Significant Sensorineural Hearing Loss Research Articles

Single-Sided Deafness and Hearing Rehabilitation Modalities: Contralateral Routing of Signal Devices, Bone Conduction Devices, and Cochlear Implants.

Single sided deafness (SSD) is characterized by significant sensorineural hearing loss, severe or profound, in only one ear. SSD adversely affects various aspects of auditory perception, including causing impairment in sound localization, difficulties with speech comprehension in noisy environments, and decreased spatial awareness, resulting in a significant decline in overall quality of life (QoL). Several treatment options are available for SSD, including cochlear implants (CI), contralateral routing of signal (CROS), and bone conduction devices (BCD). The lack of consensus on outcome domains and measurement tools complicates treatment comparisons and decision-making. This narrative overview aims to summarize the treatment options available for SSD in adult and pediatric populations, discussing their respective advantages and disadvantages. Rerouting devices (CROS and BCD) attenuate the effects of head shadow and improve sound awareness and signal-to-noise ratio in the affected ear; however, they cannot restore binaural hearing. CROS devices, being non-implantable, are the least invasive option. Cochlear implantation is the only strategy that can restore binaural hearing, delivering significant improvements in speech perception, spatial localization, tinnitus control, and overall QoL. Comprehensive preoperative counseling, including a discussion of alternative technologies, implications of no treatment, expectations, and auditory training, is critical to optimizing therapeutic outcomes.

Hearing assessment and treatment outcome in ENT tuberculosis at a tertiary hospital in India

AimAn attempt has been made to observe the response of anti-Koch’s treatment and the effect of anti-Koch’s treatment on hearing in ENT tuberculosis patients by pure tone audiometry.Material and methodsA prospective observational study was carried out at the Government Medical College Hospital. A total of 200 cases diagnosed with tuberculosis in the head and neck regions were included in the study. Sputum/pus of patients were sent for cartridge-based nucleic acid amplification test (CBNAAT). Hearing assessment was done with PTA1 at the start of the treatment, PTA2 after the intensive phase (IP) at 2 months for newly diagnosed patients and at 3 months for previously treated patients, and PTA3 at the end of the AKT treatment given as per guidelines.ResultsAmong 200 ENT tuberculosis patients, 176 patients were cured with a new case regimen, and one patient was a defaulter. Among 19 patients who were previously treated cases (CAT II regimen), two patients had treatment failure, and the other 17 patients were cured. Four patients diagnosed to have multidrug-resistant tuberculosis (MDR-TB) on the initial drug susceptibility testing (DST) were given 24 months’ treatment according to the recent guidelines of MDR-TB treatment regimen, and the patients were cured. In the present study of 200 patients, in the first PTA, 192 patients had normal hearing; in the second PTA, 106 patients had normal hearing; and in the third PTA, 35 patients had normal hearing. 21.7% of cases who were on aminoglycoside therapy developed significant sensorineural hearing loss in high frequency.ConclusionAnti-Koch’s treatment (AKT) has proven effective in ENT tuberculosis. All patients taking anti-Koch’s treatment should be regularly monitored for hearing loss, adherence to treatment, drug-resistant variants, and local recurrences.

Assessment of Hearing Impairment in Sick Newborns: A Prospective Observational Study.

Undiagnosed neonatal hearing loss causes severe language, cognitive, and behavioral problems in children. Sick newborns who spend 48 hours in the neonatal intensive care unit (NICU) have a 10- to 20-fold increased risk of permanent hearing loss.The aim of this study is to identify hearing impairment in high-risk sick newborns who spend at least 48 hours in the NICU. The present prospective observational study was conducted at a single center.All sick neonates admitted to the NICU for a minimum of 48 hours at the JK Lon Hospital, Zanana Hospital, and Mahila Chikitsalaya, Sawai Man Singh (SMS) Medical College, Jaipur,Rajasthan, India, from June 2017 to May 2018 were considered eligible for the study.The primary outcome was the assessment of hearing loss of high-risk newborns using otoacoustic emissions at the time of discharge, six weeks of age, and automated auditory brainstem response (AABR) at three months of chronological age. The secondary outcome was to analyze the association of hearing loss with different risk factors in high-risk neonates. A total of 150 infants who had one or more risk factors were studied, 60 were female and 90 were male. No statistically significant difference in hearing loss was observed based on birth weight, sex, and gestational age.The firstdistortion-product otoacoustic emission(DPOAE) screening was done on infants at the time of discharge from the NICU. Eighty-three infants (55.33%) had "refer" on the first DPOAE and the remaining 67 (44.67%) were passed results at the time of discharge.At six weeks of life, on repeat screening with a second DPOAE test, 36% "refer"on the first screen had a"refer"result on the second DPOAE. However, 4.4% "pass" on the first screen turned out to be"refer"on the second screen. These 33 infants who had"refer"results on the second screen were subjected to testing.At 10 weeks of life, AABR wasperformedon 33 infants. Eleven infants out of 33 had sensorineural hearing loss (SNHL) on AABR. Hearing impairment with the DPOAE test was observed with risk factors neonatal hyperbilirubinemia (NHH), hypoxic ischemic encephalopathy (HIE), and very low birth weight (VLBW) and was statistically significant among all risk factors.But, no such association (between hearing impairment and risk factors) was observed with the AABR test. In our study, we found that the duration of mechanical ventilation in mean days 7.67±6.24 had statistically significant SNHL compared to the lesser duration of mechanical ventilation (p<0.001). Two-stage DPOAEdone prior to AABR is helpful in the early detection of hearing loss.

Peculiarities of the acoustic reflex registration thresholds in relation to the parameters of the thresholds tone audiometry in patients with acute combat trauma

Aim: study of threshold ratios between indicators of acoustic impedance and threshold tonal audiometry in military personnel – participants in hostilities. Materials and methods: the results of audiometric and impedancemetric examinations of 43 servicemen aged 18 to 42 years who received combat acute trauma in the period March-August 2022 and 20 patients with severe sensorineural deafness (2, 3, 4 stages according international classification) of non-acutraumatic genesis as a comparison group. Results and their discussion: During the examination of 43 soldiers, it was found that with significant sensorineural hearing loss on both sides (3 and 4 stages according international classification), the patients registered a full-fledged acoustic reflex during ipsilateral and contralateral stimulation. When conducting an impedanceometric examination of patients in the comparison group, it was found that in most patients the acoustic reflex was not registered at all. Conclusions: 1. In combat acute acoustic trauma, it is possible to register acoustic middle-ear-muscle reflex with severe hearing loss, which may be caused by damage to the receptor part of the auditory analyzer and the accompanying symptom of the phenomenon of accelerated loudness, by a central mechanism, or by a combination of central and peripheral mechanisms. 2. Dissociation of acoustic middle-ear-muscle reflex threshold values and threshold tonal audiometry is a characteristic feature of acutraumatic damage to the auditory analyzer. 3. Identified features, such as registration of acoustic middle-ear-muscle reflex in severe hearing loss, dissociation of acoustic reflex thresholds and tonal audiometry may be useful for further study of the pathogenesis of combat acute trauma, which, in turn, will contribute to improving the quality of diagnosis and finding ways to correct disorders of the auditory system in individuals, who suffered as a result of hostilities.

Is COVID-19 to Blame for Sensorineural Hearing Deterioration? A Pre/Post COVID-19 Hearing Evaluation Study.

Here, we aimed to (a) determine whether a clinically significant sensorineural hearing loss (SNHL) change could be detected in post-coronavirus disease (COVID-19) hearing levels on comparing them with pre-infection hearing levels after controlling for the effect of age and (b) to identify risk factors, such as hypertension, diabetes, and smoking, which increase the likelihood of hearing loss in COVID-19 patients. We retrospectively analyzed hearing thresholds in unvaccinated patient's pre- and post-COVID-19 infection. Thresholds were controlled for age and the duration between the pre- and post-COVID-19 hearing evaluations. Correlations between additional COVID-19-related symptoms, hypertension, diabetes, and smoking and hearing threshold changes were analyzed. A significant (but not clinical) threshold elevation was found post-COVID-19 infection. However, on controlling for age and the duration between the pre- and post-COVID-19 hearing evaluations, no significant threshold elevation was found. No significant correlation was found between hearing threshold changes and additional COVID-19-related symptoms, hypertension, diabetes, or smoking. COVID-19 did not lead to a significant hearing threshold elevation in our cohort, even among patients with additional COVID-19 symptoms, hypertension, or diabetes mellitus or among those who smoked. nonrandomized controlled cohort, follow-up study Laryngoscope, 133:1976-1981, 2023.

OTHR-03. Oxaliplatin as a hearing-sparing alternative to carboplatin in tandem autologous stem cell transplants in pediatric CNS malignancy

Abstract BACKGROUND: Intensive chemotherapy with tandem autologous stem cell transplants (autoSCT) is shown to improve survival for children with CNS malignancy. Platinum-based chemotherapeutic agents in these regimens, mainly cisplatin and carboplatin, have resulted in significant sensorineural hearing loss. Oxaliplatin, a newer platinum-based agent, is considered less ototoxic. Empiric substitution of oxaliplatin for carboplatin in preparative regimens for autoSCT have been tried. However, the survival and ototoxicity outcomes have not been studied. OBJECTIVE: To compare the overall survival and ototoxicity of oxaliplatin versus carboplatin preparatory chemotherapy regimens in children who received tandem autoSCT for treatment of CNS malignancy. METHODS: We performed a retrospective chart review of all pediatric patients with primary CNS tumors who received tandem autoSCT from 2011 to 2018 at Children’s Hospital Colorado. Demographics, clinical outcomes, and medication administration records were extracted from electronic medical records. Hearing evaluations, performed at pre-transplant, after each transplant episode, and at follow-up visits, were reviewed and graded by an audiologist. Comparisons were performed using Fisher’s exact tests and log rank test statistics. RESULTS: 32 pediatric patients with CNS tumors met inclusion criteria. Seven patients received oxaliplatin in place of carboplatin in one or more preparatory regimens. There was no statistically significant difference in overall survival between the two groups (p=0.99). A total of 85 follow-up audiograms were available for assessment, including long-term follow up. Of the 13 audiograms that showed hearing loss, one (8%) had prior oxaliplatin exposure, compared to 18/72 (25%) audiograms without hearing loss had prior oxaliplatin exposure (p=0.28). CONCLUSIONS: Oxaliplatin is effective and well-tolerated when used in lieu of carboplatin in preparatory regimen for autoSCT for pediatric CNS malignancy. This study is limited by its small size. A larger, multi-center study is warranted to confirm oxaliplatin’s safety and effect on survival and ototoxicity in pediatric autoHSCT.

Outcomes in primary and revision surgery for pediatric onset stapedial pathology

ObjectivesTo analyze audiometric outcomes of surgery for pediatric onset stapedial pathology (POSP). Study designRetrospective cohort study. SettingSingle-institution database. MethodsRetrospective analysis of 809 stapes procedures performed at a single high-volume tertiary referral otology practice, 75 of which were POSP cases. ResultsOval window drillout for thick footplate and aborting the procedure were more common in POSP cases compared to the rest of the cohort (28.0% versus 9.8% [p < .001] and 5.3% versus 1.2% [p = .007], respectively). Postoperative complications were rare. Postoperative Air-Bone Gap (pABG) closure to ≤20 dB was significantly lower in the POSP group (80.0% versus 89.0%, p = .021). Rates of sensorineural hearing loss (SNHL) were not different between the two groups. Poor audiometric outcomes in the POSP group were largely driven by revision cases; pABG≤10 dB was 60.3% in primary cases but only 11.8% in revisions (p < .001), and postoperative SNHL was significantly higher in revisions (29.4% versus 0.0%, p < .001). In multivariate analysis, POSP was not a predictor of successful closure of the pABG at either level, nor did it predict significant postoperative SNHL. ConclusionsSurgery for pediatric onset stapedial pathology had significantly worse audiometric outcomes, particularly in revision cases, as compared to the rest of the cohort.

Do Perioperative Systemic Corticosteroids Improve Cochlear Implant Hearing Preservation Outcomes?

Do Perioperative Systemic Corticosteroids Improve Cochlear Implant Hearing Preservation Outcomes?

Endoscopic Ossiculoplasty for the Management of Isolated Congenital Ossicular Chain Malformation: Surgical Results in 16 Ears

The aim of this study is to describe the clinical characteristics and intraoperative findings and further evaluate the efficacy of endoscopic ossiculoplasty for the management of isolated congenital ossicular chain malformation. A retrospective study was performed on 16 ears (15 patients) with the isolated congenital ossicular chain malformation who underwent endoscopic ossiculoplasty in our department from May 2017 to January 2019. Endoscopic exploratory tympanotomy was conducted to check the ossicular chain; at the same time, endoscopic ossiculoplasty was performed depending on intraoperative findings. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps (ABGs) were measured before and after surgery, and the hearing outcome was assessed at 6 months postoperatively. The most common malformations of ossicular chain were the missing of the incus long process and stapes suprastructure. A serial assessment of the hearing status was conducted before and 6 months after surgery. It showed the mean postoperative pure-tone average (PTA) was significantly reduced, and the mean postoperative ABG was obviously closed, respectively (P < .001). The mean PTA gain was 36.3 ± 8.6 dB, and the ABG closure was 35.1 ± 8.3 dB; ABG closure to 20 dB or less and ABG closure to 10 dB or less were achieved in 14 cases (87.5%) and 5 cases (31.3%), respectively. No differences were observed in postoperative hearing outcome between type Ⅲ cases and type Ⅳ cases; however, cases with partial ossicular replacement prosthesis implantation showed a larger hearing gain (P = .049) and a higher proportion of postoperative ABG less than 10 dB (P = .021). No facial palsy and significant sensorineural hearing loss occurred; all patients completed the surgery without the need of canalplasty, and the chorda tympani nerve was preserved in all patients. This research showed endoscopic surgery was effective in the diagnosis and management of isolated congenital ossicular chain malformation; the endoscopic ossiculoplasty provides an alternative method to manage congenital ossicular chain malformation, with comfortable hearing outcome and the advantage of excellent vision and less invasion.

Thiamine-responsive megaloblastic anemia syndrome: A case report

Thiamine-responsive megaloblastic anemia (TRMA) syndrome is a rare autosomal recessive disorder characterized by a cardinal triad consisting of megaloblastic anemia, sensorineural deafness, and diabetes mellitus. TRMA is caused by mutations in the gene SLC19A2 encoding a high-affinity thiamine transporter, which disturbs the active thiamine uptake into cells. We report here on a 1-year and 9-month-old female baby with megaloblastic anemia, thrombocytopenia, and diabetes mellitus. Our patient had significant sensorineural hearing loss that was late to appear. Diagnosis was based on clinical features and dramatic response of anemia, thrombocytopenia, and glycemic control to thiamine therapy. In view of the clinical history of the patient, targeted gene sequencing of genes causing monogenic diabetes was performed. The genes selected comprised 40 gene loci and were sequenced by Illumina sequencing platform. We found a novel homozygous deletion mutation of complete exon 2 of the SLC19A2 gene (ENS0236137), which we believe has not been described to be associated with TRMA. Exon 2 of SLC19A2 gene includes amino acid from 69 to 269. Thiamine resulted in rapid normalization of the hemoglobin level with improvement in glycemic control. TRMA syndrome should be kept in mind in the differential diagnosis of megaloblastic anemia, deafness, and diabetes mellitus. Early introduction of high-dose thiamine can reverse anemia and allow more glycemic control for diabetes. We conclude that genetic analysis confirms the diagnosis of TRMA. As exogenous thiamine is shown to reverse some of the clinical features of the disease, a genetic diagnosis of TRMA syndrome is extremely important.

Does Mucosal Chronic Otitis Media Leads to Sensorineural Hearing Loss.

Sensori-neural hearing loss (SNHL) results from inner ear damage or injury to the neural pathways that relay signals from the inner ear to the brain. A serious sequelae of COM is damage to the inner ear. This study aimed at finding the occurrence of SNHL in unilateral mucosal COM. One Hundred patients who had unilateral mucosal COM were enrolled in the study and underwent pure tone audiometry using Audio 4002 system in acoustically treated room. Bone conduction (BC) threshold less than 20dB was taken as normal. Significant SNHL of 23% was seen in mucosal COM. Unilateral mucosal COM leads to significant impact on BC thresholds. The risk of SNHL increases with larger perforation size and increasing duration and active stage of disease.

Sensorineural hearing loss in chronic otitis media: analysis of an overlooked entity!

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; In developing countries, hearing loss continues to be the major public health problem. Among the various causes of hearing loss, chronic otitis media (COM) is one of the commonest. In COM, the presence of sensorineural hearing loss is well established. Our study intends to determine the prevalence of sensorineural hearing loss in chronic otitis media.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; This was a cross sectional descriptive study of 151 subjects between the age group of 10 and 60 years. Patients having unilateral chronic otitis media of both mucosal and squamosal types were selected. All patients were evaluated clinically and audiologically. The type, duration of disease and type of perforation is correlated with degree of sensorineural hearing loss. Interaural differences in bone conduction thresholds at 500 Hz, 1 kHz, 2 kHz and 4 kHz were also noted. &lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results:&lt;/strong&gt; A statistically significant correlation was noted between type of disease and perforation with hearing loss. A significant interaural difference was noted across the speech frequencies. Sensorineural hearing loss of 19.1% was seen.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Significant sensorineural hearing loss is seen in chronic otitis media which was greater in squamosal disease. Statistically significant correlation was noted between type of disease and type of perforation with hearing loss.&lt;/p&gt;

EVALUATION OF SENSORINEURALHEARING LOSS IN TUBOTYMPANIC TYPE OF CHRONIC SUPPURATIVE OTITIS MEDIA

Background: &#x0D; &#x0D; To know the prevalence of sensorineural hearing loss among chronic suppurative otitis media patients&#x0D; To study the correlation between duration and clinical findings of CSOM with the degree of hearing loss.&#x0D; &#x0D; Materials and Methods &#x0D; Hospital based cross sectional study of patients aged between 10 to 45years with unilateral chronic suppurative otitis media attending Tirumala Super speciality Hospital, Kadapa from December 2015 to November 2017 with sample size of 100 cases were taken.&#x0D; Results: Out of the 100 cases, 10 patients were having sensorineural hearing loss. Thus the percentage is 10%. Out of the 10 cases of sensorineural hearing loss, no cases with sensorineural hearing loss between 10-15yr and 16-20yr age group. 2 were between in the age group of 26-30yr, 31-35yr and 36-40yr age group and 4 patients were in the age group of 41-45yr. Out of 100 patients 52 were males and 48 were females. Out of 10 patients with sensorineural hearing loss 6 were males and 4 were females. The duration of ear discharge ranged from 3 months to more than 15 years. Among the 100 patients studied 64 had ear discharge from 3months to 5yr. 16 had ear discharge from 6-10yr, 11 had ear discharge from 11-15yr and 9 had ear discharge of more than 15years.&#x0D; Conclusion: Our study shows presence of significant (10%) sensorineural hearing loss in patients with chronic suppurative otitis media.&#x0D; Duration of ear discharge correlates well with sensorineural hearing loss.&#x0D; No significant correlation between sex and sensorineural hearing loss.&#x0D; Keywords: Chronic suppurative otitis media, tubotympanic disease, sensorineural hearing loss.

Zebrafish Model for Nonsyndromic X-Linked Sensorineural Deafness, DFNX1.

Hereditary deafness is often a neurosensory disorder and affects the quality of life of humans. Only three X-linked genes (POU class 3 homeobox 4 (POU3F4), phosphoribosyl pyrophosphate synthetase 1 (PRPS1), and small muscle protein X-linked (SMPX)) are known to be involved in nonsyndromic hearing loss. Four PRPS1 missense mutations have been found to associate with X-linked nonsyndromic sensorineural deafness (DFNX1/DFN2) in humans. However, a causative relationship between PRPS1 mutations and hearing loss in humans has not been well studied in any animal model. Phosphoribosyl pyrophosphate synthetase 1 (PRS-I) is highly conserved in vertebrate taxa. In this study, we used the zebrafish as a model to investigate the auditory role of zebrafish orthologs (prps1a and prps1b) of the human PRPS1 gene with whole mount in situ hybridization, reverse transcription polymerase chain reaction, phenotypic screening, confocal imaging, and electrophysiological methods. We found that both prps1a and prps1b genes were expressed in the inner ear of zebrafish. Splice-blocking antisense morpholino oligonucleotides (MO1 and MO2) caused exon-2 skip and intron-2 retention of prps1a and exon-2 skip and intron-1 retention of prps1b to knock down functions of the genes, respectively. MO1 and MO2 morphants had smaller otic vesicles and otoliths, fewer inner ear hair cells, and lower microphonic response amplitude and sensitivity than control zebrafish. Therefore, knockdown of either prps1a or prps1b resulted in significant sensorineural hearing loss in zebrafish. We conclude that the prps1 genes are essential for hearing in zebrafish, which has the potential to help us understand the biology of human deafness DFNX1/DFN2. Anat Rec, 303:544-555, 2020. © 2019 American Association for Anatomy.

Cochlea CT radiomics predicts chemoradiotherapy induced sensorineural hearing loss in head and neck cancer patients: A machine learning and multi-variable modelling study

ObjectivesImmediately or after head-and-neck (H&N) cancer chemoradiotherapy (CRT), patients may undergone significant sensorineural hearing loss (SNHL) which could affect their quality of life. Radiomic feature analysis is proposed to predict SNHL induced by CRT. Material and methods490 image features of 94 cochlea from 47 patients treated with three dimensional conformal RT (3DCRT) for different H&N cancers were extracted from CT images. Different machine learning (ML) algorithms and also least absolute shrinkage and selection operator (LASSO) penalized logistic regression were implemented on radiomic features for feature selection, classification and prediction. Also, LASSO penalized logistic model was used for outcome modelling. ResultsThe predictive power of ten ML methods was more than 70% (in accuracy, precision and area under the curve of receiver operating characteristic curve (AUC)). According to the LASSO penalized logistic modelling, 10 of the 490 radiomic features selected as the associated features with SNHL status. All of the 10 features were statistically associated with SNHL (all of adjusted P-values < .001). ConclusionCT radiomic analysis proposed in this study, could help in the prediction of hearing loss induced by chemoradiation. Our study also, demonstrates that combination of radiomic features with clinical and dosimetric variables can model radiotherapy outcome such as SNHL.

Third-generation bisphosphonates for cochlear otosclerosis stabilizes sensorineural hearing loss in long-term follow-up.

ObjectiveTo assess long‐term hearing outcomes in patients treated with third‐generation bisphosphonates for otosclerosis‐related progressive sensorineural hearing loss (SNHL).Study DesignRetrospective case series reviewMethodsWe performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC‐PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long‐term follow‐up was analyzed. Significant change in BC‐PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance.ResultsSeven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3 ± 10.3 years. The mean duration between treatment with bisphosphonate administration and long‐term post‐treatment follow‐up audiometry was 87.6 ± 18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC‐PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy.ConclusionTreatment with third‐generation bisphosphonates is associated with stability in otosclerosis‐related sensorineural hearing over 5‐ to 9‐year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis.Level of Evidence4 (Case series).

MEDU-34. INTELLECTUAL OUTCOME IN CHILDREN WITH SENSORINEURAL HEARING LOSS FOLLOWING TREATMENT FOR EMBRYONAL BRAIN TUMORS.

Abstract Neurological side effects associated with childhood brain tumors and their treatments contribute to long term neurocognitive morbidity. The aims of this study were to identify the incidence of sensorineural hearing loss (SNHL) in a large sample of children treated for malignant embryonal brain tumors, and to evaluate the effect of SNHL on intellectual functioning following the completion of treatment. We conducted a single center review of 119 prospectively followed patients treated for malignant embryonal brain tumors at the Hospital for Sick Children, between 1996–2015, to evaluate the impact of significant SNHL (Chang > 2b) on intellectual function. Hearing and intellectual function were assessed post-treatment, at comparable time points (median ages: hearing assessment 13.5y (+4.5); intellectual function 12.8y (+ 4.1)). The median interval since treatment was 5.9 y + 3.7. Significant SNHL was identified in half the patients (48.7%, n = 58/119), of which 53% required a hearing aid (31/58). We identified a subset of patients (n = 61) who had assessments of intellectual function. In these patients, intellectual function was significantly affected by severe SNHL, even after controlling for the effect of craniospinal radiation (severe SNHL 22.4 Gy + 13.3, no/mild hearing loss 20.4 Gy +12.8) and boost dose. Severe hearing loss resulted in lower overall IQ (severe SNHL: 72.4 + 16.6; no/mild hearing loss: 92.0 + 20.5) p<0.001 and in significantly lower verbal comprehension (severe SNHL: 78.7 + 15.9; no/mild hearing loss: 94.7 + 13.8) p<0.001, and working memory (severe SNHL: 78.2+ 17.6; no/mild hearing loss: 94.8 + 16.4) p<0.001. Hearing loss is a much more significant complication in children with embryonal tumors than previously estimated. We show the profound impact of hearing loss on intellectual deficit in children. Our results have major implications on future trial design of children being treated with intensive protocols.

Neurocognitive outcome in children with sensorineural hearing loss after treatment of malignant embryonal brain tumors.

2029 Background: Neurological side effects associated with childhood brain tumors and their treatments contribute to long term neurocognitive morbidity. The aims of this study were to identify the incidence of sensorineural hearing loss (SNHL) in a large sample of children treated for malignant brain tumors, and to evaluate the potential relationship between SNHL and intellectual functioning following the completion of treatment. Methods: We conducted a prospective follow-up study at a single center with review of 119 patients treated for embryonal brain tumors at the Hospital for Sick Children, between 1996-2015, to analyze the impact of significant SNHL (Chang &gt; 2b) on intellectual function. Hearing was assessed post-treatment (median age: 13.5y (+4.5)) and the median age for neurocognitive testing was 12.8y (+ 4.1). The median interval from time of diagnosis was 5.8y (+ 3.7). Results: Severe SNHL was identified in half the patients (50.4%, n = 60/119). We identified a subset of patients (n = 61) who had assessments of intellectual function. In this cohort, intellectual function was significantly poorer in the group with severe SNHL, even after controlling for the effect of craniospinal radiation (severe SNHL 22.4 Gy + 13.3, no or mild hearing loss 20.4 Gy +12.8) and boost dose and volume. Children experiencing severe SNHL had lower overall IQ (severe SNHL 72.4 + 16.6; no/mild hearing loss 92.0 + 20.5) p &lt; 0.001 and in significantly lower verbal comprehension (severe SNHL 78.7 + 15.9; no/mild hearing loss 94.7 + 13.8) p &lt; 0.001, and working memory (severe SNHL 78.2+ 17.6; no/mild hearing loss 94.8 + 16.4) p &lt; 0.001, scores. Conclusions: Hearing loss is a much more significant complication in children with embryonal brain tumors than previously estimated. We show the profound impact of hearing loss on intellectual deficit in children. Namely, patients with severe SNHL have difficulty using and understanding verbal language, and they have a reduced ability to concentrate and manipulate information in short-term memory. Our results have implications on future trial designs and follow-up of children treated for embryonal brain tumors.

Preliminary analysis of the effects of tailor-made notched music therapy on chronic idiopathic tinnitus

Objective: Tailor-made notched music was applied to alleviate the symptoms of chronic idiopathic tinnitus and compared its effectiveness with other existing sound treatment of tinnitus. Methods: Subjects (n=43; ears=75 )were recruited during June 2015 to October 2016 from the out-patients of our hospital. These patients had chronic (longer than 6months) and idiopathic tinnitus, with or without significant sensorineural hearing loss. In the prospective design, the patients were randomly divided into group A (treated with tailor-made notched music) and group B (treated with analogous sound masking), and received the treatment for 3 months. The tinnitus scale, tinnitus questionnaire and audiological findings were evaluated before treatment, and at one month and three months after treatment started. Results: After onemonth of treatment, the effective rate between the two groups was 40.9% and 42.9%, there was no significant difference between the two groups (χ(2)= 0.017, P=0.897). The average VAS for patients in group A showed more decrease in group A than in group B(VAS: 1.8 vs. 0.8, the percentage : 29.5% vs. 13.6%), but there was no significant difference between the two groups (t=-1.450, P=0.155). After 3 months of treatment, the effective rates were 68.2% and 23.8%, respectively. There was significant difference between the two groups (χ(2)= 8.503, P=0.004). The difference of the VAS scores between the two groups was statistically significant (t=-3.263, P=0.002), and the VAS score of group A was less.After 3 months of treatment, there was significant decreaseinthe average tinnitus loudness for patients in group A(t=5.569, P<0.01), and there was no significant changein group B(t=-0.953, P=0.374). There was also significant decreasein the scores of tinnitus handicap inventory (THI) (F=7.334, P<0.05), loudness visual analog scale (VAS) (F=20.48, P<0.001), and the proportion of patients with moderate to severe tinnitus(χ(2)=11.289, P<0.05) in the group A, and there was no significant change in group B(F=2.198, F=0.989, χ(2)=1.651; P=0.120, P=0.378, P=0.438>0.05). Conclusions: Our resultssuggest that long-term normalized listening to tailor-made notched music, can significantly reduce the perceived tinnitus loudness in varying degrees and improve the quality of life of patients. The effects and possible mechanism of this method were discussedin this paper.

Surgical treatment of patients with the cochlear form of otosclerosis

The article focuses on the surgical treatment of the patients with the new-onset cochlear form of otosclerosis, and the patients, in which, according to the tonal threshold audiometry, a significant sensorineural hearing loss had occurred after stapedotomy. This article provides an algorithm of differential diagnostic of the primary (true) and the secondary (false) sensorineural hearing loss, comprising the sensorineural hearing loss component revealed in the patients with the cochlear form of otosclerosis. The authors present detailed description of the method of stapedectomy with substitution of a part of perilymph with physiological saline solution, providing its theoretical substantiation and the preliminary functional results of the surgery.