n = 31 092). TEOAE pass/fail was analysed in detail. In an assessment performed 10 years after the start of the 6-year UNHS, prevalence, degree and type of congenital hearing loss were studied. RESULTS: The proportion of screened newborns was high, i.e. 98%. Multiple TEOAE recordings minimized the need for clinical ABR. Fifty-seven (0.18%) subjects showed bilateral hearing loss (exceeding ≈ 30 dB HL); median ABR threshold = 60 dB nHL (at 2.5 months of age). Bilateral and unilateral sensorineural hearing loss was found in 0.17% (n = 52; 56% males) and 0.06% (n = 18; 61% left ears, 56% males) of the screened newborns, respectively. Higher TEOAE pass percentages (p < 0.01) were demonstrated in right ears and in females than in left ears and males.

Characteristics of normal newborn transient-evoked otoacoustic emissions: ear asymmetries and sex effects.

Transient-evoked otoacoustic emissions (TEOAEs) were recorded from more than 30000 newborns over a six year period. Analysis was performed on all the TEOAEs that passed the bedside universal hearing screen (n=60431), in order to characterize the normal properties of neonatal TEOAEs and to study ear and sex effects. Short recording times (median=33 s) were observed in combination with high entire TEOAE level (median=18.8 dB SPL for an 81.8 dB SPL peak stimulus), and high reproducibility (median=86%). Signal-to-noise ratio (S/N) of the TEOAE was highly frequency-dependent, being poorer at low frequencies. Prolonged averaging increased median reproducibility to 97%, but the minor S/N-improvement at low frequencies did not justify the longer test time. Highly significant mean lateral asymmetries (right >left) and sex differences (female >male) existed in entire TEOAE level, S/N TEOAE, and in half-octave frequency bands (700-4000 Hz). Mean lateral and sex entire TEOAE level differences were 1.1 dB and 1.3 dB, respectively. Stimulus levels were not affected by ear or sex. Hence, physiological differences at the level of organ of Corti were demonstrated in newborns.

The effect of intravenously administered mexiletine on tinnitus - a pilot study.

The effect of intravenously administered mexiletine on subjective tinnitus and hearing was studied in six patients, who initially responded positively to lidocaine. Distinct mexiletine-induced decreases in tinnitus loudness were demonstrated in three subjects, as reflected by maximum VAS (visual analogue scale) level reduction of 34%, 95%, and 100%, respectively. One subject reported change in tinnitus pitch, another one showed a slight (18% on VAS) tinnitus reduction, and one subject disclosed no effect. Side effects were seen only during one of seven infusions. Mexiletine induced shifts in pure-tone threshold, transient evoked otoacoustic emission, and acoustic reflex threshold, probably reflecting a reversible interference in the function of organ of Corti. The concentration effect relationship remained unclear and no general 'therapeutic' level could be identified. This study confirms the effect of mexiletine on the auditory function and its potential as a possible therapeutic agent or a model for further development in tinnitus pharmacotherapy.