Cases Of Sensorineural Hearing Loss Research Articles

Sudden Sensorineural Hearing Loss after COVID-19 Vaccination: A Review of the Available Evidence through the Prism of Causality Assessment.

Sudden sensorineural hearing loss (SSNHL), a rare audiological condition that accounts for 1% of all cases of sensorineural hearing loss, can cause permanent hearing damage. Soon after the launch of global COVID-19 vaccination campaigns, the World Health Organization released a signal detection about SSNHL cases following administration of various COVID-19 vaccines. Post-marketing studies have been conducted in different countries using either pharmacovigilance or medico-administrative databases to investigate SSNHL as a potential adverse effect of COVID-19 vaccines. Here, we examine the advantages and limitations of each type of post-marketing study available. While pharmacoepidemiological studies highlight the potential association between drug exposure and the event, pharmacovigilance approaches enable causality assessment. The latter objective can only be achieved if an expert evaluation is provided using internationally validated diagnostic criteria. For a rare adverse event such as SSNHL, case information and quantification of hearing loss are mandatory for assessing seriousness, severity, delay onset, differential diagnoses, corrective treatment, recovery, as well as functional sequelae. Appropriate methodology should be adopted depending on whether the target objective is to assess a global or individual risk.

Comparative analysis of allele frequencies of 15 deafness gene variants between hearing-loss and normal populations in Henan, China

BackgroundHearing loss is found in more than 5 % of cases worldwide. Hearing loss is divided into three types: Sensorineural hearing loss, Combined hearing loss and Conductive hearing loss. Among them, no less than 50 % of pediatric cases of sensorineural hearing loss are genetic. In Henan, China, there are no statistics on the allele frequency of deafness gene variants. MethodsWe divided 2178 subjects enrolled at the Third Affiliated Hospital of Zhengzhou College from January 2019 to March 2021 into a hearing loss group and a normal control group. We performed array and pathogenicity classification for screening the 15 deafness gene variants, calculated and compared the allele frequency of the deafness gene variants, and then compared the hearing loss diagnosis rate between the hearing loss group and the normal control group. ResultsWe found that in the hearing loss group, the overall allele frequency of all detected variants was 16.6 %. Comparative analysis showed that the allele frequencies of GJB2 c.235delC variant, GJB3 c.538C > T variant and SLC26A4 c.919-2A > G variant were significantly higher than those of the East Asian population average in the gnomAD database. At the same time, our study confirmed that GJB3 c.538C > T variant may not be the disease-causing variant of hearing loss. ConclusionsThese results support genetic counseling and rational prediction of risk for deafness.

Efficacy of S53P4 Bioactive Glass for the Secondary Obliteration of Chronically Discharging Radical Cavities.

Present the results of the secondary obliteration of chronically discharging radical cavities using S53P4 bioactive glass (BAG). Retrospective cohort study. Single-center study. A single-center retrospective cohort study was conducted of all patients that underwent secondary obliteration of persistently draining radical cavities using S53P4 BAG between 2011 and 2022. Patients with middle ear cholesteatoma were excluded. The main outcome was postoperative otorrhea, as indicated by Merchant grading. In total, 97 patients were included. The median postoperative follow-up time was 3.9 years (range 0.5-10.4). Average time between the original canal wall down surgery and the secondary obliteration was 25.3 years (SD 11.7, range 2-66). At the most recent follow-up visit, a Merchant grade of 0 to 1 was observed in 95% of the cases. There were no cases of sensorineural hearing loss or facial palsy, one case developed a retro auricular skin defect and 1 patient developed CSF leakage. Minor complications were seen in 10 patients (10%). Ossicular chain reconstruction with a titanium prosthesis was performed in 42 cases, resulting in a median improvement of 11.2 dB in air conduction thresholds. In 9/42 cases (21%), closure of the postoperative air-bone gap to ≤20 dB was achieved. Twenty-five percent of cases could be discharged from out-patient visits. Revision of persistently draining radical cavities with BAG obliteration is feasible and results in a dry and safe ear in 95% of the patients, thereby enabling wearing of a conventional hearing aid. Out-patient visits could be ceased in 25% of the cases.

Sudden Bilateral Sensorineural Hearing Loss due to Basilar Artery Occlusion (P3-5.027)

<h3>Objective:</h3> We describe a case of a patient with occlusion of the basilar artery and bilateral anterior inferior cerebellar arteries (AICAs) who presented with a chief complaint of sudden bilateral sensorineural hearing loss (SNHL). <h3>Background:</h3> Sudden bilateral SNHL, defined as SNHL developing in less than 72 hours, is a rare phenomenon representing less than 5% of all acute SNHL cases. Whereas more frequent unilateral presentation is relatively benign, sudden bilateral SNHL represents a medical emergency and warrants immediate evaluation for life-threatening and/or reversible causes. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 56-year-old right-handed African American woman with personal medical history of hypertension, hyperlipidemia, chronic obstructive pulmonary disease, stroke, tobacco abuse, and obesity presented with sudden onset bilateral hearing loss preceded by one week of persistent vertigo and nausea. On admission she was hypertensive to 240/122 mm Hg, physical examination was significant for saccadic pursuit with spontaneous bilateral horizontal and vertical down-beating nystagmus, truncal ataxia, and bilateral SNHL confirmed by audiogram. CT of head alongside CT angiography showed occlusion of V4 segment of right vertebral artery extending through most of the basilar artery, and near-occlusion of bilateral AICAs. MRI showed late acute/early subacute ischemic infarcts in bilateral anterior cerebellum, cerebellar peduncles, and pons. Patient was out of time window for thrombolysis with rt-PA, mechanical thrombectomy was deferred due to &gt;24h from symptom onset and stable neurological examination throughout hospital admission. Blood pressure was gradually lowered and antiplatelet therapy with aspirin was initiated prior to discharge to rehabilitation facility. <h3>Conclusions:</h3> Sudden bilateral SNHL in isolation or accompanied by vestibular, cerebellar and/or brainstem signs particularly in patients with significant risk factors for cerebrovascular disease should alarm a clinician of a possibility of vertebrobasilar occlusion and prompt appropriate and timely medical management as delay in optimal medical treatment is associated with increased morbidity and mortality in this patient population. <b>Disclosure:</b> Dr. Buciuc has nothing to disclose. Dr. Gonzalez has nothing to disclose. Dr. Ali has nothing to disclose. Dr. Nankee has nothing to disclose. Dr. Holmstedt has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astrazeneca. The institution of Dr. Holmstedt has received research support from NIH. The institution of Dr. Holmstedt has received research support from Patient-Centered Outcomes Research Institute. The institution of Dr. Holmstedt has received research support from CSPC Pharmaceuticals . Dr. Holmstedt has received personal compensation in the range of $5,000-$9,999 for serving as a Study Adjudicator with Ischemia Care.

Assessing the Brighton Collaboration Case Definition of Sensorineural Hearing Loss in Ghana.

The Brighton Collaboration has developed a case definition to assess sensorineural hearing loss (SNHL) as an adverse event of special interest for Lassa fever vaccines. We applied the level of diagnostic certainty (LOC) criteria to 300 SNHL patients aged 18 to 59 years at a tertiary hospital in Ghana from January 2017 through June 2020 and evaluated the applicability of this definition. Most SNHL cases were assessable (85.0%) and assigned level 1 LOC (84.3%); missing information on otoscopy (86.7%) was the main reason for being unable to classify cases. Consistency of LOC classification between assessors was 99.3%. Cases with electronic medical records (EMRs) were less assessable than those with paper records (30.9% versus 93.8%). These findings indicate that the SNHL definition would be applicable to retrospectively ascertain and classify cases in resource-limited settings. Developing an EMR template to document otoscopy results may improve the feasibility at this hospital to ascertain SNHL.

A prospective cohort study of sensorineural hearing loss associated with Kawasaki disease.

About 60 cases of sensorineural hearing loss (HL) have been reported in patients with Kawasaki disease (KD), but the current estimate of its prevalence is uncertain. The present study aimed to determine the prevalence and risk factors of sensorineural HL associated with KD. The present, prospective cohort study, conducted from May 2019 to May 2020, evaluated patients with a diagnosis of KD who received the initial therapy and underwent two auditory brainstem response (ABR) tests. HL was defined as a threshold of 40dB or more, and borderline hearing was defined as a threshold of 30dB. In total, 107 patients were enrolled, and 75 underwent two ABR tests. Thirty-one patients (30.0%) received prednisolone with their initial intravenous immune globulin and acetylsalicylic acid therapy. HL was present in only one patient who had congenital conductive HL. Five patients had borderline hearing but had normal hearing behavior. There was no significant difference between the patients with normal hearing and those with borderline hearing in terms of the clinical variables. In Japan, the prevalence of sensorineural HL after KD is not high. It may therefore be unnecessary to perform routine hearing tests for all patients with KD. Association between Kawasaki Disease and Sensorineural Hearing Loss. UMIN000037019 (the date of registration: June 11, 2019).

ENT characteristics and therapeutic results in multisystemic disorders of mitochondrial encephalomyopathy

Here we report the evaluation of the frequency of subjective and objective otolaryngologic findings and therapeutic results in 32 patients with mitochondrial encephalomyopathy (MEM) from September 2001 to June 2021. Our analysis included studying the patients’ family histories, the clinical manifestations of MEM, and the therapeutic effects of treatments. The patients’ ages ranged from 2 to 77 years, with a median age of 12.3 years. We found that MEM ENT symptoms were characterized by hearing loss, dysphagia, and facial weakness. Most cases of sensorineural hearing loss were bilateral symmetrical progressive or sudden deafness since adolescence, which were often underestimated. Associated neuromuscular symptoms required mtDNA testing. Dysphagia and facial weakness occurred preferentially in middle-aged patients, and muscle biopsies were advised. Distortion product otoacoustic emissions and auditory brainstem responsetesting were more sensitive and reliable than pure tone averages for objective monitoring of pathogenesis. Administration of the mitochondrial synthase complex benefited patients with acute episodes. If patients did not fully recover and exhibitedresidual language deficits, hearing aids or cochlear implants were recommended. Counsel was given regarding synthetical treatments for facial weakness, endoscopic circopharyngealmyotomy for dysphagia, and surgical correction of ptosis. This study demonstrates that increased awareness of these symptoms is important to address appropriate interventions and avoid complications such as ablepsia, aphasia, social isolation, malnutrition, aspiration pneumonia, and heart failure in the setting of MEM.

A Review of Sensorineural Hearing Loss in Congenital Cytomegalovirus Infection.

The intrauterine transmission of the TORCH group (toxoplasmosis, rubella cytomegalovirus (CMV), herpes simplex, and HIV) produces severe complications in the fetus, leading to major life challenges for the newborns. CMV is considered one of the leading causes of congenital hearing loss in babies born to infected mothers. The majority of the cases are asymptomatic but a certain proportion show symptoms, which may not be present until later. The human cytomegalovirus (HCMV) infection can also be transferred to the baby postnatally through breastfeeding. Mothers facing primary infection from the CMV have a greater tendency to transmit the infection to the fetus, whereas secondary infection due to reactivation of the virus in females who were affected before pregnancy, delivers asymptomatic babies in most cases. Vertical transmission of HCMV,that is via the placenta from infected mother to fetus, is one of the leading causes of congenital sensorineural hearing loss (SNHL) and neurodevelopmental complications in newborns. It accounts for up to 10% of SNHL cases in newborns, of both unilateral and bilateral types. Antiviral therapy is helpful in such cases if administered within the first month of life, and for hearing impairment, cochlear implants have been used to treat children who develop profound hearing loss. The child can present with hearing loss at birth or it may be late in onset and of progressive type. Suspected children should be evaluated regularly for the early detection of hearing loss and to provide the appropriate treatment.

Assessment of seasonal pattern of idiopathic sudden sensorineural hearing loss: a retrospective cross-sectional study.

A seasonal trend of patients with idiopathic sudden sensorineural hearing loss may direct research into possible aetiology. This study reviewed data from the medical records of patients who presented from 2004 to 2019 and who were diagnosed with new-onset idiopathic sudden sensorineural hearing loss. Seasonal pattern was assessed using chi-square and Rayleigh tests, and further confirmed by Monte Carlo simulation. The study included 740 patients with a mean age of 48.3 years and a median age of 49 years. There was no statistical evidence for a difference in the distribution of sensorineural hearing loss cases for the four seasons of each year or with the cumulative data. New-onset idiopathic sudden sensorineural hearing loss cases averaged around 11 per month; there was no statistical evidence for a seasonal difference, as determined either by the Rayleigh test or with Monte Carlo simulation. There was no evidence to support the claim that idiopathic sudden sensorineural hearing loss incidence displays a seasonal pattern. More research is necessary to explore potential external factors such as climate or infection.

Analysis of appeals of patients with sensorineural hearing loss to the otolaryngological department during 2015-2021

Patients with perceptual hearing impairment occupy a significant place among patients of the otolaryngology department. They often have psycho-emotional disorders, social and family disorders. It is important to understand the etiopathogenesis of the disease. Some cases of sensorineural hearing loss (SHL) remain with an unknown etiopathogenetic mechanism of development, which requires more in-depth examination of patients. Aim: to analyze the number and character of appeals of patients with sensorineural hearing impairment to the otolaryngology department of the Alexander Clinical Hospital in Kyiv during 2015-2021. Methods and materials: We analysed 13,147 inpatients 'medical records, from which 1,138 patients' cards with perceptual hearing impairments were selected. Results: According to the analysis, the absolute and relative number of patients in the otolaryngology department of Alexander Clinical Hospital in Kyiv in 2020 and 2021 decreased significantly due to the temporary re-profiling of the otolaryngology department in these years for infectious patients with COVID-19. The most common changes in bone conduction according to audiometry occurred against the background of acute or exacerbation of chronic otitis media (509 cases). But this figure may be due to inflammatory changes, rather than true sensorineural hearing loss. 155 patients could not name a specific cause. These patients required further in-depth examination. In the third most frequent place indicated hypothermia, acute respiratory diseases (135 cases). In 106 patients, acute sensorineural hearing loss was caused by vascular disorders, and 11 patients reported emotional stress as the cause. Acute trauma caused perceptual disorders in 16, surgery in 3, neuroma in 1, barotrauma and rupture of the tympanic membrane in 7, the use of chemotherapy in 3 cases. The average interval between the onset of the first symptoms of SHL and hospitalization in the otolaryngology department of the Alexander Clinical Hospital in Kyiv for 2025-2021 was 8.19 days. The lowest interval was in 2016 – 7.5 days, the largest – 10 days in 2021. The average duration of inpatient treatment of patients for 2015-2021 was 9.4 days. The lowest figure was in 2020 – 8.1 days, the highest – 10.4 days in 2021. There were 495 patients with bilateral lesions and 354 patients with left lesions, 289 patients had lesions on the right side. 39 patients with SHL and type 2 diabetes mellitus had diabetes, 460 had tinnitus and 44 had vestibular disorders. Conclusions: Patients with acute sensorineural hearing loss receive inpatient treatment in the otolaryngology department than with the progression or exacerbation of the chronic form. Otitis media and acute respiratory diseases were the most common disorders of bone conduction, but not all of these cases can be interpreted as sensorineural hearing loss. The average interval for the study period between the onset of the first symptoms and the start of treatment was 8.19 days, which exceeds the recommended time of this indicator. The average duration of inpatient treatment was 9.4 days and did not change significantly during the study period.

Otologic disorders and management strategies in Turner syndrome

Objective: To analyze the incidence and risk factors of otologic disorders in patients with Turner syndrome (TS), so as to provide management strategies for ear health. Methods: This study is a prospective study based on questionnaires and a cross-sectional study. The TS patients who visited our hospital from 2010 January to 2021 March were included (A total of 71 patients with TS were included in this study. the age of TS diagnosed was 3- to 11-year-old, age of visiting ENT department was 4- to 27-year-old) and the incidence of otologic diseases in different age groups was investigated by questionnaires. The cross-sectional study included ear morphology and auditory function assessment, and further analysis of the risk factors that related to ear disease. Prism was used for data analysis. Results: The investigation found that the incidence of acute otitis media in patients aged 3-6 and 7-12 years was higher than that of patients over 12 years old, which was 33.8%(24/71), 42.9%(30/70)and 23.5%(8/34), respectively; 21.1% (15/71) of patients were recurrent acute otitis media in patients aged 3-6 years, and about 46.6% (7/15)of them persisted beyond 6-year. The prevalence of otitis media with effusion in the three groups was 32.4%(23/71), 34.3%(24/70)and 38.2%(13/34), respectively; the recurrence rate of tympanocentesis was 100%(7/7), 42.9%(3/7)and 50.0%(1/2), which was significantly higher than that of grommet insertion. For age groups of 3-6 and 7-12 years, the prevalence of acute otitis media and secretory otitis media was lower in the X chromosome structure abnormal patients; while for patients older than 12 years, otitis media with effusion was the highest prevalence in Y-chromosome-containing karyotypes. In addition, the prevalence of acute otitis media and otitis media with effusion in patients with other system diseases were increased significantly. A cross-sectional study found that 7.0% (5/71)of the lower auricular, 4.2% (3/71)of the external auditory canal narrow, and 38.0% (27/71)of the tympanic membrane abnormality. 35.2%(25/71) had abnormal hearing, including 17 cases of conductive deafness, 6 cases of sensorineural hearing loss, and 2 cases of mixed deafness. The rest of the patients had normal hearing, but 6 of them had abnormalities in otoacoustic emission. Eustachian tube function assessment found that the eustachian tube dysfunction accounted for 38%(27/71). Hearing loss and abnormal Eustachian tube function were not significantly related to karyotype(Chi-square 2.83 and 2.84,P value 0.418 and 0.417), but significantly related to other system diseases(Chi-square 13.43 and 7.53,P value<0.001). Conclusions: The incidence of TS-related otitis media and auditory dysfunction is significantly higher than that of the general population. It not only occurs in preschool girls, but also persists or develops after school age. Accompanied by other system diseases are risk factors for ear diseases. Clinicians should raise their awareness of TS-related ear diseases and incorporate ear health monitoring into routine diagnosis and treatment.

Effects of cytomegalovirus infection on infants' hearing and speech development

Objective:To investigate the effects of cytomegalovirus（CMV） infection on infants' hearing and speech development. Methods:A total of 192 infants with cytomegalovirus infection were selected as research objects（CMV group）. Among 320 normal infants who received physical examinations in the Second Affiliated Hospital of Zhengzhou University during the same period were selected as the control group. Using transiently evoked otoacoustic emission to conduct initial hearing screening. Jointing automatic auditory brainstem response screening method to follow up for infants infected with cytomegalovirus. Those who failed to pass the screening were diagnosed with auditory brainstem response and acoustic immittance examination. The two groups of infants were evaluated for follow-up at the age of 12, 24, 36 months using the Gesell Development scale. Results:Hearing screening（initial hearing screening and 42 d hearing re-screening）: CMV group retrospectively failed rates 28.65%（55/192）, 31.77%（61/192）, normal control group retrospectively failed rates 9.06%（29/320）, 4.06%（13/320）, the results of the two groups' hearing screening showed statistically significant differences（P<0.05）. 48 cases of diagnostic ABR were abnormal in CMV group in 3 month's hearing diagnosis, including 11 cases of secretory otitis media, 37 cases of sensorineural hearing loss. Follow-up for 36 months, 192 infants with CMV infection were confirmed congenital SNHL 37 cases（19.27%）, 21 cases of delayed SNHL（10.94%）, a total of diagnosis with SNHL 58 cases（30.21%）. The development quotient（DQ） of CMV group were respectively 92.05±4.68, 86.53±4.46, 85.92±4.82 in 12, 24, 36 months, and the DQ value of the normal control group were respectively 93.10±4.56, 94.35±4.52, 95.03±4.16. At the age of 24, 36 months, the DQ value of two groups' differences were statistically significant（P<0.05）. Conclusion:CMV infection is hearing loss' risk factors. It had the characteristics of volatility, delay and progressive decline. Follow-up should be strengthened for hearing and speech development.

Mechanism and Prevention of Spiral Ganglion Neuron Degeneration in the Cochlea

Sensorineural hearing loss (SNHL) is one of the most prevalent sensory deficits in humans, and approximately 360 million people worldwide are affected. The current treatment option for severe to profound hearing loss is cochlear implantation (CI), but its treatment efficacy is related to the survival of spiral ganglion neurons (SGNs). SGNs are the primary sensory neurons, transmitting complex acoustic information from hair cells to second-order sensory neurons in the cochlear nucleus. In mammals, SGNs have very limited regeneration ability, and SGN loss causes irreversible hearing loss. In most cases of SNHL, SGN damage is the dominant pathogenesis, and it could be caused by noise exposure, ototoxic drugs, hereditary defects, presbycusis, etc. Tremendous efforts have been made to identify novel treatments to prevent or reverse the damage to SGNs, including gene therapy and stem cell therapy. This review summarizes the major causes and the corresponding mechanisms of SGN loss and the current protection strategies, especially gene therapy and stem cell therapy, to promote the development of new therapeutic methods.

Regenerative Therapy for Sensorineural Hearing Loss: Recent Progress and Future Directions

Sensorineural Hearing Loss (SNHL) continues to be a significant public health problem with over 270 million affected people worldwide and an incidence that increases with age [1,2]. Some cases of SNHL have a genetic cause, but the majority of SHNL is the result of ototoxic insult [3]

Comprehensive molecular-genetic analysis of mid-frequency sensorineural hearing loss

The genetic heterogeneity of sensorineural hearing loss (SNHL) is a major hurdle to the detection of disease-causing variants. We aimed to identify underlying causal genes associated with mid-frequency hearing loss (HL), which contributes to less than about 1% of SNHL cases, by whole exome sequencing (WES). Thirty families segregating mid-frequency SNHL, in whom biallelic GJB2 mutations had been previously excluded, were selected from among 851 families in our DNA repository of SNHL. DNA samples from the probands were subjected to WES analysis and searched for candidate variants associated with SNHL. We were able to identify the genetic aetiology in six probands (20%). In total, we found three pathogenic and three likely pathogenic variants in four genes (COL4A5, OTOGL, TECTA, TMPRSS3). One more proband was a compound heterozygote for a pathogenic variant and a variant of uncertain significance (VUS) in MYO15A gene. To date, MYO15A and TMPRSS3 have not yet been described in association with mid-frequency SNHL. In eight additional probands, eight candidate VUS variants were detected in five genes (DIAPH1, MYO7A, TECTA, TMC1, TSPEAR). Seven of these 16 variants have not yet been published or mentioned in the available databases. The most prevalent gene was TECTA, identified in 23% of all tested families. Furthermore, we confirmed the hypothesis that a substantive portion of cases with this conspicuous audiogram shape is a consequence of a genetic disorder.

Diotic and Antiphasic Digits-in-noise Testing as a Hearing Screening and Triage Tool to Classify Type of Hearing Loss.

The digits-in-noise test (DIN) is a popular self-test measure that has traditionally been used to screen for hearing loss by providing either a pass or refer result. Standard approaches either tested each ear monaurally or used a binaural diotic version where identical digits and noise were presented simultaneously to both ears. Recently, a dichotic, antiphasic version was developed, increasing sensitivity of the DIN to unilateral or asymmetric sensorineural hearing loss (SNHL) and conductive hearing loss (CHL). The purpose of this study was to determine predictors and normative ranges of the antiphasic and diotic DIN and to determine if a combination of diotic and antiphasic DIN could accurately categorize hearing into (1) normal, (2) bilateral SNHL, or (3) unilateral SNHL or CHL. The analytical sample consisted of 489 participants between the ages of 18 and 92 years with varying types, symmetry, and degrees of hearing loss. Degree and type of hearing loss were determined based on standard clinical four-frequency (0.5-4 kHz) pure-tone air and bone conduction threshold averages. The sample consisted of bilateral normal hearing (n = 293), bilateral SNHL (n = 172), unilateral SNHL (n = 42), and CHL (n = 32). All participants (n = 489) first completed an antiphasic DIN (digit stimuli 180° out-of-phase between ears), while 393 of the sample also completed a diotic DIN. Two procedures were assessed for their ability to categorize hearing into one of the three hearing groups. The first used a fixed antiphasic cutoff combined with a cutoff formed by a linear combination of antiphasic and diotic speech recognition threshold (SRT) or binaural intelligibility-level difference. Poorer ear pure-tone average was the strongest predictor of antiphasic DIN score, whereas better ear pure-tone average explained more of the variance in diotic SRT. The antiphasic DIN sensitivity and specificity was 90% and 84%, respectively, for detecting hearing loss, with outstanding area under the receiver operating characteristics values exceeding 0.93 to identify hearing loss in the poorer ear. The first fixed SRT cutoff procedure could categorize 75% of all participants correctly, while the second procedure increased correct categorization to 79%. False negative rates for both procedures were below 10%. A sequential antiphasic and diotic DIN could categorize hearing to a reasonable degree into three groups of (1) normal hearing; (2) bilateral SNHL; and (3) unilateral asymmetric SNHL or CHL. This type of approach could optimize care pathways using remote and contactless testing, by identifying unilateral SNHL and CHL as cases requiring medical referral. In contrast, bilateral SNHL cases could be referred directly to an audiologist, or nontraditional models like OTC hearing aids.

A High Risk of Missing Congenital Cytomegalovirus-Associated Hearing Loss through Newborn Hearing Screening in Japan.

It remains unclear to what extent newborn hearing screening (NHS) detects congenital cytomegalovirus (cCMV)-associated sensorineural hearing loss (SNHL) in Japan. This study aimed to clarify the NHS results and audiological characteristics of patients with cCMV-associated SNHL. A total of 541 individuals with unilateral or bilateral hearing loss of unknown etiology were examined for cCMV infection. cCMV infection was defined by the presence of CMV DNA in the dried umbilical cord detected using real-time quantitative PCR. NHS results and audiological data were retrospectively obtained from medical records. Forty-four cases (8.1%) were positive for cCMV infection. Of them, 33 cases underwent NHS and 13 cases (39.4%) passed NHS bilaterally. The pure-tone audiograms of 21 patients were obtained. There were seven cases of unilateral SNHL, five cases of asymmetric bilateral SNHL, and nine cases of symmetric bilateral SNHL. cCMV-related hearing loss is highly heterogeneous, and there is a high risk of missing this condition through NHS.

English

Chronic kidney disease (CKD) is becoming a major health concern due to its increasing incidence among adults. There are few studies that suggest the possible relation between hearing loss and chronic kidney disease. So far only a small number of large population - based studies have assessed the relation between CKD and hearing loss. The global prevalence of CKD was 9.1 % (697.5 million cases) in 2017. The age and sex wise global prevalence of CKD was higher in women (9.5 %) than in men (7.3 %). In India, prevalence of sensorineural hearing loss (SNHL) is around 28 % to 77 % among CKD patients. Studies found that the incidence was 77 % for mild to very mild hearing loss and the incidence was 46 % for moderate to severe hearing loss. Various theories behind SNHL in CKD patients are structural similarity between ear and kidney, increased blood viscosity because of hypertension and finally electrolyte imbalance which are all thought to play a role in development of SNHL in CKD patients. Major risk factors for SNHL in CKD patients are duration of CKD, hypertension, diabetes mellitus, serum urea and creatinine levels, electrolyte imbalance, packed cell volume (PCV), ototoxic drugs. CKD being a long-term illness and majority of cases of SNHL in CKD patients are permanent, it has a great negative impact on the patient’s quality of life adding to the disability burden.

BERA as a screerning tool for evaluating cases of SNHL due to suspected retrocochlear pathology

Background: BERA is an objective study for assessing hearing loss on patients with inconsistent responses on pure tone audiogram. BERA is a non-invasive and the most cost-effective method for diagnosing retrocochlear lesion, not affected by sedation, anesthesia or age. Which helps to identify retrocochlear hearing loss. BERA is most accurate and sensitive for diagnosis of lesions in VIII nerve and auditory pathway in brainstem, especially in adults. Objectives: Screening tool for evaluating cases of sensorineural hearing loss due to suspected retrocochlear pathology. Objective test in cases of inconsistent response for pure tone audiometry. Role in evaluating brainstem lesions. Method and Aim: This is a cross sectional prospective cohortHospital based study, review of 50 patient subjected to BERA for SNHL in any age group referred to department of ENT Vijayanagara institute of medical sciences, Bellary. We aim to find Brainstem evoked response audiometry (BERA) andits applications in ENT. Result: In this study threshold and latency measures were obtained from 50 cases (100 ears) by brainstem evoked response audiometry. No restrictions were imposed on age, sex, degree of hearing loss, or audiometric configuration. The data was analyzed separately for pediatric age group (36 cases) and adults(16 cases). out of 36 pediatric cases screened 15 cases (41.7%) had normal hearing in 13 cases had profound hearing loss without any risk factors. 6 cases ( 16.7 %) hearing loss due meningitis. 2 cases (5.6%) hearing loss due to neonatal jaundice. In adult age group (14 cases) BERA was done to identify cases of retocochlear pathology and in cases of threshold estimation of hearing. Conclusion: BERA can be used to screen retrocochlear pathologies as seen in our study. Patients with hearing loss with tinnitus and giddiness screened and BERA performed had grossly degraded wave with identification of only wave I and absence of other waves and increased latencies and interaural difference in wave latencies suggests lesion in the auditory pathway. Depending on the absence of specific wave site of the lesion can also be made out. In our study 3 cases of retrocochlear pathology previously confirmed by MRI was taken and BERA was performed showing presence of only wave I and absence of remaining waves. BERA is screening test and should be confirmed by MRI.

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit.

BackgroundAutoimmune inner ear disease (AIED) represents less than 1% of all cases of sensorineural hearing loss (SNHL) but its frequency may be underestimated due to lack of specific clinical and laboratory criteria. AIED can be associated with a systemic autoimmune disease (SAID) in 15%-30% of the cases. The objective of the present study was to characterize the clinical and prognostic factors of a cohort of patients with AIED.Materials and methodsThe authors conducted a retrospective descriptive analysis of a cohort of patients with AIED referred from the otorhinolaryngology department to a systemic immune-mediated diseases unit between March 2013 and November 2020. A consecutive sample of 39 patients with suspected AIED was referred. SNHL was defined as a fall of the hearing threshold of at least 30 decibels in three consecutive frequencies. Eight patients were excluded for not meeting the audiometric criteria or having confounding factors. The remaining 31 patients were included with a total of 50 affected ears. To classify the intensity of hearing loss, an arithmetic mean of pure tone was calculated. Normal hearing or mild hearing loss at the last pure tone audiometry of the follow-up were an indicator of good prognosis and were considered the outcome of interest.ResultsThirty-two percent of the patients had an associated SAID. There were no differences regarding demographic and clinical characteristics when comparing patients with AIED alone and patients with AIED and a SAID, except for the positivity of antinuclear antibodies (ANA). ANA positivity was superior in patients with the association of AIED and a SAID when compared with patients with AIED alone (90% vs 50%; p=0.037). The SAID was diagnosed after the AIED in 70% of the patients, in which diagnosis of the SAID occurred a median of 4,2 (IQR 0.8-5.1) years after the diagnosis of the AIED. Normal audiometric evaluation or a mild hearing loss was achieved in 31% of the ears at the last audiometric evaluation. A normal audiometry or a mild hearing loss at the time of diagnosis was independently associated with a better outcome (31%, 14%, CI 1.71-273.69; p=0.018). Bilateral hearing loss was independently associated with a worse outcome (54%, 79%, CI 0.01-0.84; p=0.035). The use of systemic corticosteroids (p=0.941), transtympanic corticosteroids (p=0.700) and non-steroid immunomodulator drugs (p=0.986) did not affect prognosis. The presence of a SIAD did not affect the prognosis (p=0.986).ConclusionsIn this cohort, SAID was present in one-third of the patients with AIED. A good prognosis was achieved in one-third of the patients. A normal audiometry or mild disease at presentation was associated with a good outcome, whilst bilateral involvement was associated with a bad one. Association of a SAID did not seem to influence the hearing-related prognosis. Positivity of ANA antibodies may justify performing a complementary investigation to determine the presence of a SAID.