Sensorineural Hearing Loss In Children Research Articles

From diagnosis to management: current perspectives on congenital cytomegalovirus infection.

Congenital CMV (cCMV) infection is the most common infection of newborns and a leading cause of hearing loss and other neurologic disabilities in children. This review focuses on the diagnosis, presentation and management of cCMV infection. Cytomegalovirus is one of the leading causes of sensorineural hearing loss in children. It also leads to neurodevelopmental disabilities and learning problems throughout childhood in both symptomatic and asymptomatic newborns. Urine and saliva PCR testing are the preferred methods of testing newborn infants for cCMV. In recent years, newborn-targeted and universal screening programs have been implemented in several states and major medical centers with the goal of identifying infected infants at risk for hearing loss. Treatment for infants diagnosed with cCMV infection should be limited to those who are moderately to severely symptomatic at birth with cCMV infection, though treatment may be beneficial for children who are asymptomatic with isolated sensorineural hearing loss. As more children with cCMV are being identified through newborn screening, understanding the clinical presentation and sequelae is important for appropriate management of children with cCMV.

Language development in sensorineural hearing loss children using hearing aids .

Language development in sensorineural hearing loss children using hearing aids .

Analysis of a mathematical model with hybrid fractional derivatives under different kernel for hearing loss due to mumps virus

ABSTRACT Genetics, birth defects, some viral diseases, persistent ear infections, medications, loud noises, and age can all cause hearing loss, a global health issue. The mumps are typically the cause of unilaterally acquired sensorineural hearing loss in children. A hybrid fractional operator model employing Constant Proportional Caputo (CPC), Constant Proportional Caputo Fabrizio (CPCF), and Constant Proportional Atangana-Baleanu Caputo (CPABC) in the Caputo sense is developed to investigate mumps induced hearing loss in children. We focused on the region that is positively invariant and well-posed in the suggested model. We assessed the endemic and disease free equilibrium, reproductive number, strength number, and parameter sensitivity, and examined the global stability of the proposed system. The existence, uniqueness, and Hilfer generalized proportional operators for the constant of proportionality of the system’s solutions are demonstrated using fixed-point theorems. We simulated a system of fractional differential equations analytically using the Laplace Adomian Decomposition Method (LADM). This study is important since it is the first to model mumps related hearing loss using a fractional order model based on hybrid fractional operators without a singular kernel. The fractional-order derivative retains the system memory effect, is nonlocal, and outperforms the integer-order derivative in this regard. This sort of study aids medical professionals in diagnosis, treatment planning, and decision-making for mumps induced hearing loss.

Unilateral Sensorineural Hearing Loss in Children: Etiology, Audiological Characteristics, and Treatment.

The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45.7% (32/70); of these, 28 (87.5%) wore a conventional hearing aid, 2 (6.3%) a CROS device, and 2 (6.3%) a cochlear implant. Regarding the choice to use a hearing device, no significant differences were found between the subcategories of hearing loss degree (p = 0.55) and audiometric configuration (p = 0.54). Most parents of children with mild-to-severe USNHL observed improved attention (90.9%), and reduced fatigue and restlessness (86.4%) using the hearing aid. These children performed significantly better on all audiological tests (speech perception in quiet and in noise conditions, and sound localization) while wearing the hearing aid (p < 0.001). More efforts should be made to raise awareness among professionals and parents about the negative consequences of uncorrected USNHL.

Study of sensorineural hearing loss in children with type 1 diabetic mellitus and the corelation with the duration of the disease in a tertiary care center

Background: Annual type 1 diabetes cases in children under the age of 15 are estimated to be around 96,000 worldwide. The pathophysiological explanation for diabetes-related hearing loss is speculative. Usually, hearing loss is bilateral, gradual onset, affecting higher frequencies. It is a well-known entity in type 2 diabetic mellitus. This study aims at knowing the prevalence of hearing loss in children with type 1 diabetic mellitus with respect to duration of diabetic mellitus. Materials and Methods: A hospital-based comparative study was conducted involving 60 diabetic children and 60 healthy (control) children. Brainstem evoked response audiometry test was done in both groups. Reports were compared and prevalence of sensorineural hearing loss in diabetic children and the corelation with the duration of disease were analyzed. Results: The mean age among cases was 11.93±2.9 years and the mean age among controls was 9.08±1.92 years. Among diabetic group, 25 (41.7%) were male, 35 (58.3%) were female. Among non-diabetic group, 33 (55%) were male, 27 (45%) were female. Although none of the diabetic children had hearing loss, the differences in absolute latency waves I, II, III, IV, and V between diabetic group and non-diabetic group in the right ear and waves I and II and III and V between diabetic group and non-diabetic group in the left ear was found to be statistically significant (p&lt;0.05). The difference in interpeak latency values of wave I and wave V between diabetic and non-diabetic group in right ear and between wave I–V and wave III–V in the left ear was found to be statistically strongly significant (p&lt;0.001). This indicates prolonged absolute latency and interpeak latency among diabetics than non-diabetics. Since none of the diabetic children had hearing loss, the corelation of hearing loss with the duration of the disease was unable to obtain. Conclusion: This study stresses on the need for frequent follow-up and hearing evaluation of the type 1 diabetic children for the early detection of hearing loss.

Genetic diagnosis of childhood sensorineural hearing loss

IntroductionCongenital/early-onset sensorineural hearing loss (SNHL) is one of the most common hereditary disorders in our environment. There is increasing awareness of the importance of an etiologic diagnosis, and genetic testing with next-generation sequencing (NGS) has the highest diagnostic yield. Our study shows the genetic results obtained in a cohort of patients with bilateral congenital/early-onset SNHL. Materials and methodsWe included 105 children with bilateral SNHL that received genetic testing between 2019 and 2022. Genetic tests were performed with whole exome sequencing, analyzing genes related to hearing loss (virtual panel with 244 genes). Results48% (50/105) of patients were genetically diagnosed. We identified pathogenic and likely pathogenic variants in 26 different genes, and the most frequently mutated genes were GJB2, USH2A and STRC. 52% (26/50) of variants identified produced non-syndromic hearing loss, 40% (20/50) produced syndromic hearing loss, and the resting 8% (4/50) could produce both non-syndromic and syndromic hearing loss. ConclusionsGenetic testing plays a vital role in the etiologic diagnosis of bilateral SNHL. Our cohort shows that genetic testing with NGS has a high diagnostic yield and can provide useful information for the clinical workup of patients.

Genetic characteristic analysis of slight-to-moderate sensorineural hearing loss in children

Objective:To analyze genetic factors and phenotype characteristics in pediatric population with slight-to-moderate sensorineural hearing loss. Methods:Children with slight-to-moderate sensorineural hearing loss of and their parents, enrolled from the Chinese Deafness Genome Project, were studied. Hearing levels were assessed using pure tone audiometry, behavioral audiometry, auditory steady state response（ASSR）, auditory brainstem response（ABR） thresholds, and deformed partial otoacoustic emission（DPOAE）. Classification of hearing loss is according to the 2022 American College of Medical Genetics and Genomics（ACMG） Clinical Practice Guidelines for Hearing Loss. Whole exome sequencing（WES） and deafness gene Panel testing were performed on peripheral venous blood from probands and validations were performed on their parents by Sanger sequencing. Results:All 134 patients had childhood onset, exhibiting bilateral symmetrical slight-to-moderate sensorineural hearing loss, as indicated by audiological examinations. Of the 134 patients, 29（21.6%） had a family history of hearing loss, and the rest were sporadic patients. Genetic causative genes were identified in 66（49.3%） patients. A total of 11 causative genes were detected, of which GJB2 was causative in 34 cases（51.5%）, STRC in 10 cases（15.1%）, MPZL2 gene in six cases（9.1%）, and USH2A in five cases（7.6%）.The most common gene detected in slight-to-moderate hearing loss was GJB2, with c. 109G>A homozygous mutation found in 16 cases（47.1%） and c. 109G>A compound heterozygous mutation in 9 cases（26.5%）. Conclusion:This study provides a crucial genetic theory reference for early screening and detection of mild to moderate hearing loss in children, highlighting the predominance of recessive inheritance and the significance of gene like GJB2, STRC, MPZL2, USH2A.

Structural and functional brain network alterations in auditory sensitive period of congenital sensorineural hearing loss children: A research on magnetic resonance imaging

Structural and functional brain network alterations in auditory sensitive period of congenital sensorineural hearing loss children: A research on magnetic resonance imaging

Risk Factors for Natural Hearing Evolution in Newborns With Congenital Cytomegalovirus Infection

Congenital cytomegalovirus (cCMV) is the major cause of congenital nonhereditary sensorineural hearing loss in children. Currently, criteria to identify infants at increased risk for unfavorable hearing outcome are lacking. To identify risk factors associated with cCMV-related hearing improvement, hearing deterioration, and late-onset hearing loss. This multicenter cohort study included patients from 6 secondary and tertiary hospitals enrolled in the Flemish CMV registry (Belgium). Newborns with untreated cCMV infection with at least 4-year audiological follow-up were included. Patients who presented with other possible causes of sensorineural hearing loss were excluded. Data were collected for 15 years (January 1, 2007, to February 7, 2022) and analyzed from September 26, 2022, to January 16, 2023. Primary outcome was hearing evolution (per-ear analysis; described as stable hearing, improvement, or deterioration). The association of gestational characteristics, clinical findings, timing of seroconversion, viral load, and hearing status at birth with hearing evolution was investigated using effect sizes (Cramer V, odds ratio [OR], or Hedges g). Of the 387 children, 205 of 385 with nonmissing data were male (53.2%), 113 (29.2%) had a symptomatic infection, and 274 (70.8%) had an asymptomatic infection. Every child was 4 years or older at final hearing evaluation. A total of 701 of 774 ears (90%) showed stable hearing (normal hearing or stable hearing loss since birth) over time. Late-onset hearing loss (normal hearing at birth followed by hearing loss) was present in 43 of 683 ears (6.3%). Among children with hearing loss present at birth, 24 of 34 ears (70.6%) had hearing deterioration, and 6 of 91 ears (6.6%) had hearing improvement. Prematurity was associated with a higher chance of hearing improvement (OR, 12.80; 95% CI, 2.03-80.68). Late-onset hearing loss was more prevalent in a first trimester infection (OR, 10.10; 95% CI, 2.90-34.48). None of the 104 ears of children with a third trimester seroconversion developed late-onset hearing loss. Findings of this cohort study support that ongoing audiological follow-up for untreated children with congenital hearing loss is important, as the majority of patients had hearing deterioration. The timing of seroconversion was associated with the risk of developing late-onset hearing loss. These insights can aid in parental counseling, patient stratification, and follow-up. Future research should focus on the effect of treatment, the influence of determined risk factors, and the study of eventual new risk factors in patients at high risk to develop hearing loss.

Sensorineural hearing loss after cardiac surgery: a systematic review.

Sensorineural hearing loss (SNHL) may occur following cardiac surgery. Although preventing post-operative complications is vitally important in cardiac surgery, there are few guidelines regarding this issue. This review aimed to characterize SNHL after cardiac surgery. This systematic review was registered on PROSPERO and conducted in accordance with PRISMA guidelines. A systematic search of the PubMed, Embase and Cochrane Library were conducted from inception. Eligibility determination, data extraction and methodological quality analysis were conducted in duplicate. There were 23 studies included in the review. In the adult population, there were six cohort studies, which included 36 cases of hearing loss in a total of 7135 patients (5.05 cases per 1000 operations). In seven cohort studies including paediatric patients, there were 88 cases of hearing loss in a total of 1342 operations. The majority of cases of hearing loss were mild in the adult population (56.6%). In the paediatric population 59.2% of hearing loss cases had moderate or worse hearing loss. The hearing loss most often affected the higher frequencies, over 6000 Hz. There have been studies indicating an association between hearing loss and extracorporeal circulation, but cases have also occurred without this intervention. SNHL is a rare but potentially serious complication after cardiac surgery. This hearing loss affects both paediatric and adult populations and may have significant long-term impacts. Further research is required, particularly with respect to the consideration of screening for SNHL in children after cardiac surgery.

Impact of inner ear malformation and cochlear nerve deficiency on the development of auditory-language network in children with profound sensorineural hearing loss.

Profound congenital sensorineural hearing loss (SNHL) prevents children from developing spoken language. Cochlear implantation and auditory brainstem implantation can provide partial hearing sensation, but language development outcomes can vary, particularly for patients with inner ear malformations and/or cochlear nerve deficiency (IEM&CND). Currently, the peripheral auditory structure is evaluated through visual inspection of clinical imaging, but this method is insufficient for surgical planning and prognosis. The central auditory pathway is also challenging to examine in vivo due to its delicate subcortical structures. Previous attempts to locate subcortical auditory nuclei using fMRI responses to sounds are not applicable to patients with profound hearing loss as no auditory brainstem responses can be detected in these individuals, making it impossible to capture corresponding blood oxygen signals in fMRI. In this study, we developed a new pipeline for mapping the auditory pathway using structural and diffusional MRI. We used a fixel-based approach to investigate the structural development of the auditory-language network for profound SNHL children with normal peripheral structure and those with IEM&CND under 6 years old. Our findings indicate that the language pathway is more sensitive to peripheral auditory condition than the central auditory pathway, highlighting the importance of early intervention for profound SNHL children to provide timely speech inputs. We also propose a comprehensive pre-surgical evaluation extending from the cochlea to the auditory-language network, showing significant correlations between age, gender, Cn.VIII median contrast value, and the language network with post-implant qualitative outcomes.

The association of bisphenol A and paraben exposure with sensorineural hearing loss in children.

Bisphenol A (BPA) and parabens (PBs) are chemicals that are extensively used in personal care products (PCPs). In early childhood development, hearing is critical to speech and language development, communication, and learning. In vitro and in vivo, BPA/PBs exhibited neurotoxicity through elevated levels of oxidative stress. BPA also has the potential to be an ototoxicant. Therefore, this study aimed to determine the association of exposure to BPA/PBs with sensorineural hearing loss in children. A cross-sectional study based on hearing tests was conducted. This study enrolled 320 children aged 6-12years from elementary school. Urinary BPA and PB concentrations were analyzed by using liquid chromatography-tandem mass spectrometry (LC-MS/MS). Logistic regression models were employed to determine the association of BPA/PB exposure with sensorineural hearing loss. Children with sensorineural hearing loss had higher BPA concentrations than normal-hearing children (0.22ng/ml vs. 0.10ng/ml, p = 0.05). After adjustment for covariates, the risk of hearing loss at middle frequencies reached 1.83-fold (95% CI: 1.12-2.99) when BPA concentrations increased by 1 log10. The risk of slight hearing loss reached 2.24-fold (95% CI: 1.05-4.78) when children had a tenfold increase in ethyl paraben (EP) concentration. This study clarifies the role of exposure to BPA/PBs in hearing loss in children. Future research needs to be expanded to include cohort designs and nationwide studies to identify causality.

Congenital Cytomegalovirus-Associated Sensorineural Hearing Loss in Children: Identification Following Universal Newborn Hearing Screening, Effect of Antiviral Treatment, and Long-Term Hearing Outcomes.

Congenital cytomegalovirus (cCMV) is the most common cause of nongenetic sensorineural hearing loss (SNHL) in children. We examined the longitudinal hearing outcomes of children with cCMV in relation to their newborn hearing screening findings, and their use of antiviral therapy. The study was based on a retrospective chart review using a database of pediatric patients (N = 445) seen at the University of Minnesota Lions clinic. Chart review identified infants with cCMV, and records were reviewed for information about universal newborn hearing screen (UNHS) results, the clinical course of SNHL, and the use of antiviral therapy. A total of 44 children were identified with cCMV. In this group, 33 (75%) had SNHL of varying degree and age at onset. Notably, 17 (39%) children passed UNHS bilaterally. Of those children, 6 (35%) ultimately acquired bilateral or unilateral SNHL, detected at a mean age of 20 months (median age, 12 months). Five out of 10 children (50%) that did not pass UNHS in one ear acquired late-onset hearing loss in the contralateral ear, identified at a mean age of 24 months (median age, 4 months). Eleven (25%) children passed UNHS bilaterally and continued to demonstrate normal hearing in both ears at their most recent follow-up visit at a mean age of 19 months (SD, 18 months). Of the 33 children with cCMV and SNHL, 18 (55%) received antiviral medication (ganciclovir and/or valganciclovir). While, on average, both treated and untreated ears experienced a progression of hearing loss over time, the group that received antiviral treatment experienced less overall hearing change compared with the untreated group (baseline-adjusted expected mean difference, -10.5 dB; 95% confidence interval, -28.1 to 7.2 dB). Among children with cCMV included in this study who passed UNHS in both ears, 35% demonstrated delayed-onset SNHL. Notably, of those children who referred unilaterally, 50% later demonstrated SNHL in the contralateral ear. These findings have implications for audiological monitoring, and potentially antiviral therapy, of children with cCMV. As implementation of universal cCMV screening moves forward, a key aspect of follow-up will be appropriate long-term audiologic monitoring.

Prevalence and Profiles of Late-Onset Hearing Loss in Preschool Children with Autism Spectrum Disorder Who Passed Newborn Hearing Screening in a South East Asian Population.

Prevalence of hearing loss in children with autism spectrum disorder (ASD) is uncertain, as it is more challenging to assess hearing function in children with developmental difficulties (DD). We aimed to determine the prevalence and profiles of hearing loss in preschool children with ASD in a Southeast-Asian population who passed newborn hearing screening. A retrospective study of preschool children with DD (ASD, Global Developmental Delay (GDD), and Speech and Language Delay (SLD)) attending the Child Development Unit (CDU) at our hospital was performed. Three hundred and thirty-three children (ASD: n = 129; GDD: n = 110; and SLD: n = 94) underwent hearing assessments. Of these, 10.8% of children (n = 36, comprising 15 with ASD, 12 with GDD and 9 with SLD) had confirmed hearing loss. Hearing loss was predominantly bilateral in children with ASD and GDD; in those with SLD, unilateral and bilateral hearing loss were equally common. Conductive hearing loss occurred as frequently as sensorineural hearing loss in children with ASD and SLD, but was the dominant subtype in those with GDD. Moderate to severe hearing loss (n = 2) was noted only in children with ASD. Children with ASD and GDD required significantly more audiology visits and procedures to obtain conclusive hearing test results, compared to those with SLD. The need to identify hearing loss and monitor for resolution is particularly important in vulnerable populations with communication deficits, such as in those with ASD.

Hearing and speech outcomes in children with cochlear nerve hypoplasia who underwent cochlear implantation

Background: Data on hearing and speech outcomes following cochlear implantation in prelingual sensorineural hearing loss (SNHL) children with cochlear nerve hypoplasia, especially in Indonesia, is still limited. Purpose: To evaluate the hearing and speech function of prelingual SNHL children who had undergone cochlear implantation. Method: A retrospective cross-sectional study was conducted on 28 children who had undergone cochlear implantation procedures at the Department of Otorhinolaryngology and Head and Neck Surgery, Dr. Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia, for more than 12 months. The hearing and speech function of the subjects were assessed using the modified Categories of Auditory Performance (CAP-II) and the speech intelligibility rating (SIR) scores. Result: Children with cochlear nerve hypoplasia showed an increase in CAP-II and SIR scores after using cochlear implants for more than 12 months (versus pre-surgery, high CAP score [≥5]: 89.3% [25/28 children] vs 3.6% [1/28 children] and high SIR score [≥3]: 82.1% [23/28 children] vs 7.1% [2/28 children]; both p&lt;0.001). Post-operative CAP-II and post-operative SIR scores were highly positively correlated (r=0.705, p&lt;0.001). Type of cochlear nerve hypoplasia (unilateral vs bilateral) were not significantly associated with post-operative CAP-II and SIR scores (p=0.382 and p=0.459, respectively). Conclusion: Prelingual SNHL children with cochlear nerve hypoplasia may still get the benefit from cochlear implantation, as shown by a remarkable improvement in hearing and speech function as assessed with the CAP-II and SIR scores.&#x0D; Keywords: auditory perception, cochlear implants, cochlear nerve hypoplasia, prelingual sensorineural hearing loss, speech intelligibility

The Relationship between Prenatal Risk Factors and The Incident of Sensorineural Deafness in Children at Polyclinic ENT- Head and Neck Zainoel Abidin Hospital Banda Aceh *,

Background: Sensorineural deafness is one of congenital disorders that caused by prenatal, natal, and postnatal factors. Sensorineural deafness is a public health problem in the world due to its high prevalence and highly negative impact on child development. Objective: To determine the relation between prenatal risk factors (family history of hearing loss, ototoxic drugs consumption, history of infection, history of Diabetes Mellitus, history of gestational hypertension) with sensorineural hearing loss in children at ENT- Head and Neck Polyclinic Dr. Zainoel Abidin Hospital. Methods: This study was analytic retrospective with cross sectional design. The sampling technique used was simple random sampling. The total samples are 47 children at the ENT-Head and Neck Polyclinic of RSUDZA who has been examined with OAE, BERA and ASSR from January 2020 to December 2021 and diagnosed with sensorineural hearing loss. Data were analyzed using SPSS which bivariate analysis using Chi- square and Fisher’s exact. Results: 47 children who had been diagnosed with sensorineural hearing loss included in the inclusion criteria. The mean age of children in this study was 3.44 years with male predominance of 55.3%. The most severe degree of hearing loss was found with the percentage of weight (93.6%) in right ear and (89.4%) in left ear. A history of maternal infection during pregnancy was the most common risk factor found in this study with profound hearing loss in both ears with p value = 0.05. Conclusion: No relation between prenatal risk factors and sensorineural hearing loss in children.

The Audiological Follow-Up of Children with Symptomatic Congenital Cytomegalovirus Infection: An Experience in Two Italian Centers.

Congenital cytomegalovirus (cCMV) infection is the leading cause of non-hereditary sensorineural hearing loss in children. While about 10% of children reportedly display symptoms at birth, 85-90% of cCMV infection cases are asymptomatic. However, 10-15% of these asymptomatic infants may later develop hearing, visual, or neurodevelopmental impairments. This study aimed to evaluate the impact of cCMV infection on newborns' hearing function with a particular emphasis on progressive and late-onset cases. This study is a retrospective chart analysis with longitudinal character and was conducted in two Italian centers: Center 1 (from 1 November 2007 to 31 December 2021) and Center 2 (from 1 January 2012 to 31 December 2021). Data collected included newborn hearing screening results, characterization of hearing loss (unilateral/bilateral, degree of impairment), and audiological follow-up. The cohort consisted of 103 children (42% males, 58% females). In total, 28 children presented with hearing impairment; 71.4% (20 out of 28) of the cases of hearing loss were severe/profound, with 35.7% of the cases due to unilateral hearing loss. Out of twenty-eight, six experienced progression of hearing loss and four had late-onset hearing loss. In the absence of universal cCMV screening, hearing screening at birth for cCMV remains a critical factor for early diagnosis. A significant percentage of children affected by cCMV with normal audiological evaluations at birth is easily lost to follow-up. Close collaboration between neonatologists, pediatricians, and audiological services is fundamental to ensure timely diagnosis and treatment of cCMV-related hearing loss.

The effect of bony cochlear nerve canal (BCNC) diameter on the degree of sensorineural hearing loss.

This study investigated the correlation between the diameter of the bony cochlear nerve canal (BCNC), as determined by Temporal bone CT, and MRI findings of cochlear nerves (CN) in children with sensorineural hearing loss (SNHL). A prospective study design was followed. Radiological data (Temporal bone CT and MRI) of fifty children with sensorineural hearing loss (age <18y) were included in the study. All patients (100 ears) underwent routine MRI protocol in addition to 3D CISS (3-D constructive interference in steady state). Based on CT findings, the BCNC was classified according to its diameter into three groups; group 1 (<1.4mm), group 2 (1.4-2.0mm), and group 3 (>2.0mm). A significant difference between the three groups at degrees of SNHL (p<0.001) was observed. Significant difference (p<0.001) was also observed in the mean level of pure tone audiometry (PTA) average in group 1 compared to group 2. The CN was absent in 20 ears of group 1 CT results (29%), CN hypoplasia was noticed in 40 ears of group 1 CT (58%). However, CN was present in 9 ears of group 1 CT (13%), while in group 2 and 3, CN was present in 100% of the cases (27, and 4 ears, respectively, p<0.001). MRI and CT imaging are valuable in the diagnosis of SNHL in children. Moreover, with BCNC stenosis, there was a high probability of CN aplasia or hypoplasia.

Analysis of clinical features and prognostic correlation factors of sudden sensorineural hearing loss in children.

This study aimed to investigate the clinical features and prognostic correlation factors of sudden sensorineural hearing loss in children (CSSNHL). From January 2016 to December 2021, the clinical data of hospitalized children presenting with sudden sensorineural hearing loss, including age, gender, the ear of onset, onset of treatment, concomitant symptoms, the degree of hearing loss, and audiogram curve type, were retrospectively collected and the effective rate of treatment and the factors affecting prognosis were statistically analyzed. The effective rate of CSSNHL was 29.97%. Univariate analyses showed that the onset of treatment, the degree of hearing loss, audiogram curve type, and tinnitus were associated with prognosis (P<0.05). Multivariate analyses showed that onset of treatment was correlated with prognosis (OR=0.939, 95% CI=0.911-0.969, P<0.001). Compared with patients in the profound group, the therapeutic performance of the severe, moderate, and mild groups were significantly different (OR=9.951, 11.264, 13.373, 95% CI=2.311-42.856, 2.818-45.028, and 5.310-33.677, P<0.05). Compared with patients with profound audiogram, ascending audiogram and flat audiogram were related to therapeutic performance (OR=13.373 and 14.481, 95% CI=5.310-33.677, 6.509-32.217, P<0.001). The prognosis of CSSNHL patients was related to the onset of treatment, the degree of hearing loss, and the audiogram curve type. Patients who received earlier treatment, had lighter hearing loss and the ascending and flat audiograms exhibited improved prognosis.

Evaluation of Cognitive Function Using P300 Latency and Amplitude in Children with Profound Sensorineural Hearing Loss Using Hearing Aids: A Comparative Cross-sectional Study

Introduction: As hearing function is directly linked to language and communication abilities, hearing loss may negatively affect children’s cognitive development. Therefore, it is important that hearing-impaired children undergo cognitive function assessments to better understand how to alleviate this comorbidity. The P300 event-related potential measurement is a neurophysiological test increasingly used to objectively measure cognitive function. Aim: The aim of the study was to compare the latency and amplitude of P300 waves in children with profound sensorineural hearing loss (SNHL) with hearing aids and normal-hearing children aged 7–15 years old. Methods: A comparative cross-sectional study involving 15 children with profound SNHL and 15 children with normal hearing was conducted. The latency and amplitude of P300 waves in these children in response to auditory and visual stimuli were recorded and analyzed. Results: The mean P300 wave latency in children with SNHL was slightly longer than that in children with normal hearing, albeit insignificant (P = 0.578). Similarly, the amplitude of the P300 waves was not significantly smaller in children with SNHL (P = 0.885). Conclusions: SNHL children with hearing aid habilitation have comparable P300 amplitude and latency compare to normal-hearing children.