Hearing screening test in neonates born to COVID-19-positive mothers.

SARS-CoV-2, the responsible virus for the COVID-19 pandemic, has demonstrated neurotropic properties indicated by cases presenting with auditory and vestibular system insults. The expression of ACE-2 receptors in the placenta and the detection of IgM antibodies against the virus in the fetuses of pregnant women suffering from COVID-19 render vertical transmission of the infection to the fetus possible. Thus, our study aims to examine whether, similar to other viruses like CMV, SARS-CoV-2 is responsible for congenital hearing loss. This is a retrospective study in a regional pediatric hospital. The medical records of newborns (n = 111) born by mothers positive for COVID-19 during pregnancy who underwent screening hearing tests with Transient Evoked Otoacoustic Emissions (TEOAE) and Automatic Auditory Brainstem Response (AABR) from February 2020 to June 2022 were reviewed. Neonates with additional aggravating factors for congenital hearing loss were excluded from the study. For the study period, nine mothers were found positive during the first trimester, twenty mothers in the second trimester, and eighty-three mothers in the third trimester. TEOAEs test and AABR test scored PASS bilaterally in all neonates tested. CONCLUSION: Infection with COVID-19 during pregnancy was not a risk factor for hearing loss, similar to other studies. WHAT IS KNOWN: • The pathogenetic mechanism of the viral-induced impairment of the organ of Corti includes direct damage to the hair cells and indirect damage due to the induction of the innate inflammatory response. • Early data suggested that the SARS-CoV-2 virus also has neurotropic properties with manifestations from the sensory epithelia. WHAT IS NEW: • Although the intrauterine infection remains controversial, the expression of the ACE-2 receptor on the placenta and the detection of IgM antibodies, as well as the covid-19 genome in fetuses, make the vertical transmission tenable. • In our study, the newborn hearing screening results indicate that COVID-19 infection during pregnancy is not a risk factor for hearing loss.

Is intrauterine exposure to COVID-19 infection a risk factor for infant hearing loss?

PURPOSE: To determine whether exposure to intrauterine COVID-19 infection causes congenital or late-onset hearing loss in infants. MATERIAL AND METHOD: The hearing screening results of infants born in a tertiary hospital between March 2020 and April 2022 with and without a history of intrauterine exposure to COVID-19 infection (36 infants each) were retrospectively analyzed within one month after birth in all infants and additionally at six months after intrauterine COVID-19 infection exposure in the study group. The automated auditory brainstem response (AABR) test was used for the hearing evaluation. RESULTS: The polymerase chain reaction test was negative in study group exposed to intrauterine COVID-19 infection. The number of infants admitted to the intensive care unit (ICU), and the length of ICU stay were significantly higher in this group (p < 0.01). Six infants (16.6 %) in the study group failed the first AABR test bilaterally, but five of these infants passed the second AABR test. A bilateral severe sensorineural hearing loss was detected in one infant (2.77 %). All the infants in the study group underwent the AABR test again at six months, and all infants, except this infant, passed the test. In the control group, five infants (13.88 %) failed the first AABR test bilaterally, but they all passed the second test. CONCLUSIONS: Exposure to COVID-19 infection in the intrauterine period does not cause congenital or late-onset hearing loss (within six months) in infants; therefore, gestational COVID-19 infection is not a risk factor for infant hearing loss.

Sequential bilateral cochlear implant: long-term speech perception results in children first implanted at an early age.

PURPOSE: The study aims to assess the benefit of sequential bilateral cochlear implantation in children with congenital bilateral profound hearing loss, submitted to the first implant at an early age. METHODS: We enrolled all the bilateral sequential cochlear implanted children who received the first implant within 48 months and the second within 12 years of age at our Institution. The children were submitted to disyllabic word recognition tests and Speech Reception Threshold (SRT) assessment using the OLSA matrix sentence test with the first implanted device (CI1), with the second implanted device (CI2), and with both devices (CIbil). Furthermore, we measured the datalogging of both devices. Then we calculated the binaural SRT gain (b-SRTgain) and checked the correlations between speech perception results and the b-SRTgain with the child's age at CI1 and CI2, DELTA and the datalogging reports. RESULTS: With the bilateral electric stimulation, we found a significant improvement in disyllabic word recognition scores and in SRT. Moreover, the datalogging showed no significant differences in the time of use of CI1 and CI2. We found significant negative correlations between speech perception abilities with CI2 and age at CI2 and DELTA, and between the SRT with CI1 and the b-SRTgain. CONCLUSIONS: From this study we can conclude that in a sequential CI procedure, even if a short inter-implant delay and lower ages at the second surgery can lead to better speech perception with CI2, children can benefit from bilateral stimulation independently of age at the second surgery and the DELTA.

Do all infants with congenital hearing loss meet the 1-3-6 criteria? A study of a 10-year cohort from a universal newborn hearing screening programme in Singapore.

OBJECTIVE: To evaluate 1) the outcomes of a local universal newborn hearing screening (UNHS) programme and its audiological follow-ups at 3- and 6-month points, 2) the education outcomes of children with congenital hearing loss (CHL). DESIGN: Retrospective study that analysed data containing hearing screening, diagnosis, intervention and schooling information from electronic databases. STUDY SAMPLE: Children aged 5 to 15 years old who were born between 2004-2014 and underwent UNHS in a local hospital. RESULTS: Over a 10-year cohort, 99.4% of 29,972 newborns underwent UNHS; approximately 90% of them were screened by 1 month of age. However, only 10% of the cohort strictly fulfilled the 1-3-6 criteria recommended by the Joint Committee on Infant Hearing. Lost to follow-up (LTF) rate was highest at post-diagnosis (35%). 80% of infants who were intervened between 6 and 48 months of age went to mainstream schools. The remaining 20% had additional disabilities or family factors. CONCLUSIONS: A high UNHS coverage rate may not translate to meeting the 1-3-6 criteria. Despite ease of access to our healthcare system, LTF at post-diagnosis remained high. In the absence of additional disabilities or family factors, infants intervened during the sensitive window could still potentially make it into mainstream schools.

[Choristoma of the middle ear]. / Khoristoma srednego ukha i sostsevidnogo otrostka.

Choristoma is one of the varieties of congenital developmental anomalies, where one or another normal tissue of the body is located in an atypical place for itself. The short literary review of choristoma of middle ear is presented in article. A rare clinical cases of salivary gland choristoma of the middle ear (5-year-old girl with left-sided conductive hearing loss of III degree) and glial choristoma of the mastoid (19-year-old man with signs of chronic suppurative otitis media of the right ear) are described.

Case report: novel PCDH15 variant causes usher syndrome type 1F with congenital hearing loss and syndromic retinitis pigmentosa.

BACKGROUND: Usher syndrome (USH) is an autosomal recessive disorder primarily responsible for deaf-blindness. Patients with subtype Usher syndrome type 1 (USH1) typically experience congenital sensorineural hearing loss, abnormal vestibular function, and retinitis pigmentosa (RP). Here we present a case of Usher syndrome type 1F (USH1F) with a novel homozygous variant in the calcium-dependent cell-cell adhesion protocadherin-15 (PCDH15) gene. CASE PRESENTATION: Ophthalmic examinations were evaluated over a course of 10 years and the disease-causing variant was identified by whole exome sequencing (WES). Initial and follow-up examination of color fundus photos after 10 years revealed an increase in bone spicule pigment deposits in both eyes. A parafoveal hyper-AF ring in both eyes was shown in fundus autofluorescence (FAF) with a progressive diameter-wise constriction observed over 8 years. Outer nuclear layer (ONL) loss was observed in parafoveal and perifoveal regions of both eyes on spectral domain-optical coherence tomography (SD-OCT). Full-field electroretinography (ffERG) showed extinguished global retinal function. WES identified a novel two-base-pair deletion, c.60_61del (p.Phe21Ter), in the PCDH15 gene, confirming the diagnosis of USH1F. CONCLUSIONS: We report a novel homozygous PCDH15 pathogenic variant expected to lead to nonsense-mediated decay (NMD) of PCDH15 mRNA. The patient exhibits a loss of function with USH1F, experiencing congenital hearing loss and syndromic RP.

Early detection of hearing loss for infants in Western Australia: Comparison to international benchmarks.

AIM: To assess the degree to which timely audiological assessment of congenital hearing loss is achieved at our institution - Perth Children's Hospital, Western Australia, and to review cases which breached this timeframe in order to address barriers to timely assessment. The benchmark used to determine timely assessment is that set out by The Joint Committee on Infant Hearing (JCIH) in which diagnostic audiological testing occurs by three months of age for those who do not pass newborn hearing screening. METHODS: A retrospective chart review of infants who underwent diagnostic auditory assessment at Perth Children's Hospital between 2016-2019. A total of 151 children were identified as meeting the inclusion criteria and their medical files were reviewed. Time to first dABR was the time point for whether testing was achieved within the 3 month timeframe. RESULTS: Of the 151 children who underwent dABR assessments, 1 was identified as having breached the 90 day time limit (tested on day 91) for which no valid reason for delay could be identified. The timely delivery of dABR assessments in 99.3% of cases within this cohort compares favourably with the literature. CONCLUSIONS: Conclusion Timely diagnostic audiological assessment is achievable for children with congenital hearing loss. The reasons for patients breaching this timeframe are explored in the paper along with factors which may help avoid delays.

Universal newborn hearing screening with automated auditory brainstem response (AABR) in Hungary: 5-year experience in diagnostics and influence on the early intervention.

PURPOSE: In 2015 a new regulation and guidelines for the universal newborn hearing screening by AABR measurement have been implemented in Hungary. The aim of our study was to analyse (1) the past 5 years of data from our diagnostic centre about the incidence and types of congenital hearing losses, and (2) the first experiences with the National Newborn Hearing Screening Registry, started in 2019, and (3) the influence of the screening on the pediatric cochlear implant program. METHODS: 1269 children referred to our diagnostic centre between 2017 and 2021 were investigated. A third AABR measurement and full audiological evaluation were performed. Furthermore, one-year period data of the screening registry, and the number of implanted children at or under the age of 3 were analysed using the national databases. RESULTS: Altogether 276 newborns (22% of the referred cases after the two-stage screening) had hearing loss, 134 (49%) out of them was conductive origin, almost twice frequent in male as in female. Permanent sensorineural hearing impairment was found in 142 (51%), 58 (40%) of them had bilateral, severe to profound hearing loss, occurring more frequently in male as in female. The national digital registration of the screening data within 12 months concerned 68%. The number of early cochlear implantation in one year increased from 1 to 23 children in the past 15 years. CONCLUSION: A third AABR after the two-stage screening increased the efficiency and filtered the 78% false-positive cases. The audiological diagnostics verified and typed the hearing losses ensuring the early intervention.

Cochlear Implantation in Inner Ear Malformations: Considerations Related to Surgical Complications and Communication Skills.

INTRODUCTION: There are particular challenges in the implantation of malformed cochleae, such as in cases of facial nerve anomalies, cerebrospinal fluid (CSF) leaks, erroneous electrode insertion, or facial stimulation, and the outcomes may differ depending on the severity of the malformation. The aim of this study was to assess the impact of inner ear malformations (IEMs) on surgical complications and outcomes of cochlear implantation. METHODS: In order to assess the impact of IEMs on cochlear implant (CI) outcomes, 2 groups of patients with similar epidemiological parameters were selected from among 863 patients. Both the study group (patients with an IEM) and control group (patients with a normal inner ear) included 25 patients who received a CI and completed at least 1 year of follow-up. Auditory performance, receptive and expressive language skills, and production and use of speech were evaluated preoperatively and at least 1 year after implantation. Types of surgical complications and rates of revision surgeries were determined in each group. RESULTS: In the study group, the most common malformation was an isolated enlarged vestibular aqueduct (EVA) (44.8%). Overall, the patients with IEMs showed significant improvement in auditory-verbal skills. In general, the patients who had normal cochleae scored significantly better compared to patients with IEMs (p < 0.05). The complication rate was significantly lower in the control group compared to the study group (p = 0.001), but the rate of revision surgeries did not differ significantly (p = 0.637). CONCLUSION: It is possible to improve communication skills with CIs in patients with IEMs despite the variations in postoperative performances. Patients with EVA, incomplete partition type 2, and cochlear hypoplasia type 2 were the best performers in terms of auditory-verbal skills. Patients with IEMs scored poorly compared to patients with normal cochleae. CSF leak (gusher or oozing) was the most common complication during surgery, which is highly likely in cases of incomplete partition type 3.

Hearing Outcomes in Stickler Syndrome: Variation Due to COL2A1 and COL11A1.

OBJECTIVES: Stickler syndrome (SS) is a heterogeneous inherited connective tissue disorder, often due to a mutation in COL2A1 or COL11A1. Mutations in these genes cause collagen abnormalities affecting ocular, auditory, orofacial, and skeletal systems, including hearing loss, micrognathia, and cleft palate. Understanding the variability of hearing phenotypes based on genetic mutation has a significant impact on treatment and long-term care. DESIGN: A retrospective chart review of pediatric patients with a confirmed diagnosis of SS between January 2003 and December 2018 at a tertiary pediatric hospital was performed. Patients were excluded if they did not have genetic evaluation, craniofacial/ear, nose, and throat evaluation, and/or audiologic testing. Charts were reviewed for the following information: age, race, sex, SS diagnosis, genetic variant of SS, and audiological testing data. RESULTS: There were 29 confirmed patients with SS who met criteria, 16 with type I (COL2A1) and 13 with type II (COL11A1). Of the 13 patients with type II, 12 (92%) demonstrated hearing loss, ranging in severity from mild to severe. In type I, 25% of patients had mild or resolved hearing loss. CONCLUSION: Results suggest that patients with type II SS are more likely to have congenital hearing loss than type I. Data also suggest that the COL11A1 mutation shows consistently more severe hearing loss than the COL2A1 mutation.

Unexplained Variation in Benefit of Treatment of Congenital Unilateral Aural Atresia: A Review of the Literature.

OBJECTIVE: A review of published data regarding binaural hearing after treatment of congenital unilateral conductive hearing loss (UCHL) due to aural atresia. Treatment options concern atresia surgery (reconstructive surgery), application of a bone conduction device (BCD), or application of a middle ear implant (MEI). DATA SOURCES: Database PubMed was searched for articles published in English and German between January 1, 1994, and January 1, 2019. STUDY SELECTION: The initial search identified 52 studies, of which 9 met the inclusion criteria. DATA SYNTHESIS: Comparison of studies was based on a structured review. Meta-analysis was not feasible because of the heterogeneity of outcome measures, the limited number of relevant papers (9), and diverse types of treatment (5). CONCLUSIONS: Treatment of UCHL results in bilateral hearing instead of binaural hearing. The large intersubject variability in benefit of treatment is unexplained with a clear improvement in the minority of listeners and a limited improvement or binaural interference in most listeners after atresia repair or amplification with a BCD or MEI.

Post-lingual non-syndromic hearing loss phenotype: a polygenic case with 2 biallelic mutations in MYO15A and MITF.

BACKGROUND: Hearing loss (HL) represents the most common congenital sensory impairment with an incidence of 1-5 per 1000 live births. Non-syndromic hearing loss (NSHL) is an isolated finding that is not part of any other disorder accounting for 70% of all genetic hearing loss cases. METHODS: In the current study, we reported a polygenic mode of inheritance in an NSHL consanguineous family using exome sequencing technology and we evaluated the possible effect of the detected single nucleotide variants (SNVs) using in silico methods. RESULTS: Two bi-allelic SNVs were detected in the affected patients; a MYO15A (. p.V485A) variant, and a novel MITF (p.P338L) variant. Along with these homozygous mutations, we detected two heterozygous variants in well described hearing loss genes (MYO7A and MYH14). The novel MITF p. Pro338Leu missense mutation was predicted to change the protein structure and function. CONCLUSION: A novel MITF mutation along with a previously described MYO15A mutation segregate with an autosomal recessive non-syndromic HL case with a post-lingual onset. The findings highlight the importance of carrying whole exome sequencing for a comprehensive assessment of HL genetic heterogeneity.

Follow-up after failed newborn hearing screening: Parental and primary care provider awareness.

PURPOSE: The current loss to follow-up rate after failed newborn hearing screening (NBHS) is 34.4%. Previous studies have found that lack of parental and primary care provider (PCP) awareness of NBHS results are significant contributors to loss to follow-up. The objective of this study was to identify factors associated with parental and PCP awareness of NBHS results. MATERIALS AND METHODS: Retrospective cohort study. A survey asking about demographics and knowledge of NBHS testing and results was offered to parents in the waiting room of an urban pediatric primary care office. Included were biological parents ≥18 years of age of children ≤10 years of age born in Pennsylvania. Each child's chart was reviewed for PCP documentation of NBHS results. The odds of knowing NBHS results were evaluated using logistic regression. RESULTS: The survey was completed by 304 parents. 74.0% were aware of their child's NBHS results. Child age ≥1 year old (OR: 0.49, 95%CI[0.29, 0.82], P = 0.007) and Hispanic ethnicity (OR: 0.38, 95%CI[0.16, 0.89], P = 0.03) were associated with decreased odds of a parent knowing NBHS results. In addition, fewer fathers knew the results of their child's NBHS compared with mothers (OR: 0.33, 95%CI[0.18, 0.62], P < 0.001). However, parental awareness was not associated with birthing facility or insurance type. 222 charts were reviewed for NBHS documentation, revealing PCP awareness in 95.5% of cases and no associations with any of the factors examined. CONCLUSIONS: Factors associated with parents not knowing NBHS results included being the parent of an older child, Hispanic, or the father.

Outcomes for a clinically representative cohort of hearing-impaired adults using the Nucleus® CI532 cochlear implant.

PURPOSE: Hearing performance data was collected from a large heterogeneous group of subjects implanted with the Cochlear™ Nucleus® CI532 with Slim Modiolar Electrode, for the purposes of postmarket clinical follow-up. Data was analysed for factors which may predict postoperative speech recognition scores. METHODS: Data was collected retrospectively from five German clinics for 159 subjects from March 2017 to August 2018. Hearing thresholds and recognition scores for monosyllabic words in quiet and sentences in noise were measured preoperatively and at 3 and 6 months postoperatively. RESULTS: There was a mean gain of 44% points (95% CI 39-49%) at 6 months in monosyllable scores in quiet for implanted ears. Preoperative hearing thresholds in implant ears increased systematically with decreasing age; however, younger subjects had better baseline monosyllable scores with hearing aids compared with older subjects. Baseline performance alone explained 14% of the variation in postoperative scores. Residual hearing was preserved on average to within 22 dB at 250 Hz and 30 dB at 500 Hz of preoperative levels. CONCLUSIONS: In a large and varied cohort of routinely treated hearing-impaired adults, speech recognition with the CI532 for German monosyllabic words in quiet at 6 months was equivalent to performance reported at one year or more in other published studies. Although younger subjects had poorer preoperative pure-tone thresholds, they had better preoperative word recognition scores compared with older subjects, and also had higher post implant scores. Further research is required to identify if this phenomenon is just applicable to German health system assessment and referral practices.

Surgical complications of cochlear implantation: a 25-year retrospective analysis of cases in a tertiary academic center.

PURPOSE: Despite the advances made in cochlear implantation techniques, the associated complication rates are still high. Here, we aimed to analyze cases, with extensive follow-up data, associated with a large sample of patients to identify complications related to cochlear implants and to present our surgical experience and the technique that we used in order to follow surgical rules/medical purpose to avoid any complications. METHODS: We retrospectively examined cases involving 2597 patients (1342 males; 1255 females; age 1-88 years) who underwent cochlear implantation procedures between November 1995 and July 2019, and we classified complications as minor and major. RESULTS: The mean age at the time of implantation was 6.48 (Min: 1/Max: 88) years. The cause of deafness was congenital in 76.5% of the patients and acquired in 16.8%. The overall rate of complications in the study was 3.7% (n = 97). The minor and major complication rates were 3.0 and 0.7, respectively. Further, while the most common minor complication we encountered was vertigo, the most common major complication was implant extrusion. CONCLUSION: Fixing the cochlear implant receiver-stimulator with the bone-recess technique and sealing the posterior tympanotomy site with a piece of muscle in order to follow surgical rules/medical purpose to avoid any complications. Following the insertion of the electrode into the cochlea, the muscle closure of the cochleostomy site or the round window restores the original anatomy and in order to follow surgical rules/medical purpose to avoid any complications. We have developed this highly effective technique with years of experience and have not had a major surgical complication in 5 years.

[Hearing loss due to mutations or lack of the gene coding protein stereocillin]. / Narushenie slukha pri mutatsiyakh ili otsutstvii gena, kodiruyushchego belok stereotsilin.

OBJECTIVE: The description of a clinical picture and audiological features at the hearing loss caused by changes of a STRC gene, coding protein stereocillin (MIM: 606440). Mutations in the numerous genes responsible for the inner ear proteins are the reason for congenital sensorineural hearing loss. The main cause of congenital bilateral sensorineural hearing loss in the Russian Federation are mutations in GJB2 gene it reaches up 68% of cases identified in infancy. GJB2 gene tests already became routine around the world. Possibilities of new methods based on sequencing of new generation (NGS, next generation sequencing) allow to conduct a research of more rare genes connected with a hearing impairment. The most often among GJB2 negative patients reveal mutations and deletion of a gene of STRC. PATIENTS AND METHODS: Full audiological examination of 5 children and one adult with a hearing loss from 2 unrelated families is provided. Mutations in STRC gene were identified. All children are examined aged before 8 years, and 3 children failed universal audiological screening in maternity hospital, to two children screening was not carried out as they were born till 2009. RESULTS: The children with the sensorineural hearing loss connected with mutations and deletion of STRC gene failed hearing screening in maternity hospital because of the OAE is not registered, what indicates the congenital nature of a hearing loss. Recently it could not be noticed earlier because of slight increase of hearing thresholds and was regarded only as the early onset. Our data emphasize that the of thresholds from 35 to 60 dB in frequencies 0,5-4 kHz is common for mutations/deletions of STRC gene. CONCLUSION: The development of molecular genetics methods confirms the hereditary causes of GJB2-negative patients and expands indications for family counseling. Special approach for child with hearing loss so early revealed is necessary and the consultation of parents frightened of screening results is very important.

Unanticipated prognosis for a patient with type 2 Usher syndrome.

PURPOSE: The Usher syndrome phenotype is comprised of ocular and audiologic anomalies. Patients characteristically experience congenital hearing loss, nyctalopia, reduced visual fields, and ultimately decreased visual acuity. However, diagnosis may initially be more difficult in cases with limited ocular findings. Here, we present a case in which an adult patient had neither subjective visual complaints nor ocular findings at the time of diagnosis aside from a moderate reduction in rod and cone function on electroretinogram testing. Nevertheless, 43 years after his initial examination, he showed severe degenerative changes in the retina. METHODS: A 63-year-old man with Usher syndrome type 2 underwent ophthalmic examination that included visual acuity, optical coherence tomography (OCT), electroretinogram (ERG), fundus photography, and Goldmann visual field testing. The patient also had genetic testing performed. We additionally reviewed the ocular findings on two of his siblings also afflicted with Usher syndrome type 2. RESULTS: Our findings documented the long-term progression of Usher syndrome in this patient. They showed that the patient was asymptomatic with only a moderate reduction on ERG testing at the time of diagnosis, but subsequently progressed to an advanced stage of retinal disease with severe visual loss. CONCLUSIONS: The patient demonstrated that the absence of visual symptoms and favorable findings on functional testing on initial presentation might yet belie a future for austere visual loss. Caution is thus warranted when predicting a visual prognosis in such a patient. Further, the value in electroretinographic testing for diagnosis is demonstrated.

Genetic screening as an adjunct to universal newborn hearing screening: literature review and implications for non-congenital pre-lingual hearing loss.

Objective: Universal newborn hearing screening (UNHS) uses otoacoustic emissions testing (OAE) and auditory brainstem response testing (ABR) to screen all newborn infants for hearing loss (HL), but may not identify infants with mild HL at birth or delayed onset HL. The purpose of this review is to examine the role of genetic screening to diagnose children with pre-lingual HL that is not detected at birth by determining the rate of children who pass UNHS but have a positive genetic screening. This includes a summary of the current UNHS and its limitations and a review of genetic mutations and screening technologies used to detect patients with an increased risk of undiagnosed pre-lingual HL.Design: Literature review of studies that compare UNHS with concurrent genetic screening.Study sample: Infants and children with HLResults: Sixteen studies were included encompassing 137,895 infants. Pathogenic mutations were detected in 8.66% of patients. In total, 545 patients passed the UNHS but had a positive genetic screening. The average percentage of patients who passed UNHS but had a positive genetic screening was 1.4%.Conclusions: This review demonstrates the positive impact of concurrent genetic screening with UNHS to identify patients with pre-lingual HL.

Sensorineural Hearing Loss in Newborns Hospitalized in Neonatal Intensive Care Unit: An Observational Study.

Children hospitalized in Neonatal Intensive Care Units (NICU) present an increased risk for Sensorineural Hearing Loss (SNHL) due to prematurity, hypoxia-ischemia, hyperventilation, low birth weight and the use of ototoxic drugs. The aim of this study was to assess the prevalence of SNHL in newborns hospitalized in a NICU using Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Responses (A-ABR) and analyze the associated risk factors. A sample of 153 newborns hospitalized in NICU underwent TEOAE, A-ABR and clinical ABR to evaluate the presence of hearing deficits. Prevalence of SNHL was calculated and odds ratio for specific risk factors was measured. One-hundred fifteen babies (86.7%) presented normal hearing at TEOAE and A-ABR. Fifteen children had a REFER response at TEOAE and a PASS response at A-ABR. Twenty-five children (16.3%) had a REFER A-ABR and were addressed to clinical ABR. A diagnosis of SNHL was made in 12 (7.8%) newborns. An increased risk of SNHL was observed in preterm children <28 weeks (p=0.0135), in children with neurological disorders (p=0.02), that underwent surgery (p=0.0002), affected from premature retinopathy (p=0.0006), craniofacial malformation (p=0.007) and that had sepsis (p=0.04). Additional risk factors for SNHL in our sample were a maternal disease during pregnancy (p=0.0011), cesarean delivery (p<0.0001) and a twin pregnancy (p<0.0001). SNHL in newborns is correlated with hospitalization in NICU. An accurate hearing screening associated to a rigorous clinical medical collection of data is necessary to promptly identify cases of SNHL in children with a special attention to those hospitalized in NICU and plan proper intervention.