Hearing Loss In Infants Research Articles

Change to Hearing Loss–Related Risks and Screening in Preterm Infants

Hearing loss is one of the most common congenital defects in infancy; it increases speech and language delays and adversely affects academic achievement and socialemotional development. The risk of hearing loss in premature infants is higher than that in normal newborns, and because of the fragility of the auditory nervous system, it is more vulnerable to different risk factors. The hearing screening guidelines in current use were proposed by the American Academy of Pediatrics and updated in 2007, but there are no uniform guidelines for hearing screening in preterm infants. This review focuses on the risk factors related to hearing loss in premature infants, hearing screening strategies, and reasons for failure. The aim is to provide a more comprehensive understanding of hearing development in preterm infants to achieve early detection and early intervention. At the same time, attention should be paid to delayed auditory maturation in preterm infants to avoid excessive intervention. KEY POINTS: · Hearing loss is very common in infancy, especially in premature infants.. · Genetic factors, infection, hyperbilirubinemia, drugs, and noise are the main causes.. · We should pay attention to the delayed hearing maturity of premature infants and avoid excessive intervention..

Effective Masking Levels for Bone Conduction Auditory Brainstem Response Stimuli in Infants and Adults with Normal Hearing.

The purpose of the present study was to investigate effective masking levels (EMLs) for bone conduction (BC) auditory brainstem response (ABR) testing in infants and adults. Early hearing detection and intervention programs aim to limit delays in identifying ear-specific type/degree of hearing loss in infants using the ABR. Ear-specific assessment poses challenges as sound delivered to one ear can travel across the skull and activate the contralateral cochlea. Wave V amplitude and latency measures ipsilateral and contralateral to the bone oscillator can be compared to isolate the test cochlea in some cases; however, when these findings are equivocal, clinical masking is required. This study aims to determine EMLs for ABRs elicited to 500- and 2000-Hz BC stimuli for normal-hearing infants (0 to 18 months) and adults. Participants were 21 adults (18 to 54 years) and 24 infants (5 to 47 weeks) with normal hearing. BC 500- and 2000-Hz brief tonal stimuli at intensities approximating normal levels were presented via a B-71 oscillator (infants: 20 dB nHL at 500 Hz and 30 dB nHL at 2000 Hz; adults: 500 and 2000 Hz at 20 and 30 dB nHL, respectively). White noise masking was presented binaurally via ER-3A earphones (22 to 82 dB SPL; 10-dB steps). The lowest level of masking to eliminate a BC response was deemed the EML. For stimuli presented at 20 dB nHL, adult mean (1 SD) EMLs for 500 and 2000 Hz were 65 (9) and 53 (6) dB SPL, respectively. Mean EMLs for infants were 80 (6) dB SPL for 500 Hz at 20 dB nHL and 64 (9) dB SPL for 2000 Hz at 30 dB nHL. Compared to adults, infants required approximately 13 dB more masking at 500 Hz but a similar amount of masking at 2000 Hz. Infants required 26 dB more masking at 500 versus 2000 Hz, whereas, adults required only 12 dB more masking. Maximum binaural EMLs for infant BC responses elicited to 500 Hz at 20 dB nHL are 82 dB SPL, and for 2000 Hz at 30 and 40 dB nHL, respectively, are 72 and 82 dB SPL. Monaural masking levels for the nontest ear (assuming 10 dB of interaural attenuation) recommended clinically are as follows: (1) 500 Hz: 72 and 82 dB SPL at 20 and 30 dB nHL, respectively; and (2) 2000 Hz: 62, 72, and 82 dB SPL at 30, 40, and 50 dB nHL, respectively. Unsafe levels of white noise would be needed to effectively mask at greater stimulus levels.

Current Approaches to the Management of Usher Syndrome for the Clinician

Purpose More than 460 million people suffer from disabling hearing loss worldwide, and about 50%–60% of hearing loss in infants are due to genetics. Usher syndrome is a genetic condition in which children are born deaf or hard of hearing and subsequently develop retinitis pigmentosa. It is the most common cause of deafness and blindness in pediatric populations, and pathogenic variants in 10 different genes are shown to be causative. The purpose of this review article is to advance the understanding of the clinical symptoms of an individual with Usher syndrome and highlight the latest research in the development of new treatments including gene therapy. The care and management of individuals with Usher syndrome require a team of multidisciplinary health care professionals, including an audiologist, a speech-language pathologist, an ophthalmologist, and a medical geneticist. We highlight the importance of early identification for better speech and developmental outcomes, specifically in pediatric individuals. Conclusion This review empowers audiologists to be aware of early signs of Usher syndrome as they are typically the first point of contact and provides important information for audiologists and other health care professionals involved in the care of individuals with hearing loss and vision loss.

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.

Four patients with D-bifunctional protein (DBP) deficiency: Expanding the phenotypic spectrum of a highly variable disease

IntroductionPeroxisomal D-bifunctional protein (DBP) deficiency is an autosomal recessive disorder historically described as a Zellweger-like syndrome comprising neonatal seizures, retinopathy, hearing loss, dysmorphic features, and other complications. The HSD17B4 gene encodes DBP which is essential for oxidation of peroxisomal substrates. We describe 4 patients - 2 unrelated female girls and 2 monozygotic twin sisters - with DBP deficiency and phenotypic diversity. Patient reportsPatient 1 presented neonatally with hypotonia and seizures, and later on developed global developmental delay and regression, sensorineural hearing loss, nystagmus and cortical blindness. The brain MRI demonstrated bilateral peri-sylvian polymicrogyria. Whole exome sequencing revealed 2 mutations in the HSD17B4 gene (c.752G>A, p.(Arg251Gln); c.868 + 1delG).Patient 2 presented with hypotonia, motor delay, and sensorineural hearing loss in infancy, considerable developmental regression during her fourth year, nystagmus, and peripheral neuropathy. Brain MRI demonstrated cerebellar atrophy and abnormal basal ganglia and white matter signal, which appeared after the age of two years. Whole exome sequencing revealed 2 mutations in the HSD17B4 gene (c.14 T>G, p.(Leu5Arg); c.752G>A, p.(Arg251Gln)).Patients 3 and 4, two female monozygotic twins, presented with hypotonia, developmental delay, and macrocephaly from birth, and later on also sensorineural hearing loss, infantile spasms and hypsarrhythmia, and adrenal insufficiency. Brain MRI demonstrated delayed myelination, and an assay of peroxisomal beta oxidation suggested DBP deficiency. Sequencing of the HSD17B4 gene revealed the same 2 mutations as in patient 1. DiscussionWe describe 4 patients with variable and diverse clinical picture of DBP deficiency and particularly emphasize the clinical, biochemical, and neuroimaging characteristics. Interestingly, the clinical phenotype varied even between patients with the exact two mutations in the HSD17B4 gene. In addition, in two of the three patients in whom levels of VLCFA including phytanic acid were measured, the levels were within normal limits. This is expanding further the clinical spectrum of this disorder, which should be considered in the differential diagnosis of every patient with hypotonia and developmental delay especially if accompanied by polymicrogyria, seizures, sensorineural hearing loss, or adrenal insufficiency regardless of their VLCFA profile.

The Hearing Status of Preterm Infant's ≤ 34 Weeks as Revealed by Otoacoustic Emissions (OAE) Screening and Diagnostic Brainstem Evoked Response Audiometry (BERA): A Tertiary Center Experience.

To know the prevalence of hearing loss in preterm infants & to evaluate the sensitivity & specificity of otoacoustic emission (OAE) in detecting hearing loss in preterm infants ≤ 34weeks. A total of 70 preterm babies from 28 to 34weeks of gestational age were enrolled in the study. Detailed prenatal, perinatal, postnatal & family history and physical examination of the babies were carried out. First OAE was done at discharge or two weeks after birth, whichever was earlier and the second OAE assessment was done at 36-40weeks of corrected age. Diagnostic brainstem evoked response audiometry (BERA) was done in all infants at 36-40weeks of corrected age, at the time of second OAE. Neonates with hearing impairment were advised for early hearing aid amplification and were referred to the rehabilitation center for further management. The sensorineural hearing loss (SNHL) in either one of the ears was identified in 13 (18.57%) preterm babies. Bilateral profound SNHL was found in 5 (7.14%) babies. Auditory neuropathy profile was found in 7 (53.8%) out of 13 babies who had hearing loss. The sensitivity, specificity, positive and negative predictive values of second TEOAE compared with diagnostic BERA was 46.15%, 85.96%, 42.85%, and 87.5% respectively. Neonatal jaundice (p = 0.009) and history of exchange transfusion (p = 0.019) were found to be significant risk factors of hearing loss in our study. Other risk factors like mode of delivery, birth asphyxia, low APGAR score, meningitis, ototoxic drugs, and seizures were not associated with hearing loss. The prevalence of hearing loss in preterm infants < 34weeks is very high. OAE alone is not an ideal screening test for high-risk neonates ≤ 34weeks because of its low sensitivity. OAE combined with diagnostic BERA should be done in all high-risk infants preterm neonates ≤ 34weeks to identify cases of auditory neuropathy spectrum disorders.

Comparison of newborn hearing screening results between well babies and neonates admitted to the neonatal intensive care unit for more than 5 days: Analysis based on the national database in Korea for 9 years.

ObjectiveThe purpose of this cohort study is to compare newborn hearing screening (NHS) results between healthy newborns and neonates who were admitted to the neonate intensive care unit (NICU) for more than 5 days based on the national database for 9 years. Ultimately, we’ve tried to analyze the associated factors necessary to manage the national NHS program according to the group, which would help to establish policy to effectively detect and support hearing impaired children and which would help to control qualities.MethodsThe Ministry of Health and Welfare (MHW) introduced a nationwide coupon-mediated program for the low-income class since 2009. The coupon consisted of two parts, the screening part and the confirming parts with the same unique number, and the MHW supported the cost of one screening test and one diagnostic auditory brainstem response (ABR) test for infants who did not pass from the screening test. We have analyzed the screening test performing rate, the referral rate according to the screening methods or institutions, the prevalence of hearing loss, and the average age of hearing loss diagnosis. Hearing loss was defined as any hearing impairment either unilateral or bilateral with the hearing threshold ≥ 40 dB nHL on the diagnostic ABR test, irrespective of its etiology.ResultsA total of 524,371 newborns were enrolled in the study, and 506,634 (96.6%) neonates were in the “well-baby group (WBG)”, while 17,737 (3.4%) were in the “high-risk group (HRG)”. The referral rate of the screening test was 1.5% in average, 1.3% in the WBG, and 7.5% in the HRG. The referral rates varied according to the screening methods and screening institutions. The adjusted prevalence of HL was 5.6/1,000 in average, 4.6/1,000 in the WBC, and 28.8/1,000 in the HRG. The screening tests were performed 4.3 ± 6.7 days after birth and the diagnostic tests were done 62.7 ± 37.5 days after birth in WBG. In HRG, dates were 17.7 ± 19.3 days and 97.6 ± 51.4 days, respectively.ConclusionsThe prevalence of hearing loss in infants who were hospitalized in NICU for more than 5 days was about seven times higher than that in healthy newborns. However, different referral rates were noted depending on both institutions and the screening methods. These differences need to be addressed in order to improve our program and ensure that all neonates with hearing loss, especially neonates with high risk factor, are detected and appropriately referred for the treatment.

Accuracy of MR Imaging for Detection of Sensorineural Hearing Loss in Infants with Bacterial Meningitis.

Bacterial meningitis most commonly affects young children and can result in critical adverse outcomes, including sensorineural hearing loss (SNHL). The purpose of this study is to determine the diagnostic accuracy of MR imaging for predicting the development of SNHL among infants with bacterial meningitis. A retrospective review was performed among infants (age <365 days) with bacterial meningitis (n = 115). Independent and consensus blinded review of brain MRIs (n = 239) performed less than 90 days from presentation were conducted. Abnormal appearance of the inner ear was defined as enhancement on postcontrast T1-weighted (T1-weighted+C) sequence and FLAIR hyperintensity. The consensus MR imaging appearance of the inner ear on FLAIR, T1-weighted+C, and combined evaluation was compared with criterion standard audiometric testing to determine the sensitivity and specificity of MR imaging for detecting SNHL. The mean age at diagnosis of bacterial meningitis was 50.6 days (range, 0-338 days) and 24.3% had SNHL. Sensitivity and specificity was 0.61/0.96, 0.50/0.94, and 0.61/0.94 for T1-weighted+C, FLAIR hyperintensity, and combined evaluation, respectively, for prediction of SNHL. There was excellent interobserver agreement for both the T1-weighted+C and FLAIR sequences and combined evaluation for presence of abnormal enhancement and hyperintense signal, respectively. Factors associated with abnormal MR imaging findings on T1-weighted+C and/or FLAIR in patients with SNHL included low CSF glucose (P = .04, .02) and high CSF protein (P = .04, .03). Abnormal enhancement and/or FLAIR hyperintensity of the inner ear demonstrate high specificity and average sensitivity for prediction of SNHL among infants with bacterial meningitis.

Congenital Cytomegalovirus and Hearing Loss

Congenital cytomegalovirus (cCMV) is the most common cause of nongenetic, sensorineural hearing loss and neurodevelopmental disorders in newborns, infants, and children. Current cCMV screening procedures for pregnant women and newborns are not standardized, which may be attributed to the lack of knowledge about the consequences of infection among clinicians and the general public and the lack of effective treatments. The purpose of this article is to provide a review and update on cCMV infection epidemiology, diagnostic tests, clinical manifestations, prevention, treatment, and nursing implications. Hearing loss is emphasized because this is a common sequela of cCMV infection for which diagnostic modalities and treatments are available.

Delayed Confirmation of Hearing Loss in Infants

With required newborn hearing screening programs, the opportunity to confirm permanent hearing deficits within the first 3 months of life allows for

Seropositivity of Anti-Rubella Antibodies as A Marker for Rubella Infection in Infants at High Risk of Congenital Deafness

Hearing loss in newborns or congenital deafness can be caused by the development of several parts of the auditorysystem. Congenital deafness is often associated with infections, such as Toxoplasmosis, Rubella, Cytomegalovirus (CMV),and Herpes (TORCH). Deafness is very difficult to be early detected. Therefore, simple but fast methods are needed. Earlydetection is based on the Newborn Hearing Screening (NHS) program. Otoacoustic Emission (OAE) and AutomatedAuditory Brainstem Response (AABR) checks are raw materials for early detection. Congenital deafness often occurs withpregnancy infections with viruses such as Rubella. Rubella infection during pregnancy, especially during the first trimester,often causes Congenital Rubella Syndrome (CRS). Rubella infection often occurs with other causes, such as Toxoplasma,CMV, and Herpes. A Serological test can be used as one of the diagnostics of this infection. This study used single RubellaIgG and IgM antibodies and double antibodies test as a marker for the infection. The authors wanted to correlate theserological examination of this infection with the auditory function. Rubella infection was detected with single serologicalanti-Rubella IgG and IgM and double multiple Rubella and TORCH serological tests. Also, the auditory function wasassessed using the OAE and AABR test in this research. The result showed 35 (77.7%) patients with positive Rubellaserological tests among 45 NICU patients at Dr. Soetomo Hospital. There were number of patients was 12 (34.2%) patientswith a single positive serological test and 23 (65.7%) patients with positive multiple TORCH serological tests. The number ofpatients with Rubella negative infection was 10 (22.2%). There were 11 (31.4%) patients of positive Rubella infections withpositive hearing loss and 24 (68.6%) patients with negative hearing loss. From the results of the study, 35 patients were athigh risk of disturbance and the statistical analysis showed that there were no significant serological differences in Rubellapositive with hearing loss (p=0.087). Hearing loss in NICU infants has a high risk of factors causing Rubella infection andother related causes. In most Rubella positive serological tests IgG was found, which can be due to maternal factors.Serology tests need to be repeated for confirmation under the surveillance program. How to follow-up the patients anddefine the next laboratory test after six months remain a great challenge. The efforts need to be strengthened in surveillanceprograms.

Parental Judgement of Hearing Loss in Infants With Cleft Palate.

To investigate whether reported parental concern is supported by hearing assessment findings in children with cleft palate. To describe this population by examining the relationship between cleft type, middle ear status, and hearing loss. Retrospective consecutive case note review. Tertiary institutional regional cleft center. Consecutive cases of 194 babies born with cleft palate and referred to the specialist center from January 2009 and December 2013. Following exclusions, data from 155 infants were included for analysis. Documented parental concern in ear, nose and throat (ENT) and speech and language therapy case notes were compared to hearing assessment findings. Findings from otoscopic examination, tympanometry, and hearing assessment were analyzed with respect to cleft type. Parental concern is not always accurately reflected by objective assessment particularly when no concern is reported. Analysis of the cohort examined suggests that cleft type is not related to middle ear findings or hearing. It is helpful to be aware of parental concern and clinicians should consider that parental reports may not be accurately reflected by test results. As cleft type was not found to substantially influence middle ear status or hearing it is not recommended to adapt speech and language advice offered to families according to cleft type. Follow-up studies to increase participant numbers would support a statistical analysis.

Evaluating the success of a newly introduced Feed and Wrap protocol in magnetic resonance imaging scanning of the temporal bone for the evaluation of congenital sensorineural hearing loss

ObjectivesMagnetic resonance imaging (MRI) of the temporal bones, specifically of the cochlea and retro-cochlear pathway, is an important investigation in the diagnostic workup and management of congenital hearing loss. However, obtaining a diagnostic quality MRI scan depends upon the need for the infant to remain still for the duration of the scan. The Feed and Wrap (F + W) technique is a method that has been used successfully in infants for scans of other parts of the body, but has not been properly studied in MRI scans performed for hearing loss.The primary aim was to evaluate the diagnostic success rate of a newly introduced Feed and Wrap MRI scanning protocol used for assessment of sensorineural hearing loss in infants at Western Australia's Tertiary Paediatric hospital over the last three years. The secondary aims were to discuss the advantages and disadvantages of this technique and to review the available published literature on this technique and compare it to our case series. MethodsAll F + W MRI scans performed for the investigation of congenital hearing loss between 2016 and 2019 at the King Edward Memorial Hospital for Women and Perth Children's Hospital were retrospectively reviewed. The primary outcome was the diagnostic utility of the MRI scan with a diagnostic scan defined as the ability to diagnose or exclude radiological causes of sensorineural hearing loss. Results53 MRI scans of the temporal bone and brain were identified. 86.8% of scans were diagnostic. The mean age of infants who underwent diagnostic scans was 6.8 weeks old compared to 10.9 weeks (p = 0.01) for those who had non-diagnostic scans. The optimal age cut off for F + W technique was ≤10 weeks. The odds of failure are 16.7 (95%CI 6.5–42.8, p = 0.003) times higher above the cut of age of 10 weeks compared to ≤10 weeks. ConclusionThe F + W technique is a viable method for obtaining diagnostic quality MRI scans of the inner ear structures in infants with hearing loss, with a greater likelihood of success when applied in younger infants.

Congenital Hearing Loss among Pre-term infants in North India

Earlier investigations have reported congenital hearing loss in pre-term infants. Present study investigated hearing tests among pre-term infants to know the percentage of pre-term infants with hearing impairment. Two stage hearing assessment of pre-term infants showed congenital profound hearing loss in 16% of the infants. Present study showed the importance of TEOAE and ABR as efficient tool for hearing screening of high risk infants. Keywords: Preterm, Infants, India, 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.

Audiological Considerations for Managing Mild Bilateral or Unilateral Hearing Loss in Infants and Young Children.

Purpose This clinical focus article describes considerations for recommending assistive hearing technology to infants and young children who have mild bilateral or unilateral hearing loss. These conditions present special challenges compared to bilateral permanent hearing losses that are moderate to profound in their degree in that the recommendation to proceed with technology is not as clear. Conclusion Current clinical practice guidelines and protocols for pediatric hearing aid fitting recommend managing these conditions on a case-by-case basis. Descriptions of key considerations for recommending assistive hearing technology for infants and young children with mild bilateral hearing loss or unilateral hearing loss are offered herein.

CMV and Hearing Loss in Infants: An Audiology Call to Action

CMV and Hearing Loss in Infants: An Audiology Call to Action

2334. Saliva Screening for Congenital Cytomegalovirus Infection in the Neonatal Intensive Care Unit: Beware!

BackgroundCongenital cytomegalovirus (cCMV) infection is the most common cause of non-genetic sensorineural hearing loss in infancy. Screening of newborns for cCMV infection has been performed utilizing saliva due to ease of collection and high sensitivity. Positive saliva screens for CMV DNA by polymerase chain reaction (PCR) testing has been reported to occur secondary to breast milk feeding without signifying congenital infection. The NICUs of Nationwide Children’s Hospital recently began universal saliva screening of all admissions. We report 3 neonates whose saliva CMV screen was positive yet the urine CMV PCR test was negative in order to inform CMV screening strategies.MethodsRetrospective review of the electronic health records of neonates admitted to the neonatal intensive unit (NICU) at Nationwide Children’s Hospital, Columbus, OH who had CMV detected by PCR from saliva specimens but not from urine. Pertinent demographic and clinical data were obtained.ResultsThree female neonates had a positive saliva CMV DNA PCR test but urine CMV PCR was negative. The first infant (gestational age [GA] 34 weeks, birth weight [BW]1790 Grams) was a monochorionic diamionic twin gestation and born vaginally with unknown duration of rupture of membranes (ROM). At 16 days of age, the infant had a positive saliva CMV PCR but a negative urine CMV PCR test. The infant received maternal milk. The twin’s CMV PCR tests of saliva and urine were negative. The second infant (GA 38 weeks, BW 2952 grams) was born vaginally after 9 hours of ROM. On the first day of age, the infant had a positive saliva CMV PCR test that was followed by a negative urine CMV PCR on the third day of age. The infant had not been breastfed. The third infant (GA 33 weeks, BW 1762 grams) was born by C-section delivery with ROM at delivery. Saliva CMV PCR screen was positive on the second day of age but urine PCR was negative twice (days 5 and 7). All 3 infants had no signs/symptoms of cCMV infection and passed the newborn hearing screen.ConclusionTesting of saliva for CMV DNA by PCR is not always confirmatory for cCMV infection as contamination of saliva specimens with CMV could result from exposure to maternal milk and possibly vaginal secretions. Definitive diagnosis of cCMV infection requires additional confirmatory testing preferably with urine.Disclosures All authors: No reported disclosures.

Virus-induced cochlear inflammation in newborn mice alters auditory function

Although human cytomegalovirus (HCMV) is a known cause of sensorineural hearing loss in infants with congenital HCMV (cCMV) infections, mechanisms that contribute to sensorineural hearing loss (SNHL) in infants with cCMV infection are not well defined. Using a murine model of CMV infection during auditory development, we have shown that peripheral infection of newborn mice with murine CMV (MCMV) results in focal infection of the cochlea and virus-induced cochlear inflammation. Approximately 50%-60% of infected mice exhibited increased auditory brainstem response (ABR) thresholds across a range of sound frequencies. Histological analyses of the cochlea in MCMV-infected mice with elevated ABR thresholds revealed preservation of hair cell (HC) number and morphology in the organ of Corti. In contrast, the number of spiral ganglion neurons (SGN), synapses, and neurites connecting the cochlear HC and SGN nerve terminals were decreased. Decreasing cochlear inflammation by corticosteroid treatment of MCMV-infected mice resulted in preservation of SGN and improved auditory function. These findings show that virus-induced cochlear inflammation during early auditory development, rather than direct virus-mediated damage, could contribute to histopathology in the cochlea and altered auditory function without significant loss of HCs in the sensory epithelium.

Parental reaction to diagnosis of infant hearing loss

ABSTRACTObjectiveThis study aimed to investigate parental reactions to a diagnosis of Permanent Childhood Hearing Loss (PCHL) and the implications for engagement in medical investigations and early intervention services. The study sought to determine the impact of type of hearing loss, presence of parental depression or anxiety, and parental reflective function on timely engagement with services for children with bilateral PCHL.MethodQuestionnaires were completed by 27 parents of babies aged 3-5 months, diagnosed with bilateral hearing loss. Measures included the DASS 21, DMRF (Diamond Maternal Reflective Function Scale), and demographic items. Clinical charts were accessed six months later to ascertain timing of engagement with medical and early intervention services.Results86% of families attended all required medical appointments following diagnosis. Early intervention services were attended by 65.5% of families. Parental anxiety was the only significant factor in engagement with early intervention services. There was no impact of reflective functioning, parent education and additional medical needs of the child on engagement. The participants also had lower levels overall of anxiety and depression (compared to normative data) but higher levels of stress on the DASS.DiscussionWhile many factors may impact on the likelihood of timely attendance at medical appointments and engagement with early intervention services (EI), support needs to be tailored to each family to ameliorate any potential barriers and ensure that access is easily available, particularly when there may be parental anxiety. This study shows that higher levels of anxiety were significantly associated with decreased engagement with EI.