Auditory Brainstem Response Audiometry Research Articles

Analysis on the correlation of auditory brain/stem response and 40 Hz auditory event related potential by pure tone audiometry in adult patients with sudden deafness

Objective:To study the value of auditory brainstem response（ABR） and 40 Hz auditory evoked potential（40Hz AERP） in adult patients with sudden deafness. Methods:Pure tone audiometry, ABR and 40 Hz AERP were performed in 184 adult patients with sudden deafness before treatment. According to the frequency and severity of hearing loss revealed by pure tone audiometry, the patients were divided into low-frequency decline type（86 cases）, high-frequency decline type（60 cases）, flat type（32 cases） and total deafness type（6 cases, statistical analysis were not conducted in this group due to the incomplete elicitation of reaction threshold）. Data from 178 ears were collected for statistical results. After treatment, pure tone audiometry, ABR and 40 Hz AERP were performed. Results:As for ABR threshold, its correlation with 500 Hz threshold of subjective pure tone test was poor（r=0.233, P=0.706）, and was worse with 1000 Hz threshold（r=0.472, P=0.345）. ABR threshold was closely correlated with 2000 Hz threshold（r=0.878, P=0.021） and 4000 Hz threshold（r=0.800, P=0.010） of subjective pure tone test. As for 40 Hz AERP threshold, its correlation with 500 Hz threshold of subjective pure tone audiometry was good（r=0.992, P=0）, and was better with 1000 Hz threshold（r=0.912, P=0.110）. 40 Hz AERP threshold was poorly correlated with 2000 Hz threshold（r=0.210, P=0.690） and 4000 Hz threshold（r=0.370, P=0.945） of subjective pure tone audiometry. ABR and subjective pure tone audiometry have high correlation at high frequency, while 40 Hz AERP and subjective pure tone audiometry have high correlation at low frequency. Conclusion:ABR and 40 Hz AERP can comprehensively reflect the severity of hearing loss in adult patients with sudden deafness.

Towards effective assessment of normal hearing function from ABR using a time-variant sweep-tone stimulus approach

Objective. The auditory brainstem response (ABR) audiometry is a means of assessing the functional status of the auditory neural pathway in the clinic. The conventional click ABR test lacks good neural synchrony and it mainly evaluates high-frequency hearing while the common tone-burst ABR test only detects hearing loss of a certain frequency at a time. Additionally, the existing chirp stimuli are designed based on average data of cochlear characteristics, ignoring individual differences amongst subjects. Approach. Therefore, this study designed a new stimulus approach based on a sweep-tone concept with a time variant and spectrum characteristics that could be customized based on an individual’s cochlear characteristics. To validate the efficiency of the proposed method, we compared its performance with the click and tone-bursts using ABR recordings from 11 normal-hearing adults. Main results. Experimental results showed that the proposed sweep-tone ABR achieved a higher amplitude compared with those elicited by the click and tone-bursts. When the stimulus level or rate was varied, the sweep-tone ABR consistently elicited a larger response than the corresponding click ABR. Moreover, the sweep-tone ABR appeared earlier than the click ABR under the same conditions. Specifically, the mean wave V peak-to-peak amplitude of the sweep-tone ABR was 1.3 times that of the click ABR at 70 dB nHL (normal hearing level) and a rate of 20 s−1, in which the former saved 40% of test time. Significance. In summary, the proposed sweep-tone approach is found to be more efficient than the traditional click and tone-burst in eliciting ABR.

Comparison Results of Automated Auditory Brainstem Response and Brainstem Evoked Response Audiometry for Hearing Loss Detection in High-risk Infants

BACKGROUND: Brainstem evoked response audiometry (BERA) is not widely used for hearing screening because it is considered less practical; however, it is often used for diagnostics. Since the founding of automated auditory brainstem response (AABR), it often uses because it is more practical, has a high sensitivity and specificity in early detection of hearing loss (HL) in high-risk infants.
 AIM: The objective of the study was to determine the differences results of AABR and BERA for HL detection in high-risk infants at neonatal intensive care unit (NICU).
 METHODS: The study was conducted from November 2014 to September 2015 with consecutive sampling. The subjects were high-risk infants treated in the NICU room of the Neonatology Division at Dr. Soetomo General Hospital Surabaya and examined using AABR or BERA to determine the existence of HL.
 RESULTS: BERA results obtained normal (negative) as many as 28 ears (73.68%) and not normal (positive) as many as 10 ears (26.32%). AABR results obtained pass (negative) as many as 23 ears (60.53%) and refer (positive) as many as 15 ears (39.47%). Detection of HL in high-risk infants in NICU with AABR obtained 40% of sensitivity and 60.71% of specificity, 26.67% of positive prediction (NPP), 73.91% of negative predictive value (NPN), 55.26% of accuracy, 39.29% of false positive error rate, and 60% of false negative error rate. The comparative test of Wilcoxon signed-rank between the results of AABR and BER obtained p = 0.236.
 CONCLUSIONS: There was no difference between AABR and BERA results for HL detection in high-risk infants at NICU.

Hearing loss in patients with mucopolysaccharidoses-1 and -6 after hematopoietic cell transplantation: A longitudinal analysis.

Hearing loss is frequently seen in mucopolysaccharidoses (MPS) patients. Although hematopoietic cell transplantation (HCT) increases overall survival, disease progression is observed in certain tissues. This study describes the course of hearing loss (HL) over time in transplanted MPS patients. Transplanted MPS patients between 2003 and 2018 were included and received yearly audiological evaluation, including auditory brainstem response (ABR) or pure tone audiometry (PTA). Twenty‐eight MPS‐1 and four MPS‐6 patients were analyzed with a median follow‐up of 5 years (range 11 months–16 years). Air conduction threshold improved significantly over time (P < .001) with a PTA 1‐year post‐HCT of 50 ± 0.7 dB to 23 ± 11 dB 13 years post‐HCT. Bone conduction threshold worsened with a PTA 1 year post‐HCT of 10 ± 7 dB to 18 ± 9 dB 13 years post‐HCT (P = .34). The degree of HL varied from mainly mild‐severe early after HCT to normal‐mild at longer follow‐up. The type of HL consisted of mainly conductive in the first years post‐HCT in contrast to mainly sensorineural at longer follow‐up. MRIs of the cerebellopontine angle did not show abnormalities. HL is still seen in patients with MPS despite HCT and consists of a conductive type early after HCT in contrast to a sensorineural type at longer follow‐up in the majority of cases. Yearly follow‐up of HL is necessary to timely intervene, as hearing is important in the speech and language development of children and their academic achievements.

Simultaneous Vestibular Schwannoma Resection and Cochlear Implantation Using Electrically Evoked Auditory Brainstem Response Audiometry for Decision-making.

The aim of the study was to evaluate the predictive value regarding postoperative hearing benefit of electrically evoked auditory brainstem response audiometry in sporadic vestibular schwannoma patients undergoing simultaneous tumor resection and cochlear implantation. Patients were included in a prospective study conducted between October 2016 and January 2019. The study was conducted at a tertiary care center. Subjects with unilateral sporadic vestibular schwannoma were screened for study participation. Patients underwent translabyrinthine vestibular schwannoma resection and cochlear implantation simultaneously. Electrically evoked brainstem response audiometry was performed during surgery before and after tumor removal using an intracochlear test electrode to objectively evaluate nerve conduction. Electrically evoked brainstem response audiometry results were correlated with postoperative sound field audiometry, word recognition tests, and speech reception thresholds. Quality of life was assessed before and 12 months after translabyrinthine tumor removal and cochlear implantation. Five patients, three male and two female, were included in the study and followed for at least 1 year after implantation. Three of the five patients are daily cochlear implant users with open set speech recognition. Two individuals with negative intraoperative electrically evoked auditory brainstem response results showed no auditory perception with cochlear implant. Simultaneous translabyrinthine vestibular schwannoma resection and cochlear implantation with intraoperative electrically evoked auditory brainstem response measurements is a feasible and promising option for sporadic vestibular schwannoma patients. Preservation of electrically evoked auditory brainstem responses seems to predict good subsequent hearing outcomes.

Binaural Interaction in Tinnitus Patients

The auditory brainstem response (ABR) is a commonly used objective clinical measure for hearing evaluation. It can be also used to draw conclusions about the functioning of distinct stages of the auditory pathway including the binaural processing stages using the binaural interaction component (BIC) of the ABR. Objective: To study binaural processing in normal hearing subjects complaining of tinnitus. Methods: Sixty cases with bilateral normal peripheral hearing were included in this work, divided into 2 groups, i.e., group 1 (comprised of 30 healthy subjects representing the control group) and group 2 (comprised of 30 subjects with tinnitus representing the study group). All of the subjects were submitted to a basic audiological evaluation (including pure tone audiometry, speech audiometry, and immittancemetry) and ABR audiometry recorded in monaural and then binaural conditions. Results: In monaural recording, the tinnitus group showed significantly delayed latencies of waves I, III, and V in addition to significantly reduced wave I and III amplitudes when compared with the controls. Similar significant findings were found when binaural ABR responses were compared between both groups. Comparing BIC between both groups showed significant earlier BIC for latencies of waves I and V in the control group, while the BIC for amplitudes showed similar results in both groups. Conclusions: These finding suggest the presence of binaural processing deficits in tinnitus patients at different levels along the ascending auditory pathway.

A Survey on Auditory Brainstem Response and Pure Tone Audiometry Modalities in Mobile Phone Users

A Survey on Auditory Brainstem Response and Pure Tone Audiometry Modalities in Mobile Phone Users

Normal Hearing Function in Children Prenatally Exposed to Zika Virus.

Introduction The association between prenatal Zika virus infection and hearing alterations in offspring has been the object of some studies, although few have assessed children without microcephaly. However, a current trend to include prenatal Zika virus exposure in the group of risk indicators for hearing loss is noted. Objective To present a series of 27 children prenatally exposed to the Zika virus submitted to multiple hearing assessments over time. Methods A cohort of children born to symptomatic mothers with laboratorial Zika virus infection confirmation during pregnancy was submitted to an otoacoustic emission test, auditory brainstem response test (automated, neurodiagnostic and frequency-specific), audiometry, and imitanciometry over a period of 36 months since birth. The hearing assessment was performed independently of the presence of microcephaly or other apparent signs of congenital Zika syndrome. Results The hearing tests presented predominantly normal results. Some children had signs of middle ear pathology. The only microcephalic child had normal electrophysiological tests, as well as preserved audiometric thresholds, but presented altered motor responses to sound. Conclusion Prenatal exposure to Zika virus does not always determine hearing impairment. This risk seems to be more associated to the severity of the central nervous system damage. Hearing screening and follow-ups of the affected children are important, as well as further research in this area.

Comparison of auditory brainstem response and auditory steady state response audiometry by evaluating the hearing thresholds obtained in children with different severity of hearing loss.

Objectives:To compare the hearing thresholds obtained with auditory brainstem response (ABR) and auditory steady state response (ASSR) audiometry in children with hearing loss.Methods:Hearing thresholds were obtained by ABR and ASSR in children who presented with suspicion of deafness at Ear, nose & throat department of Al-Nafees Medical College Hospital Islamabad, between January to August 2018. The mean hearing thresholds obtained by two tests were compared within each category of severity of deafness. Time taken by both tests was also compared.Results:A total of 57 patients (114 ears) were included in the study. Among them 27 (47.4%) were male and 30 (52.6%) were female. The mean age of patients at presentation was 42 months (±30.9) with age range from one to 12 years. Mean hearing thresholds obtained by click ABR, chirp ABR, ASSR (1, 2, 4 kHz) & ASSR (0.5, 1, 2, 4 kHz) was 56.25 (±27.61), 58.88 (±27.44), 58.03 (±21.26) & 56.35 (±22.86) respectively. Mean thresholds were comparable between click ABR & ASSR (1, 2, 4 kHz) and between chirp ABR & ASSR (0.5, 1, 2, 4 kHz) in all degrees of hearing loss categories except in those patients with normal hearing thresholds. The mean time taken by clicks ABR, chirp ABR and ASSR were four minutes seven seconds, three minutes 15 seconds and 16 minutes and 7 seconds respectively.Conclusions:Hearing thresholds obtained by ABR and ASSR are comparable in all categories of severity of hearing loss. The time taken by ABR is less as compared to ASSR.

Cochlear implantation in patients afflicted with inner ear malformations – the Pécs experience

Purpose: To successfully analyze the surgical and audiological results of cochlear implantation in patients afflicted with inner ear malformations. This study briefly presents our surgical strategies, the selected implants and the audiological results.Method: Objective hearing threshold was measured using an auditory brainstem response and middle latency response audiometry. Structural imaging of high resolution temporal bone computer tomography and inner ear MR scan was performed in support of all patients. Images revealed bilateral cochlear malformation in seven patients. Three ears with common cavity deformity, two ears with incomplete partition type I and three ears suffering from cochlear hypoplasia were all rehabilitated using cochlear implantation. The type of the electrode and the surgical technique were individually determined reflective of the type of malformation. The success of the surgery and potential postoperative complications were duly noted and fully registered. Postoperative audiological measurements were performed and the results were analyzed.Results: The average audiological follow-up was 40.3 months. The average free field, pure-tone threshold of speech frequencies (0.5–3 kHz) gained postoperatively were within the range of 32.5–41.6 dB. In two of the procedures, a cerebrospinal fluid gusher did occur and was successfully managed intraoperatively, all without any postoperative complications.Conclusion: Temporal bone high resolution CT and inner ear MRI imaging are mandatory to detect and characterize the inner ear malformation. Early implantation of malformed cochlea is crucial towards achieving ideal postoperative audiological results. Notably, an intraoperative gusher is not regarded as a contraindication of the cochlear implantation.

Longitudinal Development of Distortion Product Otoacoustic Emissions in Infants With Normal Hearing.

The purpose of this study was to describe normal characteristics of distortion product otoacoustic emission (DPOAE) signal and noise level in a group of newborns and infants with normal hearing followed longitudinally from birth to 15 months of age. This is a prospective, longitudinal study of 231 infants who passed newborn hearing screening and were verified to have normal hearing. Infants were enrolled from a well-baby nursery and two neonatal intensive care units (NICUs) in Cincinnati, OH. Normal hearing was confirmed with threshold auditory brainstem response and visual reinforcement audiometry. DPOAEs were measured in up to four study visits over the first year after birth. Stimulus frequencies f1 and f2 were used with f2/f1 = 1.22, and the DPOAE was recorded at frequency 2f1-f2. A longitudinal repeated-measure linear mixed model design was used to study changes in DPOAE level and noise level as related to age, middle ear transfer, race, and NICU history. Significant changes in the DPOAE and noise levels occurred from birth to 12 months of age. DPOAE levels were the highest at 1 month of age. The largest decrease in DPOAE level occurred between 1 and 5 months of age in the mid to high frequencies (2 to 8 kHz) with minimal changes occurring between 6, 9, and 12 months of age. The decrease in DPOAE level was significantly related to a decrease in wideband absorbance at the same f2 frequencies. DPOAE noise level increased only slightly with age over the first year with the highest noise levels in the 12-month-old age range. Minor, nonsystematic effects for NICU history, race, and gestational age at birth were found, thus these results were generalizable to commonly seen clinical populations. DPOAE levels were related to wideband middle ear absorbance changes in this large sample of infants confirmed to have normal hearing at auditory brainstem response and visual reinforcement audiometry testing. This normative database can be used to evaluate clinical results from birth to 1 year of age. The distributions of DPOAE level and signal to noise ratio data reported herein across frequency and age in normal-hearing infants who were healthy or had NICU histories may be helpful to detect the presence of hearing loss in infants.

Characterization of vertigo and hearing loss in patients with Fabry disease

BackgroundFabry Disease (FD) is an X-linked hereditary lysosomal storage disorder which leads to a multisystemic intralysosomal accumulation of globotriaosylceramid (Gb3). Besides prominent renal and cardiac organ involvement, patients commonly complain about vestibulocochlear symptoms like high-frequency hearing loss, tinnitus and vertigo. However, comprehensive data especially on vertigo remain scarce. The aim of this study was to examine the prevalence and characteristics of vertigo and hearing loss in patients with FD, depending on renal and cardiac parameters and get hints about the site and the pattern of the lesions.MethodsSingle-center study with 57 FD patients. Every patient underwent an oto-rhino-laryngological examination as well as videonystagmography and vestibular evoked myogenic potentials (VEMPs) and audiological measurements using pure tone audiometry and auditory brainstem response audiometry (ABR). Renal function was measured by eGFR, cardiac impairment was graduated by NYHA class.ResultsMore than one out of three patients (35.1%) complained about hearing loss, 54.4% about vertigo and 28.1% about both symptom. In 74% a sensorineural hearing loss of at least 25 dB was found, ABR could exclude any retrocochlear lesion. Caloric testing showed abnormal values in 71.9%, VEMPs were pathological in 68%. A correlation between the side or the shape of hearing loss and pathological vestibular testing could not be revealed.ConclusionsHearing loss and vertigo show a high prevalence in FD. While hearing loss seems due to a cochlear lesion, peripheral vestibular as well as central nervous pathologies cause vertigo. Thus, both the site of lesion and the pathophysiological patterns seem to differ.

Consensus Statement of the Indian Academy of Pediatrics on Newborn Hearing Screening.

Hearing impairment is one of the most critical sensory impairments with significant social and psychological consequences. Evidence-based, standardized national guidelines are needed for professionals to screen for hearing impairment during the neonatal period. The meeting on formulation of national consensus guidelines on developmental disorders was organized by Indian Academy of Pediatrics in Mumbai, on 18th and 19th December, 2015. The invited experts included Pediatricians, Developmental Pediatricians, Pediatric Neurologists and Clinical Psychologists. The participants framed guidelines after extensive discussions. To provide guidelines on newborn hearing screening in India. The first screening should be conducted before the neonate's discharge from the hospital - if it 'fails', then it should be repeated after four weeks, or at first immunization visit. If it 'fails' again, then Auditory Brainstem Response (ABR) audiometry should be conducted. All babies admitted to intensive care unit should be screened via ABR. All babies with abnormal ABR should undergo detailed evaluation, hearing aid fitting and auditory rehabilitation, before six months of age. The goal is to screen newborn babies before one month of age, diagnose hearing loss before three months of age and start intervention before six months of age.

Normal neonatal hearing screening did not preclude sensorineural hearing loss in two-year-old very preterm infants.

Very preterm infants are at risk of neonatal hearing loss. However, it is unknown whether infants with a normal neonatal hearing screening result risk sensorineural hearing loss (SNHL) at a later age. This cohort study was conducted at the Erasmus Medical University Center Rotterdam, the Netherlands, on 77 very preterm infants born between October 2005 and September 2008. All infants underwent auditory brainstem response audiometry during neonatal hearing screening and at two years of corrected age. The frequency of SNHL in infants with a normal neonatal hearing screening was analysed and the risk factors associated with newly diagnosed SNHL in these infants were examined. We found that 3.9% (3/77) of the very preterm infants showed permanent hearing loss during their neonatal hearing screening. In addition, a relatively high prevalence of newly diagnosed SNHL (4.3%) was found in three of the 70 infants followed up at the age of two. The total prevalence rate of permanent hearing loss in the cohort was approximately 8%. A normal outcome of neonatal hearing screening did not guarantee normal hearing at two years of age in this very preterm cohort and paediatricians should be alert to the possibility of late-onset SNHL.

Advances in auditory function research of Meniere disease

Meniere disease is a unique, progressive disease of the inner ear. The complex manifestation presents diagnostic challenges. It is difficult to diagnose due to the absence of typical presentations especially in the early stages. The cochlear symptoms often present prior to vertigo and tend to be ignored. Therefore, focusing on the cochlear symptoms and functions may provide information for early diagnosis and may protect the patients from acquiring a hearing disability. This review focused on the pure-tone audiometry, glycerin test, distortion product otoacoustic emissions, auditory brainstem response, electrocochleogram, multifrequency tympanometry, and speech audiometry, which hopefully could emphasize the importance of the symptomatic and functional findings of cochlear, and be helpful in diagnosis for Meniere disease.

Hearing Loss in Children With Asymptomatic Congenital Cytomegalovirus Infection.

To assess the prevalence, characteristics, and risk of sensorineural hearing loss (SNHL) in children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected children. We included 92 case-patients and 51 controls assessed by using auditory brainstem response and behavioral audiometry. We used Kaplan-Meier survival analysis to estimate the prevalence of SNHL, defined as ≥25 dB hearing level at any frequency and Cox proportional hazards regression analyses to compare SNHL risk between groups. At age 18 years, SNHL prevalence was 25% (95% confidence interval [CI]: 17%-36%) among case-patients and 8% (95% CI: 3%-22%) in controls (hazard ratio [HR]: 4.0; 95% CI: 1.2-14.5; P = .02). Among children without SNHL by age 5 years, the risk of delayed-onset SNHL was not significantly greater for case-patients than for controls (HR: 1.6; 95% CI: 0.4-6.1; P = .5). Among case-patients, the risk of delayed-onset SNHL was significantly greater among those with unilateral congenital/early-onset hearing loss than those without (HR: 6.9; 95% CI: 2.5-19.1; P < .01). The prevalence of severe to profound bilateral SNHL among case-patients was 2% (95% CI: 1%-9%). Delayed-onset and progression of SNHL among children with asymptomatic congenital cytomegalovirus infection continued to occur throughout adolescence. However, the risk of developing SNHL after age 5 years among case-patients was not different than in uninfected children. Overall, 2% of case-patients developed SNHL that was severe enough for them to be candidates for cochlear implantation.

Auditory-steady-state Response Reliability in the Audiological Diagnosis After Neonatal Hearing Screening

Introduction and objectivesConventional audiometry is the gold standard for quantifying and describing hearing loss. Alternative methods become necessary to assess subjects who are too young to respond reliably. Auditory evoked potentials constitute the most widely used method for determining hearing thresholds objectively; however, this stimulus is not frequency specific. The advent of the auditory steady-state response (ASSR) leads to more specific threshold determination.The current study describes and compares ASSR, auditory brainstem response (ABR) and conventional behavioural tone audiometry thresholds in a group of infants with various degrees of hearing loss. MethodsA comparison was made between ASSR, ABR and behavioural hearing thresholds in 35 infants detected in the neonatal hearing screening programme. ResultsMean difference scores (±SD) between ABR and high frequency ABR thresholds were 11.2dB (±13) and 10.2dB (±11). Pearson correlations between the ASSR and audiometry thresholds were 0.80 and 0.91 (500Hz); 0.84 and 0.82 (1000Hz); 0.85 and 0.84 (2000Hz); and 0.83 and 0.82 (4000Hz). ConclusionThe ASSR technique is a valuable extension of the clinical test battery for hearing-impaired children.

Speech auditory brainstem response audiometry in adults with sensorineural hearing loss

Background: Listeners with sensorineural hearing loss (SNHL) have a reduced ability to use temporal fine structure cues due to broadening of the tuning curves. Auditory evoked potentials (AEPs) can be used to assess the functional consequences of auditory deprivation. Objectives: This work aimed to study the effect of mild to moderate SNHL on speech processing at brainstem levels. Subjects and method: This study included two groups: control group (GI) which consisted of 20 subjects with normal peripheral hearing adults and study group (GII) consisted of 40 patients with mild to moderate SNHL. Speech auditory brainstem response (S-ABR) was recorded in both groups using two speech stimuli: /da/ and /ba/. Results: In both groups, S-ABR in response to /ba/ showed statistically significant delayed latencies compared to /da/. On the other hand, there was no statistically significant difference as regards amplitudes between /da/ and /ba/ stimuli in both groups. Comparing both groups, there were statistically significant differences in the S-ABR onset latencies but not in FFR latencies for both stimuli in the SNHL group compared to the control group, while there was no statistically significant difference in amplitudes. Conclusion: SNHL affects speech processing at the level of brainstem.

Audiological findings in a group of neurologically compromised children: A retrospective study

Background . Hearing loss is more prevalent in developing countries. Later diagnosis of hearing loss will result in delayed access to rehabilitation. It is typically more difficult to obtain subjective information required in a hearing test from neurocompromised children, causing audiologists to frequently turn to objective measures such as the auditory brainstem response (ABR) measure to obtain this information. Objective . To describe the ABR results in a group of neurologically compromised children and to establish a relationship between ABR findings and behavioural audiometry results, where these existed. Methods . A retrospective review was conducted on 40 ABR patient records of neurologically compromised participants aged 5 months to 10 years. Behavioural audiometry results were sought where these existed. Hearing status was described per ear for objective and behavioural results, and descriptive statistics were conducted. Results . Behavioural audiometry results were obtained in 72.5% of ears. Results correlated between ABR and behavioural audiometry for only 7.5% of ears, which were all diagnosed with normal hearing. About 12.5% of ears were misdiagnosed with behavioural audiometry. Premature infants were most likely to cope with behavioural audiometry. Hearing loss was highest in participants with cerebral palsy, Down syndrome, prematurity and retroviral disease. Conclusions . Behavioural audiometry appears to be a largely unreliable method of testing the hearing of children diagnosed with neurological disorders, as results were obtained in only 27.5% of the study sample; however, it remains the gold standard in paediatric hearing testing to evaluate the entire auditory system and provides information on how a child processes sound. Hearing thresholds should be established via objective testing. Conditioning should continue for a behavioural audiological test battery, with adaptations for the child’s developmental ability.

Universal newborn hearing screening in Umbria region, Italy

ObjectiveIn Italy, universal newborn hearing screening (UNHS) was first introduced in some regions from 1997. Umbria Region has launched a UNHS program in all hospitals, which has been implemented throughout the region since July 2010. Before UNHS implementation in Umbria region, the average age of identification of congenital hearing loss was around 32 months of age with an average age of initial amplification treatment at least 2 months later. The coverage rate of newborn screening was only 34.4% in 2006.The aim of this study was to examine the results of this program and its evolution in the first 2.5 years since implementation in our region. MethodsSince July 2010, all 11 birth centers and hospitals in Umbria region have been involved in a UNHS program. The screening involves the automated otoacoustic emissions (AOAE) test and automatic auditory brain stem response (AABR) audiometry. The number of screening stages and tests used were different depending on whether the infants had audiological risk factors or not. ResultsA total of 20,841 babies were born in the hospitals involved of whom 20,051 were well born babies (WB), while 790 babies (3.8%) presented identified audiological risk factors (BRF). The overall coverage rate in the study period was 93.8%. The prevalence of hearing loss was 2‰ for WB infants and 4.3% for BRF. Mean age at diagnosis was 5.31±3.95 and 11.28±7.73 months in the WB and BRF groups, respectively. ConclusionsUNHS has allowed us to substantially increase the coverage rates and decrease the mean age at diagnosis and subsequent treatment. The identification of audiological risk factors is very important for adequate screening and follow-up. However the Joint Committee on Infant Hearing 2007 quality indicators and benchmarks for screening have not yet been fully achieved and there is still scope for some improvement. This could be achieved with a closer cooperation among institutions, parents, pediatricians, and ENT doctors.