OBJECTIVES:

: Assessment of auditory and vestibular function in Wolfram Syndrome (WS) patients, using a standardized protocol.

DESIGN:

: Prospective cohort study of 11 patients using otoscopic inspection, tympanometry, otoacoustic emissions, pure tones, speech in noise (SIN), the Speech Intelligibility Index, and rotational chair testing.

RESULTS::

Mean SNHL diagnosis was 7.3 years with 55% prevalence. Four patients with a Speech Intelligibility Index less than 0.75 (better ear) routinely used bilateral amplification devices. Two patients with normal-hearing sensitivity exhibited abnormal SIN scores. The only patient with significant vestibular dysfunction also had a distinctive low-frequency component to her SNHL.

CONCLUSIONS::

Hearing loss may occur earlier than previously suspected, and comprehensive testing including SIN testing may reveal deficits not apparent with pure-tone testing. Particular configurations of hearing loss may indicate a need for comprehensive vestibular assessment. Because SNHL can be the first symptom of WS, audiologists and otolaryngologists should be vigilant about referring patients with hearing loss for ophthalmologic examination.

OBJECTIVES:

: The aim of the study was to investigate whether children with bilateral conductive hearing loss benefit from their second device (i.e., the bilateral bone conduction device [BCD]).

DESIGN:

: Speech recognition in noise was assessed in 10 children fitted with bilateral BCDs during childhood. Speech recognition was measured in 2 conditions with both BCDs active. Spatial resolution was tested with the Minimum Audible Angle test in the bilateral and monaural listening conditions.

RESULTS::

Children demonstrated an improvement in speech recognition when speech was presented from the front and noise was presented from the right-hand side as compared with both speech and noise being presented from the front. The minimum audible angle decreased from 57° in the best monaural condition to 13° in the bilateral condition.

CONCLUSIONS::

The audiological outcomes demonstrate the advantage of bilateral BCD fitting in children with bilateral conductive hearing loss.

OBJECTIVES:

: The prevalence of personal music (PM) system use with earphones is high among young adults. Although previous research has demonstrated that the majority of these adults may not be listening at levels or for durations considered dangerous, the long-term consequences of PM system use are not fully understood. In this report the relationship between self-reported PM use (i.e., non, light, moderate, or heavy) and peripheral auditory function as assayed through distortion product otoacoustic emission (DPOAE) components and fine structure was explored. The purpose was to evaluate the relationship between PM system use and peripheral auditory function.

DESIGN:

: One hundred and one (N = 101) young adults, 18 to 30 years of age, with normal hearing. Survey data were first obtained to determine PM system user status along with type of earphones used, most common listening volume, and most common listening duration. Once normal-hearing sensitivity was confirmed with a hearing screening, the DPOAE research protocol was administered. DPOAEs (2f1 to f2) were measured between 1 and 6 kHz with stimulus levels fixed at L1, L2 = 55,40 dB SPL and f2/f1 = 1.22. DPOAE level as well as fine-structure depth and spacing in 1/3rd octave (oct) bands around 1, 1.5, 2, 3, 4, and 6 kHz were compared across various PM system user groups. The ear-canal DPOAE was separated into the so-called generator and characteristic frequency (CF) components. The characteristics of the components were also compared across user groups.

RESULTS::

Almost all participants reported listening to a PM system between < 1 hr/d to 3 hr/d, and 67% of the participants reported medium/comfortable as the most common volume used during a typical day. There was no significant effect of user status on 3rd oct DPOAE data, 1/3rd oct band component data, or fine-structure spacing data. Women exhibited significantly higher DPOAE as well as generator and CF component levels in the lower half of the frequency range evaluated. There were no significant gender differences for fine-structure spacing or depth. The slope of the CF component was significantly steeper in women.

CONCLUSIONS::

In this cross-sectional study, there were no significant effects of self-reported PM use on either the ear-canal DPOAE or its constituent parts. Fine-structure depth varied across user groups in a nonsystematic manner. Women exhibited higher DPOAE and component levels in the lower portion of the frequency range evaluated. It is interesting to note that a greater proportion of men reported listening to their PM system at loud or very loud levels. The size of the effect relative to the sample size of each user group may be masking the deleterious effects of PM system use on the peripheral auditory system.

OBJECTIVES:

: The purpose of the present investigation was to define for young, middle-aged, and older adults the optimal frequency (cies) to record both the cervical vestibular-evoked myogenic potential (cVEMP) and the ocular vestibular-evoked myogenic potential (oVEMP). Further, this study aimed to describe age-related changes in the tuning of these two vestibular-evoked myogenic potentials.

DESIGN:

: This was a prospective study. Participants were 39 healthy adults (mean age 46.3 ± 15.7 years; range = 22 to 78 years; 15 men) equally divided into 3 age groups of 13 participants each: young adult (18 to 39 years), middle age (40 to 59 years), and old adult (≥60 years). cVEMPs and oVEMPs were recorded using air-conduction tone bursts at stimulus frequencies of 125, 250, 500, 750, 1000, 1500, and 2000 Hz presented at 127 dB pSPL.

RESULTS::

There was a significant main effect of age group and frequency on the amplitude of both the cVEMP and the oVEMP. Amplitudes were largest for the Young adult group for the cVEMP and for the young adult and Middle age group for the oVEMP. The largest average peak-to-peak amplitude occurred in response to a 750 Hz tone burst for both responses. No significant differences in mean amplitude of the cVEMP or oVEMP were observed for 500, 750, or 1000 Hz stimuli. There was a significant interaction of age group and frequency for the cVEMP, suggesting a loss of tuning for the old adult group. Compared with the young adult group, the tuning of the cVEMP and oVEMP for the older adjults appeared to shift to a higher frequency.

CONCLUSION:

: There is no sharp tuning in the saccule and utricle. Instead, there is a range of best frequencies that may be used to evoke the cVEMP and oVEMP responses. The results of the present investigation also demonstrate that the optimal stimulus frequency to elicit a VEMP may change with age. Accordingly, 500 Hz may not be the ideal frequency to elicit VEMPs for all age groups. For this reason, in cases where the VEMP response is absent at 500 Hz it is recommended that attempts be made to record the VEMP for tone-burst frequencies of 750 or 1000 Hz.

OBJECTIVES:

: This study documented the ability of experienced pediatric cochlear implant (CI) users to perceive linguistic properties (what is said) and indexical attributes (emotional intent and talker identity) of speech, and examined the extent to which linguistic (LSP) and indexical (ISP) perception skills are related. Preimplant-aided hearing, age at implantation, speech processor technology, CI-aided thresholds, sequential bilateral cochlear implantation, and academic integration with hearing age-mates were examined for their possible relationships to both LSP and ISP skills.

DESIGN:

: Sixty 9- to 12-year olds, first implanted at an early age (12 to 38 months), participated in a comprehensive test battery that included the following LSP skills: (1) recognition of monosyllabic words at loud and soft levels, (2) repetition of phonemes and suprasegmental features from nonwords, and (3) recognition of key words from sentences presented within a noise background, and the following ISP skills: (1) discrimination of across-gender and within-gender (female) talkers and (2) identification and discrimination of emotional content from spoken sentences. A group of 30 age-matched children without hearing loss completed the nonword repetition, and talker- and emotion-perception tasks for comparison.

RESULTS::

Word-recognition scores decreased with signal level from a mean of 77% correct at 70 dB SPL to 52% at 50 dB SPL. On average, CI users recognized 50% of key words presented in sentences that were 9.8 dB above background noise. Phonetic properties were repeated from nonword stimuli at about the same level of accuracy as suprasegmental attributes (70 and 75%, respectively). The majority of CI users identified emotional content and differentiated talkers significantly above chance levels. Scores on LSP and ISP measures were combined into separate principal component scores and these components were highly correlated (r = 0.76). Both LSP and ISP component scores were higher for children who received a CI at the youngest ages, upgraded to more recent CI technology and had lower CI-aided thresholds. Higher scores, for both LSP and ISP components, were also associated with higher language levels and mainstreaming at younger ages. Higher ISP scores were associated with better social skills.

CONCLUSIONS::

Results strongly support a link between indexical and linguistic properties in perceptual analysis of speech. These two channels of information appear to be processed together in parallel by the auditory system and are inseparable in perception. Better speech performance, for both linguistic and indexical perception, is associated with younger age at implantation and use of more recent speech processor technology. Children with better speech perception demonstrated better spoken language, earlier academic mainstreaming, and placement in more typically sized classrooms (i.e., >20 students). Well-developed social skills were more highly associated with the ability to discriminate the nuances of talker identity and emotion than with the ability to recognize words and sentences through listening. The extent to which early cochlear implantation enabled these early-implanted children to make use of both linguistic and indexical properties of speech influenced not only their development of spoken language, but also their ability to function successfully in a hearing world.

OBJECTIVES:

: The purpose of this study was to determine how the bandwidth of the hearing aid (HA) fitting affects bimodal speech recognition of listeners with a cochlear implant (CI) in one ear and severe-to-profound hearing loss in the unimplanted ear (but with residual hearing sufficient for wideband amplification using National Acoustic Laboratories Revised, Profound [NAL-RP] prescriptive guidelines; unaided thresholds no poorer than 95 dB HL through 2000 Hz).

DESIGN:

: Recognition of sentence material in quiet and in noise was measured with the CI alone and with CI plus HA as the amplification provided by the HA in the high and mid-frequency regions was systematically reduced from the wideband condition (NAL-RP prescription). Modified bandwidths included upper frequency cutoffs of 2000, 1000, or 500 Hz.

RESULTS::

On average, significant bimodal benefit was obtained when the HA provided amplification at all frequencies with aidable residual hearing. Limiting the HA bandwidth to only low-frequency amplification (below 1000 Hz) did not yield significant improvements in performance over listening with the CI alone.

CONCLUSIONS::

These data suggest the importance of providing amplification across as wide a frequency region as permitted by audiometric thresholds in the HA used by bimodal users.

OBJECTIVES:

: It is often difficult to use an adaptive approach to evaluate speech reception in noise in cochlear implant (CI) users, because of variations in performance. Thus, two studies were conducted to develop an alternative method for scoring the Cantonese Hearing In Noise Test (CHINT) and to use this method for evaluating speech reception in CI users.

DESIGN:

: In Study 1, 20 normal-hearing adults were tested using four scoring methods, including three modified and the standard HINT scoring method. The speech was presented in the front, and noise originated from the front, the right, or the left loudspeakers, as a standard HINT protocol. Threshold signal-to-noise ratio (S/N) and word intelligibility percent score (word score) were measured in each CHINT noise condition using each rule. In Study 2, depending on the word score of the individual CI user in the quiet CHINT condition, different adaptive rules were selected to score the responses from 12 CI users in the three noise conditions. The percentage of CI users who could successfully be tested using these scoring methods was evaluated.

RESULTS::

In Study 1, threshold S/Ns obtained using different rules were significantly different and consistent with expectations. The slopes of the performance-intensity function relating mean word scores and threshold S/Ns were linear and agreed well with previous findings. These results showed that the modified rules could be used to adaptively measure CHINT thresholds in noise. In Study 2, these modified rules were successful in measuring CHINT thresholds in 10 of the 12 participants, whose word scores in quiet exceeded 40%.

CONCLUSIONS::

These modified rules could be used in CI users whose speech reception ability could not have been measured otherwise.

OBJECTIVE::

A growing number of studies on deaf children with cochlear implant (CI) document a significant improvement in receptive and expressive language skills after implantation, even if they show language delay when compared with normal-hearing peers. Data on language acquisition in CI Italian children are still scarce and limited to only certain aspects of language. The purpose of this study is to prospectively describe the trajectories of language development in early CI Italian children, with particular attention to the transition from first words to combinatorial speech and to acquisition of complex grammar in a language with rich morphology, such as Italian.

DESIGN:

: Six children, with profound prelingual deafness, provided with CI, between 16 and 24 months of age were prospectively assessed and followed over a mean period of up to 34.8 months postimplant. During follow-up, each child received between four to five individual language evaluations through a combination of indirect procedures (parent reports of early lexical and grammar development) and direct ones (administration of standardized receptive and expressive language tests with Italian norms and collection of spontaneous language samples).

RESULTS::

In relation to chronological age, the acquisition of expressive vocabulary was delayed. However, considering the duration of hearing experience, most CI participants showed an earlier start and faster growth of expressive rather than receptive vocabulary in comparison with typically developing children. This quite atypical result persisted right up until the end of the follow-up. The acquisition of expressive grammar was delayed relative to chronological age, though all but one CI participant achieved the expected grammar level after approximately 3 years of CI use. In addition, the rate of grammar acquisition was not homogeneous during development, showing two different paces: one comparable with normal hearing in the transition from holophrastic to primitive combinatorial speech and a much slower one to attain more advanced levels of morphosyntactic control.

CONCLUSION:

: From a rehabilitative viewpoint, our results suggest the importance of implementing rehabilitation in lexical comprehension, even when expressive vocabulary appears to be within normal range. Moreover, assessment of language acquisition in CI Italian children should focus on those grammar aspects that are more vulnerable to early acoustic deprivation (such as free and bound morphology) to ensure enhanced language therapy planning.

OBJECTIVES:

: The aim of this research is to describe the development of an adaptive Australian Sentence Test in Noise and to validate the test in terms of test-retest reliability and efficiency using data obtained from its clinical application.

DESIGN:

: The relative intelligibility of 1264 Bamford-Kowal-Bench (BKB)-like sentences in the presence of competing four-talker babble was assessed with cochlear implant recipients. Intensity adjustments to the babble segments were made to reduce intersentence variability. Computer software was developed to administer an adaptive speech reception threshold (SRT) test using these adjusted sentence/babble pairs and test-retest SRT data from a separate group of 23 cochlear implant recipients was analyzed, comparing different SRT calculation and test stopping rules.

RESULTS::

The adjusted sentence/babble pairs were used in clinical studies to obtain an SRT by presenting 32 sentences. Analysis of test-retest pairs of SRT data from 23 recipients indicated that a psychometric fit SRT calculation rule provided better reliability than did the Hearing in Noise Test (HINT) calculation rule, or rules based on mean turns. This rule, using the morpheme correct scores for each sentence, gave a standard deviation for a single SRT of 0.76 dB. Further analyses revealed that the test could be shortened to 20 sentences with an increase of 0.19 dB in variability, while reducing the median test time by approximately 2 min.

CONCLUSIONS::

This article reports validation data for a new Australian Sentence Test In Noise. When 20 BKB-like sentences are used with a psychometric fit calculation rule, a standard deviation of approximately 1 dB is obtained in approximately 3 min 36 sec.

OBJECTIVES:

: Animal data indicate that xylene induces cochlear dysfunction, characterized by the loss of outer hair cells. There is little evidence regarding xylene-induced ototoxicity in humans. The aim of the study was to investigate the possible adverse effects of xylene on the peripheral and central auditory system in humans.

DESIGN:

: A total of 30 medical laboratory workers who had been exposed to a mixture of xylene isomers, together with 30 nonexposed control participants matched for gender, age, and educational level were selected. Participants of both groups were not exposed to noise levels above 85 dBA time-weighted average. All participants were evaluated with a comprehensive audiological test battery, which included measures of peripheral and central auditory function. Peripheral auditory measures included pure-tone audiometry and distortion product otoacoustic emissions. Behavioral measures of central auditory function included a pitch pattern sequence test, an adaptive test of temporal resolution, a dichotic digit test, and a masking level difference test. The auditory brainstem response was used to objectively evaluate the function of the auditory pathways at the brainstem level. Speech perception in quiet and in noise was evaluated using the Hearing In Noise Test (HINT). The xylene-exposed participants were extensively evaluated with regard to their exposure to both noise and xylene. Noise dosimetry was conducted over an 8-hr work shift to obtain noise-exposure levels for each xylene-exposed worker. Airborne xylene concentrations were obtained at 11 different workstations throughout the medical laboratories, and methyl hippuric acid levels per gram of creatinine in urine were obtained for each xylene-exposed subject. Finally, a detailed interview exploring current and past solvent and noise exposure was conducted.

RESULTS::

The xylene-exposed participants showed significantly worse pure-tone thresholds in comparison with the nonexposed participants. The xylene-exposed participants demonstrated significantly worse results than the control group participants for the pitch pattern sequence test, dichotic digit test, HINT, and the auditory brainstem response (absolute and interpeak latencies). No significant differences between the xylene-exposed and nonexposed participants were observed for distortion product otoacoustic emissions, adaptive test of temporal resolution, or the masking level difference test. A significant correlation between the concentrations of methyl hippuric acid in urine and pure-tone thresholds (2 to 8 kHz) was found in xylene-exposed workers. Also, participants with high cumulative dose of xylene exposure presented with poorer test results than participants with low cumulative dose of xylene exposure.

CONCLUSIONS::

The results of the present research suggest that xylene is associated with adverse central auditory effects and poorer sound detection abilities in humans. A major limitation of the study is that the results found among xylene-exposed participants cannot be proved to be permanent, and thus further research should be conducted to clarify this limitation. Workers exposed to this chemical should be routinely evaluated with a comprehensive audiological test battery, to detect early signs of auditory dysfunction.