Short-term outcomes of infants with hyperbilirubinemia-associated auditory neuropathy spectrum disorder in neonatal intensive care unit.

OBJECTIVES: Hyperbilirubinemia is a high-risk factor for auditory neuropathy spectrum disorder (ANSD) as well as hearing loss in general. This study described the outcomes of hyperbilirubinemia-associated ANSD infants diagnosed in hearing screening in the neonatal intensive care unit (NICU). METHODS: A total of 578 children with hyperbilirubinemia admitted to the NICU between October 2020 and October 2021 were included in this study. The distortion product otoacoustic emission (DPOAE) and automatic auditory brainstem response (AABR) were combined for hearing screening, and those who failed the DPOAE or/and AABR underwent an auditory brainstem response (ABR) test. Infants with ANSD were followed up for 12 months. RESULTS: Forty infants (40/578, 6.9%) failed the DPOAE or/and AABR tests, of which, 13 (13/578, 2.2%) were diagnosed as ANSD, and 27 (27/578, 4.7%) were diagnosed as having sensorineural hearing loss (SNHL). Of the 13 ANSD infants followed up for 12 months, 7 recovered, 3 improved, 3 did not recover, and 1 was lost, equating to improved or recovered hearing in 75% (9/12) of ANSD infants at 12 months of age. Moreover, the maximum bilirubin in recovered or improved ANSD infants was 408.6 ± 129.0 µmol/L, while the maximum bilirubin in unrecovered ANSD infants was 749.3 ± 323.0 µmol/L. Furthermore, poorly differentiated and absent ABR waveforms were observed in 6 and 14 ears at 1 month, 2 ears were lost, 6 (6/6, 100.0%) and 6 (6/12, 50.0%) ears were recovered or improved at 12 months of age. CONCLUSION: s: The incidence of hyperbilirubinemia associated-ANSD was 2.2% of infants screened in the NICU. ANSD caused by hyperbilirubinemia may be transient, with most infants improving or recovering hearing by 12 months of age. Infants with poorly differentiated ABR waveforms and low bilirubin concentration are more likely to recover and hearing aids are not recommended in hyperbilirubinemia-associated ANSD below 12 months of age.

Cochlear nerve deficiency is an important cause of auditory neuropathy spectrum disorder at a population level in children.

OBJECTIVES: To determine the incidence, prevalence and describe risk factors and etiology for childhood Auditory Neuropathy Spectrum Disorder using population level data from a statewide universal newborn hearing program. METHODS: A retrospective statewide universal newborn hearing screening database review and descriptive analysis from 2012 to 2019 of demographic, risk factors and hearing loss etiology for babies with sensorineural hearing loss and ANSD was completed. A 2 stage aABR protocol was used and ANSD was classified when click evoked ABR were absent or grossly abnormal but otoacoustic emissions and or cochlear microphonics were present. Medical evaluation and investigation by a pediatrician or otolaryngologist was performed and etiology was assigned using a coding scheme. Next generation genetic sequencing was not available. RESULTS: From 2012 to 2019, 487 636 babies were screened for congenital hearing loss (99.1%) and 1150 were confirmed to have permanent SNHL, 80 of whom were diagnosed with ANSD (52 unilateral and 28 bilateral). The prevalence of ANSD was 7.0% and population prevalence was 0.16 per 1000 live births. The only demographic or risk factor significantly more likely to be associated with ANSD than SNHL was hyperbilirubinemia. The most common etiology for ANSD was hypoplasia or absence of the cochlear nerve with 37 cases (46.3%), and it was significantly more likely with unilateral than bilateral ANSD. CONCLUSION: At a population level, ANSD was more likely to be unilateral and the only perinatal risk factor significantly associated was hyperbilirubinemia. Cochlear nerve deficiency was the most common etiology. Given that this can occur in well babies, this provides further evidence for aABR as a preferred mode for newborn hearing screening.

Prevalence, risk factors, and audiological characteristics of auditory neuropathy.

OBJECTIVE: The objective of this study was to determine the prevalence, risk factors, and audiological characteristics of auditory neuropathy spectrum disorder (ANSD) in the pediatric population. DESIGN: A retrospective review of medical charts was conducted for children visiting two hospitals in Saudi Arabia. STUDY SAMPLE: Medical records of 1025 patients with sensorineural hearing loss (SNHL) were reviewed. We analyzed the databases for results of audiological examinations, risk factors, and outcomes of intervention including hearing aid (HA) and cochlear implantation (CI). RESULTS: Out of 1025 children with SNHL, 101 patients (9.85%) were identified to have ANSD. Audiological characteristics of the ANSD group revealed a severe-to-profound degree of hearing loss, all showed type A tympanogram and absent reflexes, absent auditory brainstem response (ABR) findings with present cochlear microphonic while otoacoustic emissions were absent in 54.5% of patients. The most prevalent risk factors for ANSD in this group were family history of hearing loss, consanguinity, hyperbilirubinemia, and low birth weight. Pure tone and speech detection thresholds improved significantly with CI compared to HA use in this sample of patients with ANSD. CONCLUSION: This study shows that ANSD is not extremely rare among Saudi children with severe to profound hearing loss, with a prevalence of 9.85%.

Prevalence, behavioural, and management outcomes of infants with auditory neuropathy spectrum disorder.

AIM: To provide an overview of electrophysiological and behavioural outcomes from a large UK centres data set on children diagnosed with auditory neuropathy (ANSD) between 2002 and March 2018. METHOD: A systematic audit was undertaken, collating the electrophysiological data from auditory brainstem response (ABR) follow-up, risk factors, and later behavioural results/management. These were then compared to look for trends between groups. The study sample consisted of 118 925 infants born, with 46 (0.039%, 0.39 per 1000 births) being diagnosed with congenital ANSD (39 bilateral, seven unilateral). RESULTS: Twenty-nine per cent of ears with ANSD had short latency components on ABR testing. Forty-four per cent of ears with present cochlear microphonics but absent transient-evoked otoacoustic emissions (TE-OAE) and no ABR went on to have profound behavioural hearing threshold levels. All but one child went on to show a hearing loss on behavioural testing. ANSD was not confined to the population from neonatal intensive care units: there were three bilateral and five unilateral cases in the typically developing infant population. INTERPRETATION: The incidence of ANSD is higher in this sample than that reported previously in the literature. Children who had cochlear microphonics with absent ABR and absent TE-OAE had significantly worse later behavioural outcomes than other patterns of electrophysiological results.

Diagnosis of Auditory Neuropathy Spectrum Disorder in the Neonatal Intensive Care Unit Population.

OBJECTIVE: To determine the incidence of auditory neuropathy spectrum disorder (ANSD) and its risk factors among the neonatal intensive care unit (NICU) population from 2009 to 2018 in the Pediatric Health Information System database. STUDY DESIGN: Retrospective national database review. SETTING: Population-based study. METHODS: The Pediatric Health Information System database was queried to identify patients ≤18 years old with NICU admission and ANSD diagnosis. Patient demographics, jaundice diagnosis, use of mechanical ventilation, extracorporeal membrane oxygenation, furosemide, and/or aminoglycosides were extracted. Multivariable linear regression was used to assess trends in incidence. Chi-square analysis was used to assess differences between patients with and without ANSD. Logistic regression was used to assess factors associated with ANSD. RESULTS: From 2009 to 2018, there was an increase in (1) NICU admissions from 14,079 to 24,851 (P < .001), (2) total ANSD diagnoses from 92 to 1847 (P = .001), and (3) annual total number of patients with ANSD and NICU admission increased from 4 to 16 (P = .005). There was strong correlation between the increases in total number of NICU admissions and total ANSD diagnoses over time (R = 0.76). The average ANSD incidence was 0.052% with no statistically significant change over 10 years. When compared with all NICU admissions, children with ANSD had a higher association with use of furosemide (P < .001) and ventilator (P < .001). CONCLUSION: Despite a statistically significant increase in NICU admissions and total ANSD diagnosis, the incidence of ANSD in the NICU population has not increased from 2009 to 2018. Furosemide and mechanical ventilator use were associated with increased likelihood of ANSD.

Association Between Central and Peripheral Age-Related Hearing Loss and Different Frailty Phenotypes in an Older Population in Southern Italy.

Importance: The association between age-related hearing loss (ARHL) and physical or cognitive frailty has been poorly explored. These associations could define new perspectives for delaying frailty-related processes in older age. Objective: To examine whether peripheral ARHL and age-related central auditory processing disorder (CAPD) are independently associated with physical or cognitive frailty. Design, Setting, and Participants: This cross-sectional study analyzed registry data from December 31, 2014, on 1929 older (≥65 years) participants of the Salus in Apulia Study (Southern Italy) who underwent audiologic, physical, and neuropsychological assessment. Data analysis was performed from December 12, 2019, to January 4, 2020. Main Outcomes and Measures: Prevalence of peripheral ARHL in older individuals with physical and/or cognitive frailty and those without frailty assessed using the Fried criteria (physical) and the Mini-Mental State Examination (cognitive). Multivariable logistic regression models were used to assess associations of audiologic variables with frailty phenotype. Results: Data from 1929 participants (mean [SD] age, 73.6 [6.3] years; 974 male [50.5%]) were eligible for the analyses. The prevalence of peripheral ARHL was higher in the physical frailty group (96 [26.6%]) than in the nonfrail group (329 [21.0%]) (difference, 5.61 percentage points; 95% CI, 0.63-10.59 percentage points) and in the cognitive frailty group (40 [38.8%]) than in the nonfrail group (385 [21.1%]) (difference, 17.75 percentage points; 95% CI, 8.2-27.3 percentage points). Age-related CAPD was more prevalent in the physical frailty group (62 [17.2%]) than in the nonfrail group (219 [14.0%]) (difference, 3.21 percentage points; 95% CI, -1.04 to 7.46 percentage points) and in the cognitive frailty group (28 [27.2%]) than in the nonfrail group (253 [13.9%]) (difference, 13.33 percentage points; 95% CI, 4.10-22.21 percentage points). In the multivariable models, age-related CAPD was associated with cognitive frailty in the fully adjusted model (odds ratio [OR], 1.889; 95% CI, 1.094-3.311). There was also an inverse association between the unitary increase in Synthetic Sentence Identification With the Ipsilateral Competitive Message scores, indicating a lower likelihood of this disorder, and cognitive frailty (OR, 0.989; 95% CI, 0.988-0.999). Peripheral ARHL was associated with cognitive frailty only in the partially adjusted model (OR, 1.725; 95% CI, 1.008-2.937). Conclusions and Relevance: In this cross-sectional study of 1929 participants, age-related CAPD was independently associated with cognitive frailty. Whether the management of ARHL may help prevent the development of different frailty phenotypes or improve their clinical consequences should be addressed in longitudinal studies and, eventually, well-designed randomized clinical trials.

New trends in the prevention of occupational noise-induced hearing loss.

Noise exposure during lifespan is one of the main causes of hearing loss. The highest risk of noise-induced hearing loss (NIHL) is related to exposures in the workplace, and affects about 7% of the population. Occupational NIHL is irreversible, thus its prevention must be considered a priority. Although current hearing conservation programs (HCPs) have proved to be very beneficial, the incidence of occupational NIHL is still high, reaching about 18% of overexposed workers. This paper reviews recent research on the effects of noise on hearing in pursuit of more effective methods for the prevention of occupational NIHL. The paper discusses the translational significance of noise-induced cochlear neuropathy, as recently shown in animals, and the concept of hidden hearing loss in relation to current NIHL damage risk criteria. The anticipated advantages of monitoring the incidents of the temporary threshold shift (TTS) in workers exposed to high levels of noise have been analyzed in regard to the preclinical diagnostics of NIHL, i.e., at the stage when hearing loss is still reversible. The challenges, such as introducing speech-in-noise audiometry and TTS computational predictive models into HCPs, have been discussed. Finally, the paper underscores the need to develop personalized medical guidelines for the prevention of NIHL and to account for several NIHL risk factors other than these included in the ISO 1999:2013 model. Implementing the steps mentioned above would presumably further reduce the incidence of occupational NIHL, as well as associated social costs. Int J Occup Med Environ Health. 2020;33(6):841-8.

Relationship research between auditory neuropathy spectrum disorder and exchange transfusion in neonates with severe hyperbilirubinemia.

OBJECTIVE: To explore the effects of exchange transfusion on auditory neuropathy spectrum disorder (ANSD) in neonates with severe hyperbilirubinemia (SH). METHODS: The clinical data of 2216 SH neonates who met the standard of exchange transfusion and 732 non severe-hyperbilirubinemia (NSH) neonates in the same period who did not require exchange transfusion in the neonatology department of Childrens' Hospital of Chongqing Medical University between January 2010 and December 2015 were retrospectively analyzed. In addition, the SH neonates were further divided into the exchange transfusion group and photography group. Hearing screening was conducted on all neonates using transiently evoked otoacoustic emission (TEOAE) and auto auditory brainstem response (AABR), and neonates who failed the above screening were performed diagnostic hearing test. And then neonates diagnosed with hearing disorder were followed up for 2-5 years. RESULTS: The pass rates of hearing screening were 80.58%, 79.71% and 87.84% in the phototherapy group, exchange transfusion group and NSH group respectively, with a significant difference(P < 0.05). Hearing loss was diagnosed in 10.15%, 12.39% and 8.54% of neonates in the phototherapy group, exchange transfusion group and NSH group. After follow-up, ultimate incidence rates of ANSD were 11.96%, 11.57% and 2.4% respectively in the 3 groups, with a significant difference (P < 0.05). CONCLUSIONS: SH is one of risk factors for ANSD. SH neonates have a lower incidence of ANSD in the exchange transfusion group than in the phototherapy group. Neonates who meet the standards of exchange transfusion adopt this therapy in early stage, which can quickly decrease bilirubin level and ultimately reduce incidence of ANSD.

The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management.

Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorineural hearing impairment (CAPOS) is a rare clinically distinct syndrome caused by a single dominant missense mutation, c.2452G>A, p.Glu818Lys, in ATP1A3, encoding the neuron-specific alpha subunit of the Na+/K+-ATPase α3. Allelic mutations cause the neurological diseases rapid dystonia Parkinsonism and alternating hemiplegia of childhood, disorders which do not encompass hearing or visual impairment. We present detailed clinical phenotypic information in 18 genetically confirmed patients from 11 families (10 previously unreported) from Denmark, Sweden, UK and Germany indicating a specific type of hearing impairment-auditory neuropathy (AN). All patients were clinically suspected of CAPOS and had hearing problems. In this retrospective analysis of audiological data, we show for the first time that cochlear outer hair cell activity was preserved as shown by the presence of otoacoustic emissions and cochlear microphonic potentials, but the auditory brainstem responses were grossly abnormal, likely reflecting neural dyssynchrony. Poor speech perception was observed, especially in noise, which was beyond the hearing level obtained in the pure tone audiograms in several of the patients presented here. Molecular modelling and in vitro electrophysiological studies of the specific CAPOS mutation were performed. Heterologous expression studies of α3 with the p.Glu818Lys mutation affects sodium binding to, and release from, the sodium-specific site in the pump, the third ion-binding site. Molecular dynamics simulations confirm that the structure of the C-terminal region is affected. In conclusion, we demonstrate for the first time evidence for auditory neuropathy in CAPOS syndrome, which may reflect impaired propagation of electrical impulses along the spiral ganglion neurons. This has implications for diagnosis and patient management. Auditory neuropathy is difficult to treat with conventional hearing aids, but preliminary improvement in speech perception in some patients suggests that cochlear implantation may be effective in CAPOS patients.

Chronic Auditory Toxicity in Late Preterm and Term Infants With Significant Hyperbilirubinemia.

BACKGROUND AND OBJECTIVES: Significant hyperbilirubinemia (SHB) may cause chronic auditory toxicity (auditory neuropathy spectrum disorder and/or sensorineural hearing loss); however, total serum bilirubin (TSB) does not discriminate neonates at risk for auditory toxicity. Our objective was to compare TSB, bilirubin albumin molar ratio (BAMR), and unbound bilirubin (UB) for their association with chronic auditory toxicity in neonates with SHB (TSB ≥20 mg/dL or TSB that met criteria for exchange transfusion). METHODS: Infants ≥34 weeks' gestational age (GA) with SHB during the first 2 postnatal weeks were eligible for a prospective longitudinal study in India. Comprehensive auditory evaluations were performed at 2 to 3 months of age by using auditory brainstem response, tympanometry, and an otoacoustic emission test and at 9 to 12 months of age by using audiometry. The evaluations were performed by an audiologist unaware of the degree of jaundice. RESULTS: A total of 93 out of 100 infants (mean GA of 37.4 weeks; 55 boys, 38 girls) who were enrolled with SHB were evaluated for auditory toxicity. Of those, 12 infants (13%) had auditory toxicity. On regression analysis controlling for covariates, peak UB (but not peak TSB or peak BAMR), was associated with auditory toxicity (odds ratio 2.41; 95% confidence interval: 1.43-4.07; P = .001). There was significant difference in the area under the receiver operating characteristic curves between UB (0.866), TSB (0.775), and BAMR (0.724) for auditory toxicity (P = .03) after controlling for covariates. CONCLUSIONS: Unconjugated hyperbilirubinemia indexed by UB (but not TSB or BAMR) is associated with chronic auditory toxicity in infants ≥34 weeks' GA with SHB.

[Auditory Neuropathy: Clinical Evaluation and Diagnostic Approach]. / Neuropatia Auditiva: Avaliação Clínica e Abordagem Diagnóstica.

INTRODUCTION: Auditory neuropathy is a condition in which there is a change in the neuronal transmission of the auditory stimuli. Our objective was to describe the patients' series within the clinical spectrum of auditory neuropathy. MATERIAL AND METHODS: We designed a transversal, retrospective study, with a description of a consecutive case series. Auditory neuropathy was defined by the presence of acoustic otoemissions plus absent/abnormal auditory brainstem responses with cochlear microphonism. RESULTS: 34 patients with bilateral hearing loss, 23 males and 11 females, were included in the study. Eighty percent of the cases had congenital onset of hearing loss. Acoustic otoemissions were absent in 67% of them. Cochlear microfonism was present in 79% of all cases. Prenatal, perinatal or ambiental factors were present in 35.2% of the cases. DISCUSSION: Medical literature shows great variability in findings related to auditory neuropathy, both in its etiology and epidemiological data. CONCLUSION: Auditory neuropathy presents a broad spectrum of changes that may result from mild to severe changes in the functioning of the auditory pathway, and in our sample we observed that 80% of Auditory neuropathy have congenital onset of hearing loss and/or with cochlear microphonism identified. 91% of patients experience significant hearing impairment and 53% suffer from severe or profound deafness.

Introdução: A neuropatia auditiva é uma condição na qual há alteração na condução neuronal do estímulo sonoro. Este trabalho pretende descrever e caracterizar a casuística de doentes com neuropatia auditiva. Material e Métodos: Realizámos um estudo transversal, retrospetivo, com descrição de uma série de casos consecutivos. O diagnóstico da neuropatia auditiva foi definido nas seguintes situações: Presença de otoemissões acústicas com potenciais auditivos de tronco encefálico ausente ou anormal e presença do microfonismo coclear independentemente da presença de otoemissões acústicas. Resultados: Foram avaliados 34 doentes com perda auditiva bilateral, 67% deles do sexo masculino. O aparecimento dos sintomas foi congênito em 80% dos casos. Na pesquisa das otoemissões acústicas, a resposta foi ausente em 67% dos doentes. O microfonismo coclear foi detetado em 79% dos doentes. Antecedentes gestacionais, perinatais ou ambientais relevantes estiveram presentes em 35,3% dos casos. Discussão: A literatura médica ainda apresenta grande variabilidade nos achados relacionados com a neuropatia auditiva, tanto na sua etiologia quanto nos dados epidemiológicos. Conclusão: A neuropatia auditiva apresenta um amplo espectro de alterações que podem resultar em disfunções leves a severas no funcionamento da via auditiva. Na nossa amostra, observámos que 80% das neuropatias auditivas terão tido origem congênita e/ou apresenta microfonismo coclear, 91% dos doentes apresenta défice auditivo significativo e 53% sofrem de surdez severa ou profunda.

Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic.

UNLABELLED: Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency. CONCLUSION: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients. WHAT IS KNOWN: • Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates. • Little data are available on the prevalence and risk factors for ANSD in healthy newborns. What is new: • The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births. • In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.

Auditory Neuropathy/Dys-Synchrony Disorder: Diagnosis and Management.

Auditory neuropathy/dys-synchrony disorder affects neural responses, either directly or indirectly. Patients may demonstrate good ability to detect sound, but have significant difficulty listening in noise. Clinical auditory physiologic measures are used to characterize cochlear, eighth nerve, and brainstem function, and are needed to accurately identify this disorder. Cochlear implants provide benefit to many patients, and some patients derive benefit from amplification. This disorder can be identified and managed in infants, may have later onset, may be a part of a syndrome, and may include fluctuation in hearing ability.

Auditory neuropathy in late preterm infants treated with phototherapy for hyperbilirubinemia.

OBJECTIVE: To evaluate the prevalence of auditory neuropathy (AN) in late preterms treated with phototherapy for hyperbilirubinemia. DESIGN: Prospective observational study comprising late preterms treated with phototherapy for hyperbilirubinemia. Newborns were screened with combined transient-evoked otoacoustic emissions (TEOAEs) / automated auditory brainstem responses (AABR). Infants who failed screening underwent diagnostic (ABR). Infants were all re-evaluated with AABR at one year. STUDY SAMPLE: Eighty-five infants with a mean serum total bilirubin concentration of 22.3 ± 1.76 mg/dl; severe-hyperbilirubinemia (SH), and 102 infants with a mean serum total bilirubin concentration of 18.6 ± 1.26 mg/dl; non-severe hyperbilirubinemia (NSH) were included. RESULTS: From 85 late preterms with SH, six (7.1%) failed screening and underwent diagnostic ABR for six weeks. AN was diagnosed in two (2%) infants with SH. Four (3.9%) of the 102 controls with NSH demonstrated failure at TEOAE/AABR. No AN was diagnosed in the control group at the diagnostic ABR. No statistically significant difference was found between infants treated with phototherapy for SH and NSH with regard to AN/AD either in the postnatal period or at one year. No correlation was found between serum bilirubin levels and ABR latencies or thresholds. CONCLUSIONS: AN (2%) in late preterms treated with phototherapy for severe-hyperbilirubinemia was not higher than in those with non-severe hyperbilirubinemia.

The prevalence of peripheral and central hearing impairment and its relation to cognition in older adults.

Age-related hearing loss (ARHL) and dementia are two highly prevalent conditions in the adult population. Recent studies have suggested that hearing loss is independently associated with poorer cognitive functioning. The aim of this study was to evaluate the prevalence of ARHL and cognitive impairment in a large sample of subjects older than 65 years and to correlate hearing function with cognitive function. A total of 488 subjects older than 65 years (mean age 72.8 years) participating in the Great Age Study underwent a complete audiological, neurological and neuropsychological evaluation as part of a multidisciplinary assessment. The prevalence of a hearing loss greater than 25 dB HL was 64.1%, of Central Auditory Processing Disorder (CAPD) was 14.3 and 25.3% of the subjects reported a hearing handicap as reported on the Hearing Handicap Inventory for the Elderly Screening Version questionnaire. Multiple logistic regression analysis corrected for gender, age and education duration showed that mild cognitive impairment (MCI) was significantly associated with hearing impairment (CAPD and hearing threshold; odds ratio 1.6, p = 0.05) and that Alzheimer's disease (AD) was significantly associated with CAPD (odds ratio 4.2, p = 0.05). Given that up to 80% of patients affected by MCI convert to AD, adding auditory tests to a screening cognitive battery might have value in the early diagnosis of cognitive decline.

Prevalence of auditory neuropathy spectrum disorder in an auditory health care service.

UNLABELLED: Auditory neuropathy spectrum disorder (ANSD) is characterized by impairment of the auditory nerve associated with preservation of outer hair cell function. OBJECTIVE: To establish the prevalence of ANSD in subjects with sensorineural hearing loss (SNHL). METHOD: This retrospective study was carried out between 2010 and 2012 and included the charts of 2,292 individuals with SNHL. Data from otolaryngological and audiological examinations based on pure-tone and speech audiometry, impedance tests, otoacoustic emissions (OAEs), and brainstem auditory evoked potentials (BAEPs) were collected. INCLUSION CRITERIA: presence of OAEs and/or cochlear microphonic (CM); absent or altered BAEPs, and normal MRI scans of the brain. RESULTS: Twenty-seven (1.2%) of the 2,292 subjects with SNHL had ANSD (37% males; 63% females). Mild SNHL was seen in 29.6% of the individuals with ANSD; 55.5% had moderate SNHL; 7.4% had severe SNHL; and 7.5% had profound SNHL. In terms of age, 14.8% were aged between zero and 20 years, 44.1% were 41 to 60 years old, and 7.4% were above the age of 60. CONCLUSION: ANSD was seen in 1.2% of the individuals with SNHL included in this study.

Prevalência do espectro da neuropatia auditiva em um serviço de saúde auditiva / Prevalence of auditory neuropathy spectrum disorder in an auditory health care service

Oespectro da neuropatia auditiva (ENA) caracteriza-se pelo acometimento do nervo auditivo, associado à preservação da função das células ciliadas externas cocleares. OBJETIVO: Determinar a prevalência do ENA em sujeitos com perda auditiva neurossensorial (PANS). MÉTODO: Estudo retrospectivo realizado de 2010 a 2012, pela análise dos prontuários de 2.292 sujeitos com PANS. Foram coletados dados das avaliações otorrinolaringológica e audiológica, por meio da audiometria tonal e vocal, imitanciometria, Emissões Otoacústicas (EOAs) e Potencial Evocado Auditivo de Tronco Encefálico (PEATE). Critérios de inclusão: presença das EOAs e/ou Microfonismo Coclear (MC); ausência ou alterações nas ondas do (PEATE) e ressonância nuclear magnética cerebral normal. RESULTADOS: Entre os 2.292 sujeitos com PANS, 27 (1,2%) apresentaram ENA, sendo 37% masculino e 63% feminino. Entre os sujeitos com ENA, 29,6% tinham PANS leve; 55,5% moderada; 7,4% grave e 7,5% profunda. Em relação à faixa etária, 14,8% estavam entre 0 e 20 anos, 33,4% entre 21 e 40 anos, 44,4% entre 41 e 60 anos e 7,4% acima de 60 anos. CONCLUSÃO: A prevalência do ENA neste estudo foi de 1,2% em sujeitos com PANS.

Auditory neuropathy spectrum disorder (ANSD) is characterized by impairment of the auditory nerve associated with preservation of outer hair cell function. OBJECTIVE: To establish the prevalence of ANSD in subjects with sensorineural hearing loss (SNHL). METHOD: This retrospective study was carried out between 2010 and 2012 and included the charts of 2,292 individuals with SNHL. Data from otolaryngological and audiological examinations based on pure-tone and speech audiometry, impedance tests, otoacoustic emissions (OAEs), and brainstem auditory evoked potentials (BAEPs) were collected. Inclusion criteria: presence of OAEs and/or cochlear microphonic (CM); absent or altered BAEPs, and normal MRI scans of the brain. RESULTS: Twenty-seven (1.2%) of the 2,292 subjects with SNHL had ANSD (37% males; 63% females). Mild SNHL was seen in 29.6% of the individuals with ANSD; 55.5% had moderate SNHL; 7.4% had severe SNHL; and 7.5% had profound SNHL. In terms of age, 14.8% were aged between zero and 20 years, 44.1% were 41 to 60 years old, and 7.4% were above the age of 60. CONCLUSION: ANSD was seen in 1.2% of the individuals with SNHL included in this study.

Clinical characteristics of children with cochlear nerve dysplasias.

OBJECTIVES/HYPOTHESIS: To describe the clinical and audiometric characteristics of children with cochlear nerve dysplasia (CND). STUDY DESIGN: Retrospective chart review of clinical database of children with inner ear anomalies treated at a tertiary care children's hospital. METHODS: Institutional review board-approved retrospective review from June 30, 2006, to July 1, 2011; 18 children were identified with magnetic resonance imaging (MRI) evidence of CND defined as a cochlear nerve 50% smaller than the adjacent facial nerve. RESULTS: Of the 18 patients, nine were girls and nine were boys. Average age at time of MRI diagnosis of CND was 4.6 years. Twelve children had cochlear nerve aplasia, and six had hypoplasia. Three were affected bilaterally: two with aplasia and one with hypoplasia. Unilateral dysplasia was found in 15 children; of these, 60% occurred on the left side. Other inner ear anomalies were found in 50%, including all patients with bilateral CND. Severe-to-profound hearing loss was found in the involved ear(s) in 14 of 18 patients, including all bilateral patients. Of the 18 patients tested, 13 (72%) had an audiometric profile of auditory neuropathy/dys-synchrony syndrome (auditory neuropathy spectrum disorder [ANSD]). Comorbid conditions were present in 56% of patients. Two patients were syndromic. Family history of hearing loss was present in 11% of patients. CONCLUSIONS: Many patients with CND have ANSD, and more than half have comorbidities. Approximately half of affected patients have other inner ear anomalies in the involved ears. Unilateral CND may be more common on the left side.

Desarrollo de una hoja de cálculo del impedimento auditivo usando Microsoft EXCEL / Developement of a spreadsheet for hearing impairment using Microsoft Excel

En nuestra actividad profesional diaria es habitual que necesitemos calcular el impedimento auditivo, para realizar informes médicos o para informar a nuestros pacientes. Hasta ahora hemos utilizado tablas y sistemas de cálculo manual que lo facilitan. Para simplificar y hacer más preciso este cálculo hemos desarrollado un sistema utilizando el programa Microsoft Excel y las fórmulas de cálculo de la American Academy of Otolaryngology. Respecto a otros sistemas, el programa de cálculo que describimos aporta 2 ventajas. La primera es su capacidad para corregir el error asociado a la aplicación directa de la fórmula de cálculo del impedimento monoaural, que con valores audiométricos normales (<25dB)da resultados negativos y en caso de hipoacusia profunda (>90dB), resultados superiores al 100%. La segunda ventaja es que no se tiene que indicar cuál es el oído mejor antes de realizar el cálculo binaural hecho que evita la introducción de los valores de «oído mejor» «oído peor» en 2 celdas y supone el ahorro de alrededor de un 20% de tiempo total de cálculo. En conclusión, consideramos que en nuestras especialidades, la hoja de cálculo de Excel adaptada para la valoración del impedimento auditivo, resulta muy adecuada, reproduce los resultados de cualquier sistema de cálculo manual y minimiza la posibilidad de error, por lo que puede aportar un ahorro de tiempo y una mayor seguridad (AU)

In daily clinical practice, the results of audiometric tests often need to be converted into the percentage of hearing impairment in order to prepare health reports or to provide information requested by patients. Until now, manual systems have been used to calculate this percentage. To simplify and add precision to this calculation, we developed a spreadsheet using Microsoft Excel software and the American Academy of Otolaryngology's formula to determine hearing handicap. Compared with other systems, our spreadsheet provides two main advantages. First, it corrects the intrinsic problem of the original formula, which gives negative results in the presence of normal audiometries (values under 25dB) or results over 100 in the case of severe hypoacusis (values over 90dB). The second advantage is that there is no need to specify the best ear in order to calculate the global hearing handicap, thus avoiding the introduction of two values and saving 20% of the overall calculation time. In conclusion, we consider that the Excel spreadsheet, adapted for the evaluation of hearing handicap, could save time and be more accurate than manual methods, thus reducing the possibility of making mistakes (AU)

Auditory neuropathy in a low-risk population: a review of the literature.

OBJECTIVE: Collect all available published evidence on the prevalence of auditory neuropathy in the well baby population and calculate the contribution of this to the false negative rate of oto-acoustic emission based newborn hearing screening programs. METHOD: PubMed and EMBASE were searched for relevant articles published between 1996 and 2010. Medical Subject Headings terms included 'Auditory disease', 'Prevalence' and 'Child' and their relevant synonyms. Included were original studies, which focused on well babies and reported the prevalence of auditory neuropathy. RESULTS: Of 519 citations 4 articles met the inclusion criteria. The population based prevalence of auditory neuropathy in children in population hearing screening was found to vary between 0.006% (SD 0.006) and 0.03% (SD 0.02). The false negative rate, caused by missed children with auditory neuropathy, is between 4 and 17%. CONCLUSION: The available information on the prevalence of auditory neuropathy in the well baby population is poor. However, if oto-acoustic emission screening is used in the first stage of a neonatal hearing screening program, children with auditory neuropathy are missed. The cost-effectiveness of population-based screening using auditory brainstem response should be studied.