Vestibular Impairment and Postural Development in Children With Bilateral Profound Hearing Loss.

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing. Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL. Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023. Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay. Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment. Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

Universal neonatal hearing screening and delayed hearing loss or late-developmental hearing loss.

OBJECTIVE: To determine the percentage of children with permanent bilateral postnatal hearing loss in order to study its incidence, related risk factors, diagnosis and treatment. METHODS: Retrospective study to collect data on children diagnosed with hearing loss outside the neonatal period in the Hearing Loss Unit of the Hospital Universitario Central de Asturias, from April 2014 to April 2021. RESULTS: 52 cases met the inclusion criteria. The detection rate of congenital hearing loss in the neonatal screening programme in the same study period was 1.5 children per thousand newborns per year, adding postnatal hearing loss results in a rate of infant bilateral hearing loss of 2.7 children per thousand (55.5% and 44.4% respectively). Thirty-five children presented risk factors for hearing loss, of which 23 were at retrocochlear risk. The mean age at referral was 91.9 (18-185) months. Hearing aid fitting was indicated in 44 cases (84.6%). Cochlear implantation was indicated in eight cases (15.4%). DISCUSSION: Although congenital hearing loss accounts for the majority of childhood deafness, postnatal hearing loss has a significant incidence. This may be mainly due to: (1) that hearing impairment may arise in the first years of life, (2) that mild hearing loss as well as hearing loss in severe frequencies are undetectable by neonatal screening in some cases, (3) that some children may have false negative results. CONCLUSION: postnatal hearing loss requires identification of risk factors and long-term follow-up of children with hearing loss, as it needs to be detected and treated early.

Fatigue in Children With Unilateral and Bilateral Hearing Loss.

OBJECTIVE: To determine whether children with unilateral hearing loss (UHL) experience similar levels fatigue as children with bilateral hearing loss (BHL) or normal-hearing (NH). DESIGN: Cross-sectional study. SETTING: Two tertiary care otolaryngology practices. PARTICIPANTS: Children, 5 to 18 years old, with UHL or BHL and their parents. MAIN OUTCOME MEASURES: PedsQL Multidimensional Fatigue Scale (MFS) survey. RESULTS: Overall response rate was 90/384 (23%). Mean age of child participants was 10.7 years old (standard deviations [SD] 3.1); 38 (42%) were men and 52 (58%) were women. Sixty-nine (77%) children had UHL, 21 (23%) had BHL. Children with BHL (mean 65, SD 21) and UHL (mean 75, SD 17) reported greater levels of fatigue than children with NH (BHL difference -15, 95% confidence interval [CI] -25 to -5; UHL difference -6, 95% CI -13-1.2). Parent-proxy reports for children with BHL (mean 67, SD 20) and UHL (mean 76, SD 20) reported more fatigue than NH (BHL difference -22, 95% CI -33 to -12; UHL difference -14; 95% CI -20 to -8). Sub-section scores for general, sleep, and cognitive fatigue were higher for children with BHL and UHL than NH. CONCLUSION AND RELEVANCE: Children with UHL and BHL reported significantly more fatigue than children with NH, and children with BHL reported more fatigue than UHL. These findings underscore the need to increase auditory rehabilitation and educational resources for children with UHL and support the use of the PedsQL MFS questionnaire as a measure to follow disability experienced by children with HL as they undergo hearing rehabilitation.

Aetiologies of profound bilateral sensorineural hearing loss among children in Ekiti State, South Western Nigeria.

INTRODUCTION: a strong need exists for the knowledge of aetiologies of diseases as this will guide the clinicians on the strategies for their treatment and prevention. In this study, we determined the aetiologies of profound bilateral sensorineural hearing loss (pbSNHL) with a view to provide the relevant data needed for preventive and therapeutic intervention among children in Ekiti State, South Western Nigeria. METHODS: medical records of children with pbSNHL seen over a ten-year period were analysed. RESULTS: in all, records of 142 children with pbSNHL were analysed. The results showed spectrum of aetiologies that were similar to those reported decades ago with the 'unknown' assuming a recurring decimal. Of the known (acquired) cases, measles takes up the largest 'chunk' with a prevalence of 45.8%. Twenty-eight (32.2%) of those with febrile illnesses had treated their fever with ototoxic antibiotics. Late diagnosis was characteristic of the pbSNHL. CONCLUSION: this study shows that there hasn't been any significant shift in the common causes of pbSNHL. Of great concern is the persistence of the 'unknown' causes which assumes a recurring decimal in this and previous studies. Also worrisome is the high prevalence of measles-induced pbSNHL despite the availability of anti-measles vaccine. We therefore suggest effective immunization against measles and other vaccine-preventable causes of pbSNHL. The need for intensive research on the unknown causes of pbSNHL is hereby stressed. Also recommended is routine hearing assessment for survivors of febrile conditions so as to detect, if any, hearing loss promptly.

Hearing outcomes in children of diabetic pregnancies.

OBJECTIVE: Children of diabetic pregnancies (CDPs) face numerous risk factors for hearing loss (HL). The objective of this study was to investigate the hearing outcomes of CDPs on a population scale. METHODS: Using the Audiological and Genetic Database, the prevalence, severity, and progression of HL in CDPs was compared against children of non-diabetic pregnancies (CNDPs) who served as controls. RESULTS: Among 311 CDPs, 71.1% demonstrated evidence of HL compared to 45.5% in CNDPs (p < 0.001). The mean age at which CDPs received audiograms was 3.6 years compared to 5.4 years for CNDPs (p < 0.001). Compared to CNDPs, CDPs were similarly affected by common otologic conditions such as acute otitis media (25.7%), chronic otitis media (38.3%), and Eustachian tube dysfunction (41.8%) (all p > 0.05). CDPs were more likely to have bilateral HL (81%) and sensorineural hearing loss (SNHL) (8%) relative to CNDPs (p < 0.001 and p = 0.004, respectively). Rates of conductive HL and mixed HL were not significantly different between groups (p = 0.952 and p = 0.058, respectively). CDPs were at significant risk for the development of HL (aOR 1.66 [1.28-2.17], SNHL (aOR 1.63 [1.01-2.52], and high-frequency HL (aOR 1.32 [1.03-1.68]). Of the comorbidities evaluated, CDPs with hyperbilirubinemia (aOR 1.85 [1.18-2.84]), perinatal asphyxia (aOR 1.90 [1.06-3.16]), or congenital heart disease (aOR 1.21 [1.07-1.37]) demonstrated higher risk of SNHL. CONCLUSION: Children of diabetic pregnancies face increased risks of developing HL, particularly bilateral and sensorineural hearing loss. Given these findings, we recommend close audiologic follow-up for these children, especially those with complicated birth histories or additional medical problems.

Prevalence of permanent neonatal hearing impairment: systematic review and Bayesian meta-analysis.

Objective: To investigate the variance in reported prevalence rates of permanent neonatal hearing impairment (HI) worldwide.Design: A systematic review and meta-analysis was performed on reported prevalence rates of sensorineural and permanent conductive or mixed HI worse than 40 dB in neonates, detected as a result of a screening programme or audiometric study.Study sample: For meta-analysis, 35 articles were selected, 25 from high-income countries and 10 from middle-income countries according to the world bank classification system.Results: The prevalence rate of permanent uni- and bilateral HI worse than 40 dB in neonates varied from 1 to 6 per 1000, the overall prevalence was 2.21 per 1000 [1.71, 2.8]. In NICU populations the prevalence rate was higher with a larger fraction of bilateral cases. Although not significant, prevalence rates were slightly higher in Asia compared to Europe and the number of infants lost to follow-up appeared higher in countries with lower gross national income.Conclusion: Substantial variations exist in prevalence rates of neonatal permanent HI across countries and regions. There is a strong need for more data from low-income countries to identify demographic factors that account for this variability in reported prevalence rates. Reporting these data in a uniform way is advocated.

Genetic Testing for Congenital Bilateral Hearing Loss in the Context of Targeted Cytomegalovirus Screening.

OBJECTIVES/HYPOTHESIS: To determine the prevalence of children with genetic hearing loss who are cytomegalovirus (CMV) positive at birth and the relative proportion of genetic and CMV etiology among children with congenital bilateral hearing loss. STUDY DESIGN: Database review. METHODS: We performed a review of clinical test results for patients undergoing comprehensive genetic testing for all known hearing loss-associated genes from January 2012 to January 2019. This population was reviewed for reported CMV status and genetic causes of congenital bilateral hearing loss. RESULTS: In the OtoSCOPE database, 61/4,282 patients were found to have a documented CMV status, and 661/4282 had documented bilateral congenital hearing loss. Two patients were identified who had both a positive CMV result and a genetic cause for their hearing loss. Forty-eight percent of patients with bilateral congenital hearing loss (320/661) were found to have a genetic etiology. In 62% (198/320), the hearing loss was associated with pathogenic variants in GJB2, STRC, SLC26A4 or an Usher syndrome-associated gene. CONCLUSIONS: We estimate that ~2% of CMV-positive newborns with hearing loss have a known genetic variant as a cause. The subcohort of CMV-positive newborns with symmetric mild-to-moderate bilateral hearing loss will have at least a 7% chance of having pathogenic gene variants associated with hearing loss. In a CMV-positive neonate who failed their newborn hearing screen bilaterally, genetic screening needs to be considered for accurate diagnosis and possible deferment of antiviral treatment. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2714-2718, 2020.

Outcomes of regional-based newborn hearing screening for 35,461 newborns for 5 years in Akita, Japan.

OBJECTIVES: Newborn hearing screening (NHS) has been actively performed since 2001 in Akita, Japan. The NHS coverage rate has increased yearly, and performance has been consistently >90% since 2012. The purpose of this study was to summarize NHS outcomes in the Akita prefecture of Japan and to obtain new insights for from our summarized data for the future. METHODS: A total of 35,461 newborns in hospitals and clinics where hearing screening was performed in Akita from 2012 to 2016 were included. The outcome data of NHS were collected for analysis. RESULTS: The overall screening coverage rate for hearing loss was 94.7%. Of the screened infants, 0.53% received a referral on the 2-stage automated auditory brainstem response (ABR), and 80.4% of referred infants had a check-up at the hospital to receive a diagnostic hearing examination. Finally, the prevalence of bilateral congenital hearing loss was 0.14%, that of bilateral moderate to profound hearing loss was 0.12%, and that of unilateral congenital hearing loss was 0.10%. Furthermore, the average consultation period in infants with risk factors was significantly later than that in infants without risk factors (p = 0.0015). Follow-up for infants diagnosed with normal hearing after diagnostic hearing examination revealed that 4.7% suffered bilateral moderate to profound hearing loss later. This percentage is significantly higher than that of the general group (p < 0.001). CONCLUSION: The prevalence of bilateral congenital hearing loss was 0.14% in Akita and 0.12% of infants were diagnosed with bilateral moderate to severe hearing loss. Medical personnel should be enlightened regarding the importance of performing hearing diagnostic examinations until 3 months of age. Even if infants were diagnosed with normal hearing after a diagnostic examination, we strongly suggest continuing follow-up until they are able to perform pure tone audiometry with accuracy.

Progressive Hearing Loss in Children With Mild Bilateral Hearing Loss.

Purpose This study investigated progressive hearing loss in a cohort of children who were identified with permanent mild bilateral hearing loss. Method This population-based study included 207 children with permanent mild bilateral hearing loss, diagnosed and followed from 2003 to 2016 in 1 region of Canada. Clinical characteristics and initial audiologic results were collected prospectively at diagnosis, and audiologic information was updated. Changes in hearing levels between the 1st and most recent assessment were analyzed to determine progressive hearing loss. Clinical characteristics were compared between children with progressive and stable hearing loss. The association between risk indicators and progressive hearing loss was explored through logistic regression. Results A total of 47.4% (94 of 207) had progressive hearing loss in at least 1 ear, and 50% (47 of 94) of those experienced more than 20-dB average drop in thresholds. For these 94 children, a total of 147 ears were affected, and 116 (78.9%) ears experienced sufficient change in thresholds to be coded as a worse category of hearing loss. In the subset of 85 children with more than 5 years of audiologic follow-up, 56.4% (53/85) showed a decrease in hearing. Of the total sample of 207 children, 137 (66.2%) continued to have mild hearing loss in the better ear. There was no association between the risk factors examined (family history of hearing loss, admission to the neonatal intensive care unit, or presence of a syndrome) and progressive hearing loss. Conclusion This study found that almost half of children with mild bilateral hearing loss showed a decrease in hearing in at least 1 ear. One third of the children first diagnosed with mild hearing loss in the better ear now have moderate or worse hearing loss in both ears. These findings point to the importance of careful long-term monitoring of children who present with mild hearing loss.

Where Do We Go From Here? Some Messages to Take Forward Regarding Children With Mild Bilateral and Unilateral Hearing Loss.

Purpose This epilogue discusses messages that we can take forward from the articles in the forum. A common theme throughout the forum is the ongoing need for research. The forum begins with evidence of potential progressive hearing loss in infants with mild bilateral hearing loss, who may be missed by current newborn hearing screening protocols, and supports the need for consensus regarding early identification in this population. Consensus regarding management similarly is a continuing need. Three studies add to the growing body of evidence that children with mild bilateral or unilateral hearing loss are at risk for difficulties in speech understanding in adverse environments, as well as delays in language and cognition, and that difficulties may persist beyond early childhood. Ambivalence regarding if and when children with mild bilateral or unilateral hearing loss should be fitted with personal amplification also impacts management decisions. Two articles address current evidence and support the need for further research into factors influencing decisions regarding amplification in these populations. A third article examines new criteria to determine hearing aid candidacy in children with mild hearing loss. The final contribution in this forum discusses listening-related fatigue in children with unilateral hearing loss. The absence of research specific to this population is evidence for the need for further investigation. Ongoing research that addresses difficulties experienced by children with mild bilateral and unilateral hearing loss and potential management options can help guide us toward interventions that are specific for the needs of these children.

Prevalence and clinical aspects of hearing loss among the South Korean adolescent: Data from a population-based study.

OBJECTIVES: Slight/mild hearing loss is detrimental to communication and academic achievements. This study aimed to describe the prevalence of hearing loss, and to investigate the factors related to noise-induced hearing loss among South Korean adolescent. METHODS: As a population-based retrospective study, 1845 South Korean adolescents aged from 12 to 19 years were analyzed using the data from Korea National Health and Nutrition Examination Survey V (KNHANES V, 2010-2012). The prevalence of hearing loss according to the side, severity, and frequency was calculated. For assessing the noise-induced hearing loss in adolescent, the prevalence of hearing loss only in high-frequency (onlyHFHL, defined as 1) thresholds at 0.5 and 1 kHz of ≤15 dB HL, and 2) maximal thresholds at 3, 4, or 6 kHz ≥ 15 dB HL higher than the highest threshold for 0.5 and 1 kHz) was analyzed. Moreover, the relevance of onlyHFHL in context of sociodemographic factors and noise exposure history was evaluated. RESULTS: The prevalence of unilateral and bilateral hearing loss based on the average of six frequencies (0.5, 1, 2, 3, 4, and 6 kHz) in South Korean adolescents were 8.56% and 1.03%, respectively, and most cases were hearing loss with slight/mild degree. The prevalence of unilateral and bilateral onlyHFHL were 32.74% and 5.53%, respectively. Factors found to be associated with onlyHFHL were sex (female) and household income (high). CONCLUSIONS: According to this population-based study, the prevalence of slight/mild hearing loss and onlyHFHL in the South Korean adolescents were considerably high. With knowledge of the factors related with onlyHFHL, paying more attention to slight/mild hearing loss will be helpful in preventing hearing loss in adolescents.

Progression of hearing loss and choice of hearing aids by patients in their 60s, 70s, and 80s and older: experience in the Japanese super-aged era.

Background: Demographic data of patients with sensorineural hearing loss (SNHL) in super-aged societies are still limited.Aims/objectives: To report audiometric statistics of SNHL and hearing aid (HA) use in patients in their 60s, 70s, and 80s and older during the super-aged era.Material and methods: Medical charts and audiograms of 2064 older patients with SNHL who visited a Japanese University Hospital in 2007-2018 were retrospectively reviewed. Among 270 patients referred to the HA service unit (HASU), the percentage of final decisions to continue using HAs was calculated.Results: The average pure tone thresholds on initial visit to the clinic were 56.9, 60.6, 69.4, and 82.4 dB HL in patients in their 60s, 70s, 80s, and 90s, respectively. The rates of progression were 0.25, 0.87, 1.19, and 1.37 dB/year in patients in their 50s, 60s, 70s, and 80s, respectively. The percentage of patients in HASU who chose to use HAs did not differ among the 60s (59.3%), 70s (51.2%), and 80s and older (58.2%).Conclusions and significance: The clinical picture of patients with SNHL in their 70s and 80s differs because progression accelerates exponentially through these ages. HAs can be recommended to older adult patients in all the age groups.

Hearing loss in ankylosing spondylitis.

AIM: Several studies have described sensorineural deafness in ankylosing spondylitis (AS) patients while conductive hearing loss has been reported to be rare. In our study we have studied the prevalence of hearing loss (HL) among AS patients and its association with non-steroidal anti-inflammatory drug (NSAID) intake. METHODS: We studied 100 AS patients and 40 controls. HL was defined as pure-tone thresholds greater than 20 dB (decibel) in at least two frequencies of the audiogram. Severity of HL was defined as given by World Health Organization. Clinical details and cumulative NSAID doses were noted. RESULTS: Ninety-six of the 100 patients were male with mean age of 32 ± 12 years and mean duration of illness of 8.2 ± 6 years. Median Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Metrology Index (BASMI) and cumulative NSAID dose were 3.5 ± 2.2, 2.75 ± 2, 3.7 ± 2.6 and 1202 ± 1290, respectively. Of the 48 with HL, 28 patients had bilateral HL. Twenty-nine patients had pure conductive HL while 16 had mixed HL (components of both sensory and conductive) and 3 had pure sensorineural HL. HL was mild in 38 patients, and moderate to severe in 10 patients. The presence of HL was associated with higher age (P ≤ 0.05). Conductive HL was at low frequency (0.25, 0.5, 1 kHz) in 70% of cases. Sensorineural HL was at high frequency (4, 8 kHz) in 75% of cases. There was no association of HL with disease parameters such as BASMI, BASDI, BASFI or cumulative NSAID dose. CONCLUSION: HL is common in AS. Conductive HL is more common than sensorineural HL. It is usually mild and occurs at low frequencies.

The emergence and prevalence of hearing loss in children with homozygous sickle cell disease.

OBJECTIVES: Many children with sickle cell disease (SCD) experience the effect of cochlear insult due to hemolytic anemia and vaso-occlusion that accompanies the disease. As a result, hearing sensitivity eventually decreases. The purpose of this study was to investigate the emergence of hearing loss and the prevalence of such in children with homozygous SCD. METHODS: A large pediatric database was utilized to gather audiometric outcomes in children with homozygous SCD. Children were identified by primary diagnosis ICD codes. Demographic and audiometric data was drawn to determine presence, type, degree, and laterality of hearing loss. Hearing loss was defined as an elevated audiometric threshold >15 dB HL for pure tone air conduction audiometry or >20 dB HL minimal response level for sound-field testing. RESULTS: One hundred and twenty-eight children were identified. The prevalence of hearing loss ranged from 28.8% to 50.8% depending on the calculation method (i.e., individual vs. ear specific prevalence and any elevated threshold vs. a three-frequency pure tone average). Conductive hearing loss and bilateral loss were most prevalent. The degree of hearing loss ranged from slight to profound. The mean age of identification of sensorineural hearing loss was 9.6 years. CONCLUSION: The prevalence of hearing loss in children with homozygous SCD is higher than in normal children. Regular hearing assessments of children with SCD is warranted and should be advocated in early infancy and as part of their ongoing care.

An Analysis of Risk Factors in Unilateral Versus Bilateral Hearing Loss.

A retrospective review of children with confirmed hearing loss identified through universal newborn hearing screening (UNHS) in Virginia from 2010 to 2014 was conducted in order to compare the incidence of Joint Committee on Infant Hearing (JCIH) risk factors in children with unilateral hearing loss (UHL) to bilateral hearing loss (BHL). Over the 5-year study period, 1004 children (0.20% of all births) developed a confirmed hearing loss, with 544 (51%) children having at least one JCIH risk factor. Overall, 18% of children with confirmed hearing loss initially passed UNHS. Of all children with risk factors, 226 (42%) demonstrated UHL and 318 (58%) had BHL. The most common risk factors for UHL were neonatal indicators (69%), craniofacial anomalies (30%), stigmata of HL syndromes (14%), and family history (14%). The most common risk factors in BHL were neonatal indicators (49%), family history (27%), stigmata of HL syndromes (19%), and craniofacial anomalies (16%). Children with the risk factor for positive family history were more likely to have BHL, while those with craniofacial anomalies were more likely to have UHL (P < .001). Neonatal indicators were the most commonly identified risk factor in both UHL and BHL populations. Children with UHL were significantly more likely to have craniofacial anomalies, while children with BHL were more likely to have a family history of hearing loss. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.

Audiometric findings in children with unilateral enlarged vestibular aqueduct.

OBJECTIVE: To evaluate the prevalence of bilateral hearing loss in children with unilateral enlarged vestibular aqueduct (EVA) at a single institution. METHODS: A retrospective case review was performed at a tertiary care pediatric referral center involving children with radiologic findings of unilateral EVA and normal labyrinthine anatomy of the contralateral ear diagnosed via CT and/or MRI. The main outcome measure of interest is the number of patients with unilateral EVA who were diagnosed with bilateral hearing loss. RESULTS: Sixty-one pediatric patients were identified. The mean audiometric follow-up was 48.2 months (0-150). Three (4.9%) patients with unilateral EVA were noted to have bilateral hearing loss, and this rate was not significantly different (p = 1.0) from the rate reported in a comparison group of patients with contralateral hearing loss (6.0%) without an EVA. The pure-tone average (defined as the average dB HL at 500, 1000, 2000, and 4000 Hz) in the group with bilateral hearing loss was 31.3 dB HL in the better hearing ear and 79.6 dB HL in the worse hearing ear, with the difference being statistically significant (p = 0.02). In the unilateral EVA patients without contralateral hearing loss (n = 56, 91.8%), the PTA was 9.4 dB HL in the better hearing ear and 51.9 dB HL in the worse hearing ear, with the difference being statistically significant (p < 0.001). Two patients (3.3%) with unilateral EVA were found to have hearing within normal limits bilaterally. The EVA was ipsilateral to the worse hearing ear in all cases. CONCLUSION: The prevalence of bilateral hearing loss in children with unilateral EVA appears to be low. Specifically, it may be no different than the rate of contralateral hearing loss in children with unilateral hearing loss without an EVA. The present report is somewhat different than the previously described prevalence in the literature. This difference could be related to the imaging type and diagnostic criteria used, the patients included, the source of the identified patents, and the overall population of patients studied.

Medical Referral Patterns and Etiologies for Children With Mild-to-Severe Hearing Loss.

OBJECTIVES: To (1) identify the etiologies and risk factors of the patient cohort and determine the degree to which they reflected the incidence for children with hearing loss and (2) quantify practice management patterns in three catchment areas of the United States with available centers of excellence in pediatric hearing loss. DESIGN: Medical information for 307 children with bilateral, mild-to-severe hearing loss was examined retrospectively. Children were participants in the Outcomes of Children with Hearing Loss (OCHL) study, a 5-year longitudinal study that recruited subjects at three different sites. Children aged 6 months to 7 years at time of OCHL enrollment were participants in this study. Children with cochlear implants, children with severe or profound hearing loss, and children with significant cognitive or motor delays were excluded from the OCHL study and, by extension, from this analysis. Medical information was gathered using medical records and participant intake forms, the latter reflecting a caregiver's report. A comparison group included 134 children with normal hearing. A Chi-square test on two-way tables was used to assess for differences in referral patterns by site for the children who are hard of hearing (CHH). Linear regression was performed on gestational age and birth weight as continuous variables. Risk factors were assessed using t tests. The alpha value was set at p < 0.05. RESULTS: Neonatal intensive care unit stay, mechanical ventilation, oxygen requirement, aminoglycoside exposure, and family history were correlated with hearing loss. For this study cohort, congenital cytomegalovirus, strep positivity, bacterial meningitis, extracorporeal membrane oxygenation, and loop diuretic exposure were not associated with hearing loss. Less than 50% of children underwent imaging, although 34.2% of those scanned had abnormalities identified. No single imaging modality was preferred. Differences in referral rates were apparent for neurology, radiology, genetics, and ophthalmology. CONCLUSIONS: The OCHL cohort reflects known etiologies of CHH. Despite available guidelines, centers of excellence, and high-yield rates for imaging, the medical workup for children with hearing loss remains inconsistently implemented and widely variable. There remains limited awareness as to what constitutes appropriate medical assessment for CHH.

Implementing universal newborn hearing screening in the French Rhône-Alpes region. State of affairs in 2016 and the 1st half of 2017.

INTRODUCTION: Universal newborn hearing screening (UNHS) started as public health policy in 2015 in the French Rhône-Alpes region, aiming to screen for unilateral and bilateral hearing loss. After a first and second screening (retest) in the maternity hospital, the diagnostic process occurred at a limited number of specialist centers. A deferred preliminary screening (T3) was proposed before the age of 1 month. The aims of this study were to assess implementation of the program, impact of T3, and present the incidence of hearing loss in this population. MATERIALS AND METHODS: The retrospective observational study was based on data transmitted routinely by the 51 maternities to the regional organization responsible for newborn screening, in 2016 and first half of 2017. RESULTS: All the facilities implemented the UNHS protocol, with 47 out of 51 using the recommended techniques. 99.7% of the 115,435 newborns were screened (excluding 0.2% of parental refusals). A retest was required for 10.2% of the babies. Among babies who didn't pass retest, 7.7% were lost to follow-up. 2.2% of the newborns were referred to diagnostic centers. The rate of T3 was 31.3% of newborns who did not pass retest. 88.6% of the infants passed T3. In the perinatal network making extensive use of T3 (75.8% versus 14.9% elsewhere), 0.6% of the infants were referred to a diagnostic center, versus 2.9% in the rest of the region (2016, p < 0.001). For 2016, the outcomes at 6 months revealed an overall hearing loss rate of 1.7‰ (4.7‰ for neonatal care unit babies), and bilateral hearing loss in 1.2‰. CONCLUSION: In Rhône-Alpes, the national and regional objectives for UNHS were exceeded, although limiting the number of infants lost to follow-up remains essential. Repeating an automated test around 2-4 weeks after birth improves the program by decreasing the false positives of the screening. It considerably limits the number of infants referred to specialist centers, without increasing the number of patients lost to follow-up.

Cross-sectional epidemiology of hearing loss in Australian children aged 11-12 years old and 25-year secular trends.

OBJECTIVE: In a national study of Australian children aged 11-12 years old, we examined the (1) prevalence and characteristics of hearing loss, (2) its demographic risk factors and (3) evidence for secular increases since 1990. METHODS: This is a cross-sectional CheckPoint wave within the Longitudinal Study of Australian Children. 1485 children (49.8% retention; 49.7% boys) underwent air-conduction audiometry. Aim 1: hearing loss (≥16 decibels hearing level (dB HL)) was defined in four ways to enable prior/future comparisons: high Fletcher Index (mean of 1, 2 and 4 kHz; primary outcome relevant to speech perception), four-frequency (1, 2, 4 and 8 kHz), lower frequency (1 and 2 kHz) and higher frequency (4 and 8 kHz); aim 2: logistic regression of hearing loss by age, gender and disadvantage index; and aim 3: P for trend examining CheckPoint and reported prevalence in studies arranged by date since 1990. RESULTS: For high Fletcher Index, the prevalence of bilateral and unilateral hearing loss ≥16 dB HL was 9.3% and 13.3%, respectively. Slight losses (16-25 dB HL) were more prevalent than mild or greater (≥26 dB HL) losses (bilateral 8.5% vs 0.8%; unilateral 12.5% vs 0.9%), and lower frequency more prevalent than higher frequency losses (bilateral 11.0% vs 6.9%; unilateral 15.4% vs 11.5%). Demographic characteristics did not convincingly predict hearing loss. Prevalence of bilateral/unilateral lower and higher frequency losses ≥16 dB HL has risen since 1990 (all P for trend <0.001). CONCLUSIONS AND RELEVANCE: Childhood hearing loss is prevalent and has risen since 1990. Future research should investigate the causes, course and impact of these changes.

Comparison between Bilateral and Unilateral Sudden Sensorineural Hearing Loss.

BACKGROUND: Bilateral sudden sensorineural hearing loss (BSSHL) is rare and assumed to be a different clinical entity compared to unilateral SSHL (USSHL). This study examined the differences between the idiopathic BSSHL and USSHL. METHODS: Forty-six sequential BSSHL patients (Se-BSSHL) and 68 simultaneous BSSHL (Si-BSSHL) were consecutively admitted between June 2008 and December 2015. Two sets of patients served as control groups: (1) USSHL patients with healthy contralateral ear and (2) USSHL patients with contralateral preexisting hearing loss (USSHLwCHL). We retrospectively analyzed differences among four cohorts using analysis of variance, Kruskal-Wallis test, Welch's t-test, and Chi-square test as appropriate before and after propensity score matching (PSM) based on age, gender, and body mass index (BMI). RESULTS: The prevalence of idiopathic BSSHL was 8.6% (114/1329) among the total SSHL patients. In the total cohort, USSHL patients tended to be younger, female, and tended to have lower BMI, renal parameters, and total cholesterol in addition to higher high-density lipoprotein compared to the other three groups. Most routine blood indicators, some coagulation markers, and immunoglobulin M (H = 13.4, P = 0.004) were significantly different among the study groups. After PSM, the major significant differences were found in audiometric characteristics. Si-BSSHL and Se-BSSHL patients demonstrated similar hearing thresholds as USSHL but were significantly better than the USSHLwCHL patients across most frequencies before and after treatment (H = 30.0, P < 0.001 for initial hearing and H = 12.0, P = 0.007 for final hearing). Moreover, the BSSHL patients showed different hearing loss distribution patterns (more descending type, χ2 = 33.8, P = 0.001) with less hearing gain (H = 17.5, P < 0.001) compared to the USSHL patients. CONCLUSIONS: Idiopathic BSSHL is a relatively rare subtype of SSHL with a higher rate of descending audiogram type and inferior hearing outcome rather than being classified as a completely different disease entity compared to USSHL.