Test accuracy and prognostic validity of multiple auditory steady state responses for targeted hearing screening

The test accuracy and prognostic validity of Multiple Auditory Steady State Responses (MSSR) and click Auditory Brainstem Responses (cABR) was compared within the context of a targeted screening protocol. A sample of 508 high-risk babies was first screened using cABR and MSSR (500 and 2000?Hz). All children (failed/pass) were called back at three to four years of age to determine their hearing status (pure-tone audiometry). Although both methods showed an equally good test performance in the first screen (sensitivity: 100% and specificity: 92–95%), the MSSR may have some potential advantage to identify low-frequency hearing loss. Furthermore, the confirmatory audiometry with MSSR predicted the child hearing status more accurately than the cABR. In conclusion, the MSSR can provide valuable information for the diagnosis and management of infants earlier detected by a screening protocol and further developed might be also useful as a screening test.

Sumario

Se comparó la eficiencia diagnóstica y el valor pronóstico de los potenciales evocados auditivos de estado estable a múltiples frecuencias (MSSR, siglas en inglés) con los potenciales evocados auditivos de tallo cerebral con clicks (cABR, siglas en inglés) en el contexto de un programa de tamizaje auditivo de niños con factores de riesgo. Inicialmente se realizó un tamizaje con cABR y MSSR (0.5 y 2 kHz) a una muestra de 508 bebés de alto riesgo. Todos los niños (los que pasaron y los que fallaron) se reevaluaron a los 3 o 4 años de edad para determinar su estado auditivo (audiometría tonal). Aunque los dos métodos mostraron igualmente buen desempeño en el primer tamizaje (sensibilidad: 100% y especificidad: 92–95%) los MSSR pueden tener una ventaja potencial para identificar hipoacusias en tonos graves. Además la prueba audiométrica confirmatoria con MSSR predijo el status auditivo del niño con mas precisión que los cABR. En conclusión, los MSSR pueden proporcionar información valiosa para el diagnóstico y tratamiento de niños previamente detectados por un programa de tamizaje y en un futuro pueden ser también útiles como prueba de tamizaje.     

Epidemiology of hearing impairment at three Flemish Institutes for Deaf and Speech Defective Children

A retrospective analysis of 190 records of hearing impaired children up to the age of 14, all educated at one of three Flemish Institutes for Deaf and Speech Defective Children in Belgium, was performed. Until 1999, the well-known behavioral test (Ewing test) was used in the Flemish national screening program for hearing losses. Because it presented a lot of disadvantages, it has been replaced by Automated Auditory Brainstem Responses (AABR), enabling the Flemish national neonatal screening program to commence. This study is an extension of the preliminary results of a recently performed retrospective analysis in one Flemish institute of the hearing loss of patients that were diagnosed in the pre-AABR era. The authors analyzed the following data: etiology, risk factors of congenital hearing impairment, the patient's history from the moment of the first suspicion to diagnosis and treatment. The median age of the children was 8.5 years (2-14 year). In 66.5% the parents and/or grandparents were the first to raise suspicion, this was at a median age of approximately 9 months. The diagnosis was often made late, at a median age of 15 months (0-88 months). The etiology was unknown in 32.6% of the cases. The only cast-iron certain diagnoses were pre- and perinatal infections, syndromal and genetic hearing loss, and acquired infections (meningitis and measles). No risk factors of hearing loss, as they are stated by the Joint Committee on Infant Hearing, were found in more than 50% of the cases. Audiometry was performed in all cases, often supplemented with Auditory Brainstem Responses and/or click-evoked otoacoustic emissions, while other diagnostic investigations (imaging, genetics, etc.) were only variably performed. Finally, the authors confirm the need for universal neonatal screening, which only recently started in Belgium, and suggest that a detailed protocol should be established to pursue a coherent diagnostic policy.     

A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: results of visual reinforcement audiometry

PURPOSE: This 3rd of 4 articles on a study of the efficacy of the 2-stage otoacoustic emission/automated auditory brainstem response (OAE/A-ABR) newborn hearing screening protocol describes (a) the behavioral audiometric protocol used to validate hearing status at 8-12 months of age, (b) the hearing status of the sample, and (c) the success of the visual reinforcement audiometry (VRA) protocol across 7 sites. METHOD: A total of 973 infants who failed OAE but passed A-ABR, in one or both ears, during newborn screening were tested with a VRA protocol, supplemented by tympanometry and OAE screening at age 8-12 months. RESULTS: VRA audiograms (1.0, 2.0, and 4.0 kHz) were obtained for 1,184 (82.7%) of the 1,432 study ears. Hearing loss was ruled out in another 100 ears by VRA in combination with OAE, for a total of 88.7% of the study sample. Permanent hearing loss was identified in 30 ears of 21 infants. Sites differed in their success with the VRA protocol. CONCLUSIONS: Continued monitoring of hearing beyond the newborn period is an important component of early detection of hearing loss. Using a structured protocol, VRA is an appropriate test method for most, but not all, infants. A battery of test procedures is often needed to adequately delineate hearing loss in infants. Examiner experience appears to be a factor in successful VRA.     

MB11 BERAphone and auditory brainstem response in newborns at audiologic risk: comparison of results

OBJECTIVE: To compare the diagnostic effectiveness of Automated Auditory Brainstem Responses (MB11 technique) with that of standard Auditory Brainstem Responses (ABR) in a sample of newborns evaluated after the second month of life. METHODS: From October 2002 to February 2005, audiologic evaluations were performed in full-term newborns who presented altered otoacoustic emissions and in newborns considered at audiologic risk admitted to the ENT (Ear Nose Throat) Unit of Giannina Gaslini Institute, Genoa, Italy. Our sample included 201 children (104 males and 97 females) who underwent on the same day an audiologic test using MB11 BERAphone Maico and standard ABR test. RESULTS: Out of the 388 ears examined, 378 (97.4%) showed agreement between the two techniques, whereas in 10 (2.6%) there was no agreement. Interobserver agreement was excellent (kappa=0.92+/-0.02 S.E., p=0.0001). The MB11 test yielded no false negatives and 10 false positives which resulted normal at ABR. The MB11 test showed very good specificity 96.8% (95% CI 94.8-98.7%) and sensitivity 100% (95% CI 93.9-100%), positive predictive value 88.2% (95% CI 79-93.9%) and negative predictive value 100% (95% CI 98.4-100%) for diagnosis of hearing loss. CONCLUSIONS: The results obtained confirm the absolute validity of MB11 screening test in subjects at audiologic risk. Furthermore, the test can be used to esclude normal hearing subjects (threshold 40 dB HL) and to refer hearing loss subjects to subsequent ABR for diagnosis.     

A test protocol for assessing the hearing status of students with special needs

Objectives

Individuals with disabilities are often reported to have a high prevalence of undetected hearing disorders/loss, but there is no standardized hearing test protocol for this population. The purposes of this study were (1) to examine the hearing status of students with special needs in Taiwan, and (2) to investigate the use of an on-site hearing test protocol that would adequately detect hearing problems in this population and reduce unnecessary referrals for off-site follow-up services.

Methods

A total of 238 students enrolled in two schools for special education and one habilitation center participated in the study. Most students had intellectual disabilities and some also had additional syndromes or disorders. A hearing screening protocol including otoscopy, tympanometry, and distortion product otoacoustic emissions was administered to examine students’ outer, middle, and inner ear functions, respectively. Pure tone tests were then administered as an on-site follow-up for those who failed or could not be tested using the screening protocol.

Results

Only 32.4% of students passed. When administered alone, the referral rate of otoscopy, tympanometry, and otoacoustic emissions were 38.7%, 46.0%, and 48.5%, respectively. The integration of these subtests revealed 52.1% of students needed follow-up services, 11.8% could not be tested, 2.5% had documented hearing loss, and 1.3% needed to be monitored because of negative middle ear pressure. The inclusion of pure tone audiometry increased the passing rate by 9.9% and provided information on hearing sensitivity for an additional 8.6% of students.

Conclusion

Hearing assessments and regular hearing screening should be provided as an integral part of health care services for individuals with special needs because of high occurrences of excessive cerumen, middle ear dysfunction, and sensorineural hearing loss. The training of care-givers and teachers of students with special needs is encouraged so that they can help identify hearing problems and reduce the negative impact of hearing disorders and hearing loss. The screening protocol needs to include subtests that examine the status of different parts of their auditory system. The addition of pure tone audiometry as an on-site follow-up tool reduced the rate of off-site referrals and provided more information on hearing sensitivity.     

Hearing screening for Japanese children and young adults using the automated auditory brainstem response

Objective

Hearing screening programs in schools are particularly important for children because they can enable early detection of hearing problems and early intervention. However, there has been little research on this topic. The MB11BERAphone^® is a novel, accurate and efficient Automated Auditory Brainstem Response device for hearing screening in infants. The aim of this study was to investigate the validity of the MB11BERAphone^® as a hearing screening device for pre-school, school-age and young-adult individuals.

Methods

Between January 2010 and March 2012, 163 normal and hearing impaired individuals, corresponding to 321 ears from subjects aged 3–22 years 11 months at Luther Aiji Kindergarten and Nakadori General Hospital in Akita, Japan, underwent primarily conditional play audiometry or conventional audiometry followed by the Automated Auditory Brainstem Response by MB11 BERAphone^®. The statistical analysis was performed with the Predictive Analytics Software (PASW Statistics 18) and presented as the mean, standard deviation (SD) and frequency distribution. The sensitivity, specificity and false-positive and false-negative rates were estimated to analyze the validity of the MB11 BERAphone^® test over audiometry.

Results

Among the normal and hearing impaired ears, 140 were scored as “REFER” and 181 were scored as “PASS” during the hearing screening examination conducted using the MB11BERAphone^® device. The specificity was 95.1%, and the sensitivity was 96.3%. The false positive rate was 5%, and the false negative rate was 4%. The overall timing without precise measurement was less than 7 min. The individuals were divided into groups: pre-schoolers (3–5 years), school-age (6–17 years) and young-adults (18–22 years). When the audiometry and MB11BERAphone^® results were compared, no statistically significant differences (p = ns) were detected among general (pre-schoolers + school-age + young-adult), pre-school, school-age, and young-adult groups.

Conclusion

The results suggest that the MB11BERAphone^® is not only useful for newborn hearing screening but also for hearing screening in older individuals, due to the low cost of the integrated electrodes and the speed with which the examination can be performed.     

Transient evoked otoacoustic emissions in hearing screening programs: protocol for developing countries

OBJECTIVES: To formulate a protocol for infant hearing screening in developing countries enabling it to be later incorporated into their national deafness screening programs. The screening tool should be sensitive in detecting hearing loss in infants with high specificity. METHODS: 2659 infants in the age range of 0-3 months who reported to the Department of Otolaryngology were included in the study. As 537 children were lost to follow up after the first screening, the remaining 2122 infants only were considered for the statistical analysis. These were divided into 3 groups with age range between 0-1, 1-2 and 2-3 months of age. All were subjected to transient evoked otoacoustic emission (TEOAE) for hearing screening. Those who failed first screening were followed up after 1-month. Pass rate for TEOAE was calculated for each. Infants who had failed the second screening underwent Brainstem Evoked Response Audiometry (BERA). The data collected was statistically analyzed. RESULTS: 77.5% of infants in 0-1-month age group passed the screening test whereas 83.4% and 92.8% of infants passed the screening test in 1-2 months and 2-3 month age groups, respectively. On the first follow up, the pass percentage of the infants who had failed screening earlier rose significantly high up to age of 3 months. Those who had failed the follow up were scheduled for Brainstem Evoked Auditory testing. CONCLUSION: The concept of this delayed hearing screening at 3 months of age would considerably decrease the number of false positive cases undergoing unnecessary investigations and wastage of resources making the universal neonatal hearing screening within 48 h of life impractical for developing countries. Combining this delayed hearing screening with the 3rd dose of universal immunization program would constitute a viable, feasible and universal hearing screening program, which can be drafted into national deafness programs of the developing countries.     

新生儿普遍听力筛查假阴性分析

目的 探讨耳声发射作为新生儿普遍听力筛查方法的可靠性及出现假阴性的原因，说明对高危儿进行听力监测的重要性。方法 收集2002年1月～2005年12月参加上海市新生儿听力筛查，并在上海儿童医学中心听力障碍诊治中心确诊为听力障碍者的资料，报道分析5例通过新生儿听力筛查、而在6～30月龄期间在该中心诊断为听力障碍者的病史、临床表现、听力学及影像学检查的结果。结果 通过新生儿听力筛查但被确诊为听力障碍者共5例，2例确诊为中重度感音神经性聋，3例确诊为极重度感音神经性聋，其中1例确诊为听神经病。耳声发射作为新生儿听力筛查方法，灵敏度是99．88％，假阴性率是0．12％。结论 耳声发射是灵敏度较高的新生儿听力筛查方法，但是有一定的假阴性率，对于各种原因造成的蜗后听觉通路病变所致的耳聋可能会漏诊。另外，对新生儿听力筛查阴性者，要警惕遗传性聋和迟发性聋的发生，尤其对高危儿应该定期随访。     

A comparison of audiometric and objective methods in hearing screening of school children. A preliminary study

Objective

In newborn hearing screening, one exclusively applies objective hearing testing methods - based on evoked potentials and/or on otoacoustic emissions. However, when testing school children, one can consider both audiometric and electrophysiological methods. The choice of methods is determined by the aims of the program. If one wants to detect conductive hearing losses, impedance audiometry seems to be the method of choice.

Methods

The aim of this study was to compare test performance measures from audiometric and objective methods (OAEs and impedance audiometry), in the hearing screening of school children. Screening protocols were applied on a group of 190 children of about 12 years of age (6th grade of primary school).

Results

For a single application of a screening procedure, the best performance was observed in the automated four-tone audiometry, followed by the tympanometry and the TEOAE-based procedures. Screening performance was enhanced using a combination of automated and impedance audiometry. A four-tone audiometry test combined with tympanometry gives a sensitivity of 65%, and the PPV of 46%, which are reasonable values, acceptable for practical use. The use of a TEOAE protocol degrades the overall performance of screening.

Conclusions

Screening of school children is feasible with a combination of automated audiometry and tympanometry with time requirements equal to 3 min per subject.     

Effect of hearing aids use on speech stimulus decoding through speech-evoked ABR

Introduction

The electrophysiological responses obtained with the complex auditory brainstem response (cABR) provide objective measures of subcortical processing of speech and other complex stimuli. The cABR has also been used to verify the plasticity in the auditory pathway in the subcortical regions.

Hearing in the elderly: a population study.

An epidemiological study comparing speech audiometry with self-assessed hearing disability and an analysis of other factors influencing the quality of life was conducted. In the Veneto region (Italy), a representative sample of 2700 independently living individuals of 65 years of age and older was selected for the study. All participants were administered a comprehensive questionnaire and a brief examination at their home, including a general physical examination, speech audiometry, Sanders' Speech Disability test, part I and III, Mini Mental State Examination, CES-D scale for depression, visual acuity, self-reported diseases and physical function. Auditory function was worst in the older individuals: auditory performance was within acceptable limits up to the 75-79 age group, while it rapidly deteriorates in the older groups. This trend is consistent with self-reported auditory disability (Sanders' test). A detailed analysis of the type of errors made in the speech audiometry was conducted for each subject. Speech audiometry is a good indicator of real hearing difficulties faced by the elderly, and it might be preferred to pure-tone audiometry, since hearing deficits with age are not always limited to an increased detection threshold, but include other aspects of hearing such as distortion of sounds, comprehension of speech and noise discrimination.     

Some thoughts on communication handicap and hearing impairment

An epidemiological study comparing speech audiometry with self-assessed hearing disability and an analysis of other factors influencing the quality of life was conducted. In the Veneto region (Italy), a representative sample of 2700 independently living individuals of 65 years of age and older was selected for the study. All participants were administered a comprehensive questionnaire and a brief examination at their home, including a general physical examination, speech audiometry, Sanders' Speech Disability test, part I and 111, Mini Mental State Examination, CES-D scale for depression, visual acuity, self-reported diseases and physical function. Auditory function was worst in the older individuals: auditory performance was within acceptable limits up to the 75–79 age group, while it rapidly deteriorates in the older groups. This trend is consistent with self-reported auditory disability (Sanders' test). A detailed analysis of the type of errors made in the speech audiometry was conducted for each subject. Speech audiometry is a good indicator of real hearing difficulties faced by the elderly, and it might be preferred to pure-tone audiometry, since hearing deficits with age are not always limited to an increased detection threshold, but include other aspects of hearing such as distortion of sounds, comprehension of speech and noise discrimination.     

The low and high frequency auditory steady state responses mature at different rates.

The age-related changes in the fast rate (70-110 Hz) auditory steady state response elicited by multiple-frequency tones (MSSR) that were amplitude-modulated (AM) are reported here. The MSSR was recorded in a sample of 64 well babies distributed into three age groups: 0-29 days (n = 25); 1-6 months (n = 26); 7-12 months (n = 13). Four simultaneously presented AM tones (0.5, 1, 2 and 4 kHz) were delivered monaurally through TDH 49 earphones, at different intensities (between 90 and 30 dB SPL). Clear developmental changes were found between birth and 12 months of age in response threshold, amplitude and detectability. Statistical analysis revealed that these changes occurred at different rates for low- and high- frequency responses. Nonetheless adult-like hearing thresholds were estimated reasonably well in most newborns and well babies, for all frequencies tested. Therefore the MSSR technique could be useful for objective frequency-specific audiometry beginning at birth.     

Bilateral sensorineural hearing disorders in children: etiology of deafness and evaluation of hearing tests

OBJECTIVE: The purpose of this study was to determine the etiology of bilateral sensorineural hearing disorders in children and to evaluate the performed hearing tests by comparison of the results of the objective and subjective tests. METHODS: The medical history and the hearing tests (behavioral observation audiometry, acoustic evoked potentials and pure tone audiometry) of 106 bilaterally hearing impaired children were analyzed in a retrospective follow-up study. RESULTS: The total group included 52 males and 54 females. The ages at first diagnosis ranged from 4 months to 11 years with a mean age of 42 months and a median of 33 months. The degree of hearing loss for the better hearing ear was mild in one child, moderate in 28 children, severe in 29 children, profound in 32 children and total in 16 children. The delay between the first examination and diagnosis ranged from 0 to 597 days with a mean of 83 days and a median of 28 days. In 47 children (44%) no cause of hearing impairment could be determined. Nineteen children (18%) had a history of familial hearing loss, 40 (38%) suffered from acquired hearing loss (seven children had prenatal causes, 21 perinatal and 12 postnatal). A comparison between behavioral observation audiometry and brainstem evoked response audiometry revealed a statistically good agreement. Twenty-nine children (32%) showed progressive hearing loss, which was defined as a threshold shift of +10 dB or more in the pure tone average in at least one ear. CONCLUSIONS: In a significant number of children with early hearing impairments the etiology still remains uncertain. Further research in the field of genetic disorders will diminish this number. Evaluation of hearing tests showed that behavioral observation audiometry still is an excellent tool in the hands of an experienced examiner. The age at identification of hearing disorders in industrialized countries still is unacceptably high. To obtain ideal care of hearing impaired children, universal neonatal hearing screening programs are mandatory.     

Früherkennung kindlicher Hörschäden Visuelle und automatische Verfahren im Vergleich

The recording of otoacoustic emissions is suitable for the detection of hearing loss in small children. The test meets the following requirements for hearing screening: it is carried out in a few minutes, specialized personnel is not necessary, the results do not depend on the vigilance of the child, and total costs are comparably low. However, the choice of a suitable device is quite essential. A test of the ILO88, ILO92, Echosensor (Otodynamics Ltd.), and Echoscreen (Madsen) devices was performed with 102 children (aged < 1 year). Additionally, a software package (Otoclass) for offline analysis of ILO88 data files was tested. The results indicate that all devices applying OAE as a screening measure was able to detect every ear (n = 25) with a hearing loss indicated by the outcome of a control BERA (stimulus level 35 dB nHL), thus reaching a sensitivity equal to 100%. The specificity of the different OAE devices depends on the underlying detection strategy. The best results were achieved with the automated Echoscreen device (95.9%) followed by the Otoclass analysis software (94.2%). The Echosensor device failed in our study to provide good specificity (77.3%). Reflex audiometry, which is favored by pediatricians in Germany, when used alone is completely inadequate as a screening method, even if conducted by a well-trained investigator. In our study, only 61.5% of the children with hearing loss were detected with reflex audiometry, and 42.7% of the children with normal hearing were misclassified. These results deviated from the results presented in ref. 13 to a large extent, as the Hanover group attested sensitivity and specificity of 100% for reflex audiometry (HNO 43, Reuter et al.). The deviating results are discussed in detail.     

Preschool hearing screening: pass/refer rates for children enrolled in a head start program in eastern North Carolina

This 4-year project investigated the pass/refer rates of preschool children in a hearing screening program. Three- and 4-year-old children who attended Head Start centers in rural, traditionally medically underserved, eastern North Carolina participated (n = 1,462). Screening procedures and pass/refer criteria were based on the Guidelines for Audiologic Screening (American Speech-Language-Hearing Association [ASHA], Panel on Audiologic Assessment, 1997). Only 54% (n = 787) of children passed the initial screening (i.e., passed all three of the screening components, which included pure-tone audiometry, tympanometry, and otoscopy), and an additional 22% (n = 323) passed the rescreening, for an overall pass rate of 76%. The initial pass rate was 90%, 71%, and 71% for otoscopy, tympanometry, and pure-tone audiometry, respectively. After the initial screening, 675 children were referred (i.e., 83%, 2%, and 15% for audiologic rescreening, medical evaluation, or both, respectively). About 71% (n = 478) received the recommended evaluation. Follow-up assessment compliance after the rescreening was poor. Slightly more than 10% of children were evaluated. The hearing status of 267 (i.e., 18.3%) children was never determined. Six (i.e., 0.5%) of the 1,195 children who completed the audiologic screening and/or received diagnostic audiologic assessment were confirmed to have hearing loss. Methodological factors that may have contributed to this high refer rate include the use of all screening techniques (pure tones, tympanometry, and otoscopy), procedural considerations in testing protocol and pass/refer criteria, and the demographic characteristics of the children screened.     

客观测听技术在鉴别伪聋中的临床应用

目的探讨聋人运动员伪聋鉴别客观、有效的测听方法。方法运用畸变产物耳声发射技术（distortion product otoacoustic emission,DPOAE）对聋人运动员进行听力初筛,初筛通过者再行中耳声导抗测试,应用声镫骨肌反射客观性推算其听敏度。对伪聋检查结果有异议的运动员和需做听神经病鉴别的运动员,再行脑干电反应测昕。结果初筛855人共有5名运动员8耳通过耳声发射检查,DPOAE耳通过率为0．47％,人通过率为0．58％。复查中耳声导抗测试3人4耳引出镫骨肌反射且阈值正常。脑干电反应测昕2人反应阈正常,2人反应阈≥100 dB nHL。结论耳声发射、中耳声导抗测试和脑干电反应测听可作为聋人运动员伪聋鉴别客观、有效的测听方法。     

von Hippel-Lindau disease: Deafness due to a non-MRI-visible endolymphatic sac tumor despite targeted screening

Abstract

Objective: Endolymphatic sac tumours (ELSTs) of the inner ear occur in 16% of patients with the hereditary tumor syndrome von Hippel-Lindau disease (vHL). ELSTs of all sizes can cause irreversible hearing loss which can, however, be prevented through early diagnosis and treatment. We aim to emphasize the challenges of prophylactic ELST screening and to explore the role of audiometry in pre-symptomatic ELST screening. Design: For a period of 17 years our patient was screened for ELSTs with inner-ear MRI (magnetic resonance imaging), audiometry, and clinical interviews. Study sample: A male vHL patient who became deaf in one ear due to a radiologically undetectable ELST. Results: Despite annual MRIs, the ELST was not visible until four months after onset of deafness when it appeared as a 1.4 × 1.4 mm tumor mass. Although his hearing was objectively within normal limits for the first 14 years, a distinct pattern of low-frequency hearing loss could retrospectively be seen at all audiometries. Conclusions: Audiometry is a candidate screening tool for detection of non-symptomatic pre-MRI-visible ELSTs, and we have initiated an international collaborative study to further determine its application. At present, we suggest an ELST screening protocol of yearly audiological assessment and inner ear MRI.