Bilateral Hearing Loss Research Articles

A Novel De Novo Missense Variant in Netrin-1 (NTN1) Associated With Chorioretinal Coloboma, Sensorineural Hearing Loss and Polydactyly.

Microphthalmia, anophthalmia and coloboma (MAC) comprise a highly heterogeneous spectrum of congenital ocular malformations with an estimated incidence of 1 in 5000 to 1 in 30 000 live births. Although there is likely to be a genetic component in the majority of cases, many remain without a molecular diagnosis. Netrin-1 was previously identified as a mediator of optic fissure closure from transcriptome analyses of chick and zebrafish and was shown to cause ocular coloboma when knocked out in both mouse and zebrafish. Here, we report the first patient with chorioretinal coloboma and microphthalmia harbouring a novel heterozygous likely pathogenic NTN1 missense variant, c.1483T>A p.(Tyr495Asn), validating a conserved gene function in ocular development. In addition, the patient displayed bilateral sensorineural hearing loss which was investigated by examining the sensory hair cells of ntn1a morphant zebrafish, suggesting a role for netrin-1 in hair cell development.

A comatose vertebrobasilar occlusion patient recovering with bilateral hearing loss following full recanalization with mechanical thrombectomy.

Acute occlusion of vertebrobasilar artery mostly presents with severe neurologic impairment. Bilateral sensorineural hearing loss (SNHL) as the first clinical symptom on onset is a rare phenomenon. Clinical benefit of mechanical thrombectomy (MT) in terms of hearing loss in these cases is poorly understood. We describe a case of a patient with vertebrobasilar occlusion who presented with only sudden bilateral SNHL and recovered poorly in terms of hearing loss after full recanalization with MT. We describe a 52-year-old right-handed male patient with an unremarkable medical history. On admission, he was hypertensive to 200/102mm Hg; neurological examination was significant for mild right facial palsy and bilateral SNHL, later confirmed by an audiogram. diffusion-weighted imaging (DWI) showed infarction of bilateral anterior cerebellum, cerebellar peduncles, and pons with negative flair. Computed tomography angiography showed occlusion in the level of the vertebrobasilar junction. Patients' neurological status deteriorated within hours into a comatose status with anarthria and quadriplegia. He was treated with MT and stenting and full recanalization was achieved. All neurological examination findings have completely resolved, except for the bilateral SNHL. Acute onset of sudden bilateral deafness in isolation or accompanied by vestibular, cerebellar, and/or brainstem signs may indicate large vessel occlusion. Although rapid recanalization with MT helps improve the symptoms, the specific impact over SNHL varies between patients.

Genotype-First Analysis in an Unselected Health System-Based Population and Phenotypic Severity of COL4A5 Variants.

Our knowledge of X-linked Alport Syndrome comes mostly from selected cohorts with more severe disease. We examined the phenotypic spectrum of X-linked Alport Syndrome in males and females with a genotype-based approach using data from the Geisinger MyCode DiscovEHR study, an unselected health system-based cohort with exome sequencing and electronic health records. Patients with COL4A5 variants reported as pathogenic or likely pathogenic in ClinVar, or protein-truncating variants, were each matched with up to 5 controls without COL4A3/4/5 variants by sociodemographics, diabetes diagnosis, and year of first outpatient encounter. Phenotypes examined included dipstick hematuria, bilateral sensorineural hearing loss, proteinuria, decreased estimated glomerular filtration rate, and kidney failure. Out of 170,856 patients, there were 29 hemizygous males (mean age 52 y [SD 20]) and 55 heterozygous females (mean age 59 y [SD 19]) with a pathogenic/likely pathogenic COL4A5 variant, including 48 with the hypomorphic variant p.Gly624Asp. Overall, penetrance (having any Alport Syndrome phenotypic feature) was highest for non-p.Gly624Asp variants (males: 94%, females: 85%), intermediate for p.Gly624Asp (males: 77%, females: 69%), compared to controls (males: 32%; females: 50%). The proportion with kidney failure was highest for males with non-p.Gly624Asp variants (44%), intermediate for males with p.Gly624Asp (15%) and females with non-p.Gly624Asp variants (10%), compared to controls (males: 3%, females 2%). Only 47% of individuals with COL4A5 had completed albuminuria screening, and a minority were taking renin-angiotensin aldosterone system inhibitors. Only 38% of males and 16% of females had a known diagnosis of Alport Syndrome or thin basement membrane disease. Using a genotype-first approach, we show that men and women with X-linked Alport Syndrome are at higher risk of related phenotypic features with a wider spectrum of severity than has been described previously and variability by genotype.

Screening for Developmental Delays in Pediatric Cochlea Implant Candidates and Recipients.

Conduct a pilot clinical improvement project to effectively screen children with hearing loss for developmental delays. Children with hearing loss and cochlear implants (CIs) are at risk for additional developmental delays; however, screening to aid in early identification and referral for developmental delays is not routinely performed at CI centers. It is important to consider all aspects of child development to maximize CI outcomes and access to language. Caregivers of 31 children completed the Ages and Stages Questionnaire (ASQ) and the Sensory Profile-2 (SP2), which are standardized questionnaires that assess developmental milestones in areas of communication, gross motor, fine motor, problem solving, personal-social, and sensory integration. Participants were prospectively evaluated at a CI center in a tertiary medical center. Participants included children, aged ≤5 years old with bilateral hearing loss who use CIs or who were CI candidates, and their families. Scores on ASQ and SP2 questionnaires. Thirty-one children were screened, and approximately 40 to 50% screened positive for risk of developmental delay in areas excluding communication and received referrals for evaluations in occupational therapy (n = 16; 51.6%), physical therapy (n = 13; 41.9%), and developmental pediatrics (n = 13; 41.9%). Of children referred and seen for evaluations, six were diagnosed with developmental delays in at least one developmental area beyond the communication domains. Routine screening in children with significant hearing loss can successfully detect developmental delays, which may go unnoticed. This proactive approach enables timely and comprehensive treatment for developmental delays beyond those solely related to communication.

Dual task iTUG to investigate increased fall risk among older adults with bilateral hearing loss

Dual task iTUG to investigate increased fall risk among older adults with bilateral hearing loss

Early Hearing Outcomes and Audiological Experiences With a Novel Fully Implanted Cochlear Implant.

To review audiological experiences and early hearing outcomes from the early feasibility study of a fully implanted cochlear implant. Prospective cohort. Tertiary academic medical center. Three adults (two-male, one female) with bilateral sensorineural hearing loss. Implantation with a fully implanted cochlear implant as part of an early feasibility study. Postoperative unaided and aided pure-tone audiometry, tympanometry, mapping parameters, speech perception, battery life, and quality-of-life assessment. All patients in the early feasibility study of this fully implanted cochlear implant now use their devices regularly. Preoperative and postoperative audiometric measurements showed that their residual hearing in the implanted ear decreased slightly after surgery but was preserved. All patients had type A tympanograms after their transient middle ear effusion resolved. Electrical stimulation levels were comparable to those routinely used in traditional cochlear implants. Two of the three patients use a hearing aid in the implanted ear for additional gain and show significant improvement in speech perception since implantation. Average battery life before recharging is 4 days. All patients are regular users with two showing improvement in quality-of-life metrics after receiving the fully implanted cochlear implant. The patient experience and hearing outcomes from the early feasibility study of a novel fully implanted cochlear implant are detailed in this study and demonstrate ease of operation and daily use by all participants. All patients obtained hearing, but two of three use a hearing aid with their device to overcome unanticipated implant circuitry noise and achieve improved speech perception scores. Current work is focused on reducing this system noise to allow for the device to be used as intended, without a hearing aid.

Fostering inclusion in medical training: accommodations for hearing loss.

This article explores two case reports of healthcare students with hearing impairments in an Italian university and the accommodations provided during their clinical training. The first student, with bilateral sensorineural hearing loss, used the Contec Visual Electronic Stethoscope CMS-VE, which amplifies the sound up to 32 times and supports earphones while wearing hearing aids. The second student, with profound bilateral sensorineural hearing loss and cochlear implants, utilized the Littmann CORE Digital Stethoscope, which amplifies the sound up to 40 times and transmits it via Bluetooth. Both students successfully acquired essential clinical skills, demonstrating that tailored technological accommodations can significantly enhance learning outcomes. This article underscores the critical role of early disability detection and the implementation of support strategies by occupational health physicians. These case reports highlight the importance of innovative solutions in promoting inclusion and ensuring equal opportunities in medical education and future workplaces.

Evaluating binaural hearing capabilities in individuals with sensorineural hearing loss through bilateral bone conduction stimulation

This study investigated the impact of bilateral bone conduction (BC) stimulation and sensorineural hearing loss on spatial release from masking, binaural intelligibility level difference, and lateralization. The study involved two groups of adults with mild to moderate sensorineural hearing loss: one group of 21 participants with symmetric hearing loss and another group of nine participants with asymmetric hearing loss. All tests were conducted through BC and air conduction (AC) headsets with non-individualized virtual positions of the sound sources and linear amplification based on individual hearing thresholds. The findings revealed a bilateral benefit for both groups of hearing-impaired individuals, with symmetric hearing loss yielding better results than asymmetric hearing loss. AC stimulation provided approximately twice the benefit in terms of dB compared to BC stimulation. A large part of this benefit originated from a favorable signal-to-noise ratio due to noise reduction from the head shadow. However, binaural processing was present in both hearing-impaired groups with bilateral BC stimulation. The ability to lateralize sounds based on interaural time delays was significantly impaired in participants with both types of hearing loss when stimulation was by BC. Despite these challenges, the study underscores the benefits of bilateral fitting of BC hearing aids, even in individuals with mild to moderate sensorineural hearing loss, whether symmetric or asymmetric.

Hearing impairment following surgically repaired congenital heart disease in children: a prospective study

ObjectivesTo determine the prevalence of sensorineural hearing loss (SNHL) in children who underwent congenital cardiac surgery (CCS) by using a pre- and postoperative hearing test, a conventional audiometry, an extended...

Rosai-Dorfman-Destombes disease in adolescence with hearing and vision loss involvement: A multidisciplinary approach.

Purpose. To report a rare case of Rosai-Dorfman-Destombes (RDD) disease in a teenage girl exhibiting mild fever, night chills, vertigo, and progressive bilateral vision and hearing loss. Method. Case report of a 15-year-old girl who underwent extensive ophthalmic multimodal imaging and a comprehensive workup evaluation. Results. Multiple choroid lesion masses in both eyes associated with systemic multifocal lymphadenopathy, involvement of the paranasal sinuses, and unusual intracranial manifestations well-shown by magnetic resonance imaging (MRI) and whole-body PET-scan. The PET-Scan and MRI imaging showed dural-based intracranial lesions, choroid-plexus signaling, and meningeal spread, as well as a focus on the paranasal sinuses, which helped determine the best biopsy location for a definitive diagnosis through immunohistochemistry. Conclusions. Here we describe a rare condition of RDD disease with bilateral visual and hearing loss associated with bilateral multiple choroid masses and neurological symptoms in an adolescent female patient. A definitive diagnosis relied on histological assessment, emphasizing the importance of interdisciplinary evaluation. RDD should be considered in the differential diagnosis of choroidal metastasis, tuberculosis, and other lymphoproliferative diseases.

Hearing loss as the first symptom of pneumonia caused by Chlamydia psittaci: a case report and literature review

BackgroundThe typical clinical symptoms of psittacosis pneumonia include fever, dry cough, and chills. Sudden sensorineural hearing loss is a relatively uncommon condition in pneumonia caused by Chlamydia psittaci. In this study, we reported a rare case of Chlamydia psittaci pneumonia presented as hearing loss.Case presentationA 65-year-old man presented to our hospital with sudden hearing loss, cough with sputum, and fever for the last three days. Chest computed tomography revealed inflammation of the left lung and poor response to broad-spectrum antibiotics. The metagenomic next-generation sequencing (mNGS) of bronchoalveolar lavage fluid identified the sequence of Chlamydia psittaci. Subsequently, antibiotic treatment was adjusted to doxycycline hydrochloride and moxifloxacin, resulting in significant improvement in both hearing loss and lung infection.ConclusionsSudden sensorineural hearing loss as an extrapulmonary feature of Chlamydia psittaci pneumonia is extremely rare. Although the exact mechanism remains unclear, this case report described a patient with sudden bilateral sensorineural hearing loss as a presenting feature of Chlamydia psittaci pneumonia, illustrating the importance of the extrapulmonary features of atypical pneumonia. The mNGS test could provide early diagnosis. Many patients had a good prognosis with prompt and effective treatment.

Comparison of Click and Level-Specific CE Chirp-Stimulated Auditory Brainstem Responses in Adults with Hearing Loss.

The Claus Elberling Chirp (CE-Chirp) stimulus used in the auditory brainstem responses (ABRs) was developed to compensate for the cochlear wave delay. As a version of broadband CE-Chirp stimulus, the use of level-specific (LS) CE-Chirp stimuli, which are created with varying delay models suitable for the intensity levels at which the sound is transmitted, is becoming increasingly common. The aim of this study was to compare click ABRs with LS CE-Chirp ABR thresholds in adults with sensorineural hearing loss. The research is a cross-sectional, analytical research. Twenty-two adult patients (n = 44 ears) with bilateral sensorineural hearing loss were included in the study. Pure-tone audiometry, click ABR, and LS CE-Chirp ABR tests were performed on adult (13 males and 9 females; 42.86 ± 14.50 years) patients with bilateral sensorineural hearing loss. Click ABR and LS CE-Chirp ABR thresholds were compared in terms of proximity to behavioral hearing thresholds of 2 kHz, 4 kHz, and 2 to 4 kHz averages. Both types of ABR stimuli were able to identify with total hearing loss (n = 6). A significant difference was found between LS CE-Chirp ABR thresholds (53.81 ± 20.28 decibel normal hearing level [dB nHL]) and click ABR thresholds (58.81 ± 19.11 dB nHL) in the other ears (n = 38) with hearing loss (p = 0.00). When both ears were evaluated together and the right and left ears were evaluated separately, no difference was found between LS CE-Chirp ABR thresholds and 4 kHz hearing thresholds (p = 0.66, 0.80, and 0.69, respectively). In adults with hearing loss, the LS CE-Chirp provides ABR thresholds closer to the behavioral hearing thresholds at 2 and 4 kHz compared with the click stimulus. Notably, there was no difference between LS CE-Chirp ABR thresholds and the 4 kHz behavioral hearing thresholds. We concluded that the LS CE-Chirp can be used effectively in the estimation of behavioral hearing thresholds in adults with hearing loss.

Changes in symptom pattern in Meniere's disease by duration: the need for comprehensive management.

This retrospective study aimed to analyze the symptom profile of Meniere's disease (MD) patients, particularly focusing on the cessation of episodic vertigo and the disease's longitudinal course and the impact of major symptoms on quality of life (QoL). The study employed a cross-sectional design and was conducted on 365 out of 560 individuals with definite MD from the Finnish Vestibular and Meniere Federation, utilizing an internet-based questionnaire. Participants were surveyed on vertigo attacks, vestibular drop attacks (VDA), balance issues, selective cognitive complaints, hearing loss, and their effects on overall quality of life (QoL). The study population comprised 79.5% females and 20.5% males, with a mean age of 63 years and an average disease duration of 15.2 years. The onset of MD was characterized by simultaneous hearing loss, vertigo, and tinnitus in 38% of participants. There was a significant delay in diagnosis for many, with 20% experiencing a delay of over 5 years. The frequency and duration of vertigo attacks generally decreased over time, with attacks becoming shorter and less severe as the disease progressed. Spontaneous remission from episodic vertigo occurred in 34% of participants variably throughout the course of MD. Of the participants 65.5% reported balance issues, and 34% experienced mild VDAs, with severe falls occurring in 10%. VDAs were more common with longer disease duration. Bilateral hearing loss developed in 34.5% of participants over the long term, with a higher risk associated with younger onset age, migraines, and family history of MD. Fatigue, anxiety, and depression were prevalent, particularly among younger participants. Cognitive impairments were linked to the severity of these symptoms and the presence of constant dizziness. QoL was significantly lower among participants with constant dizziness, with factors like fatigue, depression, VDA, and hearing loss contributing to this reduction. The study highlights the complexity of MD. While vertigo may spontaneously remit, other symptoms such as VDAs, balance issues, cognitive complaints, and hearing loss often persist and worsen over time. Assessing MD solely on primary symptoms like vertigo and hearing loss is insufficient; a comprehensive evaluation is necessary for effective management.

Polygenic Risk Scores and Hearing Loss Phenotypes in Children

Monogenic causes of childhood hearing loss are well established, as are polygenic risk contributions to age-related hearing loss. However, an untested possibility is that polygenic risk scores (PRS) also contribute to childhood hearing loss of all severities, alongside environmental and/or monogenic causes. To examine the association between a PRS for adult hearing loss and childhood hearing loss phenotypes. This cross-sectional study used a unique population-based dataset spanning normal hearing to profound loss, combining 2 contemporaneous population cohorts in Australia. This included the Child Health CheckPoint, a national population-based cross-sectional study nested within the Longitudinal Study of Australian Children, and the Victorian Childhood Hearing Longitudinal Databank (VicCHILD), a statewide population-based longitudinal data bank open to every child with congenital hearing loss in Victoria, Australia. The analysis took place from March to August 2023. Genotype data were generated from saliva- or blood-derived DNA using global single-nucleotide variations arrays. Based on genotype data, PRS was computed using published UK Biobank genome-wide association study results for self-reported hearing difficulty in individuals aged 40 to 69 years. Hearing outcomes were classified by laterality (bilateral, unilateral), severity (mild, moderate, severe or worse) and types (sensorineural, conductive, mixed, auditory neuropathy, atresia). Analyses included multinominal logistic regressions of PRS with hearing outcomes. Overall, 1488 CheckPoint study children (49.8% boys, aged 11-12 years) and 527 VicCHILD study children (55.2% boys, aged 0-13 years) with hearing and genotype data were included. A 1-SD increment in PRS was associated with higher odds of mild (odds ratio [OR], 1.3; 95% CI, 1.0-1.6), moderate (OR, 5.1; 95% CI, 3.2-8.1), and severe or worse (OR, 5.3; 95% CI, 3.9-7.3) unilateral hearing loss compared with normal hearing. Similarly, the PRS was associated with increased odds of mild, moderate, and severe or worse bilateral hearing loss (per-SD ORs, 3.9-6.6) and all hearing loss types (per-SD ORs, 8.5-10.6). In this cross-sectional study, a PRS initially developed for adult hearing difficulty was associated with wide-ranging childhood hearing loss phenotypes, partly explaining hearing phenotype variations despite shared genetic and environmental factors (eg, preterm birth). Large-scale studies with objectively defined hearing phenotypes are crucial for refining PRS and predicting high-risk children.

Natural History of Auditory Function in Patients with Alport Syndrome: A Case Series Study.

Background: Alport syndrome (AS) is a genetic disorder characterized by progressive renal disease, ocular abnormalities, and sensorineural hearing loss. However, the audiological profile of patients with AS remains elusive. Thus, this study aims to evaluate the natural history of auditory function in patients with AS. Methods: Exome or targeted sequencing for deafness genes was performed to confirm the pathogenic variants in patients with AS. Results: We identified fifteen individuals with AS who carried pathogenic variants of COL4A3, COL4A4, or COL4A5. Among fifteen, twelve (80%) showed hematuria, and six (40%) showed proteinuria. The patients exhibited bilateral sensorineural hearing loss, which was progressive and symmetric. The hearing thresholds increased according to age and plateaued at the level of 53 dB HL, indicating the hearing loss did not reach the severe-to-moderate level. The auditory dysfunction showed a distinct natural history depending on the inheritance pattern, but there was no remarkable difference between males and females among X-linked AS. Conclusions: Auditory dysfunction in AS is progressive up to the level of moderate hearing loss. Precise auditory rehabilitation for patients with AS is warranted depending on the inheritance pattern and genetic predisposition.

Perrault syndrome: a forgotten presentation for infertile women.

Perrault syndrome (PRLTS) is an uncommon hereditary condition distinguished by ovarian failure in females and sensorineural hearing loss. Infertility can be the presenting problem for a serious disease. History and physical examination is very essential among infertile couples. Perrault syndrome (PRLTS) is an uncommon hereditary condition distinguished by ovarian failure in females and sensorineural hearing loss. This case presentation describes a 22-year-old female from Saudi Arabia with PRLTS. The patient presented with progressive bilateral hearing loss since childhood, impacting her academic achievement. Additionally, she experienced amenorrhea since the age of 18 years, with previous investigations showing no hormonal imbalances. Other laboratory tests, including bone mineral density, kidney and liver function, electrolytes, and lipid profile, showed mostly normal results, except for a slightly abnormal lipid profile with low high-density lipoprotein and high low-density lipoprotein levels. This case highlights the challenges faced by individuals with PRLTS, specifically progressive hearing loss and gonadal dysfunction, leading to infertility. Further evaluation and management are warranted to address the patient's hearing impairment and fertility concerns, with a multidisciplinary approach involving audiology, endocrinology, and reproductive medicine.

Molecular and Clinical Characterization of a Cohort of Autosomal Recessive Sensorineural Hearing Loss in Egyptian Patients

Hearing loss (HL) is one of the most common health problems worldwide. Autosomal recessive non-syndromic sensorineural hearing loss (ARNSHL) represents a large portion of congenital hereditary HL. Our study was conducted on 13 patients from 13 unrelated families. The majority of patients presented with congenital severe to profound bilateral sensorineural HL. All patients were subjected to detailed family history and three-generation pedigree analysis to exclude any environmental cause and to ensure an autosomal recessive mode of inheritance. Molecular analysis was performed using the whole exome sequencing (WES) technique for the recruited patients. Three variants in the MYO7A and OTOF genes were reported for the first time in patients with ARNSHL (one nonsense, one frameshift, and one splice variant). Ten previously reported variants were detected in seven genes (GJB2, MYO15A, BSND, OTOF, CDH23, SLC26A4, and TMIE). They varied between missense, nonsense, frameshift, and splice variants. This study expands the molecular spectrum of two types of autosomal recessive deafness (types 2 and 9).

Evaluation of auditory system in vitiligo patients: A case-control study

BackgroundVitiligo is a common acquired pigmentary disorder characterized by well-defined areas of depigmentation due to loss of melanocytes. Vitiligo can affect melanocytes in the auditory system and potentially hearing. ObjectiveThe aim of this study was to investigate hearing changes in vitiligo patients. MethodsTwenty-six with vitiligo patients and 26 demographically matched healthy controls were included in this study. Audiological function was evaluated using pure tone audiometry (PTA), speech audiometry and tympanometry tests in all subjects. ResultsThe mean hearing threshold of the left ear at frequencies of 250 and 8000 Hz in vitiligo patients was significantly higher than the control group. Sensorineural hearing loss (SNHL) was seen in 14 (53.8 %) vitiligo patients and 5(19.2 %) control group (p < 0.05). Most affected patients (10/14; 71 %) had bilateral hearing loss especially in high frequency (8000 Hz). A significant association was identified between vitiligo and SNHL (OR 4.9; 95 % CI, 1.41–16.98, P = 0.01). There was a statistically significant correlation between hearing impairment and age in both groups. Gender, severity, activity and duration of vitiligo had no a significant effect on the results of audiometric tests. ConclusionThis study confirmed that the auditory system was affected in vitiligo patients. Vitiligo patients might be at a higher risk for auditory abnormalities at a younger age than control subjects. Auditory evaluation of vitiligo patients should be considered for early detection of hearing loss.

The Otoacoustic Emissions in the Universal Neonatal Hearing Screening: An Update on the European Data (2004 to 2024).

Background: The reported data on European universal neonatal hearing screening (UNHS) practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected unofficial data from 38 collaborating European institutions. The objectives of this systematic review were as follows: (a) to identify the most recent (in a 20-year span) literature information about UNHS programs in Europe and (b) to provide data on the procedures used to assess the population, the intervention policies, and on the estimated prevalence of congenital hearing loss with emphasis on the bilateral hearing loss cases. Methods: Queries were conducted via the Pubmed, Scopus and Google Scholar databases for the time period of 2004-2024. The Mesh terms used were "OAE", "Universal Neonatal Hearing Screening", "congenital hearing loss" and "well babies". Only research articles and review papers of European origin were considered good candidates. The standard English language filter was not used, in order to identify information from non-English-speaking scientific communities and groups. Results: Very few data and reports were identified in the literature search. Eleven manuscripts were identified corresponding to eight UNHS programs. Except in Poland, most of the data refer to regional and not national programs. The screening coverage estimates of all programs exceed 90%; infants were mostly assessed by a three-stage protocol (TEOAE + TEOAE + AABR), followed by a clinical ABR test. The average prevalence (i.e., from well babies AND NICU infants) of bilateral hearing loss ranged from 0.5 to 20.94 per 1000 (Zurich sample). Infants presenting unilateral or bilateral hearing losses were first rehabilitated by hearing aids and consequently (>15 mo) by cochlear implants. Conclusions: Even though UNHS programs are well-established clinical practices in the European States, the amount of information in the literature about these programs is surprising low. The existing data in the timespan 2004-2024 corroborate the international UNHS data in terms of coverage and bilateral hearing loss prevalence, but there is a strong need to supplement the existing information with the latest developments, especially in the area of hearing loss rehabilitation.

Prospective long-term follow-up after grommet insertion: Hearing and functional health outcomes in children

ObjectivesTo assess hearing levels and functional health outcomes of children two years after routine grommet surgery with standard care follow-up (discharge to General Practitioner (GP) care or Ear Nose and Throat (ENT) clinic appointment at 4–8 weeks). MethodsProspective cohort study of 89 children (average age of 7.98 years) recalled for audiological assessment 2 years after grommet surgery in a large ENT outpatient service in South Auckland, New Zealand. Functional health was assessed using parent-reported responses to the OMQ20 questionnaire. Results46 (51.7 %) children had hearing loss in at least one ear with 28 (31.5 %) children having bilateral hearing loss. Māori children had statistically higher odds compared to non-Māori children (odds ratio 5.491, p = .003) to have bilateral hearing loss after controlling for age, household deprivation, gender, season, and mode of follow-up. Most parents reported concerns with their child's hearing, speech, attention-seeking behaviours, and nasal symptoms, but not ear problems. Bilateral hearing loss (p < .001) was found to significantly predict functional health status (total OMQ20 score). ConclusionsThe high prevalence of hearing loss and functional health concerns suggest that the standard follow-up care is not adequately managing children after routine grommet insertion. Māori children are disproportionately impacted, and post-grommet follow-up schedules must consider individualised approaches to address these inequities in outcomes.