Sensorineural Loss Research Articles

Incidence of Hearing Loss in Patients With Neurofibromatosis Type 1 at a Tertiary Care Pediatric Hospital

BackgroundHearing loss has not been thoroughly investigated as a comorbidity in larger cohorts with neurofibromatosis type 1 (NF1). MethodsAvailable audiometric data were reviewed from patients with NF1 seen at a tertiary pediatric hospital to assess prevalence and risk factors for hearing loss. ResultsOf 1172 patients with NF1 seen between 2010 and 2022, 90 had available audiometric data and 48 of 90 patients (53%) had one or more audiogram revealing hearing loss. Those not referred to audiology were presumed to have normal hearing, resulting in a conservative hearing loss estimate of 4% for children and young adults with NF1. Of 90 patients with audiograms, 29 (32%) had conductive loss (CHL), 15 (17%) had sensorineural loss (SNHL), and 3 (3%) had mixed hearing loss. Hearing loss type was undetermined for one patient. For children with CHL, six had permanent CHL secondary to plexiform neurofibroma, 19 CHL were transient due to active middle ear dysfunction, and four CHL cases were indeterminate in etiology. For three children with SNHL or mixed hearing loss, etiology included history of ototoxic chemotherapy and/or family history of SNHL. In the 16 patients with SNHL or mixed hearing loss with more than one audiogram over time, progressive hearing decline was noted in eight of 16, and 26 of 178 hearing thresholds (15%) progressed. ConclusionsOur findings suggest that audiometric evaluations should be considered for at least a subset of children with NF1, given the higher-than-expected rate of hearing loss in patients with NF1 compared with the general population.

Peripheral Auditory Pathway and ABR Characterization in Adults with Williams Syndrome.

Introduction Williams syndrome (WS) is a genetic disorder caused by a microdeletion in chromosome 7, affecting ∼ 28 genes. Studies have demonstrated conductive losses seemingly related to the absence of the elastin gene and mild to profound sensorineural losses due to cochlear fragility. Objective To characterize and compare the peripheral auditory system and auditory brainstem response (ABR) of adults with WS and neurotypical adults matched by age and gender. Methods We conducted a cross-sectional observational study with 30 individuals of both sexes, aged 18 to 37 years - 15 of them with WS (study group) and 15 with neither the syndrome nor hearing complaints (control group), matched for sex and age. The subjects underwent pure-tone and speech audiometry, acoustic immittance, transient-evoked otoacoustic emissions (TEOAEs), and ABR. Results Early-onset sensorineural hearing loss was found in 53.3% of the study sample, mostly mild, occurring above 3 kHz. The TEOAEs were absent in 53.3% of assessed subjects; for those in whom they were present, the signal-to-noise responses were significantly lower than in the control group. In the ABR, increased absolute latencies were observed in waves I and III. Conclusion Individuals with WS have early and progressive cochlear impairments, mainly affecting the basal region of the cochlea. They may have low brainstem changes which seem to begin in adulthood.

Human Cytomegalovirus Infection and Neurocognitive and Neuropsychiatric Health.

A common infection, human cytomegalovirus (HCMV) has been associated with a variety of human diseases, including cardiovascular disease and possibly certain cancers. HCMV has also been associated with cognitive, psychiatric, and neurological conditions. Children with congenital or early-life HCMV are at risk for microcephaly, cerebral palsy, and sensorineural hearing loss, although in many cases sensorineural loss may resolve. In addition, HCMV can be associated with neurodevelopmental impairment, which may improve with time. In young, middle-aged, and older adults, HCMV has been adversely associated with cognitive function in some but not in all studies. Research has linked HCMV to Alzheimer's and vascular dementia, but again not all findings consistently support these associations. In addition, HCMV has been associated with depressive disorder, bipolar disorder, anxiety, and autism-spectrum disorder, although the available findings are likewise inconsistent. Given associations between HCMV and a variety of neurocognitive and neuropsychiatric disorders, additional research investigating reasons for the considerable inconsistencies in the currently available findings is needed. Additional meta-analyses and more longitudinal studies are needed as well. Research into the effects of antiviral medication on cognitive and neurological outcomes and continued efforts in vaccine development have potential to lower the neurocognitive, neuropsychiatric, and neurological burden of HCMV infection.

A Large-Scale Study of the Relationship Between Degree and Type of Hearing Loss and Recognition of Speech in Quiet and Noise.

Understanding speech in noise (SIN) is the dominant complaint of individuals with hearing loss. For decades, the default test of speech perception in routine audiologic assessment has been monosyllabic word recognition in quiet (WRQ), which does not directly address patient concerns, leading some to advocate that measures of SIN should be integrated into routine practice. However, very little is known with regard to how SIN abilities are affected by different types of hearing loss. Here, we examine performance on clinical measures of WRQ and SIN in a large patient base consisting of a variety of hearing loss types, including conductive (CHL), mixed (MHL), and sensorineural (SNHL) losses. In a retrospective study, we examined data from 5593 patients (51% female) who underwent audiometric assessment at the Stanford Ear Institute. All individuals completed pure-tone audiometry, and speech perception testing of monaural WRQ, and monaural QuickSIN. Patient ages ranged from 18 to 104 years (average = 57). The average age in years for the different classifications of hearing loss was 51.1 (NH), 48.5 (CHL), 64.2 (MHL), and 68.5 (SNHL), respectively. Generalized linear mixed-effect models and quartile regression were used to determine the relationship between hearing loss type and severity for the different speech-recognition outcome measures. Patients with CHL had similar performance to patients with normal hearing on both WRQ and QuickSIN, regardless of the hearing loss severity. In patients with MHL or SNHL, WRQ scores remained largely excellent with increasing hearing loss until the loss was moderately severe or worse. In contrast, QuickSIN signal to noise ratio (SNR) losses showed an orderly systematic decrease as the degree of hearing loss became more severe. This effect scaled with the data, with threshold-QuickSIN relationships absent for CHL, and becoming increasingly stronger for MHL and strongest in patients with SNHL. However, the variability in these data suggests that only 57% of the variance in WRQ scores, and 50% of the variance in QuickSIN SNR losses, could be accounted for by the audiometric thresholds. Patients who would not be differentiated by WRQ scores are shown to be potentially differentiable by SIN scores. In this data set, conductive hearing loss had little effect on WRQ scores or QuickSIN SNR losses. However, for patients with MHL or SNHL, speech perception abilities decreased as the severity of the hearing loss increased. In these data, QuickSIN SNR losses showed deficits in performance with degrees of hearing loss that yielded largely excellent WRQ scores. However, the considerable variability in the data suggests that even after classifying patients according to their type of hearing loss, hearing thresholds only account for a portion of the variance in speech perception abilities, particularly in noise. These results are consistent with the idea that variables such as cochlear health and aging add explanatory power over audibility alone.

Audiologic Consequences of Gunshot Wounds to the Temporal Bone Show No Relation to Concomitant Neurologic or Vascular Injuries.

To evaluate audiologic consequences of gunshot wounds (GSWs) to the temporal bone (TB), and to correlate hearing outcomes with neurologic and vascular injuries adjacent to the temporal bone. Retrospective case series. University-based level-one trauma center. Retrospective review of 35 patients surviving TB ballistic injury, 2012 to 2021. Main outcomes were audiologic results. Demographics, concomitant injuries, CT, and interventions were reviewed. Mean age was 30.7 years; 80% male. Seventeen patients (48.6%) underwent audiologic testing. Mean pure tone average (PTA) was 75 ± 35 dB, bone line average 41 ± 26 dB, and speech discrimination score (SDS) 60 ± 43%. Nineteen (54.3%) demonstrated facial nerve injury (FNI), who were more likely to show SNHL especially anacusis, though their mean PTA and SDS were not statistically different from those without (P = .30 and .47, respectively). Radiographic review of those with sensorineural loss (SNHL, 6/17) revealed otic capsule-disrupting fracture (n = 2), pneumolabyrinth (n = 2), intracranial hemorrhage (n = 3). Those with mixed loss (6/17) showed otic capsule-sparing fracture (n = 6), EAC injury (n = 5), ossicular discontinuity (n = 2), and intracranial hemorrhage (n = 4). Two with mastoid tip fractures alone had normal audiograms. Audiometric outcomes were not predicted by concomitant CSF leak, spinal injuries, vascular injuries, cranial neuropathies, or traumatic brain injury. All patterns of hearing loss-conductive, sensorineural, mixed and normal-may be seen following TB ballistic injuries. Trauma severe enough to disrupt the facial nerve is more likely to cause anacusis. However, all should be formally evaluated, since ballistic injuries complicated by neurologic or vascular damage do not necessarily correlate with worse audiologic outcomes, while patients with minimal fractures may demonstrate losses.

E016 Erosive psoriatic arthritis causing deafness

Abstract Background/Aims Hearing loss from systemic psoriatic disease is rare, and cases reported in the literature have been reported with sensorineural deficits. Conductive hearing loss due to psoriatic arthritis has not been specifically reported in the literature, and this is believed to be the first reported case of erosive psoriatic arthritis causing conductive hearing loss. Presented is an illustrated case of a patient complaining of worsening bilateral hearing loss who underwent investigations under otolaryngology and audiology, and subsequent CT imaging which identified erosion of the temporal bone, posterior to the left temporomandibular joint, resulting in subluxation into the external auditory canal leading to complete occlusion of the canal. Methods 58yo female with psoriatic arthritis, psoriasis, and Raynaud’s phenomenon presenting with an 18-month history of hearing loss, worst on the left, but with some improvement on mouth opening. Treatment of her psoriatic arthritis has been with Ixekizumab since July 2021 with good overall disease control. Previous DMARD therapy includes methotrexate, sulfasalazine, and hydroxychloroquine, and previous biologic therapy of adalimumab and secukinumab. She had undergone bilateral reshaping of the mandibular condyle & fossa 20 years ago for presumed degenerative arthropathy that was non-responsive to conservative management; further details of the surgery will be presented. Results Patient was referred to audiology and otolaryngology who noted significant narrowing of both ear canals on examination. Audiological investigations revealed a right sided sensorineural hearing loss, and mixed pattern of hearing loss on the left. The patient proceeded to have skull vault imaging to assess for a structural cause of her left sided hearing loss. The finding of the CT petrous bones revealed soft tissue swelling associated with erosion of the anterior wall of the temporal and mandibular bones, causing subluxation and mass effect into the external auditory canal with no other cause for left-sided hearing loss demonstrated. Interestingly her hearing improved slightly with jaw opening and dynamic MRI may be indicated if surgical intervention is an option. Conclusion Our patient has a mixed hearing loss. The specific cause of the sensorineural component in our patient is not known but sensorineural loss has been reported in patients with psoriatic disease. The pathogenesis of psoriasis associated sensorineural hearing loss remains unclear however emerging evidence may implicate the downregulation of gap junction beta-2 protein encoding connexin 26 an integral cell signalling gap protein. We believe this is the first reported case where conductive hearing loss has resulted from a direct result of psoriatic arthritis from erosive temporomandibular joint disease leading to mass effect on the audio vestibular anatomy. It is a stark reminder of the consequences of erosive disease and the importance of surveillance of temporomandibular joint function in psoriatic arthritis patients given this rare but potential complication. Disclosure A.K. Coulton: None. W.R. Gubbins: None. S. Young-Min: None.

Clinical and Imaging Evaluation of COVID-19-Related Olfactory Dysfunction.

Olfactory dysfunction has been reported in 47.85% of COVID patients. It can be broadly categorized into conductive or sensorineural olfactory loss. Conductive loss occurs due to impaired nasal air flow, while sensorineural loss implies dysfunction of the olfactory epithelium or central olfactory pathways. The aim of this study was to analyze the clinical and imaging findings in patients with COVID-related olfactory dysfunction. Additionally, the study aimed to investigate the possible mechanisms of COVID-related olfactory dysfunction. The study included 110 patients with post-COVID-19 olfactory dysfunction, and a control group of 50 COVID-negative subjects with normal olfactory function. Endoscopic nasal examination was performed for all participants with special focus on the olfactory cleft. Smell testing was performed for all participants by using a smell diskettes test. Olfactory pathway magnetic resonance imaging (MRI) was done to assess the condition of the olfactory cleft and the dimensions and volume of the olfactory bulb. Olfactory dysfunction was not associated with nasal symptoms in 51.8% of patients. MRI showed significantly increased olfactory bulb dimensions and volume competed to controls. Additionally, it revealed olfactory cleft edema in 57.3% of patients. On the other hand, radiological evidence of sinusitis was detected in only 15.5% of patients. The average olfactory bulb volumes were significantly higher in the patients' group compared to the control group, indicating significant edema and swelling in the olfactory bulb in patients with COVID-related olfactory dysfunction. Furthermore, in most patients, no sinonasal symptoms such as nasal congestion or rhinorrhea were reported, and similarly, no radiological evidence of sinusitis was detected. Consequently, the most probable mechanism of COVID-related olfactory dysfunction is sensorineural loss through virus spread and damage to the olfactory epithelium and pathways.

Evaluation of the Effects of Dyslipidemia on Hearing in Patients with Type 2 Diabetes Mellitus.

Modernization has led to change in life style and physical inactivity which are playing a major role in increasing the number of people with diabetes and dyslipidaemia, Different types of hearing loss in diabetic patients have been reported which shows progressive, gradual, bilateral sensorineural loss. The primary aim of the present study is to evaluate the effects of dyslipidemia on hearing in patients with type 2 diabetes mellitus. A comparative study was conducted where patients were divided into 4 groups-Type II diabetes mellitus with dyslipidemia,Type II diabetes mellitus with normal lipid profile, Only Dyslipidemia,Normal subjects. A total of 128 participants were enrolled in the study. The Diabetes patient was determined by the FBS, PPBS and Hba1c levels. Dyslipidemia determined by LDL,HDL,VLDL.Patients with Type 2 diabetes mellitus and dyslipidaemia was evaluated for the presence of hearing loss with the help of PTA. Prevalence of hearing loss among patients with Diabetes and Dyslipidaemia was 65.7%, 40.6% in Type II DM with normal lipid profile patients, 18.75% in patients with dyslipidaemia. Statistically significant association of hearing loss was noted in patients with diabetes mellitus and dyslipidaemia. Although hearing loss is multifactorial, control of the risk factors like dyslipidaemia in diabetes mellitus definitely halt the process of auditory damage. As observed from this study, poor glycaemic control, associated other co-morbidities were contributing factors for hearing loss. Healthy life style with early recognition of these diseases helps in preventing further damage.

Olfaction Now and in the Future in CRSwNP.

Chronic rhinosinusitis (CRS) is the leading cause of olfactory dysfunction in the general population. Olfactory dysfunction is more common in patients with CRS with nasal polyposis (CRSwNP) compared to those without polyps. The present review aims to summarize the current literature on the mechanism behind olfactory dysfunction in CRSwNP and the impact of therapy on olfactory outcomes in this patient population. A comprehensive review of the available literature on olfaction in CRSwNP was performed. We evaluated the most recent evidence from studies on the mechanisms behind smell loss in CRSwNP and the impact of medical and surgical therapy for CRS on olfactory outcomes. The mechanism behind olfactory dysfunction in CRSwNP is not completely understood, but evidence from clinical research and animal models suggests both an obstructive component causing conductive olfactory loss and an inflammatory response in the olfactory cleft leading to sensorineural olfactory loss. Oral steroids and endoscopic sinus surgery have both shown efficacy in improving olfactory outcomes in CRSwNP in the short term; however, the long-term response of these treatments remains uncertain. Newer targeted biologic therapies, such as dupilumab, have also shown remarkable and durable improvement in smell loss for CRSwNP patients. Olfactory dysfunction is highly prevalent in the CRSwNP population. Although significant advances have been made in our understanding of olfactory dysfunction in the setting of CRS, additional studies are needed to elucidate cellular and molecular changes mediated by type 2-mediated inflammation in the olfactory epithelium with potential downstream effects on the central olfactory system. Further identification of these underlying basic mechanisms will be vital for developing future therapies targeted to improve olfactory dysfunction in patients with CRSwNP.

Sensorineural hearing loss in chronic suppurative otitis media - tubotympanic disease : A retrospective study.

Introduction: Hearing loss in patients with tubotympanic type of chronic suppurative otitis media is classically observed as conductive deafness. However, a concomitant presence of sensorineural hearing loss can be seen in some cases.The factors contributing to the development of this type of loss needs to be evaluated. Aim of the study: To study the incidence of sensorineural loss in patients with chronic suppurative otitis media tubotympanic disease and to assess the possible associated factors. Materials and methods:322 patients with chronic suppurative otitis media- tubotympanic disease admitted in a tertiary care centre over a period of five years. Clinical evaluation sheet, discharge summary and audiological evaluation sheet were reviewed to collect the data according to the proforma and analyzed statistically after entering in SPSS software. Data was collected regarding clinical presentation, audiological evaluation and otomicroscopy findings. Results: Out of 319 cases with hearing loss, a significant number of patients (67 patients;20.8%) had mixed hearing loss. There was statistically significant correlation between the type of hearing loss and size of perforation (p value – 0.039). 42 out of 67 cases with mixed loss had large perforations.The pattern of hearing loss showed variation with the site of perforation with a significant difference in incidence between purely anterior and purely posterior perforations (p value – 0.035). Conclusion:The statistical analysis of the data collected showed a significant incidence of sensorineural loss in tubotympanic disease and it was mainly associated with the site as well as increasing size of the perforation.

Considering gene therapy to protect from X-linked deafness DFNX2 and associated neurodevelopmental disorders.

Mutations and deletions in the gene or upstream of the gene encoding the POU3F4 transcription factor cause X-linked progressive deafness DFNX2 and additional neurodevelopmental disorders in humans. Hearing loss can be purely sensorineural or mixed, that is, with both conductive and sensorineural components. Affected males show anatomical abnormalities of the inner ear, which are jointly defined as incomplete partition type III. Current approaches to improve hearing and speech skills of DFNX2 patients do not seem to be fully effective. Owing to inner ear malformations, cochlear implantation is surgically difficult and may predispose towards severe complications. Even in cases where implantation is safely performed, hearing and speech outcomes remain highly variable among patients. Mouse models for DFNX2 deafness revealed that sensorineural loss could arise from a dysfunction of spiral ligament fibrocytes in the lateral wall of the cochlea, which leads to reduced endocochlear potential. Highly positive endocochlear potential is critical for sensory hair cell mechanotransduction and hearing. In this context, here, we propose to develop a therapeutic approach in male Pou3f4 -/y mice based on an adeno-associated viral (AAV) vector-mediated gene transfer in cochlear spiral ligament fibrocytes. Among a broad range of AAV vectors, AAV7 was found to show a strong tropism for the spiral ligament. Thus, we suggest that an AAV7-mediated delivery of Pou3f4 complementary DNA in the spiral ligament of Pou3f4 -/y mice could represent an attractive strategy to prevent fibrocyte degeneration and to restore normal cochlear functions and properties, including a positive endocochlear potential, before hearing loss progresses to profound deafness.

Neural signatures of auditory hypersensitivity following acoustic trauma.

Neurons in sensory cortex exhibit a remarkable capacity to maintain stable firing rates despite large fluctuations in afferent activity levels. However, sudden peripheral deafferentation in adulthood can trigger an excessive, non-homeostatic cortical compensatory response that may underlie perceptual disorders including sensory hypersensitivity, phantom limb pain, and tinnitus. Here, we show that mice with noise-induced damage of the high-frequency cochlear base were behaviorally hypersensitive to spared mid-frequency tones and to direct optogenetic stimulation of auditory thalamocortical neurons. Chronic two-photon calcium imaging from ACtx pyramidal neurons (PyrNs) revealed an initial stage of spatially diffuse hyperactivity, hyper-correlation, and auditory hyperresponsivity that consolidated around deafferented map regions three or more days after acoustic trauma. Deafferented PyrN ensembles also displayed hypersensitive decoding of spared mid-frequency tones that mirrored behavioral hypersensitivity, suggesting that non-homeostatic regulation of cortical sound intensity coding following sensorineural loss may be an underlying source of auditory hypersensitivity. Excess cortical response gain after acoustic trauma was expressed heterogeneously among individual PyrNs, yet 40% of this variability could be accounted for by each cell's baseline response properties prior to acoustic trauma. PyrNs with initially high spontaneous activity and gradual monotonic intensity growth functions were more likely to exhibit non-homeostatic excess gain after acoustic trauma. This suggests that while cortical gain changes are triggered by reduced bottom-up afferent input, their subsequent stabilization is also shaped by their local circuit milieu, where indicators of reduced inhibition can presage pathological hyperactivity following sensorineural hearing loss.

An Overview of Nanoparticle Drug Delivery for Ototoxin and Noise Mediated Hearing Loss Treatment

Abstract: Efficient delivery of therapeutic material to target site of the inner ear is considered as the most challenging process due to their poor blood flow and inaccessibility sensitivity towards chemical stimuli, inability of drug to be delivered as well. Novel nanoparticlebased drug transport approaches have emerged for overcoming the restriction associated with inner ear therapeutic material delivery. The main focus of this article is to highlight the potential benefits, pre-clinical level otoprotective effect of different types of NPs based payload delivery strategies in mitigating sensorineural hearing impairment resulting from medication (cisplatin and antibiotic treatment) and noise exposure. This review converses about advantage of nanocarrier assisted targeted cell specific inner ear drug delivery approach and demonstrates that targeted NPs delivery systems have the capability to be utilized as vehicle to transport therapeutic materials into the OHCs within the cochlea in controlled and sustained way and maximize following therapeutic effects. Efficacy of systemic and minimally invasive intratympanic and RWM approach for delivering steroids/ genetic material to the inner ear has also been compared in this article. Understanding the importance (design, formulation, cochlear biocompatibility and cell specific binding efficiency of NPs, mechanistic pathways, route of delivery, positive therapeutic outcomes) and hitches (adverse effects after treatment, negative therapeutic outcome) of currently available NPs based drug delivery systems offer new opportunity to develop best treatment methods for sensorineural hearing dysfunction. Keywords: Nanocarriers, Otoprotective agents, Sensorineural heating loss, Ototoxicity, Noise trauma, Ototoxin.

Sleep, Sensory Integration/Processing, and Autism: A Scoping Review.

The prevalence of sleep dysfunction is considerably higher in the autistic population than in the non-autistic. Similarly, the incidence of sensory reactivity differences in autism exceeds that in the neurotypical population. The basis of sleep disorders in autism is multifactorial, but sensory integration/processing concerns may play a role. Research that investigates this interplay for autistic individuals is limited but vital. In this scoping review, we examined literature addressing the following research question: What is the relationship between sleep and sensory integration/processing in autism? We included articles if they were peer-reviewed, English or Spanish, purposefully addressed sensory integration/processing differences, were sleep focused and included autism as the primary diagnosis or population. Articles were excluded if the language was not English or Spanish, research was conducted with animals, they were non-peer-reviewed, the primary population was not autistic, the sensory focus reflected a specific sensorineural loss (e.g., blindness, or deafness), there was not a clear inclusion of sensory integration/processing or sleep. We searched six databases and included all citations from the inception of each database through June 2021. The search strategy identified 397 documents that were reduced to 24 included articles after exclusion criteria were applied. The majority of studies we identified characterized the relation between sleep and sensory integration/processing differences in autism. Investigators found multiple sleep concerns such as bedtime resistance, sleep anxiety, delayed sleep onset, night awaking, and short sleep duration in autistic individuals. Identified sensory concerns focused on reactivity, finding hyper- and hypo-reactivity as well as sensory seeking across sensory domains. Co-existence of sleep concerns and sensory integration/processing differences was frequently reported. Few intervention studies showed a clear sensory focus; those that did emphasized pressure, movement, touch, and individual sensory preferences/needs. Swimming programs and massage showed promising results. No studies were of high quality. At a minimum, there is a co-existence of sensory reactivity differences and sleep concerns in autistic children, and possibly autistic adults. The relationship between poor sleep and sensory integration/processing differences is complex and multi-faceted, requiring additional research. Interventions that purposefully include a central sensory component have not been well studied in autistic children or adults. Overall studies with greater rigor and purposeful use of sensation and sensorimotor supports as a component of intervention are needed. This study was not funded.

A Newborn with an Incidental Finding on Cranial Ultrasonography.

A Newborn with an Incidental Finding on Cranial Ultrasonography.

Assessment of Frequencies of Physiological Imperilments as Consequence of Burns due to Domestic Violence in a Tertiary Care Hospital

Background: There is no impact without an imprint. Such an imprint can range from a simple scar to functio laesa of an organ. The burns are much more notorious than any other sort of trauma/impact. Most common physiological imperilments of burns can be permanent scarring, disfigurement, mobility restrictions, loss of sensations and permanent pain etc. The frequency of all these imperilments is described in the current research. Methodology: The study was carried out in year 2018 from January to September in AED of Jinnah Hospital Lahore. There were 300 surviving victims of burns of domestic violence collected through non-probability consecutive purposive sampling for a descriptive cross-sectional study. Results: The study revealed the permanent pain and discomfort as the most frequent physiological imperilments reported to be 27% followed by mobility restrictions occurring in 23% third commonest was the sensorineural loss observed in 17% approximately among all the cases participating in the current study. Conclusion: We concluded escaping the burns incident uneventfully may be possible if the burns were minor or medical care services were up to the par and delivered well in time. Keywords: Physiological Imperilments, Domestic, Burns

DIAGNOSTIC IMPORTANCE OF THE CARHART NOTCH IN PATIENTS WITH CHRONIC OTITIS MEDIA

Objective
 The false sensorineural loss caused by otosclerosis
 due to stapes fixation is known as the Carhart notch.
 To examine the changes in air-bone gap (ABG)
 and bone-conduction (BC) hearing thresholds by
 comparing the preoperative and postoperative
 audiometry results in patients with chronic otitis media
 (COM), to investigate the effects of air conduction
 (AC) on BC, to determine the effects of postoperative
 improvement in AC on BC, to detect the presence
 of the Carhart notch in COM, and to determine the
 effects of surgery on the Carhart notch.
 Material and Methods
 A total of 104 patients who underwent type 1
 tympnoplasty between January 2012 - March 2017
 in our clinic were included in this retrospective study.
 Parameters measured during the preoperative and
 postoperative sixth month audiometric evaluation
 comprised AC hearing thresholds at the frequencies
 of 250-8,000 Hz, BC hearing thresholds at 500-4,000
 Hz, and ABG values at 500-4,000 Hz intervals.
 Results
 Before surgery, the Carhart notch was present in
 46 (44.2%) of the 104 patients. Postoperatively, the
 Carhart notch was observed to have been corrected
 in 25 (54.3%) of these patients (p=0.029).
 Conclusion
 After tympanoplasty, significant improvement in ABG
 may lead to improvement in BC at 2,000 Hz in COM
 cases with an intact and mobile ossicular chain. The
 Carhart notch may also be present in COM.

A Systematic Review of Auditory Loss and Its Management

Auditory loss is a highly under recognised undermined issue which needs to be studied in extensive detail, as according to World Health Organisation, more than 45% of the global population has loss of auditory. The high percentage can be lowered if auditory enhancement techniques such as auditory aids or implants of cochlea were not underused. In order to increase the usage of these devices we must first understand the inner workings of the ear and the pathologies that may cause it to malfunction and then move on to proper management. Auditory loss as defined by WHO as the inability to hear as well as someone with normal auditory, these are persons having auditory thresholds of 20 dB or better than 20 dB in both ears. Auditory loss is often classified into two types, conductive auditory loss and sensorineural auditory loss which is a broad classification into which we can separate the various pathologies which bring about same outcomes. In conductive auditory loss, there is often involvement of external or middle ear where there is obstruction in relay of sound waves, hence, quite literally a problem with the conduction. Conductive auditory loss can be both congenital or acquired but most often can be resolved in a manner which will result in recovery of auditory. Sensorineural auditory loss occurs when there is dysfunction in the inner ear which often leads to progressive and permanent auditory loss. Though it can be congenital, it is most often acquired, especially when it is age related more commonly known as presbycusis which affects a large amount of the aging population. The management can be done through various processes including oral medications, surgical interventions and other manual procedures. Various assistive technologies like auditory aids and cochlear implants are also vital in preservation of auditory.

Hearing impairment in patients of renal failure

<p class="abstract"><strong>Background:</strong> Kidney diseases are emerging public health problems in developing countries. A common complication of renal failure is sensorineural hearing loss which is leading to poor quality of life. The aim of the study was to determine the prevalence of hearing loss in renal failure patients and to see the correlation of dialysis, ototoxic drugs, and creatinine levels.</p><p class="abstract"><strong>Methods:</strong> This observational study was conducted at the department of otorhinolaryngology, tertiary care center, Jabalpur (Madhya Pradesh) for a period of 18 months i.e.; from March 2019 to August 2020. 70 patients with renal failure were registered for study. In all patients hematological tests include hemoglobin, urea, creatinine, random blood sugar, serum electrolytes and pure tone audiometry was done. </p><p class="abstract"><strong>Results:</strong> Out of 70 patients, 51 patients (72.9%) of renal failure had sensorineural hearing loss. Hypertension and diabetes mellitus as comorbidity play important role in hearing loss in renal failure patients. Acoustic reflex was absent in 14.3% of cases which signifying a profound hearing loss and these patients had creatinine level above 6 mg/dl.</p><p class="abstract"><strong>Conclusions:</strong> Senorineural hearing loss is more prevalent in renal failure patients. Significant association was present between raised creatinine level and sensorineural hearings loss. Diabetes mellitus and hypertension were the common comorbidities that have a significant role in hearing loss in renal failure patients. All patients having renal failure have a risk of developing sensorineural hearing loss. So, these patients should be kept under follow up by doing regular pure tone audiometry and taking preventive measures, so that the hearing loss doesn't occur.</p>

A pathogenic deletion in Forkhead Box L1 (FOXL1) identifies the first otosclerosis (OTSC) gene

Otosclerosis is a bone disorder of the otic capsule and common form of late-onset hearing impairment. Considered a complex disease, little is known about its pathogenesis. Over the past 20 years, ten autosomal dominant loci (OTSC1-10) have been mapped but no genes identified. Herein, we map a new OTSC locus to a 9.96 Mb region within the FOX gene cluster on 16q24.1 and identify a 15 bp coding deletion in Forkhead Box L1 co-segregating with otosclerosis in a Caucasian family. Pre-operative phenotype ranges from moderate to severe hearing loss to profound sensorineural loss requiring a cochlear implant. Mutant FOXL1 is both transcribed and translated and correctly locates to the cell nucleus. However, the deletion of 5 residues in the C-terminus of mutant FOXL1 causes a complete loss of transcriptional activity due to loss of secondary (alpha helix) structure. FOXL1 (rs764026385) was identified in a second unrelated case on a shared background. We conclude that FOXL1 (rs764026385) is pathogenic and causes autosomal dominant otosclerosis and propose a key inhibitory role for wildtype Foxl1 in bone remodelling in the otic capsule. New insights into the molecular pathology of otosclerosis from this study provide molecular targets for non-invasive therapeutic interventions.