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This diagnostic study assesses the performance of the Apple Watch Noise application in comparison with a class 1 sound level meter.

ObjectivesLate-life depression is a comorbidity that may co-occur in older adults with hearing loss-each has prevalent and independent modifiable risk factors for dementia.MethodsUsing data from 1,820 Health, Aging and Body Composition study participants (74 ± 2.8 years, 38% Black race), we compared the hearing loss-dementia/cognitive decline relationship between those with normal hearing/mild hearing loss and those with moderate or greater hearing loss. Using linear mixed-effects and Cox proportional hazard models, we investigated if the associations between hearing loss and cognitive decline or dementia (Modified Mini-Mental State [3MS] Examination and Digit Symbol Substitution Test [DSST]) differed by the presence or absence of depressive symptoms. Depressive symptoms were defined as Center for Epidemiologic Study-Depression scale 10 ≥10 at one or more visits from Years 1-5. Algorithmic incident dementia was defined using medication use, hospitalizations, and cognitive test scores. Audiometric hearing loss was measured at Year 5 and categorized as normal/mild versus moderate or greater hearing loss.ResultsHaving both hearing loss and depressive symptoms (vs. having neither) was associated with faster rates of decline in 3MS Examination (β = -0.30; 95% confidence interval [CI]: -0.78, -0.19) and DSST (β = -0.35; 95% CI: -0.67, -0.03) over 10 years of follow-up. Having both hearing loss and depressive symptoms (vs. neither) was associated with increased risk (hazard ratio [HR]: 2.91; 95% CI: 1.59, 5.33 vs. HR: 1.54; 95% CI: 1.10, 2.15 hearing loss only and HR: 2.35; 95% CI: 1.56, 3.53 depressive symptoms only) of incident dementia in multivariable-adjusted Cox proportional hazards models.DiscussionComorbid conditions among hearing-impaired older adults should be considered and may aid in dementia prevention and management strategies.

Speech perception is thought to rely on a cortical feedforward serial transformation of acoustic into linguistic representations. Using intracranial recordings across the entire human auditory cortex, electrocortical stimulation, and surgical ablation, we show that cortical processing across areas is not consistent with a serial hierarchical organization. Instead, response latency and receptive field analyses demonstrate parallel and distinct information processing in the primary and nonprimary auditory cortices. This functional dissociation was also observed where stimulation of the primary auditory cortex evokes auditory hallucination but does not distort or interfere with speech perception. Opposite effects were observed during stimulation of nonprimary cortex in superior temporal gyrus. Ablation of the primary auditory cortex does not affect speech perception. These results establish a distributed functional organization of parallel information processing throughout the human auditory cortex and demonstrate an essential independent role for nonprimary auditory cortex in speech processing.

ObjectivesTo describe a cohort of patients presenting with long-term sudden sensorineural hearing loss (SSNHL) treated with prophylactic migraine and intratympanic steroid therapy.MethodsPatients presenting to a neurotology clinic at least 6 weeks from SSNHL onset were included. All patients received migraine prophylactic medication (nortriptyline, topiramate, and/or verapamil) and lifestyle changes for at least 6 weeks, as well as intratympanic steroid injections, if appropriate.ResultsTwenty-one patients (43% female) with a mean age of 64 ± 11 years who presented 9 ± 8 months (median = 5) from symptom onset were included. Posttreatment hearing thresholds were significantly improved compared with pretreatment thresholds at 500 Hz (49 ± 19 dB versus 55 ± 20 dB, p = 0.01), 1000 Hz (52 ± 19 dB versus 57 ± 21 dB, p = 0.03), low-frequency pure-tone average (53 ± 15 dB versus 57 ± 17 dB, p = 0.01), and speech-frequency pure-tone average (57 ± 13 dB versus 60 ± 15 dB, p = 0.02). Posttreatment word-recognition-score (WRS) and speech-recognition-threshold (SRT) were also significantly improved (45 ± 28% versus 70 ± 28% and 57 ± 18 dB versus 50 ± 16 dB, respectively, both p 50% WRS with an average improvement of 39 ± 9%.ConclusionsMigraine medications in addition to intratympanic steroid injections significantly improved SRT and hearing frequencies in 40% and 29% of SSNHL patients, respectively, while significant WRS recovery was observed in most (68%) patients. This suggests SSNHL may be an otologic migraine phenomenon, which may be at least partially reversible even after the traditional 30-day postonset window.

ObjectivesTo describe a cohort of patients presenting with long-term sudden sensorineural hearing loss (SSNHL) treated with prophylactic migraine and intratympanic steroid therapy.MethodsPatients presenting to a neurotology clinic at least 6 weeks from SSNHL onset were included. All patients received migraine prophylactic medication (nortriptyline, topiramate, and/or verapamil) and lifestyle changes for at least 6 weeks, as well as intratympanic steroid injections, if appropriate.ResultsTwenty-one patients (43% female) with a mean age of 64 ± 11 years who presented 9 ± 8 months (median = 5) from symptom onset were included. Posttreatment hearing thresholds were significantly improved compared with pretreatment thresholds at 500 Hz (49 ± 19 dB versus 55 ± 20 dB, p = 0.01), 1000 Hz (52 ± 19 dB versus 57 ± 21 dB, p = 0.03), low-frequency pure-tone average (53 ± 15 dB versus 57 ± 17 dB, p = 0.01), and speech-frequency pure-tone average (57 ± 13 dB versus 60 ± 15 dB, p = 0.02). Posttreatment word-recognition-score (WRS) and speech-recognition-threshold (SRT) were also significantly improved (45 ± 28% versus 70 ± 28% and 57 ± 18 dB versus 50 ± 16 dB, respectively, both p 50% WRS with an average improvement of 39 ± 9%.ConclusionsMigraine medications in addition to intratympanic steroid injections significantly improved SRT and hearing frequencies in 40% and 29% of SSNHL patients, respectively, while significant WRS recovery was observed in most (68%) patients. This suggests SSNHL may be an otologic migraine phenomenon, which may be at least partially reversible even after the traditional 30-day postonset window.

OBJECTIVES:To characterize the progression of mid-frequency sensorineural hearing loss (MFSNHL) over time. METHODS:A retrospective chart review spanning 2012 to 2017 was performed at a tertiary care audiology and neurotology center. Our cohort included 37 patients met the criteria for MFSNHL also known as "cookie bite hearing loss." It was defined as having a 1, 2, and 4 kHz average pure tone audiometry greater than 10 dB in intensity compared with the average threshold at 500 Hz and 8 kHz. RESULTS:Average age at initial presentation was 11.8 years (range, 8 mo to 70 yr). Across all individuals, the average mid-frequency threshold was 47 dB, compared with 27 dB at 500 Hz and 8 kHz. Twenty-three patients (62%) had multiple audiograms with 4-year median follow up time. Average values across all frequencies (0.5, 1, 2, 4, 8 kHz) in the initial audiogram was 37 dB, compared with an average of 39 dB demonstrated on final audiogram. Of those with serial audiograms, only five patients demonstrated threshold changes of 10 dB or more. Of these five patients, only one was found to have clinical worsening of MFSNHL. CONCLUSIONS:MFSNHL is an uncommon audiometric finding with unspecified long-term outcomes. We demonstrated that most patients (96%) with MFSNHL do not experience clinical worsening of their hearing threshold over almost 4 years of follow up. Future prospective studies aimed at collecting longer-term data are warranted to further elucidate the long-term trajectory of MFSNHL patients.

OBJECTIVES:To characterize the progression of mid-frequency sensorineural hearing loss (MFSNHL) over time. METHODS:A retrospective chart review spanning 2012 to 2017 was performed at a tertiary care audiology and neurotology center. Our cohort included 37 patients met the criteria for MFSNHL also known as "cookie bite hearing loss." It was defined as having a 1, 2, and 4 kHz average pure tone audiometry greater than 10 dB in intensity compared with the average threshold at 500 Hz and 8 kHz. RESULTS:Average age at initial presentation was 11.8 years (range, 8 mo to 70 yr). Across all individuals, the average mid-frequency threshold was 47 dB, compared with 27 dB at 500 Hz and 8 kHz. Twenty-three patients (62%) had multiple audiograms with 4-year median follow up time. Average values across all frequencies (0.5, 1, 2, 4, 8 kHz) in the initial audiogram was 37 dB, compared with an average of 39 dB demonstrated on final audiogram. Of those with serial audiograms, only five patients demonstrated threshold changes of 10 dB or more. Of these five patients, only one was found to have clinical worsening of MFSNHL. CONCLUSIONS:MFSNHL is an uncommon audiometric finding with unspecified long-term outcomes. We demonstrated that most patients (96%) with MFSNHL do not experience clinical worsening of their hearing threshold over almost 4 years of follow up. Future prospective studies aimed at collecting longer-term data are warranted to further elucidate the long-term trajectory of MFSNHL patients.

ObjectivesTo characterize the progression of mid-frequency sensorineural hearing loss (MFSNHL) over time.MethodsA retrospective chart review spanning 2012 to 2017 was performed at a tertiary care audiology and neurotology center. Our cohort included 37 patients met the criteria for MFSNHL also known as "cookie bite hearing loss." It was defined as having a 1, 2, and 4 kHz average pure tone audiometry greater than 10 dB in intensity compared with the average threshold at 500 Hz and 8 kHz.ResultsAverage age at initial presentation was 11.8 years (range, 8 mo to 70 yr). Across all individuals, the average mid-frequency threshold was 47 dB, compared with 27 dB at 500 Hz and 8 kHz. Twenty-three patients (62%) had multiple audiograms with 4-year median follow up time. Average values across all frequencies (0.5, 1, 2, 4, 8 kHz) in the initial audiogram was 37 dB, compared with an average of 39 dB demonstrated on final audiogram. Of those with serial audiograms, only five patients demonstrated threshold changes of 10 dB or more. Of these five patients, only one was found to have clinical worsening of MFSNHL.ConclusionsMFSNHL is an uncommon audiometric finding with unspecified long-term outcomes. We demonstrated that most patients (96%) with MFSNHL do not experience clinical worsening of their hearing threshold over almost 4 years of follow up. Future prospective studies aimed at collecting longer-term data are warranted to further elucidate the long-term trajectory of MFSNHL patients.

OBJECTIVES:To characterize the progression of mid-frequency sensorineural hearing loss (MFSNHL) over time. METHODS:A retrospective chart review spanning 2012 to 2017 was performed at a tertiary care audiology and neurotology center. Our cohort included 37 patients met the criteria for MFSNHL also known as "cookie bite hearing loss." It was defined as having a 1, 2, and 4 kHz average pure tone audiometry greater than 10 dB in intensity compared with the average threshold at 500 Hz and 8 kHz. RESULTS:Average age at initial presentation was 11.8 years (range, 8 mo to 70 yr). Across all individuals, the average mid-frequency threshold was 47 dB, compared with 27 dB at 500 Hz and 8 kHz. Twenty-three patients (62%) had multiple audiograms with 4-year median follow up time. Average values across all frequencies (0.5, 1, 2, 4, 8 kHz) in the initial audiogram was 37 dB, compared with an average of 39 dB demonstrated on final audiogram. Of those with serial audiograms, only five patients demonstrated threshold changes of 10 dB or more. Of these five patients, only one was found to have clinical worsening of MFSNHL. CONCLUSIONS:MFSNHL is an uncommon audiometric finding with unspecified long-term outcomes. We demonstrated that most patients (96%) with MFSNHL do not experience clinical worsening of their hearing threshold over almost 4 years of follow up. Future prospective studies aimed at collecting longer-term data are warranted to further elucidate the long-term trajectory of MFSNHL patients.

ObjectiveTo describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations.MethodWe report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition.ResultsIn this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments.ConclusionGPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

ObjectiveTo describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations.MethodWe report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition.ResultsIn this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments.ConclusionGPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

IntroductionHearing loss substantially impacts pediatric development, and early identification improves outcomes. While intervening before school-entry is critical to optimize learning, early-childhood hearing screening practices are highly variable. Conditioned play audiometry (CPA) is the gold standard for preschool hearing screening, but otoacoustic emission (OAE) testing provides objective data that may improve screening outcomes.ObjectivesTo compare outcomes of a community-based low-income preschool hearing program before and after implementation of OAE in a single-visit, two-tiered paradigm. We hypothesized that this intervention would reduce referral rates and improve follow-up while maintaining stable rates of diagnosed sensorineural hearing loss.MethodsWe performed a cohort study of 3257 children screened from July 2014-June 2016. Department of Public Health data were analyzed pre- and post-implementation of second-line OAE testing for children referred on CPA screening with targeted follow-up by DPH staff. Primary outcomes included referral rates, follow-up rates, and diagnosis of sensorineural hearing loss.ResultsDemographics, pure-tone pass rates, and incidence of newly-diagnosed permanent hearing loss were similar across years. After intervention, overall pass rates increased from 92% to 95% (P = 0.0014), while only 0.7% remained unable to be tested (P

IntroductionHearing loss substantially impacts pediatric development, and early identification improves outcomes. While intervening before school-entry is critical to optimize learning, early-childhood hearing screening practices are highly variable. Conditioned play audiometry (CPA) is the gold standard for preschool hearing screening, but otoacoustic emission (OAE) testing provides objective data that may improve screening outcomes.ObjectivesTo compare outcomes of a community-based low-income preschool hearing program before and after implementation of OAE in a single-visit, two-tiered paradigm. We hypothesized that this intervention would reduce referral rates and improve follow-up while maintaining stable rates of diagnosed sensorineural hearing loss.MethodsWe performed a cohort study of 3257 children screened from July 2014-June 2016. Department of Public Health data were analyzed pre- and post-implementation of second-line OAE testing for children referred on CPA screening with targeted follow-up by DPH staff. Primary outcomes included referral rates, follow-up rates, and diagnosis of sensorineural hearing loss.ResultsDemographics, pure-tone pass rates, and incidence of newly-diagnosed permanent hearing loss were similar across years. After intervention, overall pass rates increased from 92% to 95% (P = 0.0014), while only 0.7% remained unable to be tested (P

In a complex auditory scene, signals of interest can be distinguished from masking sounds by differences in source location [spatial release from masking (SRM)] and by differences between masker-alone and masker-plus-signal envelopes. This study investigated interactions between those factors in release of masking of 700-Hz tones in an open sound field. Signal and masker sources were colocated in front of the listener, or the signal source was shifted 90° to the side. In Experiment 1, the masker contained a 25-Hz-wide on-signal band plus flanking bands having envelopes that were either mutually uncorrelated or were comodulated. Comodulation masking release (CMR) was largely independent of signal location at a higher masker sound level, but at a lower level CMR was reduced for the lateral signal location. In Experiment 2, a brief signal was positioned at the envelope maximum (peak) or minimum (dip) of a 50-Hz-wide on-signal masker. Masking was released in dip more than in peak conditions only for the 90° signal. Overall, open-field SRM was greater in magnitude than binaural masking release reported in comparable closed-field studies, and envelope-related release was somewhat weaker. Mutual enhancement of masking release by spatial and envelope-related effects tended to increase with increasing masker level.

A given speech sound will be realized differently depending on the context in which it is produced. Listeners have been found to compensate perceptually for these coarticulatory effects, yet it is unclear to what extent this effect depends on actual production experience. In this study, whether changes in motor-to-sound mappings induced by adaptation to altered auditory feedback can affect perceptual compensation for coarticulation is investigated. Specifically, whether altering how the vowel [i] is produced can affect the categorization of a stimulus continuum between an alveolar and a palatal fricative whose interpretation is dependent on vocalic context is tested. It was found that participants could be sorted into three groups based on whether they tended to oppose the direction of the shifted auditory feedback, to follow it, or a mixture of the two, and that these articulatory responses, not the shifted feedback the participants heard, correlated with changes in perception. These results indicate that sensorimotor adaptation to altered feedback can affect the perception of unaltered yet coarticulatorily-dependent speech sounds, suggesting a modulatory role of sensorimotor experience on speech perception.

ObjectiveTo record envelope following responses (EFRs) to monaural amplitude-modulated broadband noise carriers in which amplitude modulation (AM) depth was slowly changed over time and to compare these objective electrophysiological measures to subjective behavioral thresholds in young normal hearing and older subjects.DesignParticipantsthree groups of subjects included a young normal-hearing group (YNH 18 to 28 years; pure-tone average = 5 dB HL), a first older group ("O1"; 41 to 62 years; pure-tone average = 19 dB HL), and a second older group ("O2"; 67 to 82 years; pure-tone average = 35 dB HL). Electrophysiology: In condition 1, the AM depth (41 Hz) of a white noise carrier, was continuously varied from 2% to 100% (5%/s). EFRs were analyzed as a function of the AM depth. In condition 2, auditory steady-state responses were recorded to fixed AM depths (100%, 75%, 50%, and 25%) at a rate of 41 Hz. Psychophysics: A 3 AFC (alternative forced choice) procedure was used to track the AM depth needed to detect AM at 41 Hz (AM detection). The minimum AM depth capable of eliciting a statistically detectable EFR was defined as the physiological AM detection threshold.ResultsAcross all ages, the fixed AM depth auditory steady-state response and swept AM EFR yielded similar response amplitudes. Statistically significant correlations (r = 0.48) were observed between behavioral and physiological AM detection thresholds. Older subjects had slightly higher (not significant) behavioral AM detection thresholds than younger subjects. AM detection thresholds did not correlate with age. All groups showed a sigmoidal EFR amplitude versus AM depth function but the shape of the function differed across groups. The O2 group reached EFR amplitude plateau levels at lower modulation depths than the normal-hearing group and had a narrower neural dynamic range. In the young normal-hearing group, the EFR phase did not differ with AM depth, whereas in the older group, EFR phase showed a consistent decrease with increasing AM depth. The degree of phase change (or phase slope) was significantly correlated to the pure-tone threshold at 4 kHz.ConclusionsEFRs can be recorded using either the swept modulation depth or the discrete AM depth techniques. Sweep recordings may provide additional valuable information at suprathreshold intensities including the plateau level, slope, and dynamic range. Older subjects had a reduced neural dynamic range compared with younger subjects suggesting that aging affects the ability of the auditory system to encode subtle differences in the depth of AM. The phase-slope differences are likely related to differences in low and high-frequency contributions to EFR. The behavioral-physiological AM depth threshold relationship was significant but likely too weak to be clinically useful in the present individual subjects who did not suffer from apparent temporal processing deficits.

ConclusionThis study described objective and subjective evaluations of the Nurotron® Venus™ Cochlear Implant System and indicated that this system produced a satisfactory performance.ObjectiveTo observe the performance of the Nurotron® Venus™ cochlear implant (CI) system via electrophysiological and psychophysical evaluations.MethodsA 26-electrode CI system was specially designed. The performance of MRI in animal and cadaveric head experiments, EABR in cats experiment, the correlation between ESRT and C level, and psychophysics evaluations in clinical trials were observed.ResultsIn the animal and cadaveric head experiments, magnet dislocation could not be prevented in the 1.5 T MRI without removal of the internal magnet. The EABR was clearly elicited in cat experiment. In the clinical trial, the ESRT was strongly correlated with C level (p

Rates of hearing impairment in individuals with Autism Spectrum Disorders (ASD) are higher than those reported in the general population. Although ASD is not caused by hearing impairment, it may exacerbate symptomatology. Participants with ASD (N = 60) and typically developing peers (N = 16) aged 5-18 years underwent a comprehensive audiological screening (pure tone audiometry, uncomfortable loudness level, tympanometry, acoustic reflexes, distortion product otoacoustic emissions, and auditory brainstem response) and assessment of communication abilities (expressive/receptive language, articulation, phonological awareness, and vocal affect recognition). Incidence of abnormal findings on at least one measure of audiological functioning was higher for the ASD group (55%) than controls (14.9%) or the general population estimate (6%). The presence of sound sensitivity was also considerably higher for the ASD group (37%) compared with controls (0%) or general population estimates (8-15%). When participants with ASD were dichotomized into groups with and without evidence of clinical audiological abnormality, no significant differences were identified on measures of communication; however, results of correlational analyses indicated that variability in hearing thresholds at middle range frequencies (2000 Hz) was significantly related to performance on all measures of speech articulation and language after correction for multiple comparisons (r = -0.48 to r = -0.53, P

ImportanceSurgical repair of congenital aural atresia and hypoplasia (CAAH) is technically challenging. Long-term surgical and audiologic outcomes of atresiaplasty are incompletely understood.ObjectivesTo review the surgical outcomes for CAAH and analyze the hearing results.Design, setting, and participantsA retrospective medical record review of CAAH outcomes was performed during an 11-year period from January 1, 2004, through December 31, 2014. The data analysis was undertaken from December 1, 2014, through January 31, 2015. The mean clinic follow-up time was 3.9 years, and the mean audiologic follow-time was 2.8 years. The study included 98 patients aged 5 to 66 years (mean age, 16.6 years) with CAAH who underwent a total of 104 operations.InterventionsSurgical repair of CAAH.Main outcomes and measuresPreoperative and postoperative pure-tone averages (PTAs), speech reception thresholds (SRTs), air-bone gaps (ABGs), and interaural PTA and SRT differences were compared. Factors that affect hearing outcomes were analyzed. The complication rates were reviewed and compared with results from similar studies.ResultsIn the 98 patients with CAAH, the mean improvement in ABGs and SRTs was 26.7 and 25.9 dB, respectively, resulting in a postoperative ABG of 30 dB or less in 4 of 5 cases. The mean postoperative PTAs and SRTs were 36.9 and 34.3 dB, respectively. Patients with a functional native ossicular chain (36 of 104 [34.6%]) had significantly superior audiometric outcomes when compared with patients in whom a reconstruction prosthesis was required during primary or revision operations. Audiometric results from hypoplasia surgery were not significantly different from those of atresia surgery; results in patients with craniofacial syndromes were similarly not significantly different from those in patients with sporadic CAAH. We report a low incidence of meatal stenosis.Conclusions and relevanceThe mean hearing outcomes for this group compared favorably with other series. The need for ossicular chain reconstruction was associated with poorer audiometric outcomes. The safety profile and the demonstrated hearing improvement of CAAH surgery suggest that it remains a favorable option for patients.

ImportanceSurgical repair of congenital aural atresia and hypoplasia (CAAH) is technically challenging. Long-term surgical and audiologic outcomes of atresiaplasty are incompletely understood.ObjectivesTo review the surgical outcomes for CAAH and analyze the hearing results.Design, setting, and participantsA retrospective medical record review of CAAH outcomes was performed during an 11-year period from January 1, 2004, through December 31, 2014. The data analysis was undertaken from December 1, 2014, through January 31, 2015. The mean clinic follow-up time was 3.9 years, and the mean audiologic follow-time was 2.8 years. The study included 98 patients aged 5 to 66 years (mean age, 16.6 years) with CAAH who underwent a total of 104 operations.InterventionsSurgical repair of CAAH.Main outcomes and measuresPreoperative and postoperative pure-tone averages (PTAs), speech reception thresholds (SRTs), air-bone gaps (ABGs), and interaural PTA and SRT differences were compared. Factors that affect hearing outcomes were analyzed. The complication rates were reviewed and compared with results from similar studies.ResultsIn the 98 patients with CAAH, the mean improvement in ABGs and SRTs was 26.7 and 25.9 dB, respectively, resulting in a postoperative ABG of 30 dB or less in 4 of 5 cases. The mean postoperative PTAs and SRTs were 36.9 and 34.3 dB, respectively. Patients with a functional native ossicular chain (36 of 104 [34.6%]) had significantly superior audiometric outcomes when compared with patients in whom a reconstruction prosthesis was required during primary or revision operations. Audiometric results from hypoplasia surgery were not significantly different from those of atresia surgery; results in patients with craniofacial syndromes were similarly not significantly different from those in patients with sporadic CAAH. We report a low incidence of meatal stenosis.Conclusions and relevanceThe mean hearing outcomes for this group compared favorably with other series. The need for ossicular chain reconstruction was associated with poorer audiometric outcomes. The safety profile and the demonstrated hearing improvement of CAAH surgery suggest that it remains a favorable option for patients.

ConclusionThis study described objective and subjective evaluations of the Nurotron® Venus™ Cochlear Implant System and indicated that this system produced a satisfactory performance.ObjectiveTo observe the performance of the Nurotron® Venus™ cochlear implant (CI) system via electrophysiological and psychophysical evaluations.MethodsA 26-electrode CI system was specially designed. The performance of MRI in animal and cadaveric head experiments, EABR in cats experiment, the correlation between ESRT and C level, and psychophysics evaluations in clinical trials were observed.ResultsIn the animal and cadaveric head experiments, magnet dislocation could not be prevented in the 1.5 T MRI without removal of the internal magnet. The EABR was clearly elicited in cat experiment. In the clinical trial, the ESRT was strongly correlated with C level (p

BackgroundExternal auditory canal exostoses are benign, bony overgrowths that arise in patients who experience chronic cold water exposure. While considerable advancement has been made in canalplasty techniques in recent decades, many patients continue to experience prolonged healing periods and recurrent stenosis following surgery.ObjectiveTo perform a retrospective outcomes analysis of our experience with a skin-preserving canalplasty technique with temporoparietal fascia grafting and use of bone wax for skin flap protection.Study designRetrospective review.Subjects and methodsThirty-four patients (41 ears) underwent canalplasty from 2008 to 2014 at a tertiary referral center. Primary outcome measures included rates of prolonged healing and restenosis, need for revision surgery, and audiometric results. Rates of intraoperative and postoperative complications were also tabulated.ResultsNo patient experienced recurrent stenosis or required a revision surgery within the follow-up period. All but one patient (97%) achieved complete healing within 8 weeks. Minimal intraoperative complications were found. Statistically significant improvements in air pure tone averages and air-bone gaps were achieved.ConclusionThe proposed technique is a safe and effective method of canalplasty for exostoses that imparts accelerated wound healing and minimizes the rate of recurrent stenosis.

Mutations in the OTOF gene encoding otoferlin result in a disrupted function of the ribbon synapses with impairment of the multivesicular glutamate release. Most affected subjects present with congenital hearing loss and abnormal auditory brainstem potentials associated with preserved cochlear hair cell activities (otoacoustic emissions, cochlear microphonics [CMs]). Transtympanic electrocochleography (ECochG) has recently been proposed for defining the details of potentials arising in both the cochlea and auditory nerve in this disorder, and with a view to shedding light on the pathophysiological mechanisms underlying auditory dysfunction. We review the audiological and electrophysiological findings in children with congenital profound deafness carrying two mutant alleles of the OTOF gene. We show that cochlear microphonic (CM) amplitude and summating potential (SP) amplitude and latency are normal, consistently with a preserved outer and inner hair cell function. In the majority of OTOF children, the SP component is followed by a markedly prolonged low-amplitude negative potential replacing the compound action potential (CAP) recorded in normally-hearing children. This potential is identified at intensities as low as 90 dB below the behavioral threshold. In some ears, a synchronized CAP is superimposed on the prolonged responses at high intensity. Stimulation at high rates reduces the amplitude and duration of the prolonged potentials, consistently with their neural generation. In some children, however, the ECochG response only consists of the SP, with no prolonged potential. Cochlear implants restore hearing sensitivity, speech perception and neural CAP by electrically stimulating the auditory nerve fibers. These findings indicate that an impaired multivesicular glutamate release in OTOF-related disorders leads to abnormal auditory nerve fiber activation and a consequent impairment of spike generation. The magnitude of these effects seems to vary, ranging from no auditory nerve fiber activation to an abnormal generation of EPSPs that occasionally trigger a synchronized electrical activity, resulting in high-threshold CAPs.

BackgroundExternal auditory canal exostoses are benign, bony overgrowths that arise in patients who experience chronic cold water exposure. While considerable advancement has been made in canalplasty techniques in recent decades, many patients continue to experience prolonged healing periods and recurrent stenosis following surgery.ObjectiveTo perform a retrospective outcomes analysis of our experience with a skin-preserving canalplasty technique with temporoparietal fascia grafting and use of bone wax for skin flap protection.Study designRetrospective review.Subjects and methodsThirty-four patients (41 ears) underwent canalplasty from 2008 to 2014 at a tertiary referral center. Primary outcome measures included rates of prolonged healing and restenosis, need for revision surgery, and audiometric results. Rates of intraoperative and postoperative complications were also tabulated.ResultsNo patient experienced recurrent stenosis or required a revision surgery within the follow-up period. All but one patient (97%) achieved complete healing within 8 weeks. Minimal intraoperative complications were found. Statistically significant improvements in air pure tone averages and air-bone gaps were achieved.ConclusionThe proposed technique is a safe and effective method of canalplasty for exostoses that imparts accelerated wound healing and minimizes the rate of recurrent stenosis.

Mutations in the OTOF gene encoding otoferlin result in a disrupted function of the ribbon synapses with impairment of the multivesicular glutamate release. Most affected subjects present with congenital hearing loss and abnormal auditory brainstem potentials associated with preserved cochlear hair cell activities (otoacoustic emissions, cochlear microphonics [CMs]). Transtympanic electrocochleography (ECochG) has recently been proposed for defining the details of potentials arising in both the cochlea and auditory nerve in this disorder, and with a view to shedding light on the pathophysiological mechanisms underlying auditory dysfunction. We review the audiological and electrophysiological findings in children with congenital profound deafness carrying two mutant alleles of the OTOF gene. We show that cochlear microphonic (CM) amplitude and summating potential (SP) amplitude and latency are normal, consistently with a preserved outer and inner hair cell function. In the majority of OTOF children, the SP component is followed by a markedly prolonged low-amplitude negative potential replacing the compound action potential (CAP) recorded in normally-hearing children. This potential is identified at intensities as low as 90 dB below the behavioral threshold. In some ears, a synchronized CAP is superimposed on the prolonged responses at high intensity. Stimulation at high rates reduces the amplitude and duration of the prolonged potentials, consistently with their neural generation. In some children, however, the ECochG response only consists of the SP, with no prolonged potential. Cochlear implants restore hearing sensitivity, speech perception and neural CAP by electrically stimulating the auditory nerve fibers. These findings indicate that an impaired multivesicular glutamate release in OTOF-related disorders leads to abnormal auditory nerve fiber activation and a consequent impairment of spike generation. The magnitude of these effects seems to vary, ranging from no auditory nerve fiber activation to an abnormal generation of EPSPs that occasionally trigger a synchronized electrical activity, resulting in high-threshold CAPs.

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.

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.

Digit recognition was measured in quiet and in two noise conditions by English-native (EN) and Chinese-native (CN) listeners. EN listeners were tested using English digits and CN listeners were tested using both English and Chinese digits. In quiet, forward digit span recall worsened for both groups as the number of digits was increased. Significant effects of language experience were observed with five or more digits. Language experience had a significant effect on digit recognition in babble but not in steady noise. These results suggest that understanding of a nonnative language can be influenced by both cognitive load and listening environment.

ObjectiveTo compare outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) treated with intratympanic (IT) dexamethasone (DEX) at either 10 mg/mL or 24 mg/mL.Study designRetrospective case series.SettingTertiary referral center.PatientsThirty-seven adults with ISSNHL.InterventionsIn addition to concurrent prednisone taper, patients received a series of IT DEX injections for 2 weeks with either 10 mg/mL or 24 mg/mL.Main outcome measureGreater than 30-dB improvement in pure-tone average (PTA).ResultsBaseline characteristics were similar between groups. Mean follow-up was 10 weeks. Ten (53%) of 19 patients treated with 24 mg/mL had greater than 30-dB improvement in PTA compared with 3 (17%) of 18 treated with 10 mg/mL (p = 0.0382, Fisher's exact test). There was a trend toward improved word recognition score outcome with 24 mg/mL. The interval between onset and initiation of IT DEX significantly affected outcome, with earlier treatment resulting in greater improvement in PTA and word recognition score. Multivariate logistic regression confirmed that IT DEX dose and interval to starting treatment were both independent predictors of PTA outcome. Change in PTA was not significantly affected by age, sex, pretreatment hearing levels, or concurrent treatment with hyperbaric oxygen.ConclusionTo our knowledge, this is the first demonstration of superiority of IT DEX at 24 mg/mL for the treatment of ISSNHL, with significantly better recovery of PTA. Our data suggest that treatment should be initiated as soon as possible. A prospective randomized trial to confirm the optimal dose is warranted.

BackgroundIdentifying factors associated with functional declines in older adults is important given the aging of the population. We investigated if hearing impairment is independently associated with objectively measured declines in physical functioning in a community-based sample of older adults.MethodsProspective observational study of 2,190 individuals from the Health, Aging, and Body Composition study. Participants were followed annually for up to 11 visits. Hearing was measured with pure-tone audiometry. Physical functioning and gait speed were measured with the Short Physical Performance Battery (SPPB). Incident disability and requirement for nursing care were assessed semiannually through self-report.ResultsIn a mixed-effects model, greater hearing impairment was associated with poorer physical functioning. At both Visit 1 and Visit 11, SPPB scores were lower in individuals with mild (10.14 [95% CI 10.04-10.25], p < .01; 7.35 [95% CI 7.12-7.58], p < .05) and moderate or greater hearing impairment (10.04 [95% CI 9.90-10.19], p < .01; 7.00 [95% CI 6.69-7.32], p < .01) than scores in normal hearing individuals (10.36 [95% CI 10.26-10.46]; 7.71 [95% CI 7.49-7.92]). We observed that women with moderate or greater hearing impairment had a 31% increased risk of incident disability (Hazard ratio [HR] =1.31 [95% CI 1.08-1.60], p < .01) and a 31% increased risk of incident nursing care requirement (HR = 1.31 [95% CI 1.05-1.62], p = .02) compared to women with normal hearing.ConclusionsHearing impairment is independently associated with poorer objective physical functioning in older adults, and a 31% increased risk for incident disability and need for nursing care in women.

Although the cochlear implant has been widely acknowledged as the most successful neural prosthesis, only a fraction of hearing-impaired people who can potentially benefit from a cochlear implant have actually received one due to its limited awareness, accessibility, and affordability. To help overcome these limitations, a 26-electrode cochlear implant has been developed to receive China's Food and Drug Administration (CFDA) approval in 2011 and Conformité Européenne (CE) Marking in 2012. The present article describes design philosophy, system specification, and technical verification of the Nurotron device, which includes advanced digital signal processing and 4 current sources with multiple amplitude resolutions that not only are compatible with perceptual capability but also allow interleaved or simultaneous stimulation. The article also presents 3-year longitudinal evaluation data from 60 human subjects who have received the Nurotron device. The objective measures show that electrode impedance decreased within the first month of device use, but was stable until a slight increase at the end of two years. The subjective loudness measures show that electric stimulation threshold was stable while the maximal comfort level increased over the 3 years. Mandarin sentence recognition increased from the pre-surgical 0%-correct score to a plateau of about 80% correct with 6-month use of the device. Both indirect and direct comparisons indicate indistinguishable performance differences between the Nurotron system and other commercially available devices. The present 26-electrode cochlear implant has already helped to lower the price of cochlear implantation in China and will likely contribute to increased cochlear implant access and success in the rest of the world. This article is part of a Special Issue entitled .

Although the cochlear implant has been widely acknowledged as the most successful neural prosthesis, only a fraction of hearing-impaired people who can potentially benefit from a cochlear implant have actually received one due to its limited awareness, accessibility, and affordability. To help overcome these limitations, a 26-electrode cochlear implant has been developed to receive China's Food and Drug Administration (CFDA) approval in 2011 and Conformité Européenne (CE) Marking in 2012. The present article describes design philosophy, system specification, and technical verification of the Nurotron device, which includes advanced digital signal processing and 4 current sources with multiple amplitude resolutions that not only are compatible with perceptual capability but also allow interleaved or simultaneous stimulation. The article also presents 3-year longitudinal evaluation data from 60 human subjects who have received the Nurotron device. The objective measures show that electrode impedance decreased within the first month of device use, but was stable until a slight increase at the end of two years. The subjective loudness measures show that electric stimulation threshold was stable while the maximal comfort level increased over the 3 years. Mandarin sentence recognition increased from the pre-surgical 0%-correct score to a plateau of about 80% correct with 6-month use of the device. Both indirect and direct comparisons indicate indistinguishable performance differences between the Nurotron system and other commercially available devices. The present 26-electrode cochlear implant has already helped to lower the price of cochlear implantation in China and will likely contribute to increased cochlear implant access and success in the rest of the world. This article is part of a Special Issue entitled .

BackgroundHearing impairment (HI) is highly prevalent in older adults and is associated with social isolation, depression, and risk of dementia. Whether HI is associated with broader downstream outcomes is unclear. We undertook this study to determine whether audiometric HI is associated with mortality in older adults.MethodsProspective observational data from 1,958 adults ≥70 years of age from the Health, Aging, and Body Composition Study were analyzed using Cox proportional hazards regression. Participants were followed for 8 years after audiometric examination. Mortality was adjudicated by obtaining death certificates. Hearing was defined as the pure-tone average of hearing thresholds in decibels re: hearing level (dB HL) at frequencies from 0.5 to 4kHz. HI was defined as pure-tone average >25 dB HL in the better ear.ResultsOf the 1,146 participants with HI, 492 (42.9%) died compared with 255 (31.4%) of the 812 with normal hearing (odds ratio = 1.64, 95% CI: 1.36-1.98). After adjustment for demographics and cardiovascular risk factors, HI was associated with a 20% increased mortality risk compared with normal hearing (hazard ratio = 1.20, 95% CI: 1.03-1.41). Confirmatory analyses treating HI as a continuous predictor yielded similar results, demonstrating a nonlinear increase in mortality risk with increasing HI (hazard ratio = 1.14, 95% CI: 1.00-1.29 per 10 dB of threshold elevation up to 35 dB HL).ConclusionsHI in older adults is associated with increased mortality, independent of demographics and cardiovascular risk factors. Further research is necessary to understand the basis of this association and whether these pathways might be amenable to hearing rehabilitation.

BackgroundSome experimental and human data suggest that exposure to polychlorinated biphenyls (PCBs) may induce ototoxicity, though results of previous epidemiologic studies are mixed and generally focus on either prenatal or postnatal PCB concentrations exclusively.ObjectivesOur aim was to evaluate the association between pre- and postnatal PCB concentrations in relation to cochlear status, assessed by distortion product otoacoustic emissions (DPOAEs), and to further clarify the critical periods in development where cochlear status may be most susceptible to PCBs.MethodsA total of 351 children from a birth cohort in eastern Slovakia underwent otoacoustic testing at 45 months of age. Maternal pregnancy, cord, and child 6-, 16-, and 45-month blood samples were collected and analyzed for PCB concentrations. At 45 months of age, DPOAEs were assessed at 11 frequencies in both ears. Multivariate, generalized linear models were used to estimate the associations between PCB concentrations at different ages and DPOAEs, adjusting for potential confounders.ResultsMaternal and cord PCB-153 concentrations were not associated with DPOAEs at 45 months. Higher postnatal PCB concentrations at 6-, 16-, and 45-months of age were associated with lower (poorer) DPOAE amplitudes. When all postnatal PCB exposures were considered as an area-under-the-curve metric, an increase in PCB-153 concentration from the 25th to the 75th percentile was associated with a 1.6-dB SPL (sound pressure level) decrease in DPOAE amplitude (95% CI: -2.6, -0.5; p = 0.003).ConclusionsIn this study, postnatal rather than maternal or cord PCB concentrations were associated with poorer performance on otoacoustic tests at age 45 months.

BackgroundSome experimental and human data suggest that exposure to polychlorinated biphenyls (PCBs) may induce ototoxicity, though results of previous epidemiologic studies are mixed and generally focus on either prenatal or postnatal PCB concentrations exclusively.ObjectivesOur aim was to evaluate the association between pre- and postnatal PCB concentrations in relation to cochlear status, assessed by distortion product otoacoustic emissions (DPOAEs), and to further clarify the critical periods in development where cochlear status may be most susceptible to PCBs.MethodsA total of 351 children from a birth cohort in eastern Slovakia underwent otoacoustic testing at 45 months of age. Maternal pregnancy, cord, and child 6-, 16-, and 45-month blood samples were collected and analyzed for PCB concentrations. At 45 months of age, DPOAEs were assessed at 11 frequencies in both ears. Multivariate, generalized linear models were used to estimate the associations between PCB concentrations at different ages and DPOAEs, adjusting for potential confounders.ResultsMaternal and cord PCB-153 concentrations were not associated with DPOAEs at 45 months. Higher postnatal PCB concentrations at 6-, 16-, and 45-months of age were associated with lower (poorer) DPOAE amplitudes. When all postnatal PCB exposures were considered as an area-under-the-curve metric, an increase in PCB-153 concentration from the 25th to the 75th percentile was associated with a 1.6-dB SPL (sound pressure level) decrease in DPOAE amplitude (95% CI: -2.6, -0.5; p = 0.003).ConclusionsIn this study, postnatal rather than maternal or cord PCB concentrations were associated with poorer performance on otoacoustic tests at age 45 months.

The primary goal of most cochlear implant procedures is to improve a patient's ability to discriminate speech. To accomplish this, cochlear implants are programmed so as to maximize speech understanding. However, programming a cochlear implant can be an iterative, labor-intensive process that takes place over months. In this study, we sought to determine whether functional near-infrared spectroscopy (fNIRS), a non-invasive neuroimaging method which is safe to use repeatedly and for extended periods of time, can provide an objective measure of whether a subject is hearing normal speech or distorted speech. We used a 140 channel fNIRS system to measure activation within the auditory cortex in 19 normal hearing subjects while they listed to speech with different levels of intelligibility. Custom software was developed to analyze the data and compute topographic maps from the measured changes in oxyhemoglobin and deoxyhemoglobin concentration. Normal speech reliably evoked the strongest responses within the auditory cortex. Distorted speech produced less region-specific cortical activation. Environmental sounds were used as a control, and they produced the least cortical activation. These data collected using fNIRS are consistent with the fMRI literature and thus demonstrate the feasibility of using this technique to objectively detect differences in cortical responses to speech of different intelligibility.

The primary goal of most cochlear implant procedures is to improve a patient's ability to discriminate speech. To accomplish this, cochlear implants are programmed so as to maximize speech understanding. However, programming a cochlear implant can be an iterative, labor-intensive process that takes place over months. In this study, we sought to determine whether functional near-infrared spectroscopy (fNIRS), a non-invasive neuroimaging method which is safe to use repeatedly and for extended periods of time, can provide an objective measure of whether a subject is hearing normal speech or distorted speech. We used a 140 channel fNIRS system to measure activation within the auditory cortex in 19 normal hearing subjects while they listed to speech with different levels of intelligibility. Custom software was developed to analyze the data and compute topographic maps from the measured changes in oxyhemoglobin and deoxyhemoglobin concentration. Normal speech reliably evoked the strongest responses within the auditory cortex. Distorted speech produced less region-specific cortical activation. Environmental sounds were used as a control, and they produced the least cortical activation. These data collected using fNIRS are consistent with the fMRI literature and thus demonstrate the feasibility of using this technique to objectively detect differences in cortical responses to speech of different intelligibility.

ObjectiveThe aim of this study is to determine the feasibility of an Apple iOS-based automated hearing testing application and to compare its accuracy with conventional audiometry.Study designProspective diagnostic study. Setting Academic medical center.Subjects and methodsAn iOS-based software application was developed to perform automated pure-tone hearing testing on the iPhone, iPod touch, and iPad. To assess for device variations and compatibility, preliminary work was performed to compare the standardized sound output (dB) of various Apple device and headset combinations. Forty-two subjects underwent automated iOS-based hearing testing in a sound booth, automated iOS-based hearing testing in a quiet room, and conventional manual audiometry.ResultsThe maximum difference in sound intensity between various Apple device and headset combinations was 4 dB. On average, 96% (95% confidence interval [CI], 91%-100%) of the threshold values obtained using the automated test in a sound booth were within 10 dB of the corresponding threshold values obtained using conventional audiometry. When the automated test was performed in a quiet room, 94% (95% CI, 87%-100%) of the threshold values were within 10 dB of the threshold values obtained using conventional audiometry. Under standardized testing conditions, 90% of the subjects preferred iOS-based audiometry as opposed to conventional audiometry.ConclusionApple iOS-based devices provide a platform for automated air conduction audiometry without requiring extra equipment and yield hearing test results that approach those of conventional audiometry.

ObjectiveThe aim of this study is to determine the feasibility of an Apple iOS-based automated hearing testing application and to compare its accuracy with conventional audiometry.Study designProspective diagnostic study. Setting Academic medical center.Subjects and methodsAn iOS-based software application was developed to perform automated pure-tone hearing testing on the iPhone, iPod touch, and iPad. To assess for device variations and compatibility, preliminary work was performed to compare the standardized sound output (dB) of various Apple device and headset combinations. Forty-two subjects underwent automated iOS-based hearing testing in a sound booth, automated iOS-based hearing testing in a quiet room, and conventional manual audiometry.ResultsThe maximum difference in sound intensity between various Apple device and headset combinations was 4 dB. On average, 96% (95% confidence interval [CI], 91%-100%) of the threshold values obtained using the automated test in a sound booth were within 10 dB of the corresponding threshold values obtained using conventional audiometry. When the automated test was performed in a quiet room, 94% (95% CI, 87%-100%) of the threshold values were within 10 dB of the threshold values obtained using conventional audiometry. Under standardized testing conditions, 90% of the subjects preferred iOS-based audiometry as opposed to conventional audiometry.ConclusionApple iOS-based devices provide a platform for automated air conduction audiometry without requiring extra equipment and yield hearing test results that approach those of conventional audiometry.

Objectives/hypothesisTo evaluate the prevalence, characteristics, and associated risk factors of tinnitus in U.S. adolescents.Study designCross-sectional analyses of U.S. representative demographic and audiometric data, the National Health and Nutrition Examination Survey (NHANES) 2005 to 2008.MethodsThe study population consisted of 3,520 individuals aged 12 to 19 years with complete tinnitus-related data. Tinnitus was defined as the presence of ringing or buzzing in the ears lasting for at least 5 minutes during the preceding 12 months. In addition, we defined a chronic tinnitus subgroup as being bothered by tinnitus for more than 3 months. Demographic and other data regarding tinnitus, smoking, body mass index (BMI), anemia, hypertension, history of ear infections, tympanostomy tube placement, otoscopy, tympanometry and hearing thresholds, history of firearm use, and recreational and occupational exposure to noise were extracted and analyzed.ResultsOverall, tinnitus lasting 5 minutes or more in the preceding 12 months was reported by 7.5% of the 12- to 19-year-old population. This represents about 2.5 million adolescents in the United States. The prevalence of chronic tinnitus was 4.7%, corresponding to about 1.6 million adolescents in the United States. Multivariable-adjusted analysis revealed that both overall and chronic tinnitus were associated with female gender, low income, exposure to passive smoking, type A tympanogram, and occupational and recreational noise exposure. History of ≥ 3 ear infections and history of tympanostomy tube placement were associated only with overall tinnitus.ConclusionsTinnitus afflicts a substantial portion of the youth population. Further investigation of the association between tinnitus and the identified risk factors is warranted.

A Bayesian adaptive procedure for estimating the auditory-filter shape was proposed and evaluated using young, normal-hearing listeners at moderate stimulus levels. The resulting quick-auditory-filter (qAF) procedure assumed the power spectrum model of masking with the auditory-filter shape being modeled using a spectrally symmetric, two-parameter rounded-exponential (roex) function. During data collection using the qAF procedure, listeners detected the presence of a pure-tone signal presented in the spectral notch of a noise masker. Dependent on the listener's response on each trial, the posterior probability distributions of the model parameters were updated, and the resulting parameter estimates were then used to optimize the choice of stimulus parameters for the subsequent trials. Results showed that the qAF procedure gave similar parameter estimates to the traditional threshold-based procedure in many cases and was able to reasonably predict the masked signal thresholds. Additional measurements suggested that occasional failures of the qAF procedure to reliably converge could be a consequence of incorrect responses early in a qAF track. The addition of a parameter describing lapses of attention reduced the likelihood of such failures.

Sound streams were generated by randomly choosing the levels of tone pips from two different distributions, A and B. Of the 18 tone pips, the first nine were drawn from distribution A and the second nine from distribution B, or the opposite. The listeners' task was to indicate order, A-B or B-A. In two conditions the A and B distributions differed in mean (condition 1) or variance (condition 2). In contrast to an ideal observer, listeners' strategies were consistent across the two conditions. Analyses suggest that listeners relied primarily on the more intense tone pips in making their decisions.

Objectives/hypothesisTo evaluate the prevalence, characteristics, and associated risk factors of tinnitus in U.S. adolescents.Study designCross-sectional analyses of U.S. representative demographic and audiometric data, the National Health and Nutrition Examination Survey (NHANES) 2005 to 2008.MethodsThe study population consisted of 3,520 individuals aged 12 to 19 years with complete tinnitus-related data. Tinnitus was defined as the presence of ringing or buzzing in the ears lasting for at least 5 minutes during the preceding 12 months. In addition, we defined a chronic tinnitus subgroup as being bothered by tinnitus for more than 3 months. Demographic and other data regarding tinnitus, smoking, body mass index (BMI), anemia, hypertension, history of ear infections, tympanostomy tube placement, otoscopy, tympanometry and hearing thresholds, history of firearm use, and recreational and occupational exposure to noise were extracted and analyzed.ResultsOverall, tinnitus lasting 5 minutes or more in the preceding 12 months was reported by 7.5% of the 12- to 19-year-old population. This represents about 2.5 million adolescents in the United States. The prevalence of chronic tinnitus was 4.7%, corresponding to about 1.6 million adolescents in the United States. Multivariable-adjusted analysis revealed that both overall and chronic tinnitus were associated with female gender, low income, exposure to passive smoking, type A tympanogram, and occupational and recreational noise exposure. History of ≥ 3 ear infections and history of tympanostomy tube placement were associated only with overall tinnitus.ConclusionsTinnitus afflicts a substantial portion of the youth population. Further investigation of the association between tinnitus and the identified risk factors is warranted.

ObjectiveTo systematically evaluate the noise generated by toys targeted for children and to compare the results over the course of 4 consecutive holiday shopping seasons.Study designExperimental study.SettingAcademic medical center.Subjects and methodsDuring 2008-2011, more than 200 toys marketed for children older than 6 months were screened for loudness. The toys with sound output of more than 80 dBA at speaker level were retested in a soundproof audiometry booth. The generated sound amplitude of each toy was measured at speaker level and at 30 cm away from the speaker.ResultsNinety different toys were analyzed. The mean (SD) noise amplitude was 100 (8) dBA (range, 80-121 dBA) at the speaker level and 80 (11) dBA (range, 60-109 dBA) at 30 cm away from the speaker. Eighty-eight (98%) had more than an 85-dBA noise amplitude at speaker level, whereas 19 (26%) had more than an 85-dBA noise amplitude at a 30-cm distance. Only the mean noise amplitude at 30 cm significantly declined during the studied period (P < .001). There was no significant difference in mean noise amplitude of different toys specified for different age groups.ConclusionOur findings demonstrate the persistence of extremely loud toys marketed for very young children. Acoustic trauma from toys remains a potential risk factor for noise-induced hearing loss in this age group, warranting promotion of public awareness and regulatory considerations for manufacture and marketing of toys.

ObjectiveTo systematically evaluate the noise generated by toys targeted for children and to compare the results over the course of 4 consecutive holiday shopping seasons.Study designExperimental study.SettingAcademic medical center.Subjects and methodsDuring 2008-2011, more than 200 toys marketed for children older than 6 months were screened for loudness. The toys with sound output of more than 80 dBA at speaker level were retested in a soundproof audiometry booth. The generated sound amplitude of each toy was measured at speaker level and at 30 cm away from the speaker.ResultsNinety different toys were analyzed. The mean (SD) noise amplitude was 100 (8) dBA (range, 80-121 dBA) at the speaker level and 80 (11) dBA (range, 60-109 dBA) at 30 cm away from the speaker. Eighty-eight (98%) had more than an 85-dBA noise amplitude at speaker level, whereas 19 (26%) had more than an 85-dBA noise amplitude at a 30-cm distance. Only the mean noise amplitude at 30 cm significantly declined during the studied period (P < .001). There was no significant difference in mean noise amplitude of different toys specified for different age groups.ConclusionOur findings demonstrate the persistence of extremely loud toys marketed for very young children. Acoustic trauma from toys remains a potential risk factor for noise-induced hearing loss in this age group, warranting promotion of public awareness and regulatory considerations for manufacture and marketing of toys.

Cochlear Implant (CI) users typically perform poorly on musical tasks, especially those based on pitch ranking and melody recognition. It was hypothesized that CI users would demonstrate deterioration in performance for a pitch ranking and a melody recognition task presented with iterated rippled noise (IRN) in comparison to pure tones (PT). In Addition, it was hypothesized that normal hearing (NH) listeners would show fewer differences in performance between IRN and PT for these two tasks. In this study, the ability of CI users and NH subjects to rank pitches and to identify melodies created with IRN and PT was assessed in free field in a sound-isolated room. CI subjects scored significantly above chance level with PT stimuli in both tasks. With IRN stimuli their performance was around chance level. NH subjects scored significantly above chance level in both tasks and with all stimuli. NH subjects performed significantly better than CI subjects in both tasks. These results illustrate the difficulties of CI subjects to rank pitches and to identify melodies.

BACKGROUND Whether hearing loss is independently associated with accelerated cognitive decline in older adults is unknown. METHODS We studied 1984 older adults (mean age, 77.4 years) enrolled in the Health ABC Study, a prospective observational study begun in 1997-1998. Our baseline cohort consisted of participants without prevalent cognitive impairment (Modified Mini-Mental State Examination [3MS] score, ≥80) who underwent audiometric testing in year 5. Participants were followed up for 6 years. Hearing was defined at baseline using a pure-tone average of thresholds at 0.5 to 4 kHz in the better-hearing ear. Cognitive testing was performed in years 5, 8, 10, and 11 and consisted of the 3MS (measuring global function) and the Digit Symbol Substitution test (measuring executive function). Incident cognitive impairment was defined as a 3MS score of less than 80 or a decline in 3MS score of more than 5 points from baseline. Mixed-effects regression and Cox proportional hazards regression models were adjusted for demographic and cardiovascular risk factors. RESULTS In total, 1162 individuals with baseline hearing loss (pure-tone average >25 dB) had annual rates of decline in 3MS and Digit Symbol Substitution test scores that were 41% and 32% greater, respectively, than those among individuals with normal hearing. On the 3MS, the annual score changes were -0.65 (95% CI, -0.73 to -0.56) vs -0.46 (95% CI, -0.55 to -0.36) points per year (P = .004). On the Digit Symbol Substitution test, the annual score changes were -0.83 (95% CI, -0.94 to -0.73) vs -0.63 (95% CI, -0.75 to -0.51) points per year (P = .02). Compared to those with normal hearing, individuals with hearing loss at baseline had a 24% (hazard ratio, 1.24; 95% CI, 1.05-1.48) increased risk for incident cognitive impairment. Rates of cognitive decline and the risk for incident cognitive impairment were linearly associated with the severity of an individual's baseline hearing loss. CONCLUSIONS Hearing loss is independently associated with accelerated cognitive decline and incident cognitive impairment in community-dwelling older adults. Further studies are needed to investigate what the mechanistic basis of this association is and whether hearing rehabilitative interventions could affect cognitive decline.