Ear Pathology Research Articles

Dual inhibition of the endothelin and angiotensin receptor ameliorates renal and inner ear pathologies in Alport mice.

Alport syndrome (AS), a type IV collagen disorder, leads to glomerular disease and, in some patients, hearing loss. AS is treated with inhibitors of the renin-angiotensin system; however, a need exists for novel therapies, especially those addressing both major pathologies. Sparsentan is a single-molecule dual endothelin type-A and angiotensin II type 1 receptor antagonist (DEARA) under clinical development for focal segmental glomerulosclerosis and IgA nephropathy. We report the ability of sparsentan to ameliorate both renal and inner ear pathologies in an autosomal-recessive Alport mouse model. Sparsentan significantly delayed onset of glomerulosclerosis, interstitial fibrosis, proteinuria, and glomerular filtration rate decline. Sparsentan attenuated glomerular basement membrane defects, blunted mesangial filopodial invasion into the glomerular capillaries, increased lifespan more than losartan, and lessened changes in profibrotic/pro-inflammatory gene pathways in both the glomerular and the renal cortical compartments. Notably, treatment with sparsentan, but not losartan, prevented accumulation of extracellular matrix in the strial capillary basement membranes in the inner ear and reduced susceptibility to hearing loss. Improvements in lifespan and in renal and strial pathology were observed even when sparsentan was initiated after development of renal pathologies. These findings suggest that sparsentan may address both renal and hearing pathologies in Alport syndrome patients. © 2023 Travere Therapeutics, Inc and The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

Auditory Deprivation during Development Alters Efferent Neural Feedback and Perception.

Auditory experience plays a critical role in hearing development. Developmental auditory deprivation because of otitis media, a common childhood disease, produces long-standing changes in the central auditory system, even after the middle ear pathology is resolved. The effects of sound deprivation because of otitis media have been mostly studied in the ascending auditory system but remain to be examined in the descending pathway that runs from the auditory cortex to the cochlea via the brainstem. Alterations in the efferent neural system could be important because the descending olivocochlear pathway influences the neural representation of transient sounds in noise in the afferent auditory system and is thought to be involved in auditory learning. Here, we show that the inhibitory strength of the medial olivocochlear efferents is weaker in children with a documented history of otitis media relative to controls; both boys and girls were included in the study. In addition, children with otitis media history required a higher signal-to-noise ratio on a sentence-in-noise recognition task than controls to achieve the same criterion performance level. Poorer speech-in-noise recognition, a hallmark of impaired central auditory processing, was related to efferent inhibition, and could not be attributed to the middle ear or cochlear mechanics.SIGNIFICANCE STATEMENT Otitis media is the second most common reason children go to the doctor. Previously, degraded auditory experience because of otitis media has been associated with reorganized ascending neural pathways, even after middle ear pathology resolved. Here, we show that altered afferent auditory input because of otitis media during childhood is also associated with long-lasting reduced descending neural pathway function and poorer speech-in-noise recognition. These novel, efferent findings may be important for the detection and treatment of childhood otitis media.

High Frequency Tympanometry in Early Infancy and Its Effect on Oto Acoustic Emissions.

The present study was conducted to study the benefit of high frequency tympanometry (HFT) in detecting middle ear pathologies in infants. This study also aims to find the effect of middle ear diseases on absent otoacoustic emissions (OAE). Our study was a prospective study which included 123 healthy neonates below 1 month of age. The babies were referred for the new born hearing screening programme. They were subjected to DPOAE followed by tympanometry with a 1000z probe tone (HFT). The study was approved by the institutions ethical committee. "Present" OAE result was obtained for 202 ears, while "absent" OAE result was obtained for 44 ears. High frequency tympanometry was also performed on all the participants, where a normal tympanogram was obtained in 170 ears and 76 ears showed an abnormal tympanogram. The following parameters were studied: peak pressure, ear canal volume and compliance. Descriptive statistics were obtained for the same. Association of OAE with HFT was studied. Out of the 44 ears with refer OAE, 34 ears had an abnormal tympanogram and among 202 ears with a present OAE, 160 ears had a normal tympanometric peak indicating a sensitivity and specificity of 77.3% and 79.2% respectively. High frequency tympanometry not only helps in early detection of middle ear pathologies but also to find the effect of middle ear diseases on absent otoacoustic emissions.

Cochlear Aqueduct Morphology in Superior Canal Dehiscence Syndrome.

The cochlear aqueduct (CA) connects the scala tympani to the subarachnoid space and is thought to assist in pressure regulation of perilymph in normal ears, however, its role and variation in inner ear pathology, such as in superior canal dehiscence syndrome (SCDS), is unknown. This retrospective radiographic investigation compared CA measurements and classification, as measured on flat-panel computerized tomography, among three groups of ears: controls, n = 64; anatomic superior canal dehiscence without symptoms (SCD), n = 28; and SCDS, n = 64. We found that in a multinomial logistic regression adjusted for age, sex, and BMI, an increase in CA length by 1 mm was associated with a lower odds for being in the SCDS group vs. control (Odds ratio 0.760 p = 0.005). Hierarchical clustering of continuous CA measures revealed a cluster with small CAs and a cluster with large CAs. Another multinomial logistic regression adjusted for the aforementioned clinical covariates showed an odds ratio of 2.97 for SCDS in the small CA cluster as compared to the large (p = 0.004). Further, no significant association was observed between SCDS symptomatology-vestibular and/or auditory symptoms-and CA structure in SCDS ears. The findings of this study lend support to the hypothesis that SCDS has a congenital etiology.

Appropriateness of Diagnosis and Management for Otitis Media With Effusion in Pediatric Urgent Care Clinics.

Otitis media with effusion (OME)'s clinical presentation is often confused with acute otitis media (AOM) by clinicians. Despite OME guidelines recommending watchful waiting with no antibiotics, rates of antibiotic use remain elevated. The aim of this study was to determine the clinician diagnosis validity and the rates of antibiotics prescribed among pediatric OME patients evaluated in 3 urgent care clinics within a pediatric health care system. We retrospectively reviewed a random sample of encounters for children aged 0 to 18 years with a billing diagnosis of OME in 2019. We recorded clinical symptoms, antibiotic prescribed, and the clinicians' diagnosis. We used the American Academy of Pediatrics guidelines to assign an AOM diagnosis and compared those with the clinicians' final diagnoses using Pearson χ 2 . Of the 912 eligible charts, clinicians' final diagnoses were as follows: AOM for 271 (29.7%) patients, OME for 638 (70.0%) patients, and no ear pathology for 3 (0.3%) patients. Antibiotics were prescribed for 519 (56.9%) patients; of those, only 242 (46.6%) had a final clinician diagnosis of AOM. Antibiotic prescribing rates were higher when a clinician diagnosed AOM compared with OME (89.3% vs 43.2%; P < 0.001). Per American Academy of Pediatrics guidelines, up to 273 (29.9%) patients qualified for an AOM diagnosis, but those were not the same as those diagnosed with AOM by clinicians ( P < 0.001). When evaluating children with a billing diagnosis of OME, a third fit a diagnosis of AOM. Clinicians commonly misdiagnosed AOM, but also prescribed antibiotics to almost half of those they diagnose with OME.

On the issue of preoperative preparation of patients with chronic suppurative otitis media

Introduction. In the otosurgeon practice, there are often cases of exacerbation of chronic suppurative otitis media (CSOM) refractory to conservative therapy. The direct irritant effect of gastric contents due to the gastroesophageal reflux disease (GERD) often leads to alteration of the middle ear mucosa and persistence of the inflammation process.Aim. To substantiate the significance of GERD therapy in the treatment of CSOM exacerbation refractory to the standard conservative therapy protocols.Materials and methods. The study group included 42 patients aged 19 to 67 years (mean age 47.6 years), 13 men and 29 women, who received treatment and daily observations of the attending otorhinolaryngologist. Of these, 31 patients were users of oral nicotine products (chewing gum, smoking, tobacco heating systems). The conservative treatment of CSOM was carried out in accordance with the medical care standards and clinical guidelines as in force on the time of the treatment process. The conservative treatment of GERD was carried out in accordance with the clinical guidelines approved by the Russian Gastroenterological Association.Results and discussion. 25 patients were diagnosed with isolated incompetence of the gastric cardia, 13 — with incompetence of the gastric cardia accompanied by reflux esophagitis, 4 — with hiatal hernia. The efficacy of anti-reflux therapy for chronic otitis media exacerbation was assessed on Day 10 after start of the therapy. Due to the gastroenterological therapy, the “dry” ear status could be achieved in 34 patients, which made it possible to offer them reconstructive intervention in the future. 5 patients achieved the CSOM remission by the end of the first month of anti-reflux therapy. 3 patients failed to achieve the state of CSOM remissionConclusions. The high efficacy of anti-reflux therapy for the management of CHSO exacerbation refractory to the standard conservative therapy regimens at the level of 81% allows us to recommend further research to prove the causal relationship between GERD and ineffectiveness of treatment of ear pathology.

Tympanometric findings among adult HIV patients undergoing a short-term treatment with high active antiretroviral therapy (HAART) in port harcourt.

In our practice as ENT specialists, people living with Human immunodeficiency Virus/acquired immunodeficiency syndrome (HIV/AIDS) have presented at the clinics with symptoms suggestive of otitis media with effusion such as the sensation of fluid in the ear, aural fullness and hearing loss. Eustachian tube dysfunction which is often the beginning of middle ear pathology could be caused by nasal allergy, upper respiratory tract infection, or obstruction by a nasal pharyngeal lesion such as lymphoid hyperplasia which is a common feature in people living with HIV/AIDS. Tympanometric findings give a measure of the objective assessment of middle ear function. This study was designed to determine tympanometric findings among adult patients undergoing short-term treatment with HAART in Port Harcourt. A hospital-based study involving 150 HIV-positive patients that received the same HAART treatment over 6 months and a control group of 150 HIV-negative individuals in Port Harcourt. The data extracted includes; the patient's ear symptoms, otoscopic findings, and tympanogram. Data were analyzed using SPSS version 20 and statistical significance was set at P > 0.05. There was a high proportion of type B-Typanogram at baseline (Rt ear 24[16.0%], left ear 23 [15.3%]) and at repeat (Rt ear 23 (15.3%), Lt ear 21 (14%) evaluations. Also, there was a relatively high proportion of type C- tympanogram at baseline {right ear 18 (12%), left ear 15 (10%)} and at repeat Rt ear 14 (9.3%), Lt ear 10 (6.7%)} evaluations. One out of every eight patients living with HIV infection may likely have Eustachian tube dysfunction while one out of every five may have developed otitis media with effusion already. There was no significant change in tympanometric findings after treatment with HAART.

A PROSPECTIVE STUDY ON ROLE OF HIGH RESOLUTION COMPUTED TOMOGRAPHY IN EVALUATION OF TEMPORAL BONE PATHOLOGIES

Background: The temporal bone is a complex anatomic structure that contains the organs of hearing and balance and has direct contact with brainstem, cerebellum and temporal lobe of brain. The application of high-resolution computed tomography (HRCT) in the temporal bone study has allowed the detailed assessment of complex bony anatomy and pathology. The aim of this study was to evaluate various temporal bone pathologies (inammatory, traumatic, neoplastic and congenital anomalies), their extent and complications on HRCT and to correlate these imaging ndings with surgical/histopathological ndings wherever possible. Materials And Methods: This was a prospective study conducted in the Department of Radiology, Silchar Medical College and Hospital, Silchar from 1st of June 2021 to 31st of May 2022 amongst 50 patients with clinically known or suspected pathologies of inner ear, middle or external ear and the temporal bone. In our study, the most common temporal bone pathologies were inamma Results: tory (62%) followed by traumatic (24%), neoplastic (10%) and congenital (4%). Most common inammatory pathology was otomastoiditis (45.16%) followed by acquired Cholesteatoma (35.48%), chronic mastoiditis (9.68%), Otitis media (6.45%) and Otitis externa (3.23%). Out of 12 traumatic cases, longitudinal fracture(58.3%) was found to be the most common type followed by transverse fracture(25%) and mixed fracture(16.67%). Out of 5 cases of neoplastic pathology, 60% of cases were benign and 40% were malignant aetiology with acoustic neuroma being the most common tumour. EAC atresia was the most common congenital anomaly. The surgical and radiological correlation showed a high sensitivity in the identication of cholesteatoma. HRCT of the temporal bone is a highly efcacious modality for a Conclusions: ccurate delineation of the anatomy and various pathologies of the temporal bone and it has revolutionized the role of radiology in diagnosis and management of temporal bone diseases.

High frequency hearing 25 years after transmyringeal ventilation tube treatment.

The main aim of the study was to compare hearing outcome between a healthy control group and patients treated with transmyringeal ventilation tubes, 25 years after primary surgery. Another aim was to analyse the relation between ventilation tube treatment in childhood and the occurrence of persistent middle ear pathology 25 years later. In 1996, children treated with transmyringeal ventilation tubes were recruited for a prospective study on the outcome of ventilation tube treatment. In 2006, a healthy control group were recruited and examined together with the original participants (case group). All participants in the 2006 follow-up were eligible for this study. A clinical ear microscopy examination including eardrum pathology grading and high frequency audiometry (10-16kHz) was carried out. A total of 52 participants were available for analysis. Hearing outcome was worse in the treatment group (n=29) compared to the control group (n=29), both in regard to standard frequency range hearing, (0.5-4kHz), and high frequency hearing (HPTA3 10-16kHz). Almost half the case group (48%) had eardrum retraction to some extent, compared to 10% in the control group. No case of cholesteatoma was found in this study and eardrum perforation was rare (<2%). In the long term, high frequency hearing (HPTA3 10-16kHz) was more often affected in the patients with transmyringeal ventilation tube treatment during childhood compared with the healthy controls. Middle ear pathology of greater clinical significance was rare.

Asymmetry of Occupational Noise Induced Hearing Loss: An Electrophysiological Approach.

Introduction The question as to whether occupational noise exposure causes symmetrical or asymmetrical hearing loss is still controversial and incompletely understood. Objective Two electrophysiological methods (cortical evoked response audiometry: CERA and auditory steady state responses: ASSR) were used to address this issue. Method 156 subjects with a well-documented history of noise exposure, a wide range of noise induced hearing loss (NIHL) and without middle ear pathology underwent both a CERA and an ASSR examination in the context of an exhaustive medicolegal expert assessment intended for possible compensation. Results Whatever the method (CERA or ASSR), the average electrophysiological hearing thresholds (1-2-3 kHz) are significantly worse in the left ear. The right - left differences in CERA and ASSR thresholds are strongly correlated with each other. No significant effect of frequency is found. No correlation is observed between right - left differences in hearing thresholds and either age or degree of hearing loss. Conclusion In NIHL, there is an actual average right - left difference of about 2.23 dB, i.e., 3.2%, the left ear being more impaired.

Tympanoplasty in the Setting of Complex Middle Ear Pathology: A Systematic Review.

To assess the prognostic factors for anatomic and hearing success after tympanoplasty in the setting of complex middle ear pathology. A systematic review was performed in January 2022. English-language articles describing outcome data for tympanoplasty repair variables including underlying pathology, perforation location, smoking status, graft technique, reconstruction material, anatomic success, and hearing success were extracted. Articles were included when tympanosclerosis, retraction pockets, adhesions, cholesteatoma, chronic suppurative otitis media, anterior perforations, and smoking were included. Underlying pathology, perforation location, smoking status, graft technique, reconstruction material, anatomic success, and hearing success were extracted. Any factors analyzed as potential indicators of success were sought out. Data sources included PubMed, OVID, Cochrane, Web of Science, Scopus, and manual search of bibliographies. Ninety-three articles met final criteria, which accounted for 6685 patients. Fifty articles presented data on both anatomic and hearing outcomes, 32 articles presented data on anatomic outcomes only, and 11 articles presented data on hearing outcomes only. This systematic review found that adhesions and tympanosclerosis were prognostic factors for poorer hearing. Additionally, smoking and tympanosclerosis may be predictive of anatomic failure; however, the significance of this finding was mixed in included studies. This analysis is significantly limited by both the heterogeneity within the patients and the lack of controls. Adhesions and tympanosclerosis were prognostic factors for poorer hearing. Clearly documented methods and outcomes for the included pathologies could lead to more definitive conclusions regarding prognostic factors for success. 3B.

Investigation of inner ear drug delivery with a cochlear catheter in piglets as a representative model for human cochlear pharmacokinetics.

Hearing impairment is the most common sensory disorder in humans, and yet hardly any medications are licensed for the treatment of inner ear pathologies. Intricate pharmacokinetic examinations to better understand drug distribution within this complex organ could facilitate the development of novel therapeutics. For such translational research projects, animal models are indispensable, but differences in inner ear dimensions and other anatomical features complicate the transfer of experimental results to the clinic. The gap between rodents and humans may be bridged using larger animal models such as non-human primates. However, their use is challenging and impeded by administrative, regulatory, and financial hurdles. Other large animal models with more human-like inner ear dimensions are scarce. In this study, we analyzed the inner ears of piglets as a potential representative model for the human inner ear and established a surgical approach for intracochlear drug application and subsequent apical sampling. Further, controlled intracochlear delivery of fluorescein isothiocyanate-dextran (FITC-d) was carried out after the insertion of a novel, clinically applicable CE-marked cochlear catheter through the round window membrane. Two, six, and 24hours after a single injection with this device, the intracochlear FITC-d distribution was determined in sequential perilymph samples. The fluorometrically assessed concentrations two hours after injection were compared to the FITC-d content in control groups, which either had been injected with a simple needle puncture through the round window membrane or the cochlear catheter in combination with a stapes vent hole. Our findings demonstrate not only significantly increased apical FITC-d concentrations when using the cochlear catheter but also higher total concentrations in all perilymph samples. Additionally, the concentration decreased after six and 24hours and showed a more homogenous distribution compared to shorter observation times.

In Silico Localization of Perilymph Proteins Enriched in Meńier̀e Disease Using Mammalian Cochlear Single-cell Transcriptomics.

Proteins enriched in the perilymph proteome of Meńier̀e disease (MD) patients may identify affected cell types. Utilizing single-cell transcriptome datasets from the mammalian cochlea, we hypothesize that these enriched perilymph proteins can be localized to specific cochlear cell types. The limited understanding of human inner ear pathologies and their associated biomolecular variations hinder efforts to develop disease-specific diagnostics and therapeutics. Perilymph sampling and analysis is now enabling further characterization of the cochlear microenvironment. Recently, enriched inner ear protein expression has been demonstrated in patients with MD compared to patients with other inner ear diseases. Localizing expression of these proteins to cochlear cell types can further our knowledge of potential disease pathways and subsequent development of targeted therapeutics. We compiled previously published data regarding differential perilymph proteome profiles amongst patients with MD, otosclerosis, enlarged vestibular aqueduct, sudden hearing loss, and hearing loss of undefined etiology (controls). Enriched proteins in MD were cross-referenced against published single-cell/single-nucleus RNA-sequencing datasets to localize gene expression to specific cochlear cell types. In silico analysis of single-cell transcriptomic datasets demonstrates enrichment of a unique group of perilymph proteins associated with MD in a variety of intracochlear cells, and some exogeneous hematologic and immune effector cells. This suggests that these cell types may play an important role in the pathology associated with late MD, suggesting potential future areas of investigation for MD pathophysiology and treatment. Perilymph proteins enriched in MD are expressed by specific cochlear cell types based on in silico localization, potentially facilitating development of disease-specific diagnostic markers and therapeutics.

Evaluation of Types of Vertigo With Electronystagmography: An Experience From a Tertiary Care Hospital in West Bengal, India.

Background Electronystagmography (ENG) is a diagnostic test that measures the electrical activity of the muscles that control eye movements. ENG has the potential to identify the cause of vertigo by assessing the function of the vestibular system. Vertigo can be of two types - peripheral or central. In addition, a combination of peripheral and central types may coexist. Peripheral vertigo is caused by pathology in the inner ear and central vertigo is caused by pathology in the brainstem or cerebellum. Objective This study aimed to evaluate the applicability of ENG in assisting the diagnosis of the type of vertigo in a remote tertiary care center in West Bengal, India. Materials and methods This cross-sectional study was conducted in tertiary care hospital in West Bengal, India. Any patient presenting first time with a complaint of vertigo was approached and recruited for the study after taking written informed consent. We collected demographics and conducted a complete ear, nose, and throat examination, including otoscopy and audiological evaluation. A consensus between two expert otorhinolaryngologists was reached for the categorization of vertigo. Then, ENG was performed to assess the vestibular function to help aid the categorization. Magnetic resonance imaging (MRI) and computed tomography (CT) scans were done in central vertigo patients according to the necessity to diagnose the cause. Data were presented in descriptive statistical terms and categorical data were compared by Chi-square test. Result A total of 84 patients (male 31, female 53) with a median age of 25 years (Q1-Q3: 21-30.25) participated in the study. We found 75% of the patients were complaining of instability, 50% rotatory objective vertigo, 29.76% falling tendency, 22.62% blackout, and 2.38% sinking sensation. The majority of the patients (63%) had two or more symptoms. A total of 68 (80.95%) patients could be categorized into peripheral (46 [54.76%]) and central (22 [26.19%]) types. When we added ENG to the tests, we could categorize all the patients and found that 48 (57.14%) had peripheral, 27 (32.14%) had central, and nine (10.71%) had mixed lesions. Conclusion ENG when used in conjunction with clinical examination, otoscopy, and an audiological examination can help to categorize all patients into peripheral, central, or mixed lesion types of vertigo. Hence, ENG can be an important tool in identifying the type of vertigo and can aid in appropriate treatment decisions.

Differential Expression of Na/K-ATPase in the Human Saccule of Patients With and Without Otologic Disease.

Na + , K + -ATPase (Na/K-ATPase) α1 subunit expression in the saccule of patients diagnosed with otologic disease is different compared with normal controls. We have recently characterized changes in the expression of Na/K-ATPase α1 subunit in the normal and pathological cochlea; however, no studies have determined the distribution Na/K-ATPase α1 subunit in the human saccule. The present study uses archival temporal bones to study the expression Na/K-ATPase α1 subunit in the human saccule. Archival celloidin formalin fixed 20-micron thick sections of the vestibule from patients diagnosed with Menière's disease (n = 5), otosclerosis (n = 5), sensorineural hearing loss, and normal hearing and balance (n = 5) were analyzed. Sections containing the saccular macula were immunoreacted with mouse monoclonal antibodies against Na/K-ATPase α1 subunit. Micrographs were acquired using a high-resolution digital camera coupled to a light inverted microscope. In the normal human saccule vestibular sensory epithelium, Na/K-ATPase α1 immunoreactivity (IR) was present in nerve fibers and calyces that surround type I vestibular hair cells and nerve terminals. The transition epithelium cells were also Na/K-ATPase α1 immunoreactive. Comparison between normal and pathological specimens showed that there was a significant reduction of Na/K-ATPase α1 IR in the saccule vestibular sensory epithelium from patients with Menière's disease, otosclerosis, and sensorineural hearing loss. The decrease of Na/K-ATPase-IR α1 in the saccule vestibular sensory epithelium from patients with otopathologies suggests its critical role in inner ear homeostasis and pathology.

Comparing Myringoplasty to Type I Tympanoplasty in Tympanic Membrane Repair: A Systematic Review and Meta-analysis.

To compare the anatomic success rates of type I tympanoplasty (tympanoplasty) versus myringoplasty. By our definition, tympanoplasty involves entering the middle ear via elevation of a tympanomeatal flap, while myringoplasty involves surgery to the drumhead without middle ear exposure. PubMed, Scopus, CINAHL, Cochrane. To be included, studies must have documented surgical technique, tympanic membrane (TM) perforation size (as % of TM), and success rate using tissue or alloplastic grafts. Exclusion criteria included series with more than 10% of patients with cholesteatoma or middle ear pathology. A meta-analysis of weighted summary proportions under the random effects model was performed, and proportion differences (PD) were calculated. A secondary analysis of hearing outcomes was performed. Eighty-five studies met inclusion, with a tympanoplasty cohort of n = 7966 and n = 1759 for myringoplasty. For perforations, less than 50% of the TM, the success rate for tympanoplasty and myringoplasty was 90.2% and 91.4%, respectively (PD: 1.2%, p = .19). In perforations greater than 50%, tympanoplasty and myringoplasty success rates were 82.8% and 85.3%, respectively (PD: 2.5%, p = .29). For both procedures, perforations less than 50% of the TM had higher success rates than perforations greater than 50% of the TM (p < .01). Both techniques endorsed significant improvements to air-bone gap (ABG) metrics. Our analysis suggests that the anatomic success rate is similar for tympanoplasty and myringoplasty, regardless of perforation size, and that smaller perforations experience higher success rates in both techniques. ABG outcomes were also similar between procedure techniques.

Wideband Acoustic Immittance in Children.

As wideband absorbance (WBA) gains popularity, it is essential to understand the impact of different middle ear pathologies on the absorbance patterns as a function of frequency in children with various middle ear pathologies. More recently, the use of wideband tympanometry has enabled clinicians to conduct WBA at ambient pressure (WBA amb ) as well as the pressurized mode (WBA TPP ). This article reviews evidence for the ability of WBA measurements to accurately characterize the normal middle ear function across a wide range of frequencies and to aid in differential diagnosis of common middle ear disorders in children. Absorbance results in cases of otitis media with effusion, negative middle ear pressure, Eustachian tube malfunction, middle ear tumors, and pressure equalization tubes will be compared to age-appropriate normative data. Where applicable, WBA amb as well as WBA TPP will be reviewed in these conditions. The main objectives of this article are to identify, assess, and interpret WBA amb and WBA TPP outcomes from various middle ear conditions in children between the ages of 3 and 12 years.

Degree of Hearing Loss in Association with Site of Tympanic Membrane Perforation: A Systematic Review

Background: Tympanic Membrane perforation is commonly seen in a middle ear pathology called chronic otitis media that affects at least 0.5% of the total world population. Aim: To evaluate the nature of hearing loss in tympanic membrane perforation (TMP) based on the site of perforation. Study design: Systemic review. Methodology: All the articles were taken from Google Scholar, Cochrane Library, Pub Med, NCBI (NLM Catalog) and HEC Digital Library within five years range: 2017-2021. All the researchers were screened widely by a well-intentioned peer review group. Results: Articles (n=19) were included in the review. In case of site of perforation, most of the articles compared anterior site with posterior and central, depicting that posterior site causes more hearing loss than anterior site, while central perforations had greater hearing loss than anterior/posterior site. The total sample size of these 19 articles collectively was (n=1,650). Most severe hearing loss with respect to site of TM perforation was demonstrated in only 1 study (5.26%) out of 19 and that was 61-80 decibels. One study (5.26%) showed no correlation between hearing loss and site of TM perforation. Practical Implication: Sometimes, it is seen in ENT practices that there was a dilemma regarding the severity of hearing loss with respect to site of perforation. It benefited otolaryngologists to find a systematic review on the provided topic, to learn and know more about the association between hearing loss and site of TMP. Conclusion: We concluded that anterior sites of perforations cause less hearing loss than posterior, central or marginal. Keywords: Hearing loss; Tympanic membrane perforation; Site of TM perforation.

Ten years' experience with bone conduction hearing aids in the Western Cape, South Africa.

Untreated conductive and mixed hearing losses as a result of middle ear pathology or congenital ear malformations can lead to poor speech, language and academic outcomes in children. Lack of access to centralised hearing healthcare in resource-constrained environments limits opportunities for children with hearing loss. Red Cross War Memorial Children's Hospital (RCWMCH) is one of only two dedicated paediatric hospitals in sub-Saharan Africa. Between 2016 and 2021, 29 children received implanted bone conduction hearing devices, and 104 children were fitted with bone conduction devices on softbands. The authors' experience at RCWMCH suggests that bone-anchored hearing devices, either fitted on softbands or on implanted abutments, can provide solutions in settings where patients have limited access to hearing healthcare and optimal classroom environments. Hearing healthcare should be accessible and delivered at the appropriate level of care to mitigate the adverse effects of hearing loss in children.Contribution:This article describes strategies employed at RCWMCH such as fitting bone conduction hearing devices on a softband immediately after hearing loss diagnosis and conducting follow-up via remote technology to make hearing healthcare more accessible to vulnerable populations.

FEATURES OF NEUROLOGICAL DISORDERS IN CHILDREN WITH CONGENITAL AND ACQUIRED SENSORINEURAL HEARING LOSS

Sensorineural hearing loss is currently the most important medical and social problem and is an urgent problem of neurology. According to various researchers, from 6% to 36% of the worlds population suffers from ear pathology, of which 60-80% have sensorineural hearing impairment (damage to the cochleo-vestibular nerve receptor - the cochlea of the inner ear).Deafness is the most common defect in sensory systems in children and occurs in 1: 750 cases, and in adults 4-36% depending on age, which reflects the interaction of environmental factors and individual genetic predisposition (Tranebjaerg L., 2000).About 400 genetic syndromes that include hearing impairment have been described (Friedman T.V., Griffith A J., 2003). KEY WORDS: sensorineural hearing loss, neurological disorders. Purpose: to study clinical and neurological features in patients with sensorineural hearing loss.