Cochlear dysfunction in patients with acute hypothyroidism

The effect of acute hypothyroidism on the cochlear function was studied prospectively, in a group of 52 patients with thyroid carcinoma who underwent total thyroidectomy. All patients were examined before surgery and 6–8 weeks postoperatively. During this period there was no replacement with levothyroxine and the magnitude of thyroxin depletion was monitored by serum thyroid-stimulating hormone levels. Pure-tone audiometry, tympanometry and transiently evoked otoacoustic emissions were performed. A group of healthy volunteers of similar age and sex were used as controls. Tympanograms were normal, either on initial or on repeat testing. Audiometry showed elevation of all postoperative hearing thresholds, whereas the thresholds varied significantly across frequency. Transiently evoked otoacoustic emission testing showed response signal-to-noise ratios lower in the postoperative session (hypothyroid state) than in the preoperative session on all measured frequencies. Emission levels varied significantly across frequency, with maximum response observed at 2 kHz. Comparison of significant pure-tone and otoacoustic emission shifts for individual ears showed more ears affected in otoacoustic emission testing, indicating subclinical involvement. Comparing hearing thresholds and otoacoustic emission levels between patients and controls showed significant differences on postoperative testing. It may be thus concluded that acute hypothyroidism causes elevation of hearing thresholds in humans and to a greater degree subclinical damage of the cochlear function.     

扩展高频测听在噪声性听力损失早期诊断中的应用

目的 探讨扩展高频测听（１０－２０ｋＨｚ）在噪声性听力损失早期诊断中的作用及不同年龄,工龄,暴露噪声的强度与１０－２０ｋＨｚ听阈损失的关系。方法 应用频率范围为０．５－２０ｋＨｚ的纯音听力计对１０００名噪声下作业工人的听力进行检测,以１２０名不同年龄的健康人作为对照组,结果在９５－１１５ｄＢ（Ａ）的噪声强度下,当０．５～６ｋＨｚ的阈值未出异常时,１０－１８ｋＨｚ的阈值明显高于对照９５－１１５ｄＢ（     

Evaluation of early hearing damage in personal listening device users using extended high-frequency audiometry and otoacoustic emissions

Although sound exposure from personal listening devices (PLDs) could potentially lead to noise-induced hearing loss (NIHL), the actual hearing risk associated with the use of these devices is still unclear. In this study, early hearing effects related to PLD usage were evaluated in 35 young adult PLD users (listening for >1 h/day, at >50 % of the maximum volume setting of their devices) and their age- and sex-matched controls using a combination of conventional and extended high-frequency audiometry as well as transient-evoked otoacoustic emission (TEOAE) and distortion product of otoacoustic emission (DPOAE) measurements. The mean listening duration of the PLD users was 2.7 ± 1.0 h/day while their estimated average listening volume was 81.3 ± 9.0 dBA (free-field corrected). Typical signs of NIHL were not detected in the audiogram of PLD users and their audiometric thresholds at most of the conventional test frequencies (0.25–8 kHz) were comparable with those obtained from controls. However, compared with the controls, mean hearing thresholds of PLD users at many of the extended high-frequencies (9–16 kHz) were significantly higher. In addition, TEOAE and DPOAE amplitudes in users were reduced compared with controls. The deterioration of extended high-frequency thresholds and the decrease in DPOAE amplitudes were more evident in the users’ right ears. These results indicate the presence of an early stage of hearing damage in the PLD user group. Preventive steps should be taken as the initial hearing damage in these users could eventually progress into permanent NIHL after many years of PLD use.     

听性稳态反应和短声诱发的听性脑干反应在客观听阈评估中的作用

目的探讨听性稳态反应(auditory steady-state responses,ASSR)和短声诱发听性脑干反应(ABR)在感音神经性聋人群客观听阈评估中的作用及其在耳聋鉴定中的价值。方法对感音神经性聋组(35耳)及正常组(22耳)分别进行纯音听阈、ABR及ASSR测试,并记录0.5、1、2及4kHz ASSR反应阈(dBHL)、纯音听阈(dBHL)及ABR反应阈(dBnHL)。结果正常组在0.5、1、2、4kHz的ASSR反应阈与纯音听阈相比差异无统计学意义(P>0.05),ABR反应阈与各频率纯音听阈差异有显著统计学意义(P<0.05);耳聋组各频率ASSR反应阈与纯音听阈差异无统计学意义(P>0.05),ABR反应阈与0.5kHz纯音听阈差异有统计学意义(P<0.05),与1、2、4kHz纯音听阈差异无统计学意义(P>0.05)。结论ASSR与ABR联合测试是临床工作中用于客观听阈评估的有效方法。     

detection of early noise-induced hearing impairment in pilots

Objective To detect early signs of noise-induced hearing loss(NIHL) in military pilots without hearing complaints. Methods Pure tone audiometry and acoustic reflex thresholds were tested in 36 military pilots (72 ears) with noise exposure history but no complaints of hearing loss. Conventional test frequencies (0.25-8 kHz) and extended high frequencies (EHF, 10 and 12.5 kHz) were included in audiometry. White noise and pure tones at 0.5, 1, 2, and 4 kHz were used for acoustic reflex tests. Twenty normal hearing subjects(40 ears) with no exposure to occupational noise were used as controls. Results Pure tone thresholds at all conventional frequencies and at EHFs were elevated in the pilots, with the maximum shift at 4 kHz, compared with controls (p < 0.01 ). The pilots also showed elevated ART to white noise and decreased differentials between white noise and pure tone ARTs (p< 0.01 ). Conclusion Early signs of NIHL are present in some symptom-free military pilots. High frequency hearing threshold shift, elevated white noise ART and decreased differential between white noise and pure tone ARTs may be objective indicators of early NIHL.     

Distortion product otoacoustic emissions in normal and hearing-impaired ears

Otoacoustic emissions of distortion products (DPOAE's) were recorded in normal and hearing-impaired human ears using relatively straightforward methods. Two pure-tone stimuli at fixed frequency levels of 73 dB HL for f1 and of 67 dB HL for f2 were used. The frequencies of the two primaries were chosen so that their geometric mean represented standard audiometric frequencies. Measurements of the emission amplitudes at 2f1-f2 and the adjacent noise floor were achieved by spectral averaging. A total of 101 subjects (199 ears) were tested. Seventy-seven ears in 46 subjects had normal hearing (hearing levels less than or equal to 20 dB at standard audiometric frequencies; average hearing levels, less than or equal to 10 dB). Thirty-six ears in 25 subjects had near-normal hearing (no hearing complaints, hearing levels less than or equal to 40 dB; average hearing levels, less than or equal to 20 dB). No significant differences in mean DPOAE values were apparent between these two groups of ears. All but two of these 113 ears (98%) showed emissions at three or more of the six frequencies tested between 1 and 6 kHz. Emissions were detected in more than 75% at each frequency between 1 and 6 kHz and in more than 85% between 1 and 4 kHz. A further 86 ears in 44 subjects exhibited varying degrees of sensorineural hearing loss caused by different pathologies. In general, emission amplitudes approximated the shapes of the audiograms, and a highly significant correlation between hearing thresholds and emission amplitudes was demonstrated in the frequency range of 1 to 4 kHz.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hearing loss and middle ear involvement in patients with juvenile idiopathic arthritis

OBJECTIVE: Evaluation of the hearing status and middle ear function of patients with juvenile idiopathic arthritis. METHODS: The study group was comprised of 38 ears of 19 patients (6 males, 13 females) aged between 5 and 23 years. The control group was comprised of 30 ears of 15 healthy subjects (5 males, 10 females) aged between 5 and 22 years. All subjects were examined audiologically using tympanometry, stapedial reflex, acoustic reflex decay, pure-tone audiometry, high frequency audiometry and transient evoked otoacoustic emission tests. RESULTS: There were statistically significant (p<0.05) number of ears (32%) with abnormal tympanograms in the patient population while all tympanograms were normal, type A in the control group. Seven type As, 2 type Ad, and 3 type C tympanograms were seen in the patient population. In pure tone audiometry tests there was no subject having neither a conductive nor sensorineural hearing loss individually in both groups. But as a group, patients with juvenile idiopathic arthritis showed statistically significant elevation of air conduction thresholds at frequencies of 250, 500, 6000, 14,000 and 16,000 Hz for right ears; and at 500, 2000, 12,500 and 16,000 Hz for left ears; and larger air-bone gaps at 500 and 2000 Hz for right ears; and at 500 Hz for left ears (p<0.05). Comparison of bone conduction thresholds and otoacoustic emission tests between both groups did not reveal any statistically significant difference (p>0.05). CONCLUSION: This study suggests a dual effect of disease on both the middle and inner ear of patients with juvenile idiopathic arthritis. Presence of abnormal tympanograms together with worse air conduction thresholds at lower frequencies as well as larger air bone gaps at frequencies of 500 and 2000 Hz suggest subclinical middle ear involvement; while hearing losses at 6000 Hz and very high frequencies of 12,500, 14,000 and 16,000 Hz suggest inner involvement at an early stage.     

Changes in otoacoustic emissions and high-frequency hearing thresholds in children and adolescents

With the aim of characterizing the loss of high frequency hearing sensitivity in children, hearing thresholds and otoacoustic emissions were measured in a group of 126 normal hearing children and adolescents aged from 6 to 25 years. The subjects were divided into four 5-year age groups. Hearing thresholds over a range of 125 Hz-12.5 kHz were similar in all age groups, the average hearing threshold at 16 kHz was significantly elevated in the oldest age group. The response values of transiently evoked otoacoustic emissions (TEOAEs) significantly declined with age; the decline was negatively correlated with the hearing loss at 16 kHz. Significantly larger TEOAE responses and average distortion-product otoacoustic emission (DPOAE) values at 6.3 kHz were present in the youngest group in comparison with the other three older groups. Spontaneous otoacoustic emissions (SOAEs) were present in 70.8% of the children (in either one or both ears) with the greatest prevalence in the 11-20-year-old subjects. In the 21-25-year-old group, the hearing loss at 16 kHz was significantly smaller in ears with SOAEs than in ears without SOAEs. The results demonstrate that the increase in the high frequency hearing threshold at 16 kHz, which starts at ages over 20 years, is correlated with a decrease in the TEOAE responses at middle frequencies.     

自动数字听力计健听儿童测定初探

目的探讨8~18岁健听儿童使用自动听力计的可行性及有效性。方法采用标准听力计和自动数字听力计,在隔音室中采集30名8~18岁健听儿童0.5、1、2、4 kHz的听阈,记录两种测试所用时间。结果8~18岁健听儿童自动测听气导听阈14.21±2.97 dB HL,骨导听阈13.38±2.98 dB HL;标准听力计气导听阈12.58±2.53 dB HL,骨导听阈11.65±2.26 dB HL,两者听阈比较无统计学差异(P>0.05)。不同频率下阈值比较显示,0.5 kHz气导听阈比较无统计学差异(P>0.05),1、2、4 kHz气导听阈比较有统计学差异(P<0.05);0.5、1、2、4 kHz骨导听阈比较有统计学差异(P<0.05)。自动测听平均测听时间为13.76±3.66分,标准听力计平均测听时间为14.15±3.21分,两者比较无统计学差异(P>0.05)。结论8~18岁儿童使用自动听力计可检测出可靠有效的听力阈值。     

Influence of a 6–8 kHz audiometric notch on transient evoked otoacoustic emissions

Thirty-eight patients with known unilateral cochlear hearing loss at 6 and/or 8 kHz were examined for transient evoked otoacoustic emisssions (TEOAEs). These findings were compared with those of the contralateral “normal hearing” ear. Statistically significant lower values of echo reproducibility and amplitude were recorded in hearing-impaired ears, together with a more narrow TEOAE spectrum. In addition to these findings, a globally reduced amplitude of the cochlear response was found that was unrelated to the frequency impaired in pure-tone audiometry (6, 8, or 6–8 kHz). Since patients’ audiometric thresholds at such frequencies could influence test results, findings could possibly be due to an altered echo travelling wave across the most basal part of the cochlea or to coexisting damage in the rest of Corti’s organ that were undetectable with standard audiometry. A significant overlap was found between the results from hearing-impaired ears and those from normally hearing ones. Although TEOAEs were not helpful in the present study in identifying patients with a unilateral hearing loss at 6 and/ or 8 kHz when compared to normal contralateral ears, they are still considered to play an important role in the follow-up of subjects at risk for hearing damage. Received: 22 December 1997 / Accepted: 14 May 1998     

Otoacoustic emissions recorded in people with chronic migraine

Migraine, a moderate to severe chronic headache occurring on one or both sides, is a common disease affects young people. Although hearing loss in subjects with migraine is not rare, the correlation of migraine with hearing loss is not clear. In this study, we examined hearing loss in young migraine subjects to determine if migraine may be a factor in causing cochlear dysfunction. Seven college students with migraine and three age matched subjects without history of migraine were assessed using extended high frequency audiometry and distortion product otoacoustic emissions (DPOAEs). There was no significant difference in regular audiometry threshold between the migraine group and the control group. However, high frequency audiometry (9–16 kHz) showed thresholds at 25 dB nHL or higher in six out of twenty ears in the migraine group. The amplitude of DPOAEs were reduced for more than 10 dB in the migraine group in comparison with the control group. These data suggest that migraine may affect cochlear dysfunction evidenced by the reduced amplitude of DPOAE and high frequency pure-tone audiometry.     

Low- and high-frequency hearing in patients with Menière's disease

Summary We determined the pure-tone hearing thresholds of 25 patients with unilateral active Menière's disease using standard clinical audiometry, high-frequency audiometry (10–18 kHz), and low-frequency audiometry (20–80 Hz). In addition to hearing loss at the conventional frequencies, all of the affected ears showed deterioration of the thresholds at both low- and high-frequency areas as compared with the reference ears. The degree of hearing loss at these frequencies correlated to the degree of the hearing loss at the conventional frequencies, where it was greatest at 0.5–1 kHz. Our findings suggest that in Menière's disease the whole cochlear sensory area is affected, and that the damage caused by Menière's disease advances simultaneously in all parts of the cochlea. Offprint requests to: T. Rahko     

Auditory steady-state responses for estimating moderate hearing loss

The auditory steady-state response (ASSR) has gained popularity as an alternative technique for objective audiometry but its use in less severe degrees of hearing loss has been questioned. The aim of this study was to investigate the usefulness of the ASSR in estimating moderate degrees of hearing loss. Seven subjects (12 ears) with moderate sensorineural hearing loss between 15 and 18 years of age were enrolled in the study. Forty-eight behavioural and ASSR thresholds were obtained across the frequencies of 0.5, 1, 2, and 4 kHz. ASSR thresholds were determined using a dichotic multiple frequency recording technique. Mean threshold differences varied between 2 and 8 dB (±7–10 dB SD) across frequencies. The highest difference and variability was recorded at 0.5 kHz. The frequencies 1–4 kHz also revealed significantly better correlations (0.74–0.88) compared to 0.5 kHz (0.31). Comparing correlation coefficients for behavioural thresholds less than 60 and 60 dB and higher revealed a significant difference. Eighty-six percent of ASSR thresholds corresponded within 5 dB of moderate to severe behavioural thresholds compared to only 29% for mild to moderate thresholds in this study. The results confirm that the ASSR can reliably estimate behavioural thresholds of 60 dB and higher, but due to increased variability, caution is recommended when estimating behavioural thresholds of less than 60 dB, especially at 0.5 kHz.     

The clinical use of oto-acoustic emissions of cochlear distortion products

Otoacoustic emissions of cochlear distortion products (DPOAEs) were measured in normally hearing and hearing-impaired human ears. A total of 133 subjects (231 ears) were tested. Two puretone stimuli f1 and f2 were delivered to a sound probe fixed in the outer ear canal. The frequencies of the two primaries were chosen so that their geometric mean represented pure-tone audiometric frequencies. The otoacoustic emission was measured at the distortion product frequency 2f1-f2 by spectral averaging. For 199 ears, the levels of the primaries were 73 dBHL for L1 and 67 dBHL for L2. Statistical analysis was carried out in 77 ears of 46 subjects with normal hearing (average hearing levels at pure-tone audiometric thresholds less than or equal to 10 dBHL) and 36 ears of 25 subjects exhibiting near-normal hearing (average hearing levels at pure-tone audiometric thresholds less than or equal to 20 dBHL). The mean DPOAE amplitudes were similar in these two groups of ears. In 111 of these 113 ears (98.2%), DPOAEs were detected at three or more of the six tested frequencies between 1 and 6 kHz. DPOAEs were measured in more than 75% of ears at each frequency between 1-6 kHz and in more than 86% between 1-4 kHz. Eighty-six hearing-impaired ears of 44 subjects with sensorineural hearing loss formed the patient group. A highly significant correlation between pure-tone audiometric thresholds and DPOAE amplitudes was demonstrated in the frequency range of 1-4 kHz. Percentiles of DPOAE amplitudes were calculated in 22 ears with a mean pure-tone threshold less than or equal to 5 dBHL and in 12 specially selected pathological ears.(ABSTRACT TRUNCATED AT 250 WORDS)     

Evidence of subtle auditory deficit in a group of patients recovered from bacterial meningitis.

OBJECTIVES: Sensorineural hearing loss of greater than 30 dB hearing loss occurs in up to 30% of patients after acute bacterial meningitis. This study investigated whether postbacterial meningitic patients with no apparent clinical sensorineural hearing loss had any evidence of more subtle subclinical cochlear deficit. DESIGN: Prospective case-controlled clinical trial. SETTING: Departments of Otolaryngology in Leicester and Nottingham, England, UK. PATIENTS: Fifty-eight controls and 20 postbacterial meningitic patients aged between 18 and 38 years were screened by a questionnaire and tympanometry to exclude hearing loss attributable to other causes. All participants fell below the 90th percentile pure-tone audiometry threshold of the Lutman and Davis UK data sets. MAIN OUTCOME MEASURES: In both ears, standard (0.25-8 kHz) pure-tone audiometry, high-frequency pure-tone audiometry (10-16 kHz), and distortion product otoacoustic emissions at 2, 4, and 6 kHz were measured. RESULTS: Mean thresholds over the range of standard pure-tone audiometry (analyzed independently) for the postbacterial meningitic patients were significantly elevated at most frequencies (p < 0.05-p < 0.001) between 4 and 7 dB in both ears above control group values. There was no evidence of significant high-frequency threshold elevation (10-16 kHz). The mean iso-distortion product values at 2, 4, and 6 kHz were elevated in both ears in the meningitis group; significantly so (p < 0.05-p < 0.01) at all three frequencies in the right ear and at 4 kHz in the left. CONCLUSIONS: Postbacterial meningitic patients with hearing below the 90th percentile range had a slight but significant subclinical threshold elevation over the standard pure-tone audiometry. This may reflect a real effect of the infection at the level of the cochlea, or it may be attributable to a mild residual cognitive defect. The moderate increases in iso-distortion product values are more likely to be real and reflect an effect on outer hair cell function in response to lower stimulus intensities.     

感音神经性聋儿童听性稳态反应与行为听阈相关性分析

目的：分析感音神经性聋儿童听性稳态反应（auditory steady－state response,ASSR）与行为听阈的相关性。方法63例（126耳）重度或极重度感音神经性听力损失儿童,年龄0．5～6岁,按年龄分为 A 组（0．5～1．0岁,21例）,B 组（1．1～3．0岁,21例）,C 组（3．1～6．0岁,21例）;分别进行 ASSR 及小儿行为听阈测试,A 组采用行为观察测听（BOA）,B 组采用视觉强化测听（VRA）,C 组采用游戏测听（PA）,采用 SPSS17．0统计软件分析二种检查结果的相关性。结果①ASSR 反应阈与行为听阈在0．5、1、2和4 kHz 处的相关系数分别为0．75、0．76、0．76、0．83,呈极显著相关（P ＜0．01）。②小儿行为听阈值通常比 ASSR 反应阈值低,随着频率的增高,两者间差距缩小。③A 组（相关系数0．70）各频率 ABR 反应阈与行为听阈相关性均较 B（相关系数0．78）、C（相关系数0．83）组低。结论0．5～6．0岁感音神经性聋儿童 ASSR 与行为听阈值均有良好的相关性,ASSR 可预测感音神经性聋儿言语频率的客观听阈,为低龄儿童及难以检测行为听力的患儿验配助听器提供参考。     

Audiometric predictions using stimulus-frequency otoacoustic emissions and middle ear measurements

OBJECTIVE: The goals of the study are to determine how well stimulus-frequency otoacoustic emissions (SFOAEs) identify hearing loss, classify hearing loss as mild or moderate-severe, and correlate with pure-tone thresholds in a population of adults with normal middle ear function. Other goals are to determine if middle ear function as assessed by wideband acoustic transfer function (ATF) measurements in the ear canal account for the variability in normal thresholds, and if the inclusion of ATFs improves the ability of SFOAEs to identify hearing loss and predict pure-tone thresholds. DESIGN: The total suppressed SFOAE signal and its corresponding noise were recorded in 85 ears (22 normal ears and 63 ears with sensorineural hearing loss) at octave frequencies from 0.5 to 8 kHz, using a nonlinear residual method. SFOAEs were recorded a second time in three impaired ears to assess repeatability. Ambient-pressure ATFs were obtained in all but one of these 85 ears and were also obtained from an additional 31 normal-hearing subjects in whom SFOAE data were not obtained. Pure-tone air and bone conduction thresholds and 226-Hz tympanograms were obtained on all subjects. Normal tympanometry and the absence of air-bone gaps were used to screen subjects for normal middle ear function. Clinical decision theory was used to assess the performance of SFOAE and ATF predictors in classifying ears as normal or impaired, and linear regression analysis was used to test the ability of SFOAE and ATF variables to predict the air conduction audiogram. RESULTS: The ability of SFOAEs to classify ears as normal or hearing impaired was significant at all test frequencies. The ability of SFOAEs to classify impaired ears as either mild or moderate-severe was significant at test frequencies from 0.5 to 4 kHz. SFOAEs were present in cases of severe hearing loss. SFOAEs were also significantly correlated with air conduction thresholds from 0.5 to 8 kHz. The best performance occurred with the use of the SFOAE signal-to-noise ratio as the predictor, and the overall best performance was at 2 kHz. The SFOAE signal-to-noise measures were repeatable to within 3.5 dB in impaired ears. The ATF measures explained up to 25% of the variance in the normal audiogram; however, ATF measures did not improve SFOAEs predictors of hearing loss except at 4 kHz. CONCLUSIONS: In common with other OAE types, SFOAEs are capable of identifying the presence of hearing loss. In particular, SFOAEs performed better than distortion-product and click-evoked OAEs in predicting auditory status at 0.5 kHz; SFOAE performance was similar to that of other OAE types at higher frequencies except for a slight performance reduction at 4 kHz. Because SFOAEs were detected in ears with mild to severe cases of hearing loss, they may also provide an estimate of the classification of hearing loss. Although SFOAEs were significantly correlated with hearing threshold, they do not appear to have clinical utility in predicting a specific behavioral threshold. Information on middle ear status as assessed by ATF measures offered minimal improvement in SFOAE predictions of auditory status in a population of normal and impaired ears with normal middle ear function. However, ATF variables did explain a significant fraction of the variability in the audiograms of normal ears, suggesting that audiometric thresholds in normal ears are partially constrained by middle ear function as assessed by ATF tests.     

Hearing deterioration in patients with a non-growing vestibular schwannoma

OBJECTIVE: The aim of this study was to document and analyse the course of several audiometric parameters in 49 patients with a non-growing unilateral vestibular schwannoma (VS). MATERIAL AND METHODS: Patients received conservative management and absence of tumour growth was ascertained by means of serial magnetic imaging studies. Pure-tone audiometry and speech audiometry were performed at yearly intervals. RESULTS: Pure-tone audiometry revealed a significant increase in thresholds at all frequencies, except for 8.0 kHz. The maximum yearly threshold increase was 2.4 dB hearing level at 1.0 and 2.0 kHz. Speech audiometry revealed a significant decrease in maximum discrimination over the course of time. No significant changes were observed in the following parameters: the intensity level at which maximum discrimination was achieved; the roll-over index; the speech reception threshold; and the slope of the curve in the speech audiogram. No change was observed in the relation between pure-tone audiometry thresholds and speech audiometry scores. Apparently, the deterioration of pure-tone perception and speech discrimination ran parallel courses. CONCLUSIONS: The results of this study indicate that hearing loss is a predominant symptom in patients with a non-growing VS, as is also known in patients with a growing lesion. Moreover, it seems unlikely that the hearing loss in VS patients is merely the result of mechanical influences on retrocochlear neural or vascular structures.     

Auditory steady-state responses for children with severe to profound hearing loss

OBJECTIVE: To investigate the clinical usefulness of the dichotic single-frequency auditory steady-state response (ASSR) for estimation of behavioral thresholds in children with severe to profound congenital sensorineural hearing loss. DESIGN: A comparative experimental research design was selected to compare behavioral and ASSR thresholds for the sample. Behavioral pure-tone audiometry served as the criterion standard. SETTING: Hearing Clinic, Department of Communication Pathology, University of Pretoria, Pretoria, South Africa. PATIENTS: A referred sample of 10 patients (20 ears), 5 girls and 5 boys aged 10 to 15 years (mean age, 13 years 4 months), with severe to profound sensorineural hearing impairment. MAIN OUTCOME MEASURES: The difference, and correlation, between 160 pure-tone behavioral and ASSR thresholds at 0.5, 1, 2, and 4 kHz. RESULTS: Mean differences between ASSR and behavioral thresholds were 6 dB for 0.5 kHz and 4 dB for 1, 2, and 4 kHz, with standard deviations varying between 8 and 12 dB. No significant differences (P<.05) were observed between ASSR and behavioral thresholds, except at 0.5 kHz, and Pearson correlation coefficients varied between 0.58 and 0.74 across the evaluated frequencies, with best correlation at 1 kHz and worst at 0.5 kHz. CONCLUSIONS: The ASSR thresholds provided reliable estimations of behavioral thresholds for children with severe to profound hearing loss and indicated an increased sensitivity for more profound hearing loss.