Hearing Loss In Pediatric Patients Research Articles

Molecular Characteristics of Cisplatin-Induced Ototoxicity and Therapeutic Interventions.

Cisplatin is a commonly used chemotherapeutic agent with proven efficacy in treating various malignancies, including testicular, ovarian, cervical, breast, bladder, head and neck, and lung cancer. Cisplatin is also used to treat tumors in children, such as neuroblastoma, osteosarcoma, and hepatoblastoma. However, its clinical use is limited by severe side effects, including ototoxicity, nephrotoxicity, neurotoxicity, hepatotoxicity, gastrointestinal toxicity, and retinal toxicity. Cisplatin-induced ototoxicity manifests as irreversible, bilateral, high-frequency sensorineural hearing loss in 40-60% of adults and in up to 60% of children. Hearing loss can lead to social isolation, depression, and cognitive decline in adults, and speech and language developmental delays in children. Cisplatin causes hair cell death by forming DNA adducts, mitochondrial dysfunction, oxidative stress, and inflammation, culminating in programmed cell death by apoptosis, necroptosis, pyroptosis, or ferroptosis. Contemporary medical interventions for cisplatin ototoxicity are limited to prosthetic devices, such as hearing aids, but these have significant limitations because the cochlea remains damaged. Recently, the U.S. Food and Drug Administration (FDA) approved the first therapy, sodium thiosulfate, to prevent cisplatin-induced hearing loss in pediatric patients with localized, non-metastatic solid tumors. Other pharmacological treatments for cisplatin ototoxicity are in various stages of preclinical and clinical development. This narrative review aims to highlight the molecular mechanisms involved in cisplatin-induced ototoxicity, focusing on cochlear inflammation, and shed light on potential antioxidant and anti-inflammatory therapeutic interventions to prevent or mitigate the ototoxic effects of cisplatin. We conducted a comprehensive literature search (Google Scholar, PubMed) focusing on publications in the last five years.

Correlation of Conductive Hearing Impairment With Sizes of Adenoids in the Pediatric Age Group: An Observational Case-Control Study.

When adenoids enlarge and elicit symptoms it is referred to as symptomatic adenoids, which is a preventable cause of hearing loss and nasal obstruction in the pediatric age group. This study was done to correlate conductive hearing loss with the size of adenoids and to emphasize the importance of screening in the pediatric age group. An observational case-control study was conducted to analyze the degree of hearing impairment in children with adenoid hypertrophy. In total, 98 patients with at least one symptom of adenoid hypertrophy aged between 5 and <15 years were recruited. Those with conductive hearing loss were in thecase group and those with normal hearing were in the control group. Audiometry, tympanogram, X-ray, and fibreoptic nasal endoscopy were conducted and compared. The mean age of presentation of conductive hearing loss with adenoids was 7.67 years. The mean conductive hearing loss on audiometry was 31.69 dB. Tympanogram showed a type B curve in 40.81% of ears and type C in 26.53%. On X-ray nasopharynx, the majority of cases had grade III hypertrophy followed by grade II and grade IV.In nasal endoscopy, most cases had second-degree adenoid hypertrophy followed by third degree, first degree, and then fourth degree. The highest degree of hearing loss of 32-48 dB was present with fourth-degree adenoids. Conductive hearing loss was five times more in patients with third- and fourth-degree adenoids. In our study, adenoid hypertrophy has a positive correlation with conductive hearing loss in pediatric patients. So proper screening and early management should be done to prevent hearing loss in children.

A review of bilateral sudden sensorineural hearing loss in pediatric patients.

Bilateral, sudden sensorineural hearing loss (SSNHL) in the pediatric population is a rare phenomenon potentially detrimental to language acquisition and social development. This study comprehensively reviews and analyzes existing literature to determine any correlation or commonality in etiologies, presentations, and management of this condition. PubMed, Cochrane, Scopus, and Web of Science databases were systematically searched for articles related to pediatric SSNHL from 1970 to 2021. Case series, case reports, and cohort studies were included. Data on patient demographics, etiology, diagnostic testing, management, and hearing recovery were collected. Excluding duplicates, 553 unique titles were identified by established search criteria, of which 342 titles were relevant to pediatric sudden hearing loss. Forty-six papers reported cases of bilateral SSNHL, totaling 145 individual cases. Not included in the analysis were 45 cases documented as non-organic hearing loss. The average age of the total 145 included patients was 8.5 years and 51 were male. Reported etiologies included cytomegalovirus (n=3), meningitis (n=13), mumps (n=5), ototoxin exposure (n=13), and enlarged vestibular aqueduct (n=9). Tinnitus (n=30) was the most reported concurrent symptom, followed by vertigo (n=21). Systemic steroid therapy was the most common treatment and, when follow up was reported, most patients (51.2%) had complete or partial recovery of hearing. This is a comprehensive review of pediatric bilateral SSNHL. Though often idiopathic, etiologies also include infectious, structural, and autoimmune. Treatment largely consists of systemic steroid therapy, with variables rates of recovery. Further studies on intratympanic administration of steroids may guide future treatment.

HEARING LOSS IN CHILDREN AFTER TYMPANIC MEMBRANE PERFORATION: CLUSTER ANALYSIS OF 27 CASES

IntroductionTympanic membrane perforation (TMP) may be caused by several factors but commonly leads to conductive hearing loss. This study aims to characterize the profiles of hearing loss in pediatric patients with TMPs.Material and methodsA retrospective analysis of the medical charts of 27 patients was conducted. Otoscopy of the TM was done and pure tone audiometry was used to assess hearing loss. Cluster analysis was applied to evaluate the profiles of hearing loss and to find possible relations between profiles of hearing loss and the location of the perforation on the TM.ResultsCluster analysis revealed three types of hearing loss. The mean hearing loss in cluster 1 (6 cases) was above 30 dB, mainly as the result of perforation after chronic otitis media. Hearing loss in clusters 2 (9 cases) and 3 (12 cases) was less than 30 dB. In cluster 2 the perforation was mostly located in the posterior quadrants, while in cluster 3 it was most commonly in the inferior quadrants. In clusters 2 and 3, perforation was usually caused by slap of the open hand, injury, or past ventilation tube.ConclusionsThree different profiles (clusters) of hearing loss resulting from TMP were identified. Force of injury, etiology of the injury, and inflammation produce different sizes of perforations. Conductive hearing loss increases with perforation size and is independent of TM location. In general, hearing loss classification methods have the potential to improve diagnostic procedures, surgery, and rehabilitation of patients with TMPs.

Hearing loss in pediatric patients with congenital rubella syndrome

Congenital rubella syndrome (CRS) is caused by rubella infection in pregnant women. It was estimated 100.000 children with CRS, with 46% found in developing countries. The CRS consists of symptoms like sensorineural hearing loss (SNHL), congenital heart disease, cataract or congenital glaucoma, and other symptoms. Sensorineural hearing loss is the commonest symptoms compared with others. This study aimed to determine the hearing loss in CRS in Dr. Kariadi Central Hospital, Semarang, Central Java. This was a descriptive study using data from medical records from CRS pediatric patients who had BERA examination from January 2019 until December 2020. The CRS was characterized with one or more symptoms: SNHL, congenital cataract, congenital heart disease, and laboratory IgG and/or IgM Rubella (+). The SNHL was described from refer OAE result, tympanometry A, and BERA with hearing threshold ≥40 dB. Follow-up evaluation was performed with Google form questionnaire. There were 55 CRS cases with 70.9% had bilateral SNHL, mostly with very severe hearing loss. Normal hearing was found in 16 children (28.1%). From 30 children who were followed up, there were 20 children who had bilateral SNHL with 30% were moderate-severe degree and 70% profound. With further multidisciplinary management in children with CRS and parental support, 65% children used hearing aid and 40% had auditory-verbal therapy (AVT). Evaluation of the AVT showed progress in 30% children. In conclusion, SNHL is found in 70.9% children with CRS. Further management using hearing aid and AVT shows progress on few children.

Gadolinium-based contrast agent for Magnetic Resonance Imaging as a predictor of postmeningitic hearing loss in children

ObjectiveTo determine if Gadolinium-based enhanced Magnetic Resonance Imaging (GdMRI) can be used to predict sensorineural hearing loss (SNHL) in pediatric patients diagnosed with bacterial meningitis. StudyDesign: Retrospective chart review. SettingPrimary Children's Hospital, Salt Lake City, Utah. Subjectsand Methods: We studied forty-two pediatric patients diagnosed with bacterial meningitis who underwent brain GdMRI during their index hospital admission and for whom ear specific audiometric data were available (August 2008–July 2018). A pediatric neuroradiologist, blinded to both disease and audiometric data, rated cochlear enhancement of each GdMRI (0–3; none to markedly enhanced). ResultsEar specific MRI scores were statistically significantly related to ear specific hearing outcomes (p < 0.01). SNHL occurred in 19 out of 82 ears (12 out of 42 patients; 2 ears were excluded due to pre-existing SNHL in one ear and inability to read the GdMRI on the other ear). Ten of 19 ears (53%) that developed SNHL showed mild/moderate/marked enhancement (MRI score of 1, 2, or 3 respectively). Fifty-three of the 63 unaffected ears (84%) showed no enhancement (MRI score of 0). Ten of 13 (77%) ears that developed severe to profound SNHL showed mild/moderate/marked enhancement. GdMRI was 58% sensitive and 84% specific in predicting which ears would develop SNHL. GdMRI was 77% sensitive and 84% specific in identifying severe to profound SNHL. ConclusionOur study demonstrates that GdMRI is a promising tool for predicting specifically severe-profound hearing loss in pediatric patients following bacterial meningitis infection.

Otitis Media and Obesity-An Unusual Relationship in Children.

Otitis media (OM) represents a public health matter, being the main cause of preventable hearing loss in pediatric patients. Besides well-established risk factors for developing OM, such as craniofacial abnormalities, prematurity, low birth weight, or tobacco exposure, there is evidence that obesity could be associated with a high incidence of OM. Our aim is to perform a literature review on the state of current published research on the relationship between OM and obesity and to discuss the interconnectivity between these two entities. We conducted an electronic search in PubMed and EMBASE databases. Out of 176 references, 15 articles were included in our study. Our findings suggest that obesity and overweight might be risk factors for developing OM, and vice versa. The main mechanisms for developing OM in obese patients include alteration in cytokine profile, increased gastroesophageal reflux, and/or fat accumulation. Conversely, ear infections exposure might increase the risk of obesity, mostly by taste changes through middle ear cavity inflammation.

Hearing Loss Risk in Pediatric Patients Treated with Cranial Irradiation and Cisplatin-Based Chemotherapy.

Cranial radiation therapy (RT) and cisplatin-based chemotherapy are essential to treating many pediatric cancers but cause significant ototoxicity. The objective of this study is to determine the relationship between the RT dose and the risk of subsequent hearing loss in pediatric patients treated with cisplatin. This retrospective study of cisplatin-treated pediatric patients examined ototoxicity from cranial RT. Ototoxicity was graded for each ear according to the International Society of Pediatric Oncology (SIOP) consensus ototoxicity scale. The RT dose to the cochlea was calculated using the mean, median, maximum, and minimum dose received to determine the most predictive parameter for hearing loss. Multivariable logistic regression models then examined risk factors for hearing loss. In 96 children (161 ears) treated with RT + cisplatin, the minimum cochlear RT dose was most predictive of hearing loss. A higher cochlear RT dose was associated with increased hearing loss (odds ratio per 10 Gy dose increase = 1.64; P = .043), with an added risk in those receiving an autologous bone marrow transplantation (hazard ratio = 10.47; P < .001). This research supports further testing of the minimum cochlear RT dose as a more predictive dose parameter for risk of ototoxicity. The cochlear RT dose was additive to the risk of hearing loss from underlying cisplatin-based chemotherapy. Exposure to autologous bone marrow transplantation was the strongest predictor of developing hearing loss, placing these children at particularly high risk for hearing loss across all cochlear doses. Future prospective studies are crucial to further inform RT dose thresholds and minimize the risk of hearing loss in childhood cancer survivors.

Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.

To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. Meta-analysis. We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021.

The Relation of Sudden Sensorineural Hearing Loss in Pediatric Patients With Recurrent Otitis Media: A Nested Case-control Study Using a National Sample Cohort.

The risk of sudden sensorineural hearing loss (SSNHL) was suggested to be increased in chronic otitis media patients. This study expanded previous findings by using pediatric patients with recurrent otitis media. A nested case-control study. Participants in the Korean National Health Insurance Service-National Sample Cohort that were less than 15 years old from 2002 to 2004 were included. Overall, 24,226 patients with ≥ 5 instances of otitis media were 1:1 matched with a group controlled for age, sex, income, and region of residence. The occurrence of SSNHL was followed until 2013. The hazard ratio (HR) for SSNHL was analyzed in the otitis media patients using a stratified Cox proportional hazard regression model. Matching variables were stratified. The histories of sinusitis and atopic dermatitis were adjusted. Subgroup analysis was performed according to age and sex. Overall, 0.14% (33/24,226) of the otitis media group and 0.06% (14/24,226) of the control group had SSNHL (p = 0.006). The otitis media group had a higher adjusted HR for SSNHL than the control group (adjusted HR = 2.13, 95% CI = 1.13-4.02). According to age, the 10- to 14-year-old group showed a 9.07-fold higher HR for SSNHL than the control group (95% CI = 1.14-72.54). According to sex, the female group showed a 3.74-fold higher HR for SSNHL than the control group (95% CI = 1.24-11.29). Recurrent otitis media in children was related to the onset of SSNHL.

NIEDOSŁUCH W OSTEOGENESIS IMPERFECTA W POPULACJI PEDIATRYCZNEJ

Hearing loss in osteogenesis imperfecta (OI) typically begins in the third decade of life. OI rarely occurs in childhood. Case reports of hearing loss in pediatric patients with OI are considered as merely incidental, and until now pediatric OI cases have not been presented in the literature. Typically, hearing loss is conductive, but over time a sensorineural component also occurs. There are some reports of profound sensorineural hearing loss, but this type of loss is believed to be due to additional genetic factors. The two case reports described in this paper show how the hearing loss due to OI can be treated by means of stapedotomy and restapedotomy

Incidence of Hearing Loss in Pediatric Patients with Complaints of Speech Disorders

Incidence of Hearing Loss in Pediatric Patients with Complaints of Speech Disorders

Long term platinum-induced ototoxicity in pediatric patients

ObjectivesPlatinum-based chemotherapy treatments are effective against a variety of pediatric malignancies. However, its use can lead to permanent hearing loss. The aim of this study was to evaluate the long-term effect of platinum chemotherapy on hearing and evaluate its progression. MethodsProspective cohort study. All records of pediatric patients receiving platinum-based chemotherapy between 2001 and 2006 were reviewed. Demographics and audiograms performed before, during, and following chemotherapy were analyzed. An updated audiogram and a video head impulse test were performed. A hearing ability questionnaire was also completed. ResultsThirty-nine patients met the inclusion criteria. Of these, 12 patients were included in the study; 14 were deceased, 8 had incomplete data and 5 were excluded for other reasons. Median age at chemotherapy was 4.3 years (range 10 months–14.2 years). Seven patients had received cisplatin, two received carboplatin and three received both agents. Five had also received cranial irradiation. With a median follow-up time of 11.9 years, 58.3% had developed hearing loss and two patients wore bilateral hearing aids; 67% of the patients with hearing loss had worsening of their hearing in the long-term. All patients referred difficulties in various subscales measured by the questionnaire. Three patients had decreased vestibulo-ocular reflex gains. ConclusionPlatinum-induced hearing loss in pediatric patients can be progressive and debilitating. A long term audiometric follow-up of at least 10 years is suggested for these patients.

Hearing Loss in Pediatric Patients With Cerebral Palsy

This study evaluates the prevalence, type, and severity of hearing impairment in children with cerebral palsy (CP) and to analyze audiologic and otologic outcomes in these patients. Retrospective analysis of the AudGen Database. Tertiary academic referral center. Pediatric patients in AudGen Database with a diagnosis of cerebral palsy. Appropriate audiologic, otologic, and demographic data were recorded.Nine hundred forty patients met inclusion criteria. Hearing loss (HL) was defined as greater than 15 dB HL at any threshold by pure tone or greater than 20 dB HL by soundfield audiometry. Other available otologic and medical conditions were documented. Of 940 patients, 367 (39%) had hearing loss. Of the 1,629 individual ears with HL, 782 (48%) had conductive, 63 (4%) had sensorineural, 410 (25%) had mixed, and 374 (23%) had unspecified hearing loss. Patients with mixed type 1 hearing loss had significantly worse PTAs. Hearing loss in CP has a large degree of sensorineural loss, with a predisposition to be bilateral. The severity of hearing loss was correlated with the degree of the motor and neurologic disability in these patients.

Audiometric assessment of pediatric patients with cystic fibrosis

BackgroundThe purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF). MethodsThis is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss. Results217 patients with CF were included in this study. 69 (31.8%) had hearing loss on initial audiogram. Chronic otitis media (OR: 2.4, 95% CI: 1.3–4.5, p<0.01), Eustachian tube dysfunction (OR: 2.4, 95% CI: 1.4–5.4, p<0.01), and otorrhea (OR: 6.3, 95% CI: 1.6–24.7, p<0.01) were positive predictors of HL. Children with a diagnosis of diabetes had more decline in hearing over time than those without diabetes (12.4±17.2dB worsening vs. −5.7±9.8dB improvement in PTA, p=0.014). ConclusionThis is the largest comprehensive analysis of all types of hearing loss in pediatric patients with CF. Our data suggest that children with more severe sinus disease may be at lower risk for inflammatory middle ear disease and subsequent hearing loss. Patients who develop complications of CF such as diabetes should be monitored frequently, and the use of ototoxic drugs should be limited if possible.

Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratios in Pediatric Patients with Idiopathic Sudden Hearing Loss.

We investigated the significance of the neutrophil-to-lymphocyte and platelet-to-lymphocyte ratios, which have recently been used in adults for the prognosis of idiopathic sudden hearing loss (ISHL), in the prognosis of idiopathic sudden hearing loss in a pediatric population. A total of 13 males and 8 females younger than19 years with idiopathic sudden hearing loss were retrospectively examined between January 2008 and August 2016.The control group consisted of 12 healthy males and 12 healthy females. Patients were divided into two groups: those who recovered after treatment and those who did not. A statistical significance for the neutrophil-to-lymphocyte ratio was detected between the patient group and control group and between patients who recovered and those who did not (p<0.05). No statistical significance for the platelet-to-lymphocyte ratio was detected between the patient group and control group and between patients with recovery and those without recovery (p>0.05). The neutrophil-to-lymphocyte ratio is an important marker for the prognosis of idiopathic sudden hearing loss in pediatric patients, similar to its use in adult patients.

Prevalence of hearing loss in children with 22q11.2 deletion syndrome

ObjectiveThe purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS). MethodsFifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicine's database with a focus on hearing loss and 22q11DS was performed. Results22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears. ConclusionHearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population.

Early detection of changes to hearing status attributed to treatment regimen with ototoxicity in the state of Qatar

Background Ototoxicity resulting from cisplatin chemotherapy constitutes a significant clinical problem and challenge. Therefore, an early and effective detection of hearing loss in pediatric oncology patients receiving ototoxic medication and early intervention are critical to help these patients in achieving these developmental milestones and improving their quality of life. Materials and methods This prospective, observational study was designed as an initial pilot study to collect and analyze data from January 2014 to January 2015 on a variety of parameters. All pediatric patients, aged less than 14 years, referred from the Pediatric Hematology/Oncology Clinic to the new Ototoxic Clinic were included in the study. Descriptive statistics were used to summarize the above data, and associations between two or more qualitative variables were assessed using the χ2-test or Fisher's exact test as appropriate. Results A total of 20 patients were included in the study. Diagnostic otoacoustic emission findings were found to be normal in 14 patients (80%); four patients (20%) had abnormal findings, and in two cases this test was not carried out. High-frequency audiometry test indicated that nine (52.9%) patients had normal findings, whereas five (29.4%) had abnormal results. High-frequency audiometry could not be carried out for six (30%) patients because of their ages. Hearing loss involving the speech frequencies affected only two patients (10%; 95% confidence interval 1.7–29.3); they were diagnosed to have β-thalassemia and retinoblastoma and are currently using xjade and cisplatin. Conclusion Early and effective identification of ototoxicity is vital in children receiving cancer treatments because of the impact acquired hearing loss has on social and educational outcomes in children. This study highlights the role of early detection of hearing loss in pediatric patient receiving ototoxic medication and of possible potential interventions to enhance their quality of life during and after treatment.

A case of bilateral enlarged vestibular aqueducts and unilateral hearing loss at birth

Screening of newborn hearing is nowadays widespread, and as many as 3.4% of all screened infants are found to have unilateral hearing loss. However, we, as clinicians and parents, usually dismiss the severity of unilateral hearing loss in pediatric patients. Recently, we experienced the case of unilateral hearing loss since birth in a 2-year-old girl who was finally found to have bilateral enlarged vestibular aqueducts when the disorder progressed to bilateral hearing loss. Proper evaluation of the cause of hearing loss, including imaging studies of the inner ear, and consecutive hearing evaluations should be mandatory in children with unilateral sensorineural hearing loss, due to the possibility of progression of hearing loss bilaterally.

Sensorineural hearing loss in pediatric patients with celiac disease.

Sensorineural hearing loss (SNHL) as an extraintestinal manifestation of celiac disease (CD) has been reported in several studies. The aim of this study was to determine presence of subclinical sensorineural hearing loss associated with CD in pediatric patients. Otoscopy, tympanometry and pure tone audiometry were performed in 44 patients with CD and 20 healthy age and sex-matched controls. Pure tone audiometry did not show significant sensorineural hearing loss over all frequencies in patients with CD compared with controls (P > 0.05). SNHL was detected in only three (6.8 %) patients with CD. In conclusion, subclinical sensorineural hearing loss was demonstrated in adult patients with CD; therefore, we recommend to perform audiometric examinations in pediatric patients for recognizing hearing loss early during the course of the disease.