Hearing Loss In Children Research Articles

Congenital Cytomegalovirus Severity Definitions and Treatment Decisions around the World: A Systematic Scoping Review of the Literature.

Congenital cytomegalovirus (cCMV) is the most common cause of congenital infection and the leading cause of non-genetic sensorineural hearing loss in childhood. While treatment trials have been conducted in symptomatic children, defining asymptomatic infection can be complex. We performed a scoping review to understand how infection severity is defined and treated globally, as well as the various indications for initiating treatment. We conducted an electronic search of MEDLINE, EMBASE, Scopus, and the Cochrane Library, using combinations of the following terms: "newborn", "baby", "child", "ganciclovir", "valganciclovir", and "cytomegalovirus" or "CMV". We included eligible prospective and retrospective studies, case series, and randomized clinical trials (RCTs) published up to May 2024. A total of 26 studies were included, of which only 5 were RCTs. There was significant heterogeneity between studies. The most commonly considered criteria for symptomatic infection were microcephaly (23/24 studies), abnormal neuroimaging (22/24 studies), chorioretinitis/ocular impairment (21/24 studies), and hearing impairment (20/24 studies). Two studies also included asymptomatic newborns in their treatment protocols. Outcome measures varied widely, focusing either on different hearing assessments or neurocognitive issues. Our literature analysis revealed significant variability and heterogeneity in the definition of symptomatic cCMV infection and, consequently, in treatment approaches. A consensus on core outcomes and well-conducted RCTs are needed to establish treatment protocols for specific groups of newborns with varying manifestations of cCMV.

Wideband tympanometry in otitis Media

BackgroundOtitis media with effusion (OME) is one of the most frequent infectious diseases in children and is the most common cause of acquired hearing loss in childhood. Diagnosis of OME is mainly dependent on the single-frequency tympanometry. Wideband tympanometry (WBT) is an emerging technology that has been utilized to overcome the limitations of conventional tympanometric procedures. This study aimed to evaluate the diagnostic value of wideband acoustic absorbance in children suffering from otitis media.MethodsThis study included a control group of 50 children with 100 ears with bilateral normal peripheral hearing and bilateral normal middle ear function and a study group which included 100 children with 176 ears complaining of otitis media. Measures included subjects history, basic audiological evaluation and wideband tympanometry. Resonant frequency, tympanometric peak pressure and middle ear absorbance were measured from wideband tympanometry and compared in both groups.ResultsThere was a statistically significant reduction of resonsnce frequency in the study group in comparison to the control group. Also, there was a statistically significant reduction of tympanometric peak pressure in the study group in comparison to the control group. As regards the absorbance, there was a statistically significant reduction of absorbance in the study group in comparison to the control group at all tested frequencies (250Hz- 8000Hz). The best area under the receiver operating characteristic curve values for diagnosing OM were obtained at all tested frequencies. Data of this work showed that higher values were mainly at 250Hz, 500Hz and 1kHz (0.84, 0.88 and 0.93 respectively).ConclusionFindings of this study suggested that wide band tympanometry can be used efficiently in various degrees of OM.

Structural and covariance network alterations of the hippocampus and amygdala in congenital hearing loss children

ObjectiveThe hippocampus and amygdala, as important components of the limbic system, play crucial roles in central remodeling in congenital hearing loss. This study aimed to investigate the morphological integrity and network properties of the subfields of hippocampus and amygdala in children with congenital hearing loss. MethodsA total of 24 children with congenital hearing loss and 17 age- and sex- matched healthy controls (HC) are included in the study. T1-weighted images are analyzed by segmenting the brain into cortical and subcortical regions. Intergroup difference of volumes were explored. Structural covariance networks for the whole brain and hippocampus-amygdala subregions were constructed. Between-group differences of network property are investigated by comparing area under a range of network sparsity. ResultsPatients with congenital hearing loss exhibited significantly larger volumes in the right dentate gyrus and CA3 of the hippocampus. However, there were no significant differences in total hippocampal or showed decreased global efficiency and increased characteristic path length, indicating reduced network integration. Lower betweenness centrality was observed in the left hippocampal fissure in the hearing loss group. The changes in volume and network topological properties are not affected by age and sex. ConclusionChildren with congenital hearing loss display specific volumetric increases in hippocampal subregions, suggesting compensatory adaptations to auditory deprivation. The hippocampus-amygdala network shows significant reorganization, potentially underpinning cognitive and behavioral development issues associated with congenital hearing loss. These findings highlight the importance of targeted neural substrates in understanding and addressing the developmental challenges faced by children with congenital hearing loss.

Navigating childhood hearing loss: the experiences of parents, grandparents and siblings

Purpose This study aimed to explore the impact of childhood hearing loss on the family unit and their resulting intervention needs. Materials & Methods Qualitative descriptive methodology was used, with in-depth interviews analysed using reflexive thematic analysis. Four family units of children with hearing loss participated in the study, including parents (n = 5), grandparents (n = 7), and siblings (n = 5). Results Five themes were developed from interview data: (1) the daily grind; (2) we’re all in this together; (3) family dynamics; (4) the early intervention experience and (5) personal growth and adaptations. Family members were impacted in multi-faceted ways and identified informational and emotional intervention needs, with an integrative theme highlighting the emotional toll of childhood hearing loss on families. Conclusions Early intervention services have a crucial role in addressing third-party disability through a multi-disciplinary service delivery model that addresses the needs of all family members, beyond the child with hearing loss.

Hearing Loss in Children with 22q11.2 Deletion Syndrome

Hearing Loss in Children with 22q11.2 Deletion Syndrome

Neonatal Markers of Prematurity as Predictors of Permanent Childhood Hearing Loss and Neurodevelopmental Impairment in Children Admitted to the Neonatal Intensive Care Unit.

Need for admission to the neonatal intensive care unit (NICU) confers an increased risk of hearing loss in the newborn and of later neurodevelopmental impairment. In this retrospective longitudinal case-controlled study, we assess how the degree of prematurity, measured via gestational age, birth weight, and z-scores, in 138 infants admitted to the NICU are associated with permanent childhood hearing loss (PCHI) and 2-year developmental outcomes. Logistic regression analyses, Kruskal-Wallis analysis of variance, and Chi-squared tests were used. Independent of prematurity, PCHI and NICU admission were predictive of poor developmental outcomes. Twenty-one (47%) children with PCHI had a moderate-to-severe developmental delay, compared to three (7%) matched controls. Days in the NICU but not z-scores predicted PCHI. Z-score was not prognostic of moderate or severe developmental impairment in children with PCHI. The odds ratio of moderate-to-severe neurodevelopmental impairment with PCHI was high, at 12.48 [95% CI = 3.37-46.40]. Children with PCHI were significantly more likely to have cerebral palsy than their matched counterparts (30% vs. 2%). These findings challenge the conventional focus on gestational age and birth weight on neurodevelopmental outcomes for children with PCHI and NICU admission. A more nuanced approach to monitoring and intervention is needed.

Situational assessment of age of suspicion, diagnosis, and intervention of hearing loss in pediatric population in India and factors that influence them

ObjectiveIn the absence of newborn hearing screening, identification of childhood hearing loss is primarily mediated by parents’ help-seeking behaviour. This study attempted to measure the variables age of suspicion, diagnosis, and intervention of congenital hearing loss in India in a large cohort to verify the efficiency of this approach. It aimed to study the influence of parental perceived awareness, accessibility, and affordability on these milestones. MethodData were collected through a survey method of research using a pre-developed questionnaire as the instrument. The study involved 384 parents of children with hearing loss receiving hearing healthcare services from various healthcare institutions and clinics in Karnataka, India. ResultsThe mean age of suspicion, diagnosis, and intervention of hearing loss after data analysis were 18.6, 25.0, and 30.6 months, respectively. Earlier parental suspicion of hearing loss was associated with earlier diagnosis and intervention in the child. While less than 4 % of the cohort was diagnosed before three months, less than 1 % received intervention within six months of age. Lack of awareness, accessibility, and affordability significantly delayed these milestones. Among the participants, 15.03 % had experienced at least one of the three barriers (lack of awareness, accessibility, and affordability), whereas 35.28 % had reported two barriers, and 41.71 % had reported all three barriers for their help-seeking behavior. ConclusionsRelying on parental help-seeking behaviour cannot result in early identification and intervention required for optimum pediatric hearing healthcare. India should implement comprehensive early hearing detection and intervention program that includes universal newborn hearing screening and addresses awareness, accessibility, and affordability of hearing healthcare services as critical components.

Socioeconomic Disparities and Other Factors Affecting Time to Dispensing of Pediatric Hearing Aids.

Pediatric hearing loss can significantly impact speech, language, social, and educational development. Providing access to speech and environmental sounds using amplification devices, such as hearing aids, can help improve developmental outcomes. However, timely rehabilitation and intervention may be delayed due to limited access to resources, further prolonging the adverse effects of childhood hearing loss. The aim of this study was to investigate socioeconomic barriers in time to dispensing hearing aids in a diverse pediatric patient population. Data from an existing internal database from a tertiary pediatric hospital were analyzed from January 2020 through August 2022 for barriers associated with hearing aid (HA) dispensing delays. Demographic and clinical characteristics were obtained. Multivariate regression and survival analysis statistics were used to identify factors associated with delayed time to dispensing hearing aids. Of the 121 patients who had been appropriately diagnosed and fit, 108 (89.3%) had received hearings aids and 13 (10.7%) had not at the time of the study. Of those who had received HA, time to dispensing was not significantly impacted by sex, race, ethnicity, language, or income level. Insurance was found to be an influencing factor in time to receiving the HA. Factors such as insurance and hearing loss laterality contribute to delays in receiving hearing aids. Identifying these specific barriers and disparities in hearing rehabilitation services will prove vital in facilitating an expedited and equitable pathway to receiving hearing aids. 3 Laryngoscope, 2024.

Mapping neonatal hearing screening services in Cape Town metro: A situational analysis.

Childhood hearing loss is a global health concern. Despite the proven benefits of neonatal hearing screening (NHS), it is not yet mandated in South Africa. The lack of awareness of hearing loss and absence of NHS leads to delayed diagnosis and adverse developmental outcomes for affected children. The study aimed to assess the availability of NHS services across primary healthcare (PHC) facilities in the City of Cape Town (CCT). Surveys were conducted with 26 PHC facilities in the CCT metropolitan areas that offer mother and child healthcare services. Surveys gathered data through online and telephone methods. The surveys aimed to assess the availability and nature of NHS services, care pathways and training of healthcare professionals regarding NHS. None of the facilities used objective screening methods to screen hearing or have standardised care pathways for at-risk babies. Instead, they relied on parental concerns, with the use of the Road to Health book. None of the respondents reported having received hearing screening training, and the majority of participants (62%) lacked confidence in their knowledge of ear and hearing care. The absence of NHS services highlights the need for standardised protocols and increased awareness among healthcare workers and caregivers. Implementing NHS services could facilitate earlier diagnosis and intervention of hearing loss for infants in the Western Cape.Contribution:This study's findings could guide efforts to improving access to NHS access at PHC level in Cape Town, ultimately providing early hearing screening services to infants.

Exploring the implementation of an educational film within antenatal care to reduce the risk of cytomegalovirus infection in pregnancy: A qualitative study

BackgroundCongenital cytomegalovirus (CMV) infection is a leading cause of sensorineural hearing loss and neuro-disability in childhood. In the absence of a licensed vaccine, adoption of hygiene-based measures may reduce the risk of CMV infection in pregnancy, however these measures are not routinely discussed with pregnant women as part of National Health Service (NHS) antenatal care in the United Kingdom (UK).MethodsAn exploratory qualitative study was conducted, underpinned by Normalization Process Theory (NPT), to investigate how an educational intervention comprising of a short film about CMV may best be implemented, sustained, and enhanced in real-world routine antenatal care settings. Video, semi-structured interviews were conducted with participants who were recruited using a purposive sample that comprised of midwives providing antenatal care from three NHS hospitals (n = 15) and participants from professional colleges and from organisations or charities providing, or with an interest in, antenatal education or health information in the UK (n = 15).FindingsMidwives were reluctant to include CMV as part of early pregnancy discussions about reducing the risk of other infections due to lack of time, knowledge and absence of guidance or policies relating to CMV in antenatal education. However, the educational intervention was perceived to be a useful tool to encourage conversations and empower women to manage risk by all stakeholders, which would overcome some identified barriers. Macro-level challenges such as screening policies and lack of official guidelines to legitimise dissemination were identified.DiscussionSuccessful implementation of education about CMV as part of routine NHS care in the UK will require an increase in awareness and knowledge about CMV amongst midwives. NPT revealed that ‘coherence’ and ‘cognitive participation’ between service members are vital to imbed CMV education in routine practice. ‘Collective action’ and ‘reflexive monitoring’ is required to sustain service changes.

Hamartomas of the Tuber Cinereum Associated with X-Linked Deafness Show Signs of Pubertas Tarda Instead of Pubertas Praecox and No Gelastic Seizures-Long-Term Follow-Up of 12 Years.

Hamartomas of tuber cinereum present as ectopic tissue in the hypothalamic region. Clinically, the usual hypothalamic hamartomas manifest themself by gelastic seizures and pubertas praecox. We observed an increased coincidence of the presence of X-linked recessive deafness DFNX2 (DFN3) and a hamartoma of the tuber cinereum. Initially five patients presented with hearing loss in childhood, two additional were already adults, not showing any characteristic symptoms for a hamartoma but signs of delayed puberty. Seven patients who underwent computed tomography imaging due to a sensorineural hearing loss and had a hamartoma of the tuber cinereum in addition to X-linked deafness DFNX2 (DFN3) were included in a retrospective study. Patients underwent initial neurologic, endocrinologic, and genetic evaluation. Long-term follow-up was performed after 10 to 12 years. The average age at the initial exam was 12.9 years (range 4-29). All patients genetically proven nonsyndromic, X-linked deafness associated with the POU3F4 gene. Three out of six patients presented signs of delayed puberty. None of all seven showed any evidence of pubertas praecox or gelastic seizures at mean age of 17 years (range 17-29 years) at any time. Hamartomas of tuber cinereum are often coincident with DFNX2. Clinically, half of the cases are-in contrary to the usual pubertas praecox-associated with growth hormone deficiency and delayed puberty, in the sense of pubertas tarda, when coincident. Clinicians' and radiologists' knowledge and awareness of this rare combination are crucial to identify children early enough for hormone-sensitive treatment.

Prevalence and Risk Factors of Hearing Loss in Children in Mumbai, India

Introduction: Hearing loss (GP) is a significant public health problem in India, especially in children. In Mumbai, the prevalence of GP among children is not yet known with certainty. This study aims to determine the prevalence and risk factors of GP in children in Mumbai. Methods: This research was conducted cross-sectionally involving 500 children aged 5-15 years in Mumbai. Data was collected through interviews and hearing examinations. Risk factors analyzed included age, gender, socioeconomic status, noise exposure, and history of ear infections. Results: The prevalence of GP among children in Mumbai is 12%. Risk factors significantly associated with GP were age, noise exposure, and history of ear infections. Conclusion: The prevalence of GP among children in Mumbai is quite high. The main risk factors for GP are age, noise exposure, and history of ear infections. Efforts to prevent GP in children in Mumbai need to focus on controlling noise exposure and treating ear infections early.

Newborn congenital cytomegalovirus screening and hearing outcomes: a systematic review of current literature.

The purpose of this review is to summarize the very recent literature surrounding hearing outcomes of children with congenital cytomegalovirus (cCMV) detected through systematic screening programs. There are several different approaches to cCMV screening including forms of targeted vs. universal screening of newborns as well as maternally-derived prenatal testing. However, many studies fail to document hearing-related outcomes both in the newborn period and further into childhood when late-onset sensorineural hearing loss (SNHL) can occur. This systematic review included studies of neonates screened for cCMV reporting hearing outcomes for at least one point in time. Hearing targeted screening appeared the most widely reported for detection of unilateral and bilateral SNHL in those with cCMV. A few studies examined these clinical findings in relation to antiviral treatment. Congenital CMV is an important and common cause of childhood hearing loss. Newborn screening programs may expand opportunities for early diagnosis and treatment of the infection and its sequelae.

Efficacy of cochlear implantation in cochlear nerve deficiency children – A single center study

PurposesThe objective of this research was to interpret and analyze the imaging, audiological features and cochlear implantation outcomes in cochlear nerve deficiency children. Material and methodsRetrospective analysis. Results25 prelingual hearing loss children diagnosed cochlear nerve deficiency (CND), the age range from 1 to 15 (mean age, 5.04), underwent cochlear implantation at Ear Nose and Throat Hospital - Ho Chi Minh City (ENT hospital – HCMC) from 2016 to 2023. All children had sensorineural hearing loss (SNHL) from severe to profound degree. Magnetic resonance imaging (MRI) showed cochlear nerve hypoplasia in 76 % and cochlear nerve aplasia in 24 % of cases. Inner ear malformations were found in 52 % of cases. The mean Categories of Auditory Performance (CAP) score at 1 year after surgery was 4.8. At 6 months and 1 year after surgery, the mean CAP score of the aplasia group was significantly lower than that of the hypoplasia group (p < 0.05). ConclusionsIn cochlear nerve deficiency children, auditory perception and speech performance still improved after cochlear implantation. However, this progress was significantly limited in cochlear nerve aplasia group.

Detecting Hearing Loss Through Targeted Surveillance: Risk Registry and Surveillance Timeframe Recommendations.

The purpose of this study was to inform the revision of a targeted surveillance risk registry by identifying which risk factors predict postnatally identified hearing loss (PNIHL) in children who pass newborn hearing screening and to determine whether hearing surveillance beyond the age of 1 year is warranted. We used retrospective analysis of the audiological outcomes of children born in the state of Queensland, Australia, between January 1, 2010, and December 31, 2019, who passed the newborn hearing screen with risk factors. Approximately one third of children were lost to follow-up and could not be included in the analysis. Risk factors that predicted PNIHL in the analyzed cohort were as follows: syndromes associated with hearing loss, craniofacial anomalies, perinatal infections, and family history of permanent childhood hearing loss. Severe asphyxia did not predict PNIHL but yielded some cases of significant bilateral hearing loss. Hearing loss in children with a history of prolonged ventilation was mild and/or unilateral in nature (except in cases where the hearing loss was due to an unrelated etiology). There were no cases of PNIHL in children with hyperbilirubinemia or neonatal bacterial meningitis. For the risk factors that predicted PNIHL, nearly all hearing losses were detected by 1 year of age, except for children with family history where one quarter of hearing losses had a later onset. The four risk factors recommended for efficient postnatal identification of hearing loss are as follows: syndromes associated with hearing loss, craniofacial anomalies, perinatal infection, and family history of permanent childhood hearing loss. Hearing surveillance through to 1 year old is sufficient except for children with a family history, where a second phase assessment is indicated. Alternative targeted surveillance protocols and models of care are required to minimize loss to follow-up.

Pre-school hearing screening is necessary to detect childhood hearing loss after the newborn period: a study exploring risk factors, additional disabilities, and referral pathways

Objective To explore strategies for detecting childhood hearing loss, aside from newborn hearing screening. Design A retrospective review of medical records on the modes of detection of hearing loss, risk factors for late-onset hearing loss, hearing loss degree, aetiology, additional disabilities, and timelines from referral to intervention. Study sample Children, born 2006 to 2015, enrolled for intervention whose hearing loss was detected up to age 7 years but not from newborn hearing screening (n = 326). Results Universal pre-school hearing screening detected 38% of the cohort at 4–5 years of age. Risk factors for late-onset hearing loss were present in 36% of children, 80% of whom had a reported family history. Sixty-nine percent had mild bilateral or unilateral hearing loss. Children with additional disabilities faced significantly longer delays from referral to intervention. Children self-referred due to parent concern had more severe degree of hearing loss than those referred from screening. Conclusion Most children with hearing loss detected after the newborn period do not have any known risk factors for late-onset hearing loss. Pre-school hearing screening is needed for comprehensive detection of hearing loss in early childhood. More work is needed towards improving timely diagnosis and intervention for children with additional disabilities.

QOL-44. EARLY SEVERE HEARING LOSS IN CANADIAN CHILDREN WITH CENTRAL NERVOUS SYSTEM TUMORS: A POPULATION-BASED COHORT STUDY

Abstract BACKGROUND Severe treatment-related hearing loss (HL) is associated with devastating psychosocial/neurocognitive outcomes. Children with central nervous system (CNS) tumors are highly vulnerable to severe HL due to tumor location and treatment exposures. This is the first study to report national CNS tumor-specific rates and predictors of early severe HL. METHODS We conducted a retrospective, population-based cohort study of all children ≤15yrs at diagnosis with CNS tumors between 2001-2019 using the Cancer in Young People in Canada registry. The primary outcome was Common Terminology Criteria for Adverse Events grade 3/4 HL ≤5yrs following diagnosis. We determined prevalence and probabilities of early HL; predictors were determined using multivariable logistic regression models. RESULTS Among 3,201 children with CNS tumors, 5.1% experienced early HL. Children with medulloblastoma (N=570) and ATRT/other embryonal tumors (N=269) experienced disproportionate rates (16.1%, 15.2%, respectively); 85.4% (medulloblastoma) and 68.8% (ATRT/other embryonal) received cisplatin, 74.6% (medulloblastoma) and 52.1% (ATRT/other embryonal) were irradiated. In children with medulloblastoma, when controlling for follow-up, age &amp;lt;=5yrs at diagnosis [odds ratio (OR) 2.4, 1.5-3.8], radiation (OR 3.5, 1.6-7.6), and cisplatin (OR 20.4, 1.3-329.7) predicted early HL. In children with medulloblastoma post cisplatin, age ≤5yrs at diagnosis doubled the probability of early HL in both irradiated (29.8%, vs 15.3% if &amp;gt;5yrs at diagnosis) and non-irradiated patients (10.6% vs 4.8% if &amp;gt;5yrs at diagnosis). In children with ATRT/other embryonal, cisplatin (OR 28.2, 1.7-472.1) was the only predictor of early HL. CONCLUSIONS In the largest population-based study of early severe HL in children with CNS tumors, we report striking rates in children with embryonal tumors. Younger irradiated children had disproportionate probabilities of early HL, suggesting a novel additive interaction between age at diagnosis and radiation. Standardized otoprotection and research focusing on therapy de-escalation in young children with embryonal tumors are urgently needed.

Validation of the Chinese Version of the Speech, Spatial, and Qualities of Hearing Scale for Parents and Children.

To translate and validate the Chinese version of the Speech, Spatial, and Qualities of Hearing Scale (SSQ) for children with hearing impairment (C-SSQ-C) and for their parents (C-SSQ-P). We translated the SSQ for children into Chinese and verified its readability and comprehensibility. A total of 105 participants with moderate-to-profound hearing loss (HL) and 54 with normal hearing were enrolled in the validation process. The participants with HL were fitted with bilateral hearing aids, bimodal hearing, or bilateral cochlear implants. The C-SSQ-P was administered to the parents of participants aged 3 to 6.9 years, and the C-SSQ-C was administered to participants aged 7 to 18 years. The internal consistency, test-retest reliability, and validity were evaluated for both questionnaires. Both C-SSQ-P and C-SSQ-C demonstrated high internal consistency (Cronbach's α >0.8) and good validity (generalized linear model revealed significant negative relationships between the C-SSQ-P subscales with aided better-hearing threshold [β = -0.08 to -0.12, p ≤ 0.001] and between the C-SSQ-C subscales with worse-hearing threshold [β = -0.13 to -0.14, p < 0.001]). Among the children with HL, the participants with bilateral cochlear implants had demonstrated better performance than those with bimodal hearing and bilateral hearing aids, as evidenced by the highest mean scores in three subscales. Both C-SSQ-P and C-SSQ-C are reliable and valid for assessing HL in children and adolescents. The C-SSQ-P is applicable in evaluating young children aged 3 to 6.9 years after a 7-day observation period, while the C-SSQ-C is appropriate for children and adolescents aged 7 to 18 years.

Association of Residual Low-frequency Hearing Loss in Children and Type of Ventilation Tube Placed During Myringotomy With Tympanostomy Tube Placement

Objective: Assess whether titanium Tytan tympanostomy tubes result in a larger air-bone gap (ABG) at lower frequencies compared to Armstrong and Paparella tubes. Study Design: Retrospective cohort study. Setting: Pediatric otolaryngology department of a 1000-bed academic center. Patients: A cohort of 110 children, aged 4–16 years, who had no sensorineural hearing loss or congenital syndromes and underwent tympanostomy tube placement between August 2018 and February 2021. Interventions: Tympanostomy procedures using Tytan, Armstrong, or Paparella tubes. Main Outcome Measure: Evaluation of pure tone average (PTA) and ABG at frequencies of 500 and 250 Hz. Results: Tytan tubes enhanced PTA and ABGs across studied frequencies. Nonetheless, post-Tytan tube placement exhibited significantly worse PTA and 500 Hz ABG compared to Armstrong and Paparella tubes (P &lt; 0.05; P &lt; 0.0001). Additionally, 250 Hz ABG post-Tytan was inferior to post-Armstrong (P &lt; 0.0001). Relative risks of persistent ABG ≥ 15 dB with Tytan versus Armstrong tubes were 2.36 (1.62–3.44) at 500 Hz and 1.49 (1.09–1.99) at 250 Hz. Against Paparella tubes, these were 2.11 (1.28–3.72) at 500 Hz and 1.12 (0.81–1.67) at 250 Hz, the latter being not significant. Conclusion: While Tytan tubes optimize PTA, they are linked to enduring ABG ≥ 15 dB at lower frequencies. Notably, they perform less favorably in terms of 500 Hz PTA and ABG compared to both Armstrong and Paparella and demonstrate poorer 250 Hz ABG when juxtaposed with Armstrong tubes.

Etiology of Childhood Profound Sensorineural Hearing Loss: The Role of Hearing Loss Gene Panel Testing.

Establishing the cause of hearing loss(HL)is important and rewarding, though not without its challenges. Whileour ability to identify the etiology for HL has improved with advances in scientific knowledge, a significant proportion of cases remain of unknown etiology. Recent protocol changes within the NHS Genomic Medicine Service support the utilization of the HL gene panel test, rather than individual gene tests. In light of these changes, determining the yield of these more extensive panel tests is important in informing future practice. Retrospective study. The Cochlear Implant (CI) Department at Great Ormond Street Hospital (GOSH). Four hundred seventy-six children with profound HL were identified from a database of referrals to the GOSH CI Department. Data on etiology of HL including genetic diagnosis was collected from hospital notes on an electronic patient records system and hospital genetics database. We identified a positive result in 163/476 (34%) cases through the gene panel test, representing an additional 19% yield to current level 1 investigations. Genetic HL, including both syndromic (including those not covered by the HL gene panel) and nonsyndromic (209/476, 44%) was the most common etiology in our cohort. Perinatal, intrauterine, ototoxicity, meningitis, and encephalitis categories altogether comprised 97/476 (20%) cases. Gene panel testing provides significant additional yield over current level 1 investigations which include GJB2 testing only. This has far-reaching implications for how we optimize investigations into HL in children and counsel families, and for future early interventions.