Tone-burst elicited auditory brainstem responses in full-term and pre-term infants.

OBJECTIVE: To investigate the reliability of tone-burst auditory brainstem response (TB-ABR) latencies and thresholds in pre-term and full-term infants. DESIGN: TB-ABRs to 500 Hz and 4000 Hz tone-burst stimuli were recorded at two-week intervals over the first six weeks of life in a group of full-term infants (40-46 weeks gestational age [GA]) and over ten weeks (36-46 weeks gestation) in a group of preterm infants. Linear mixed model analyses evaluated within-subject changes and the effects of the subject group, age at assessment, and stimulus frequency on ABR latency and threshold. STUDY SAMPLE: Twenty-four infants participated. Nine were full-term (GA: 39-41 weeks) and fifteen were healthy pre-term (GA: 28-34 weeks) at birth. RESULTS: TB-ABR wave V latencies at 70 dBnHL decreased throughout the study (p < 0.001) in pre-term babies for both test frequencies by approximately 0.5 ms. There were, however, no group or GA (at birth) effects indicating that response latency normalized in these children by the due date. Similarly, TB-ABR threshold levels in the premature group were elevated (p = 0.001) by approximately 5 dB in pre-term evaluation but were equivalent to those of full-term participants in the post-term assessment period. CONCLUSIONS: In healthy, pre-term infants, tone-burst ABR testing is reliable from 36 weeks gestation.

The reliability of remote diagnostic hearing assessment in school-entry-aged children.

OBJECTIVE: To explore the feasibility of remote diagnostic hearing testing within schools, for school-entry aged children (4-7 years). DESIGN: A prospective cross-sectional comparative study design was used to establish the reliability of remote hearing assessment by comparing traditional face-to-face pure tone audiometry (PTA) with remote PTA, traditional otoscopy with remote video otoscopy and interpretation of tympanograms in-person with remote analysis. STUDY SAMPLE: 464 school-entry year level children from 18 schools. RESULTS: Air conduction thresholds established by remote testing were within a clinically acceptable range of ±10 dB HL from traditional thresholds in 98% of cases at 1 kHz and 97.8% at 4 kHz. Bone conduction thresholds varied by ≤10 dB HL in 100% of cases at 1 kHz and 95.7% at 4 kHz. Remote otoscopy and tympanometry interpretation had 78.6% agreement (moderate; κ = 0.6) and 92.2% agreement (almost perfect; κ = 0.9), respectively. CONCLUSIONS: We found there to be good reliability between face-to-face PTA and remote PTA performed within the school environment. Furthermore, assessment of the outer and middle ear via video otoscopy and remote tympanometry classification were both viable. Remote diagnostic hearing tests in schools can extend reach of paediatric services in rural areas.

Evaluating benefits of remote microphone technology for adults with hearing loss using behavioural and predictive metrics.

OBJECTIVE: To investigate the benefit of remote-microphone (RM) systems for adults with sensory hearing loss. DESIGN: Speech recognition in quiet and in background noise was assessed. Participants with hearing loss underwent testing in two device conditions: hearing aids (HAs) alone and HAs with a RM. Normal hearing participants completed testing in the unaided condition. Predictive speech intelligibility modelling using the Hearing-Aid Speech Perception Index (HASPI) was also performed on recordings of HA processed test material. STUDY SAMPLE: Twenty adults with sensory hearing loss and 10 adults with normal hearing participated. RESULTS: Speech recognition for participants with hearing loss improved significantly when using the RM compared to HAs alone fit to Phonak's proprietary prescription. Largest benefits were observed in the most challenging conditions. At the lowest signal-to-noise ratio, participants with hearing loss using a RM outperformed normal hearing listeners. Predicted intelligibility scores produced by HASPI were strongly correlated to behavioural results. CONCLUSIONS: Adults using HAs who have significant difficulties understanding speech in noise will experience considerable benefits with the addition of a RM. Improvements in speech recognition were observed for all participants using RM systems, including those with relatively mild hearing loss. HASPI modelling reliably predicted the speech perception difficulties experienced.

The Assistive Benefits of Remote Microphone Technology for Normal Hearing Children With Listening Difficulties.

OBJECTIVES: Children often present with listening difficulties (LiD) but with normal sound detection thresholds. These children are susceptible to learning challenges, and struggle with the suboptimal acoustics of standard classrooms. Remote microphone technology (RMT) is one way to improve the listening environment. The aim of this study was to determine the assistive potential of RMT for speech identification and attention skills in children with LiD, and to investigate whether the benefits obtained by these children were greater than for those with no listening concerns. DESIGN: A total of 28 children with LiD and 10 control participants with no listening concerns aged 6 to 12 years were included in this study. Children attended two laboratory-based testing sessions, where their speech intelligibility and attention skills were behaviorally assessed with and without the use of RMT. RESULTS: There were significant improvements in speech identification and attention skills when RMT was used. For the LiD group, use of the devices improved speech intelligibility to being comparable or better than control abilities without RMT. Auditory attention scores also improved from being poorer than controls without RMT to comparable to control performance with device assistance. CONCLUSIONS: Use of RMT was found to have a positive effect on both speech intelligibility and attention. RMT should be considered a viable option for addressing common behavioral symptoms of LiD, including for the many children that present with concerns of inattentiveness.

Evaluation of a game-based hearing screening program for identifying hearing loss in primary school-aged children.

OBJECTIVES: To evaluate a tablet-based hearing screening game in primary school aged children. To examine the prevalence of middle/outer ear pathology, hearing loss and spatial processing disorder in primary school aged children. DESIGN: The automated hearing test Sound Scouts was used as a screening tool, which measures hearing abnormalities through tests of speech-in-quiet/noise and tone-in-noise. Children who failed the screenings underwent follow up testing with pure tone audiometry, tympanometry, otoscopy, and the Listening in Spatialised Noise-Sentences test. Results of each test were compared to measure efficacy. STUDY SAMPLE: 1256 children aged 4-13 years from 8 primary schools. RESULTS: 111 children (8.84%) presented with evidence of middle/outer ear pathologies. 21 children (1.67%) had hearing loss in at least one ear. 30 children (2.52%) were diagnosed with spatial processing disorder. False positive rate was 5.01%, indicating that a relatively small proportion of the children who failed the screenings were subsequently shown to have normal auditory function. CONCLUSIONS: A game based program testing sound detection and binaural speech processing can be effective in detecting undiagnosed hearing deficits, in large format school-based hearing screenings. Prevalence of hearing abnormalities in Victorian primary school aged children were established, highlighting the value of school hearing screening programs.

Vestibular function and balance performance in children with sensorineural hearing loss.

OBJECTIVE: Balance difficulties are common in children with sensorineural hearing loss (SNHL). For some of these children, concomitant vestibular deficits may impact postural control. This study aimed to explore vestibular function, functional balance and postural control, and the relationship between these measures in children with SNHL. DESIGN: Cross-sectional study quantifying peripheral vestibular function (vestibular evoked myogenic potentials [VEMP], video head impulse test), functional balance (Bruininks-Oseretsky Test of Motor Proficiency [BOT]) and postural control (static posturography with modified sensory inputs). The relationship between the degree of vestibular impairment, functional balance and postural control was explored. STUDY SAMPLE: Eleven with SNHL, and 11 with normal sound detection (NSD) between 5 and 12 years of age. RESULTS: Children with SNHL had varying degrees of vestibular dysfunction and differences in overall balance performance. Across all children, greater degrees of vestibular impairment were associated with significantly poorer functional balance and postural control performance for complex standing conditions (BOT percentile rank p = 0.001; compliant surface eyes open [EO]: p = 0.027; compliant surface eyes closed: p = 0.048). CONCLUSIONS: Vestibular dysfunction in children with SNHL was variable. Vestibular impairment predicted poorer functional balance performance and postural control abilities, including differences in postural sway patterns.

Interviews with musicians with hearing aids.

Objectives: Musicians who wear hearing aids are a unique subset of hearing-impaired individuals. There are a number of issues musicians experience with hearing aids, making effective hearing rehabilitation a challenge. Research suggests hearing aid satisfaction in musicians is lower partly due to their advanced listening skills, however, qualitative research addressing musicians who wear hearing aids for music is scarce. The current study aimed to record the barriers encountered by musicians who wear hearing aids when playing their instrument/singing, listening to recorded music and listening to live music.Design: Professional musicians who wear hearing aids were interviewed. Participants were questioned about their experiences with hearing loss and hearing aids, with particular emphasis on experiences listening to recorded and live music, and playing or performing music with the hearing aids.Study Sample: Eight professional musicians were interviewed, using a semi-structured interview style, with a question and prompt guide.Results: Thematic analysis revealed three main themes in the data: the musicians' journey, communication, and flexibility/adjustability.Conclusions: The results have implications for future research into specific fitting parameters for hearing aids for musicians (particularly for music performance), the need for evidence-based rehabilitation programs for musicians with hearing loss, and the need for a glossary of terms to assist communication between Audiologists and musicians.

A randomized controlled trial of remote microphone listening devices to treat auditory deficits in children with neurofibromatosis type 1.

BACKGROUND: A high proportion of patients with neurofibromatosis type 1 (NF1) present with functional hearing deficiency as a result of neural abnormality in the late auditory brainstem. METHODS: In this randomized, two-period crossover study, we investigated the hypothesis that remote-microphone listening devices can ameliorate hearing and communication deficits in affected school-aged children (7-17 years). Speech perception ability in background noise was evaluated in device-active and inactive conditions using the CNC-word test. Participants were then randomized to one of two treatment sequences: (1) inactive device for two weeks (placebo), followed by active device use for two weeks, or (2) active device for 2 weeks, followed by inactive device for 2 weeks. Listening and communication ratings (LIFE-R Questionnaire) were obtained at baseline and at the end of each treatment phase. RESULTS: Each participant demonstrated functional hearing benefits with remote-microphone use. All showed a speech perception in noise increase when the device was activated with a mean phoneme-score difference of 16.4% (p < 0.001) and reported improved listening/communication abilities in the school classroom (mean difference: 23.4%; p = 0.017). DISCUSSION: Conventional hearing aids are typically ineffective as a treatment for auditory neural dysfunction, making sounds louder, but not clearer for affected individuals. In this study, we demonstrate that remote-microphone technologies are acceptable/tolerable in pediatric patients with NF1 and can ameliorate their hearing deficits. CONCLUSION: Remote-microphone listening systems offer a viable treatment option for children with auditory deficits associated with NF1.

Fiber-Specific Changes in White Matter Microstructure in Individuals With X-Linked Auditory Neuropathy.

OBJECTIVES: Auditory neuropathy (AN) is the term used to describe a group of hearing disorders, in which the hearing impairment occurs as a result of abnormal auditory nerve function. While our understanding of this condition has advanced significantly over recent years, the ability to determine the site of lesion and the extent of dysfunction in affected individuals remains a challenge. To this end, we investigated potential axonal degeneration in the white matter tracts of the brainstem in individuals with X-linked AN. We hypothesized that individuals with X-linked AN would show focal degeneration within the VIII nerve and/or auditory brainstem tracts, and the degree of degeneration would correlate with the extent of auditory perceptual impairment. DESIGN: This was achieved using a higher-order diffusion magnetic resonance imaging (dMRI)-based quantitative measure called apparent fiber density as obtained from a technique called single-shell 3-tissue constrained spherical deconvolution and analyzed with the fixel-based analysis framework. Eleven subjects with genetically confirmed X-linked AN and 11 controls with normal hearing were assessed using behavioral and objective auditory measures. dMRI data were also collected for each participant. RESULTS: Fixel-based analysis of the brainstem region showed that subjects with X-linked AN had significantly lower apparent fiber density in the VIII nerve compared with controls, consistent with axonal degeneration in this region. Subsequent analysis of the auditory brainstem tracts specifically showed that degeneration was also significant in these structures overall. The apparent fiber density findings were supported by objective measures of auditory function, such as auditory brainstem responses, electrocochleography, and otoacoustic emissions, which showed VIII nerve activity was severely disrupted in X-linked AN subjects while cochlear sensory hair cell function was relatively unaffected. Moreover, apparent fiber density results were significantly correlated with temporal processing ability (gap detection task) in affected subjects, suggesting that the degree of VIII nerve degeneration may impact the ability to resolve temporal aspects of an acoustic signal. Auditory assessments of sound detection, speech perception, and the processing of binaural cues were also significantly poorer in the X-linked AN group compared with the controls with normal hearing. CONCLUSIONS: The results of this study suggest that the dMRI-based measure of apparent fiber density may provide a useful adjunct to existing auditory assessments in the characterization of the site of lesion and extent of dysfunction in individuals with AN. Additionally, the ability to determine the degree of degeneration has the potential to guide rehabilitation strategies in the future.

The frequency-following response as an assessment of spatial processing.

Objective: It is important to detect children with difficulties distinguishing speech-in-noise early. Prompt identification may be assisted by an evoked potential. The aims of the present study were: 1) to evaluate the frequency-following response (FFR) as a measure of binaural processing and spatial listening and, 2) to investigate the relationship between the FFR and a behavioural measure of binaural processing and spatial listening. Design: A single group, repeated measures design. The FFR was recorded in two different spatial conditions and amplitudes compared to spatial listening ability. Study Sample: Thirty-two children (aged 6.0 to 13.1 years) with a range of spatial processing abilities as measured behaviourally using the Listening in Spatialised Noise Sentences test (LiSN-S). Results: FFR waveforms were elicited using speech-like stimuli in co-located and separated conditions. A significant (p≤0.005) spatial advantage effect was observed with larger amplitudes in the separated condition. No correlations were observed between FFR amplitude and LiSN-S results. Conclusions: The FFR shows promise as a measure of binaural processing and spatial listening, but could be measuring different processes to those measured by the LiSN-S.

Wireless binaural hearing aid technology for telephone use and listening in wind noise.

OBJECTIVE: To assess the speech perception benefits of binaural streaming technology for bilateral hearing aid users in two difficult listening conditions. DESIGN: Two studies were conducted to compare hearing aid processing features relating to telephone use and wind noise. Speech perception testing was conducted in four different experimental conditions in each study. STUDY SAMPLE: Ten bilaterally-aided children in each study. RESULTS: Significant improvements in speech perception were obtained with a wireless feature for telephone use. Significant speech perception benefits were also obtained with wireless hearing aid features when listening to speech in simulated wind noise. CONCLUSIONS: Binaural signal processing algorithms can significantly improve speech perception for bilateral hearing aid users in challenging listening situations.

Remediation of spatial processing disorder (SPD).

OBJECTIVE: To determine the efficacy of deficit-specific remediation for spatial processing disorder, quantify effects of remediation on functional listening, and determine if remediation is maintained. DESIGN: Participants had SPD, diagnosed using the Listening in Spatialised Noise-Sentences test. The LiSN and Learn software was provided as auditory training. Post-training, repeat LiSN-S testing was conducted. Questionnaires pre- and post-training acted as subjective measures of remediation. A late-outcome assessment established long-term effects of remediation. STUDY SAMPLE: Sixteen children aged between 6;3 [years; months] and 10;0 completed between 20 and 146 training games. RESULTS: Post-training LiSN-S improved in measures containing spatial cues (p ≤ 0.001) by 2.0 SDs (3.6 dB) for DV90, 1.8 SDs for SV90 (3.2 dB), 1.4 SDs for spatial advantage (2.9 dB) and 1.6 SDs for total advantage (3.3 dB). Improvement was also found in the DV0 condition (1.4 dB or 0.5 SDs). Post-training changes were not significant in the talker advantage measure (1.0 dB or 0.4 SDs) or the SV0 condition (0.3 dB or 0.1 SDs). The late-outcome assessment demonstrated improvement was maintained. Subjective improvement post-remediation was observed using the parent questionnaire. CONCLUSIONS: Children with SPD had improved ability to utilise spatial cues following deficit-specific remediation, with the parent questionnaire sensitive to remediation. Effects of the remediation also appear to be sustained.

Measuring Inhibition and Cognitive Flexibility in Friedreich Ataxia.

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder with subtle impact on cognition. Inhibitory processes and cognitive flexibility were examined in FRDA by assessing the ability to suppress a predictable verbal response. We administered the Hayling Sentence Completion Test (HSCT), the Trail Making Test, and the Stroop Test to 43 individuals with FRDA and 42 gender- and age-matched control participants. There were no significant group differences in performance on the Stroop or Trail Making Test whereas significant impairment in cognitive flexibility including the ability to predict and inhibit a pre-potent response as measured in the HSCT was evident in individuals with FRDA. These deficits did not correlate with clinical characteristics of FRDA (age of disease onset, disease duration, number of guanine-adenine-adenine repeats on the shorter or larger FXN allele, or Friedreich Ataxia Rating Scale score), suggesting that such impairment may not be related to the disease process in a straightforward way. The observed specific impairment of inhibition and predictive capacity in individuals with FRDA on the HSCT task, in the absence of impairment in associated executive functions, supports cerebellar dysfunction in conjunction with disturbance to cortico-thalamo-cerebellar connectivity, perhaps via inability to access frontal areas necessary for successful task completion.

Consequences of Early Conductive Hearing Loss on Long-Term Binaural Processing.

OBJECTIVES: The aim of the study was to investigate the long-term effects of early conductive hearing loss on binaural processing in school-age children. DESIGN: One hundred and eighteen children participated in the study, 82 children with a documented history of conductive hearing loss associated with otitis media and 36 controls who had documented histories showing no evidence of otitis media or conductive hearing loss. All children were demonstrated to have normal-hearing acuity and middle ear function at the time of assessment. The Listening in Spatialized Noise Sentence (LiSN-S) task and the masking level difference (MLD) task were used as the two different measures of binaural interaction ability. RESULTS: Children with a history of conductive hearing loss performed significantly poorer than controls on all LiSN-S conditions relying on binaural cues (DV90, p = <0.001 and SV90, p = 0.003). No significant difference was found between the groups in listening conditions without binaural cues. Fifteen children with a conductive hearing loss history (18%) showed results consistent with a spatial processing disorder. No significant difference was observed between the conductive hearing loss group and the controls on the MLD task. Furthermore, no correlations were found between LiSN-S and MLD. CONCLUSIONS: Results show a relationship between early conductive hearing loss and listening deficits that persist once hearing has returned to normal. Results also suggest that the two binaural interaction tasks (LiSN-S and MLD) may be measuring binaural processing at different levels. Findings highlight the need for a screening measure of functional listening ability in children with a history of early otitis media.

Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy.

The effects of inner ear abnormality on audibility have been explored since the early 20th century when sound detection measures were first used to define and quantify 'hearing loss'. The development in the 1970s of objective measures of cochlear hair cell function (cochlear microphonics, otoacoustic emissions, summating potentials) and auditory nerve/brainstem activity (auditory brainstem responses) have made it possible to distinguish both synaptic and auditory nerve disorders from sensory receptor loss. This distinction is critically important when considering aetiology and management. In this review we address the clinical and pathophysiological features of auditory neuropathy that distinguish site(s) of dysfunction. We describe the diagnostic criteria for: (i) presynaptic disorders affecting inner hair cells and ribbon synapses; (ii) postsynaptic disorders affecting unmyelinated auditory nerve dendrites; (iii) postsynaptic disorders affecting auditory ganglion cells and their myelinated axons and dendrites; and (iv) central neural pathway disorders affecting the auditory brainstem. We review data and principles to identify treatment options for affected patients and explore their benefits as a function of site of lesion.

Functional hearing in the classroom: assistive listening devices for students with hearing impairment in a mainstream school setting.

OBJECTIVE: To assess the benefit of assistive listening devices (ALDs) for students with hearing impairment in mainstream schools. DESIGN: Speech recognition (CNC words) in background noise was assessed in a typical classroom. Participants underwent testing using four device configurations: (1) HA(s)/CI(s) alone, (2) soundfield amplification, (3) remote microphone (Roger Pen) on desk and (4) remote microphone at the loudspeaker. A sub-group of students subsequently underwent a 2-week classroom trial of each ALD. Degree of improvement from baseline [HA(s)/CI(s)] alone was assessed using teacher and student Listening Inventory for Education-Revised (LIFE-R) questionnaires. STUDY SAMPLE: In all, 20 students, aged 12.5-18.9 years, underwent speech recognition assessment. In total, 10 of these participated in the classroom trial. Hearing loss ranged from mild-to-profound levels. RESULTS: Performance in each ALD configuration was higher than for HAs/CIs alone (p < 0.001). Teacher and student LIFE-R results indicated significant improvement in listening/communication when using the remote microphone in conjunction with HAs/CIs (p < 0.05). There was no difference between the soundfield system and the baseline measurement (p > 0.05). CONCLUSION: Speech recognition improvements were demonstrated with the implementation of both remote microphones and soundfield systems. Both students and teachers reported functional hearing advantages in the classroom when using the remote microphone in concert with their standard hearing devices.

A new locus for X-linked dominant Charcot-Marie-Tooth disease (CMTX6) is caused by mutations in the pyruvate dehydrogenase kinase isoenzyme 3 (PDK3) gene.

Hereditary motor and sensory disorders of the peripheral nerve form one of the most common groups of human genetic diseases collectively called Charcot-Marie-Tooth (CMT) neuropathy. Using linkage analysis in a three generation kindred, we have mapped a new locus for X-linked dominant CMT to chromosome Xp22.11. A microsatellite scan of the X chromosome established significant linkage to several markers including DXS993 (Zmax = 3.16; θ = 0.05). Extended haplotype analysis refined the linkage region to a 1.43-Mb interval flanked by markers DXS7110 and DXS8027. Whole exome sequencing identified a missense mutation c.G473A (p.R158H) in the pyruvate dehydrogenase kinase isoenzyme 3 (PDK3) gene. The change localized within the 1.43-Mb linkage interval, segregated with the affected phenotype and was excluded in ethnically matched control chromosomes. PDK3 is one of the four isoenzymes regulating the pyruvate dehydrogenase complex (PDC), by reversible phosphorylation, and is a nuclear-coded protein located in the mitochondrial matrix. PDC catalyzes the oxidative decarboxylation of pyruvate to acetyl CoA and is a key enzyme linking glycolysis to the energy-producing Krebs cycle and lipogenic pathways. We found that the R158H mutation confers enzyme hyperactivity and binds with stronger affinity than the wild-type to the inner-lipoyl (L2) domain of the E2p chain of PDC. Our findings suggest a reduced pyruvate flux due to R158H mutant PDK3-mediated hyper-phosphorylation of the PDC as the underlying pathogenic cause of peripheral neuropathy. The results highlight an important causative link between peripheral nerve degeneration and an essential bioenergetic or biosynthetic pathway required for the maintenance of peripheral nerves.

The Impact of Auditory Processing and Cognitive Abilities in Children.

OBJECTIVES: To examine the links between auditory processing (AP) test results, functional deficits, and cognitive abilities. DESIGN: One hundred and fifty-five children, ages 7-12 years, comprising 50 control children and 105 children referred for AP assessment, all with normal peripheral hearing, completed an AP and cognitive (sustained attention, auditory working memory, and nonverbal intelligence) test battery. Functional outcome measures of listening ability (developed using questionnaires from parent, teacher, and child respondents) and reading fluency were also collected. RESULTS: AP scores for dichotic digits, frequency pattern, and listening in spatialized noise-sentences test baseline scores showed significant intertask correlations, and significant correlations with functional outcomes. The gaps in noise task showed correlation with reading fluency only. The AP tasks of masking level differences and spatial advantage showed no correlation with listening ability or reading fluency. Results showed significantly poorer cognitive abilities overall in the children referred for AP assessment compared with the control group. Within the referred group, children diagnosed with an auditory processing disorder had significantly poorer cognitive abilities than those passing the test battery. Correlation and regression studies showed significant associations between AP and cognitive scores. The results of multilinear regression analyses showed that the associations of AP scores with listening and academic results were no longer significant when cognitive scores were also included as predictors. CONCLUSIONS: A complex interaction of cognitive abilities and AP scores is evident. For many children with listening difficulties, who perform poorly on AP tasks, cognitive deficits are also in place. Although the direction of causality is unclear, it is likely that these cognitive deficits are causing the perceived difficulty and/or are having a significant effect on the test results. Interpretation of AP tests requires consideration of how cognitive abilities may have impacted on not only task results but also the functional difficulties experienced by the child.

The use of listening devices to ameliorate auditory deficit in children with autism.

OBJECTIVES: To evaluate both monaural and binaural processing skills in a group of children with autism spectrum disorder (ASD) and to determine the degree to which personal frequency modulation (radio transmission) (FM) listening systems could ameliorate their listening difficulties. STUDY DESIGN: Auditory temporal processing (amplitude modulation detection), spatial listening (integration of binaural difference cues), and functional hearing (speech perception in background noise) were evaluated in 20 children with ASD. Ten of these subsequently underwent a 6-week device trial in which they wore the FM system for up to 7 hours per day. RESULTS: Auditory temporal processing and spatial listening ability were poorer in subjects with ASD than in matched controls (temporal: P = .014 [95% CI -6.4 to -0.8 dB], spatial: P = .003 [1.0 to 4.4 dB]), and performance on both of these basic processing measures was correlated with speech perception ability (temporal: r = -0.44, P = .022; spatial: r = -0.50, P = .015). The provision of FM listening systems resulted in improved discrimination of speech in noise (P < .001 [11.6% to 21.7%]). Furthermore, both participant and teacher questionnaire data revealed device-related benefits across a range of evaluation categories including Effect of Background Noise (P = .036 [-60.7% to -2.8%]) and Ease of Communication (P = .019 [-40.1% to -5.0%]). Eight of the 10 participants who undertook the 6-week device trial remained consistent FM users at study completion. CONCLUSIONS: Sustained use of FM listening devices can enhance speech perception in noise, aid social interaction, and improve educational outcomes in children with ASD.

Long-term hearing deficits after childhood middle ear disease.

OBJECTIVES: To investigate the long-term impact of childhood otitis media on listening ability in school-aged children. DESIGN: Speech perception in background noise was measured in two groups of 35 children, aged 6 to 12 years, with normal middle ear function and sound detection at assessment. The first consisted of children who had previously suffered middle ear disease; the second those with no history of middle ear disease. RESULTS: Binaural speech perception ability was significantly poorer in the children with prior middle ear disease. Furthermore, spatial listening (the ability to selectively attend to a sound signal from one location) was also significantly impaired. Significant correlations were demonstrated between both the age of onset and the duration of childhood otitis media and speech perception ability (onset: r = -0.58, p < 0.001; duration: r = -0.63, p < 0.001). CONCLUSIONS: These results demonstrate the risk of long-term functional hearing deficit for children with middle ear disease history in childhood. They also indicate that this risk is increased with earlier onset and longer duration. The findings highlight the need for early intervention and an awareness of the potential for reduced functional listening ability even after sound detection has returned to normal.