Laboratory evaluation of an optimised internet-based speech-in-noise test for occupational high-frequency hearing loss screening: Occupational Earcheck.

OBJECTIVE: The "Occupational Earcheck" (OEC) is a Dutch online self-screening speech-in-noise test developed for the detection of occupational high-frequency hearing loss (HFHL). This study evaluates an optimised version of the test and determines the most appropriate masking noise. DESIGN: The original OEC was improved by homogenisation of the speech material, and shortening the test. A laboratory-based cross-sectional study was performed in which the optimised OEC in five alternative masking noise conditions was evaluated. STUDY SAMPLE: The study was conducted on 18 normal-hearing (NH) adults, and 15 middle-aged listeners with HFHL. RESULTS: The OEC in a low-pass (LP) filtered stationary background noise (test version LP 3: with a cut-off frequency of 1.6 kHz, and a noise floor of -12 dB) was the most accurate version tested. The test showed a reasonable sensitivity (93%), and specificity (94%) and test reliability (intra-class correlation coefficient: 0.84, mean within-subject standard deviation: 1.5 dB SNR, slope of psychometric function: 13.1%/dB SNR). CONCLUSIONS: The improved OEC, with homogenous word material in a LP filtered noise, appears to be suitable for the discrimination between younger NH listeners and older listeners with HFHL. The appropriateness of the OEC for screening purposes in an occupational setting will be studied further.

Speech perception and quality of life of open-fit hearing aid users.

OBJECTIVE: To relate the performance of individuals with hearing loss at high frequencies in speech perception with the quality of life before and after the fitting of an open-fit hearing aid (HA). METHODS: The WHOQOL-BREF had been used before the fitting and 90 days after the use of HA. The Hearing in Noise Test (HINT) had been conducted in two phases: (1) at the time of fitting without an HA (situation A) and with an HA (situation B); (2) with an HA 90 days after fitting (situation C). STUDY SAMPLE: Thirty subjects with sensorineural hearing loss at high frequencies. RESULTS: By using an analysis of variance and the Tukey's test comparing the three HINT situations in quiet and noisy environments, an improvement has been observed after the HA fitting. The results of the WHOQOL-BREF have showed an improvement in the quality of life after the HA fitting (paired t-test). The relationship between speech perception and quality of life before the HA fitting indicated a significant relationship between speech recognition in noisy environments and in the domain of social relations after the HA fitting (Pearson's correlation coefficient). CONCLUSIONS: The auditory stimulation has improved speech perception and the quality of life of individuals.

Speech perception and quality of life of open-fit hearing aid users

ABSTRACT Objective To relate the performance of individuals with hearing loss at high frequencies in speech perception with the quality of life before and after the fitting of an open-fit hearing aid (HA). Methods The WHOQOL-BREF had been used before the fitting and 90 days after the use of HA. The Hearing in Noise Test (HINT) had been conducted in two phases: (1) at the time of fitting without an HA (situation A) and with an HA (situation B); (2) with an HA 90 days after fitting (situation C). Study Sample Thirty subjects with sensorineural hearing loss at high frequencies. Results By using an analysis of variance and the Tukey’s test comparing the three HINT situations in quiet and noisy environments, an improvement has been observed after the HA fitting. The results of the WHOQOL-BREF have showed an improvement in the quality of life after the HA fitting (paired t-test). The relationship between speech perception and quality of life before the HA fitting indicated a significant relationship between speech recognition in noisy environments and in the domain of social relations after the HA fitting (Pearson’s correlation coefficient). Conclusions The auditory stimulation has improved speech perception and the quality of life of individuals.

Effects of steep high-frequency hearing loss on speech recognition using temporal fine structure in low-frequency region.

The present study examined the effects of steep high-frequency sensorineural hearing loss (SHF-SNHL) on speech recognition using acoustic temporal fine structure (TFS) in the low-frequency region where the absolute thresholds appeared to be normal. In total, 28 participants with SHF-SNHL were assigned to 3 groups according to the cut-off frequency (1, 2, and 4 kHz, respectively) of their pure-tone absolute thresholds. Fourteen age-matched normal-hearing (NH) individuals were enrolled as controls. For each Mandarin sentence, the acoustic TFS in 10 frequency bands (each 3-ERB wide) was extracted using the Hilbert transform and was further lowpass filtered at 1, 2, and 4 kHz. Speech recognition scores were compared among the NH and 1-, 2-, and 4-kHz SHF-SNHL groups using stimuli with varying bandwidths. Results showed that speech recognition with the same TFS-speech stimulus bandwidth differed significantly in groups and filtering conditions. Sentence recognition in quiet conditions was better than that in noise. Compared with the NH participants, nearly all the SHF-SNHL participants showed significantly poorer sentence recognition within their frequency regions with "normal hearing" (defined clinically by normal absolute thresholds) in both quiet and noisy conditions. These may result from disrupted auditory nerve function in the "normal hearing" low-frequency regions.

Clinical feasibility of fast psychophysical tuning curves evaluated using normally hearing adults: success rate, range of tip shift, repeatability, and comparison of methods used for estimation of frequency at the tip.

OBJECTIVE: Psychophysical tuning curves (PTCs) have been used predominantly in laboratory settings to assess frequency selectivity in the auditory system. Recently, a fast-PTC procedure has been developed for diagnosis of cochlear dead regions. In this study the clinical feasibility of using fast PTCs in adults was investigated. Success rate (the number of successes per number of attempts) and repeatability were assessed. The range of the tip frequency (ftip) shift was established and different methods of ftip estimation compared. DESIGN: Fast PTCs were measured for signal frequencies (fs) of 500, 1000, 2000, 3000, and 4000 Hz on two occasions using an upward-sweeping masker. Five methods were used to estimate ftip for each PTC. STUDY SAMPLE: Thirty-two adults with normal hearing were tested. RESULTS: All participants were able to successfully complete the task, the majority of them with minimal training. The moving average, quadratic function, and double lowpass filtering methods had the highest success rate in ftip estimation. The quadratic function method had the smallest 95% range of - 3.4% fs to 10.2% fs and the best test-retest reliability of 5.1% fs. CONCLUSIONS: Fast PTCs show potential for clinical use due to a high success rate with minimal training required. We suggest the quadratic function method for routine clinical use as it had the smallest 95% range, a high success rate in ftip estimation and the best test-retest reliability. For fast PTCs measured for signal frequencies from 500 to 4000 Hz using an upward-sweeping masker, we suggest the normative range of ftip to be - 3% fs to 10% fs with a test-retest reliability of 5% fs.

Effects of auditory training in individuals with high-frequency hearing loss.

OBJECTIVE: To determine the effects of a formal auditory training program on the behavioral, electrophysiological and subjective aspects of auditory function in individuals with bilateral high-frequency hearing loss. METHOD: A prospective study of seven individuals aged 46 to 57 years with symmetric, moderate high-frequency hearing loss ranging from 3 to 8 kHz was conducted. Evaluations of auditory processing (sound location, verbal and non-verbal sequential memory tests, the speech-in-noise test, the staggered spondaic word test, synthetic sentence identification with competitive ipsilateral and contralateral competitive messages, random gap detection and the standard duration test), auditory brainstem response and long-latency potentials and the administration of the Abbreviated Profile of Hearing Aid Benefit questionnaire were performed in a sound booth before and immediately after formal auditory training. RESULTS: All of the participants demonstrated abnormal pre-training long-latency characteristics (abnormal latency or absence of the P3 component) and these abnormal characteristics were maintained in six of the seven individuals at the post-training evaluation. No significant differences were found between ears in the quantitative analysis of auditory brainstem responses or long-latency potentials. However, the subjects demonstrated improvements on all behavioral tests. For the questionnaire, the difference on the background noise subscale achieved statistical significance. CONCLUSION: Auditory training in adults with high-frequency hearing loss led to improvements in figure-background hearing skills for verbal sounds, temporal ordination and resolution, and communication in noisy environments. Electrophysiological changes were also observed because, after the training, some long latency components that were absent pre-training were observed during the re-evaluation.

Effects of auditory training in individuals with high-frequency hearing loss

OBJECTIVE: To determine the effects of a formal auditory training program on the behavioral, electrophysiological and subjective aspects of auditory function in individuals with bilateral high-frequency hearing loss. METHOD: A prospective study of seven individuals aged 46 to 57 years with symmetric, moderate high-frequency hearing loss ranging from 3 to 8 kHz was conducted. Evaluations of auditory processing (sound location, verbal and non-verbal sequential memory tests, the speech-in-noise test, the staggered spondaic word test, synthetic sentence identification with competitive ipsilateral and contralateral competitive messages, random gap detection and the standard duration test), auditory brainstem response and long-latency potentials and the administration of the Abbreviated Profile of Hearing Aid Benefit questionnaire were performed in a sound booth before and immediately after formal auditory training. RESULTS: All of the participants demonstrated abnormal pre-training long-latency characteristics (abnormal latency or absence of the P3 component) and these abnormal characteristics were maintained in six of the seven individuals at the post-training evaluation. No significant differences were found between ears in the quantitative analysis of auditory brainstem responses or long-latency potentials. However, the subjects demonstrated improvements on ...

Exploring the limits of frequency lowering.

PURPOSE: This study examined how frequency lowering affected sentence intelligibility and quality for adults with postlingually acquired, mild-to-moderate hearing loss. METHOD: Listeners included adults aged 60-92 years with sloping sensorineural hearing loss and a control group of similarly aged adults with normal hearing. Sentences were presented in quiet and babble at a range of signal-to-noise ratios. Intelligibility and quality were measured with varying amounts of frequency lowering, implemented using a form of frequency compression. RESULTS: Moderate amounts of compression, particularly with high cutoff frequencies, had minimal effects on sentence intelligibility. Listeners with the greatest high-frequency hearing loss showed the greatest benefit. Sentence intelligibility decreased with more compression. Listeners were more affected by a given set of parameters in noise than in quiet. In quiet, any amount of compression resulted in lower speech quality for most listeners, with the greatest degradation for listeners with better high-frequency hearing. Quality ratings were lower with background noise, and in noise, the effect of changing compression parameters was small. CONCLUSIONS: The benefits of frequency lowering in adults were affected by the compression parameters as well as individual hearing thresholds. The data are consistent with the idea that frequency lowering can be viewed in terms of improved audibility versus increased distortion trade-off.

Frequency discrimination in ears with and without contralateral cochlear dead regions.

OBJECTIVE: The purpose of this study was to test the ability to discriminate low-frequency pure-tone stimuli for ears with and without contralateral dead regions, in subjects with bilateral high-frequency hearing loss; we examined associations between hearing loss characteristics and frequency discrimination of low-frequency stimuli in subjects with high-frequency hearing loss. DESIGN: Cochlear dead regions were diagnosed using the TEN-HL test. A frequency discrimination test utilizing an adaptive three-alternative forced choice method provided difference limens for reference frequencies 0.25 kHz and 0.5 kHz. STUDY SAMPLE: Among 105 subjects with bilateral high-frequency hearing loss, unilateral dead regions were found in 15 subjects. These, and an additional 15 matched control subjects without dead regions, were included in the study. RESULTS: Ears with dead regions performed best at the frequency discrimination test. Ears with a contralateral dead region performed significantly better than ears without a contralateral dead region at 0.5 kHz, the reference frequency closest to the mean audiogram cut-off, while the opposite result was obtained at 0.25 kHz. CONCLUSIONS: Results may be seen as sign of a contralateral effect of unilateral dead regions on the discrimination of stimuli with frequencies well below the audiogram cut-off in adult subjects with bilateral high-frequency hearing loss.

Electrophysiological evidence of augmented interaural asymmetry in middle-aged listeners.

BACKGROUND: Various dimensions of auditory processing, especially the perception of speech in the presence of background competition, have been shown to deteriorate with age. A persistent problem in the assessment of these age-related changes has been the high prevalence of age-related high-frequency hearing loss in elderly persons. Some investigators have suggested that a more fruitful approach to the study of age-related decline might be to study middle-aged, rather than elderly, persons, where confounding high-frequency hearing loss is less prevalent. PURPOSE: To determine whether an increase in the left-ear disadvantage (LED) in dichotic listening could be demonstrated in a group of middle-aged persons. RESEARCH DESIGN: The N400 component of the auditory event-related potential (AERP) was utilized to evaluate interaural asymmetry in a quasi-dichotic competing speech task. Electrophysiological responses were obtained on a word-pair semantic categorization task presented through a front loudspeaker while the listener ignored competing speech presented through either left (competition left [CL]) or right (competition right [CR]) loudspeakers. Study Samples: Twenty young (18-24 yr) and 20 middle-aged (44-57 yr) females with normal hearing sensitivity. DATA COLLECTION AND ANALYSIS: Individual, as well as grand-averaged, AERP waveforms and scalp topographies were analyzed for the word pairs. Peak amplitude and latency measures of the N400 component were subjected to a mixed design analysis of variance (ANOVA). RESULTS: No significant interaural asymmetry was found in the AERP waveform for the reference word condition in either age group. In response to the second word of the pair, however, middle-aged females showed significantly greater N400 negativity in the CR condition than in the CL condition. No significant laterality effect was found in the young females. CONCLUSIONS: The study of young versus middle-aged participants may be an effective way of avoiding the confound of high-frequency hearing loss in elderly persons when studying age effects on auditory processing.

High-frequency hearing impairment assessed with cochlear microphonics.

CONCLUSION: Cochlear microphonic (CM) measurements may potentially become a supplementary approach to otoacoustic emission (OAE) measurements for assessing low-frequency cochlear functions in the clinic. OBJECTIVE: The objective of this study was to investigate the measurement of CMs in subjects with high-frequency hearing loss. Currently, CMs can be measured using electrocochleography (ECochG or ECoG) techniques. Both CMs and OAEs are cochlear responses, while auditory brainstem responses (ABRs) are not. However, there are inherent limitations associated with OAE measurements such as acoustic noise, which can conceal low-frequency OAEs measured in the clinic. However, CM measurements may not have these limitations. METHODS: CMs were measured in human subjects using an ear canal electrode. The CMs were compared between the high-frequency hearing loss group and the normal-hearing control group. Distortion product OAEs (DPOAEs) and audiogram were also measured. RESULTS: The DPOAE and audiogram measurements indicate that the subjects were correctly selected for the two groups. Low-frequency CM waveforms (CMWs) can be measured using ear canal electrodes in high-frequency hearing loss subjects. The difference in amplitudes of CMWs between the high-frequency hearing loss group and the normal-hearing group is insignificant at low frequencies but significant at high frequencies.

Change in echolocation signals with hearing loss in a false killer whale (Pseudorca crassidens).

The echolocation signals of a false killer whale (Pseudorca crassidens) were collected during a wall thickness discrimination task and compared to clicks recorded during an identical experiment in 1992. During the sixteen year time period, the subject demonstrated a loss of high frequency hearing of about 70 kHz. Clicks between the two experiments were compared to investigate the effect of hearing loss on echolocation signals. There was a significant reduction in the peak frequency, center frequency and source level of clicks between the two time periods. Additionally, the subject currently produces more signals with low frequency peaks and fewer signals with high frequency peaks than she did in 1992. These results indicate the subject changed its echolocation signals to match its range of best hearing.

Functional and structural aspects of tinnitus-related enhancement and suppression of auditory cortex activity.

The steady-state auditory evoked magnetic field was recorded in tinnitus patients and controls, both either musicians or non-musicians, all of them with high-frequency hearing loss. Stimuli were AM-tones with two modulation frequencies and three carrier frequencies matching the "audiometric edge", i.e. the frequency above which hearing loss increases more rapidly, the tinnitus frequency or the frequency 1 1/2 octaves above the audiometric edge in controls, and a frequency 1 1/2 octaves below the audiometric edge. Stimuli equated in carrier frequency, but differing in modulation frequency, were simultaneously presented to the two ears. The modulation frequency-specific components of the dual steady-state response were recovered by bandpass filtering. In both hemispheres, the source amplitude of the response was larger for contralateral than ipsilateral input. In non-musicians with tinnitus, this laterality effect was enhanced in the hemisphere contralateral and reduced in the hemisphere ipsilateral to the tinnitus ear, especially for the tinnitus frequency. The hemisphere-by-input laterality dominance effect was smaller in musicians than in non-musicians. In both patient groups, source amplitude change over time, i.e. amplitude slope, was increasing with tonal frequency for contralateral input and decreasing for ipsilateral input. However, slope was smaller for musicians than non-musicians. In patients, source amplitude was negatively correlated with the MRI-determined volume of the medial partition of Heschl's gyrus. Tinnitus patients show an altered excitatory-inhibitory balance reflecting the downregulation of inhibition and resulting in a steeper dominance hierarchy among simultaneous processes in auditory cortex. Direction and extent of this alteration are modulated by musicality and auditory cortex volume.

Middle-ear function at high frequencies quantified with advanced bone-conduction measures.

Auditory thresholds with standardized clinical procedures are obtained over a much narrower frequency range by bone conduction than by air conduction. As a result, diagnostic information for both sensorineural and conductive-mechanism function is incomplete for high frequencies. A new magnetostrictive bone-conduction transducer that has the potential for improved output in the high-frequency range was evaluated in the laboratory and in a variety of subjects with normal hearing (N=11) or sensorineural hearing loss (N=9). Laboratory results indicated that harmonic distortion and acoustic radiation were both sufficiently low to allow accurate threshold measurements. Auditory thresholds obtained with this magnetostrictive bone-conduction transducer can be measured accurately under conventional clinical conditions for frequencies up to 16 kHz and levels up to 85 dB HL. These measures can be used to accurately characterize sensorineural hearing sensitivity for high frequencies and, when combined with standard air-conduction measures for high frequencies, to accurately characterize conductive-mechanism function for frequencies higher than possible with current diagnostic bone-conduction technology.

Plasticity of tonotopic maps in humans: influence of hearing loss, hearing aids and cochlear implants.

CONCLUSION: This paper reviews psychoacoustical and electrophysiological evidence for reorganization of the human central auditory system in case of auditory deprivation and rehabilitation. OBJECTIVE: To investigate the plasticity of cortical tonotopic maps in cochlear-damaged subjects. METHODS: Frequency discrimination scores were analysed in subjects with high frequency hearing loss to test for potential perceptual correlates of auditory deprivation- and rehabilitation-induced plasticity. In cochlear implant patients, electrically evoked auditory cortical responses were obtained using EEG to study scalp potential maps. RESULTS: Perceptual changes in frequency discrimination were observed at the lesion-edge frequency of steeply sloping hearing loss. Although these results are not direct proof of cortical plasticity, no peripheral phenomenon has been found to explain them. The reversal of such auditory deprivation-induced plasticity, a phenomenon that may be termed rehabilitation plasticity, can be studied in hearing-impaired subjects fitted with a hearing aid. Cochlear implant subjects provide another interesting model for studying rehabilitation plasticity in that even profound to total deafness is made partially reversible by cochlear implantation. We found that the auditory cortex of deaf subjects with at least 3 months of cochlear implant experience is organized in a way similar to the tonotopy described in normal-hearing subjects.

Hyperactive auditory efferent system and lack of acoustic reflexes in Williams syndrome.

The auditory efferent system and acoustic reflexes have been investigated in patients with Williams syndrome (WS). Twenty-one patients aged 6-26 years with a genetically confirmed diagnosis of WS and with reported hyperacusis were compared with 21 normally developing age-matched subjects. The medial olivocochlear (MOC) efferent system was tested by stimulation of the contralateral ear with increasing levels of white noise, while recording transient evoked otoacoustic emissions (TEOAE) in the ipsilateral ear. The suppression effect on the amplitudes of the TEOAE was computed for each contralateral stimulus level. This measure reflects the strength of the MOC efferent system. In addition, the thresholds of ipsilateral and contralateral acoustic reflexes in response to 1, 2 and 4 kHz tones as well as to broadband stimuli were also recorded. Results showed that patients with WS had a significantly higher suppression effect of the MOC reflex on TEOAE. Ipsilateral and contralateral acoustic reflexes to tonal and broadband stimuli presented at maximum stimulus intensities were absent in 62-86% of the patients with WS. In the remainder, acoustic reflexes were elicited at lower auditory sensation thresholds than in controls. Hyperexcitability of the MOC efferent system coupled with absence of acoustic reflexes may contribute to the hyperacusis in WS and the consequent high-tone hearing loss induced by environmental noise. Both measures can be used for objective detection and thus, intervention of hyperacusis in the early stages of life.

High-frequency amplification and sound quality in listeners with normal through moderate hearing loss.

PURPOSE: One factor that has been shown to greatly affect sound quality is audible bandwidth. Provision of gain for frequencies above 4-6 kHz has not generally been supported for groups of hearing aid wearers. The purpose of this study was to determine if preference for bandwidth extension in hearing aid processed sounds was related to the magnitude of hearing loss in individual listeners. METHOD: Ten participants with normal hearing and 20 participants with mild-to-moderate hearing loss completed the study. Signals were processed using hearing aid-style compression algorithms and filtered using two cutoff frequencies, 5.5 and 9 kHz, which were selected to represent bandwidths that are achievable in modern hearing aids. Round-robin paired comparisons based on the criteria of preferred sound quality were made for 2 different monaurally presented brief sound segments, including music and a movie. RESULTS: Results revealed that preference for either the wider or narrower bandwidth (9- or 5.5-kHz cutoff frequency, respectively) was correlated with the slope of hearing loss from 4 to 12 kHz, with steep threshold slopes associated with preference for narrower bandwidths. CONCLUSION: Consistent preference for wider bandwidth is present in some listeners with mild-to-moderate hearing loss.

Contribution of high frequencies to speech recognition in quiet and noise in listeners with varying degrees of high-frequency sensorineural hearing loss.

PURPOSE: The contribution of audible high-frequency information to speech-understanding performance in listeners with varying degrees of high-frequency sensorineural hearing loss was examined. METHOD: Thirty-six elderly hearing-impaired (EHI) and 24 young normal-hearing (YNH) listeners were tested in quiet (+20 dB speech-to-noise ratio [SNR]) and noise (+5 dB SNR) and under different bandpass conditions (narrow, 200-1600 Hz; midband, 200-3200 Hz; broadband, 200-6400 Hz), both without and with spectral shaping of the stimuli. Monosyllabic word-recognition performance was examined through use of both whole-word scoring and phoneme scoring. The 36 EHI listeners were divided into 3 groups of 12 listeners each, with the groups differing in the amount of high-frequency hearing loss. The 24 YNH participants were separated into 2 groups, each serving as a reference group for either the unshaped or spectrally shaped speech listening conditions. RESULTS: Results for spectrally shaped speech, in both quiet and noise, revealed that the 3 EHI groups performed equivalently in the different bandwidth conditions and demonstrated no change (increase or decrease) in word-recognition performance between the midband and broadband conditions. The YNH groups, however, demonstrated improved speech understanding attributable to the higher frequencies for the broadband condition in both the unshaped and shaped conditions. CONCLUSIONS: Data from the EHI listeners revealed that performance for unshaped speech was correlated moderately and negatively with degree of high-frequency hearing loss. Alternatively, recognition performance for shaped speech was related to neither the performance for unshaped speech nor the amount of high-frequency hearing loss.

Age-related differences in discrimination of an interval separating onsets of successive tone bursts as a function of interval duration.

The study measured listener sensitivity to increments in the inter-onset interval (IOI) separating pairs of successive 20-ms 4000-Hz tone pulses. A silent interval between the tone pulses was adjusted across conditions to create reference tonal IOI values of 25-600 ms. For each condition, a duration DL for increments of the tonal IOI was measured in listeners comprised of young normal-hearing adults and two groups of older adults with and without high-frequency hearing loss. Discrimination performance of all listeners was poorest for the shorter reference IOIs, and improved to stable levels for longer reference intervals exceeding about 200 ms. Temporal sensitivity of the young listeners was significantly better than that of the elderly listeners in each condition, with the largest age-related differences observed for the shortest reference interval. Age-related differences were also observed for duration DLs measured using single 4000-Hz tone bursts set to three reference durations in the range 50-200 ms. The tone DLs of all listeners were smaller than the corresponding tone-pair IOI DLs, particularly for the shorter reference stimulus durations. There were no significant performance differences observed between the older listeners with and without hearing loss for either discrimination task.

Cochlear function in facioscapulohumeral muscular dystrophy.

OBJECTIVE: Facioscapulohumeral muscular dystrophy (FSHD) is commonly associated with high-frequency hearing impairment. Our objective was to evaluate a group of normally hearing patients with FSHD using otoacoustic emissions. STUDY DESIGN: Prospective, randomized clinical trial. SETTING: A tertiary University-based referral center in Athens, Greece. PATIENTS: The study group consisted of a consecutive sample of 24 patients diagnosed on clinical, histopathologic, and genetic grounds as having FSHD. All subjects were selected on the basis of normal to near normal audiometric pure tone thresholds. Controls consisted of 40 age-matched healthy volunteers. INTERVENTIONS: Transiently evoked otoacoustic emissions were performed. Whole reproducibility and total response were measured, as well as partial scores at the octave bands centered at 1, 2, 3, 4, and 5 kHz. MAIN OUTCOME MEASURES: Transiently evoked otoacoustic emission measurements were compared between the two groups. RESULTS: The audiometric findings were normal to near normal for both groups. Compared with controls, most patients had diminished scores in both whole and partial reproducibility scores and overall and partial response scores. CONCLUSION: Despite normal hearing, subclinical involvement of the cochlea is quite common in patients with FSHD. Our findings support the genetic homogeneity of this disorder and its association with cochlear damage. Otoacoustic emissions might provide a useful tool in the clinical workup and follow-up of these patients.