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Objectives: When there is a difference in hearing on both ears, where to perform the first cochlear implantation (CI) becomes an important issue. The purpose of the study was to evaluate which ear should be chosen for the first implantation in sequential bilateral CI with a long inter-implant period. Methods: The study population consisted of 34 severe-to-profound sensorineural hearing loss pediatrics with the inter-implant period of ≥3 years between the first CI (CI-1) and the second CI (CI-2) before the age of 19 (mean of inter-implant period: 7.1-year). The patients were classified into Group A (CI-1 was performed on the ear with better hearing), Group B (CI-1 on the ear with worse hearing), or Group C (symmetrical hearing in both ears). Speech intelligibility test results were compared between the groups. Results: The monosyllabic word scores of CI-1 were excellent in Groups A (91.7±7.9%) and B (92.5±3.6%) but slightly lower in Group C (85.7±14.9%) before the second implantation (P =.487). At 3 years after the second implantation, all groups demonstrated excellent scores in the bilateral CI condition (95.9±3.0% in Group A; 99.1±.8% in Group B; 97.5±2.9% in Group C, P =.600). However, when the patients were tested in using CI-2 only in Groups A and B after using bilateral CI for 3 years, the scores were inconsistent in Group A (79.6±23.9%; range: 22.2-94.4%), while those were higher and more constant in Group B (92.9±4.8%; 86.8-100.0%). Conclusions: The first CI is strongly recommended to perform on a worse hearing ear if they had different hearing levels between ears. Even with the first CI on a worse hearing ear, its performance never deteriorates. In addition, if they receive the second CI several years later, it will be likely that the second one functions better. [ABSTRACT FROM AUTHOR]

Significance Statement: Primary middle ear inverted papilloma is an exceedingly rare finding posing a diagnostic challenge, as symptomatology often mimics common clinical entities like serous otitis media. Clinical and radiological findings are not specific, whilst high recurrence rates, local destruction, and potential malignant transformation constitute an overall demanding surgical challenge, requiring aggressive procedures. We report the case of a primary inverted papilloma of the middle ear, discussing diagnostic and therapeutic issues. [ABSTRACT FROM AUTHOR]

Since the issuance of the emergency use authorization (EUA) of 3 coronavirus disease 2019 (COVID-19) vaccines, there have been over 180 million individuals fully vaccinated in the United States (US). With the increasing administration of COVID-19 vaccinations, there have been over 550,000 adverse events reported in the Vaccine Adverse Event Reporting System (VAERS) with approximately 230,000 experienced after receipt of the Pfizer-BioNTech COVID-19 Vaccine as of September 23rd 2021. Audio-vestibular symptoms (including Sudden Sensorineural Hearing Loss (SSNHL)) secondary to immunizations has previously been evaluated. However, this report describes the first case of bilateral sudden sensorineural hearing loss potentially due to the Pfizer-BioNTech COVID-19 vaccine. We further review the available literature regarding the treatment of Sudden Sensorineural Hearing Loss, and the association of SSNHL with previous immunizations and COVID-19 infection. Lastly, we hypothesize the underlying potential mechanisms between SSNHL and the Pfizer-BioNTech COVID-19 vaccine. [ABSTRACT FROM AUTHOR]

Bacckground: Approximately 80% to 85% of sudden sensorineural hearing loss (SSNHL) is idiopathic, but immune-mediated mechanisms are thought to be involved. Behçet disease is an autoinflammatory vasculitis that may involve vessels of the inner ear. It can cause sensorineural hearing loss (SNHL) and an increased risk of SSNHL. Study Sample: We report a 21-year-old man who was diagnosed with Behçet disease in childhood and presented to our clinic with a 10-day history of abrupt hearing deterioration in both ears. Pure-tone audiometry showed severe to profound bilateral SNHL. Results: Oral prednisolone was prescribed for 3 weeks. Concurrent intratympanic steroid injections (ITSIs) were administered in each ear every 2 days for 5 days. A total of 15 daily sessions of hyperbaric oxygen therapy (HBOT) were completed. Acupuncture was performed every 2 days for 1 month. After these combined therapies, the patient's hearing threshold by 20 dB and his speech recognition threshold were improved. Conclusions: Some patients with SSNHL recover no hearing improvement after routine treatment, and alternative treatments including ITSI, HBOT, and acupuncture can be considered as optional. We used an aggressive multimodal approach to treat severe bilateral SSNHL in patient with Behçet disease. [ABSTRACT FROM AUTHOR]

Objective: The etiology of sudden sensorineural hearing loss (SSNHL) remains unknown; inflammation may be one cause. We retrospectively studied SSHNL cases in terms of the inflammatory parameters involved. Methods: We reviewed 169 SSNHL cases. The control group contained 132 patients with vocal cord polyps. The C-reactive protein (CRP) level, CRP/serum albumin (Alb) ratio, and neutrophil/lymphocyte ratio (NLR) were compared between the 2 groups. The relationships between these parameters and the time to treatment after SSHNL onset, hearing level, and therapeutic effects were also analyzed. Results: The CRP level, CRP/Alb ratio, and NLR were higher in SSNHL patients than in controls, but only the NLR differed significantly between the 2 groups. We found no significant differences between patients with different hearing levels, those evaluated at various times after SSNHL onset, and those with different outcomes. Conclusion: The NLR is a reliable SSNHL diagnostic marker. The CRP level and CRP/Alb ratio are not useful indicators. [ABSTRACT FROM AUTHOR]

Objective: The presence of Carhart's notch at 2000 Hz in otosclerosis links the changed bone conduction for this frequency with the otosclerotic process occurring in the oval window. The aim of this study is to perform an audiometric assessment of the effectiveness of surgical treatment of otosclerosis depending on the incidence of Carhart's notch. Methods: The analysis included 116 patients treated surgically for the first time due to otosclerosis. Patients were divided into 4 groups depending on the occurrence of Carhart's notch, determined by pure-tone audiometry (PTA) before the surgery and 36 months afterward. The mean value of bone conduction thresholds was calculated for 500 Hz, 1000 Hz, 2000 Hz, and 3000 Hz in the groups in which the Cahart's notch was observed. This value of bone conduction (BC) was a reference point for further analysis in patients who had no preoperative or postoperative Carhart's notch. Results: The analysis indicated that Cahart's notch in preoperative PTA is a statistically significant improvement factor for average BC. It was found that over a longer observation period, the presence of Carhart's notch has adverse effects on the size of the postoperative air-bone gap, and consequently on hearing improvement after surgical treatment. A comparison between patients from the two groups without preoperative Carhart's notch found that no beneficial effects of the surgery on speech comprehension were observed regarding high-level sensorineural hearing loss (SNHL). Conclusions: (1) In a long-term observation post-stapedotomy, average BC values were found to improve. Nevertheless, the improvement is less evident in patients with preoperative Carhart's notch. (2) Disappearance of Cahart's notch after surgical treatment of otosclerosis is a good prognosis of improvement in speech audiometry. (3) Deep SNHL in the absence of Carhart's notch in PTA constitutes a bad prognostic factor for improvement in speech audiometry in patients qualified for surgical treatment of otosclerosis. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study is to assess the association between atrial fibrillation (AF) and sudden sensorineural hearing loss (SSNHL). Methods: This study was conducted by searching the longitudinal health insurance database of the Taiwan National Health Insurance Program for relevant information from January 1, 2000, to December 31, 2011. Patients with AF were matched with non-AF controls with a 1:1 strategy according to propensity scores. Multiple logistic regression analyses were performed to determine the risk of SSNHL. Results: In total, 14 698 patients with AF were matched with the same number of non-AF patients as controls. After propensity score matching, the use of antiplatelet or anticoagulation medications and the occurrence of SSNHL were found to have a significant difference between AF and non-AF patients. The occurrence of SSNHL was found to be higher in men, those of 45 to 74 years old, and patients with hypertension in both AF and non-AF groups. Multiple logistic regression analyses revealed that male gender, age between 45 and 74 years, hyperlipidemia, and hypertension are risk factors for SSNHL. The use of aspirin was found to reduce the rate of SSNHL (odds ratio [OR]: 0.67, 95% CI: 0.49-0.94, P =.019), but AF was not found to be a risk factor for SSNHL (OR: 0.89, 95% CI: 0.64-1.23, P =.467). Conclusion: The association between AF and SSNHL is not significant. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the role of normal weight central obesity (NWCO) in the prognosis of sudden sensorineural hearing loss (SSNHL). Methods: We retrospectively investigated 807 cases of SSNHL from January of 2008 to August of 2019 from the Department of Otorhinolaryngology at Kaohsiung Medical University Hospital in southern Taiwan. We analyzed the association between overweight and obesity, NWCO, and the prognosis of SSNHL. The demographic and clinical characteristics, audiometry results, and outcomes were also reviewed. Results: The nonobese (body mass index [BMI] < 24 kg/m2) and overweight and obese groups (BMI ≥ 24 kg/m2) comprised 343 (42.50%) and 464 (57.50%) patients, respectively. The favorable prognosis rates in the nonobese and the overweight and obese groups were 45.48% and 45.91%, respectively, without a significant difference (P =.9048). Multivariate logistic regression revealed that BMI (adjusted odds ratio [aOR] = 1.00, 95% CI = 0.948-1.062, P =.9165) was not significantly associated with SSNHL recovery. The normal weight noncentral obesity (NWNCO) and NWCO groups comprised 266 (77.55%) and 77 (22.45%) patients, respectively, and had favorable prognosis rates of 48.50% and 35.06%, respectively. The difference between the groups was significant (P =.0371). Multivariate logistic regression analysis revealed that NWCO (aOR = 2.51, 95% CI = 1.292-5.019, P =.0075) was significantly associated with SSNHL recovery. Conclusions: NWCO may significantly affect the prognosis of SSNHL. [ABSTRACT FROM AUTHOR]

Objective: Hyperbaric oxygen therapy (HBOT) is an accepted treatment option for sudden sensorineural hearing loss (SSNHL), but it is still recommended in combination with corticosteroids. We investigated the efficacy of salvage HBOT in refractory SSNHL that does not respond to corticosteroid combination therapy. Methods: Eighty-four patients were included, who had unilateral SSNHL with an improvement of pure-tone average (PTA) less than 10 dB after using intratympanic plus systemic corticosteroids (combined therapy) as the initial therapy. The control group (n = 66) received no further treatment, and the HBOT group (n = 18) received additional treatment with HBOT (10 sessions in total with 2.5 atmospheres absolute for 1 hour). Results: No differences in PTA or WDS were found between the 2 groups. However, the mean hearing gain in the HBOT group (16.8 ± 4.49 dB) was significantly higher than that in the control group (4.45 ± 1.03 dB) (P =.015). The proportion of patients with hearing recovery (hearing gain of 10 dB or more) after treatment was significantly higher in HBOT group (38.9%) than in the control group (10.6%). Conclusions: In patients with refractory SSNHL after steroid combined therapy, salvage HBOT showed a significant effect on hearing gain and recovery rate. [ABSTRACT FROM AUTHOR]

Background: Machine Learning models have been applied in various healthcare fields, including Audiology, to predict disease outcomes. The prognosis of sudden sensorineural hearing loss is difficult to predict due to the variable course of the disease. Hence, researchers have attempted to utilize ML models to predict the outcome of patients with sudden sensorineural hearing loss. The objectives of this study were to review the performance of these machine learning models and assess their applicability in real-world settings. Methods: A systematic search was conducted in PubMed, Web of Science and Scopus. Only studies that built machine learning prediction models were included, and studies that used algorithms such as logistic regression only for the purpose of adjusting for confounding variables were excluded. The risk of bias was assessed using the Prediction model Risk of Bias Assessment Tool (PROBAST). Results: After screening, a total of 7 papers were eligible for synthesis. In total, these studies built 48 ML models. The most common utilized algorithms were Logistic Regression, Support Vector Machine (SVM) and boosting. The area under the curve of the receiver operating characteristic curve ranged between 0.59 and 0.915. All of the included studies had a high risk of bias; hence there are concerns regarding their applicability. Conclusion: Although these models showed great performance and promising results, future studies are still needed before these models can be applied in a real-world setting. Future studies should employ multiple cohorts, different feature selection methods, and external validation to further validate the models' applicability. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to evaluate the graft outcomes of endoscopic perichondrium-cartilage myringoplasty with preserving of anterior margins for repairing anterior perforation with 3 year followup. Study design: Prospective case series. Setting: Tertiary university hospital. Materials and Methods: We performed a prospective study in 47 patients with anterior perforation who underwent perichondrium-cartilage myringoplasty with preserving of anterior margins and tuck grafts. The operation time, graft success rate, hearing outcome, and complications were evaluated at 6 months and 3 years after surgery. Results: A total of 47 ears with anterior marginal perforation were included in the study. The perforation size was subtotal in 2 (4.3%) eras, large in 11 (23.4%) ears, medium in 27 (57.4%) ears, and small in 7 (14.9%). The mean operation time was 41.2 ± 5.4 minutes. All patients completed 6 months of follow-up. Residual perforation was observed in 2 patients with medium perforations, the graft success rate was 95.7% (45/47). The mean preoperative and postoperative AC PTAs were 38.1 ± 7.3 dB and 25.4 ± 4.6 dB (P <.05), while the mean preoperative and postoperative BC PTAs were 9.0 ± 4.6 dB and 9.6 ± 1.9 dB (P =.672). The functional success was 91.5% (43/47). None of the patients reported sensorineural hearing loss, altered taste, facial nerve palsy, vertigo, or tinnitus during the follow-up period. In addition, 34 (72.3%) patients completed 3 years followup and performed temporal bone CT examination, the mean followup time was 39.1 ± 2.7 months, CT revealed the well pneumatization of mastoids and middle ear. Conclusions: Endoscopic perichondrium-cartilage myringoplasty with preserving of anterior margins and tuck grafts is a safe, suitable, and reliable method for repair of anterior perforation with few risk of anterior blunting and lateralization. [ABSTRACT FROM AUTHOR]

Background: Universal newborn hearing screening (UNHS) is effective in identifying newborns with possible hearing loss (HL). Outpatient follow-up for newborns referred after hospital-based screening remains a potential area of improvement. In this study, we evaluate the efficacy of a community health worker (CHW) intervention in promoting adherence to outpatient rescreening for newborns referred after initial UNHS. Methods: A mixed prospective-retrospective cohort study was performed to evaluate a CHW intervention at an academic medical center. Caregivers of referred newborns were contacted by CHWs prior to discharge and educated about HL and the importance of follow-up screening. The CHW outreach intervention was performed for 297 referred newborns between May 2020 and June 2021 and compared to a cohort of 238 newborns without the CHW intervention between March 2019 and June 2021. Statistical analyses were conducted using 2 × 2 Chi-square tests, two-tailed unpaired t -tests, multinomial logistic regression, and multiple linear regression. Results: In the intervention group, 236 of 297 newborns (79.5%) completed their outpatient follow-up rescreening; in the comparison group, 170 of 238 newborns (71.4%) completed their follow-up rescreening (P =.031, OR = 1.55 with regression P =.04). In the intervention group, the average time to follow-up was 13.4 days versus 12.5 days for the comparison group (P =.449, multiple R 2 =.02 with P =.78). Conclusions: CHW outreach intervention may increase adherence to outpatient follow-up rescreening for newborns referred after initial, hospital-based UNHS. Expansion of nursery teams to include CHWs may thus improve completion of recommended follow-up hearing screens. [ABSTRACT FROM AUTHOR]

Objectives: 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. Study design: Retrospective case series. Setting: University-based level-one trauma center. Methods: 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Background: The spiral ganglion hypothesis suggests that pathogenic variants in genes preferentially expressed in the spiral ganglion nerves (SGN), may lead to poor cochlear implant (CI) performance. It was long thought that TMPRSS3 was particularly expressed in the SGNs. However, this is not in line with recent reviews evaluating CI performance in subjects with TMPRSS3-associated sensorineural hearing loss (SNHL) reporting overall beneficial outcomes. These outcomes are, however, based on variable follow-up times of, in general, 1 year or less. Therefore, we aimed to 1. evaluate long-term outcomes after CI implantation of speech recognition in quiet in subjects with TMPRSS3-associated SNHL, and 2. test the spiral ganglion hypothesis using the TMPRSS3-group. Methods: This retrospective, multicentre study evaluated long-term CI performance in a Dutch population with TMPRSS3-associated SNHL. The phoneme scores at 70 dB with CI in the TMPRSS3-group were compared to a control group of fully genotyped cochlear implant users with post-lingual SNHL without genes affecting the SGN, or severe anatomical inner ear malformations. CI-recipients with a phoneme score ≤ 70% at least 1-year post-implantation were considered poor performers and were evaluated in more detail. Results: The TMPRSS3 group consisted of 29 subjects (N = 33 ears), and the control group of 62 subjects (N = 67 ears). For the TMPRSS3-group, we found an average phoneme score of 89% after 5 years, which remained stable up to 10 years post-implantation. At both 5 and 10-year follow-up, no difference was found in speech recognition in quiet between both groups (p = 0.830 and p = 0.987, respectively). Despite these overall adequate CI outcomes, six CI recipients had a phoneme score of ≤ 70% and were considered poor performers. The latter was observed in subjects with residual hearing post-implantation or older age at implantation. Conclusion: Subjects with TMPRSS3-associated SNHL have adequate and stable long-term outcomes after cochlear implantation, equal to the performance of genotyped patient with affected genes not expressed in the SGN. These findings are not in line with the spiral ganglion hypothesis. However, more recent studies showed that TMPRSS3 is mainly expressed in the hair cells with only limited SGN expression. Therefore, we cannot confirm nor refute the spiral ganglion hypothesis. [ABSTRACT FROM AUTHOR]

Objective: Systemic administration of glucocorticoid steroids is the most common initial treatment for idiopathic sudden sensorineural hearing loss (ISSNHL); however, due to the prevalence of coronavirus disease, the indications for this treatment must be carefully determined. The aim of this study was to investigate the efficacy of intratympanic steroid therapy as an initial treatment for idiopathic SSNHL. Methods: Sixty-eight patients with idiopathic ISSNHL who were treated with intravenous or intratympanic steroids were included in this study. Patients were retrospectively evaluated regarding preoperative grade, type of additional treatment, outcome of treatment, and side effects of each treatment. Results: In 46 cases, patients received intravenous steroid therapy as the initial treatment, while 22 patients received intratympanic steroid therapy; 10 patients underwent salvage treatment due to inadequate improvement of symptoms. Regarding additional treatment, intravenous steroid monotherapy was used in 37 patients. The outcomes were similar after both treatments; 16 (43%) and 11 (52%) patients treated exclusively with intravenous and intratympanic steroids, respectively, were completely cured. There were no significant differences in the effects between the 2 treatments, indicating that they were almost equally effective. The side effects observed in patients treated with intravenous steroid therapy were increased blood pressure, acute gastric mucosal disorder, and insomnia. None of these side effects were observed in any of the patients treated with intratympanic steroids; however, 1 case of perforation of the tympanic membrane occurred due to the procedure. Conclusion: There were no significant differences in posttreatment outcomes between patients treated with either intratympanic or intravenous steroids. The therapeutic effects were comparable, and no severe side effects were observed; therefore, intratympanic steroid therapy may be considered useful as an initial treatment for ISSNHL in the context of widespread coronavirus disease. [ABSTRACT FROM AUTHOR]

Objectives: With the implementation of Universal Newborn Hearing Screening, early diagnosis and referral has been expedited. Many patients who refer screening pass subsequent testing with otoacoustic emissions (OAE) or auditory brainstem response (ABR). The objective of our study was to identify the incidence and etiology of hearing loss in infants who refer initial testing in an urban, tertiary care pediatric otolaryngology practice. Methods: We performed a chart review of infants who were evaluated after referring newborn hearing screening from 2017 to 2021. Data collected included birth history, hospital screening results, subsequent audiology and otolaryngology visit findings, final hearing diagnoses, interventions, and outcomes. Results: Of the 450 patients, 83.8% (n = 377) had normal hearing bilaterally after repeat testing (OAE and/or ABR). Thirty five patients were diagnosed with otitis media with effusion (OME) (7.8%) and 17 patients (3.8%) were diagnosed with sensorineural hearing loss. Twenty seven patients (6.0%) were diagnosed with obstructing cerumen/vernix, many times in addition to another diagnosis. Of the 17 patients with sensorineural hearing loss, 2 had genetic syndromes and 2 had congenital cytomegalovirus. Sensorineural hearing loss was significantly associated with the presence of a deafness syndrome (P =.004) and in-utero infections (P =.04). About 11 (2.4%) underwent myringotomy with tube placement, 5 (1.1%) were fitted with hearing aids, 2 (0.4%) were referred for hearing aids, 4 (0.9%) had both myringotomy with tube placement and hearing aids, 1 child had a soft band/Bone Anchored Hearing Aid (BAHA) (0.2%), and 1 child (0.2%) had a cochlear implant. Conclusion: Our incidence of sensorineural hearing loss was 3.8% (95% CI 2.0, 5.5), compared to rates of 0.44 to 68% in the published literature. Most patients had normal hearing, usually identified after only 1 repeat test. OME requiring myringotomy tube insertion was the most common pathology requiring intervention. Close observation for resolution and intervention, if warranted, is critical to prevent sequelae. [ABSTRACT FROM AUTHOR]

Introduction: Aminoglycoside antibiotics such as gentamicin are bactericidal and effective against gram negative organisms and act synergistically against gram positive organisms, including S taphylococcus aureus. However, they have serious adverse effects such as nephrotoxicity and ototoxicity. Gentamicin ototoxicity may occur after a single dose and results in decreased vestibular function, which is frequently debilitating and often permanent. Objective: To emphasize the risk of gentamicin ototoxicity and suggest alternative antibiotics in penicillin-allergic patients undergoing surgery. Case Summary: We present a case of a woman with preexisting Meniere's Disease who received gentamicin 400 mg perioperatively for a sigmoidectomy due to a penicillin allergy listed in the patient's medical record. The patient developed severe ototoxicity preventing her from working or driving. Physical examination was remarkable for a broad-based gait requiring assistance to walk and bilateral corrective saccades. Vestibular testing revealed high-grade bilateral vestibular loss associated with all semicircular canals, a considerable decline compared to her function 3 years prior. Discussion: Gentamicin is indicated for surgical prophylaxis when a patient has a true allergy to penicillins and cannot receive cephalosporins, though alternatives exist. True allergies include IgE-mediated illness (anaphylaxis, bronchospasm, or urticaria 30-60 minutes after administration) or exfoliative reactions (Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis). The authors encourage more prudent use of gentamicin, especially in patients susceptible for debilitating otologic insults, and offer recommendations for alternative agents prior to using gentamicin. [ABSTRACT FROM AUTHOR]

Although peripheral facial paralysis and sudden sensorineural hearing loss are not as common as anosmia, they are reported neurological manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We present a 62-year-old, serologically COVID-19 positive woman with seventh and eighth nerve involvement showed electrophysiologically with Auditory Brainstem Response and electroneurography and radiologically with internal acoustic canal magnetic resonance imaging. This single case report suggests a possible association between the SARS-CoV-2 infection with simultaneous sudden sensorineural hearing loss and isolated facial paralysis. However, further studies are needed to determine whether this relationship is coincidental or occasional. [ABSTRACT FROM AUTHOR]

The article discusses a study which investigated the adverse effects of COVID-19 vaccines on the auditory and vestibular system. The vaccines studied are Comirnaty from Pfizer, Spikevax from Moderna and Vaxzevria from AstraZenica. Some of the reported audio-vestibular adverse effects of the vaccines include vertigo, tinnitus, hearing loss and middle ear diseases.

Objectives: To assess and compare complication rates of symptomatic external auditory canal (EAC) exostoses treated with drill versus osteotome canalplasty. Databases Reviewed: PubMed/Medline, OVID, EMBASE, Web of Science, Google Scholar. Methods: A systematic review and meta-analysis in accordance with PRISMA guidelines and standardized bias assessment using the JBI critical appraisal checklist was performed. Studies containing original outcome data on drill and osteotome canalplasty were included. The primary study outcome was complication rates. Results: Fifteen studies were included, encompassing 1399 total patients (1788 ears) with 530 and 1258 ears in the osteotome and drill groups, respectively. Ten studies used a drill, 2 used an osteotome, and 3 used both. The most frequently reported complications were tympanic membrane (TM) perforation (osteotome group: 5.3% [95% CI: 1.7%-10.9%]; drill group: 3.8% [1.5%-7.1%]), sensorineural hearing loss (SNHL) (0.69% [0.07%-1.9%]; 4.3% [2.2%-7.0%]), and postoperative stenosis (1.1% [0.0005%-4.3%]; 4.1% [1.9%-7.0%]). Use of the osteotome was associated with a lower rate of SNHL (P <.05) and stenosis (P <.05), and a higher rate of TM perforation (P <.05). Heterogeneity of the studies included in the analyzed complications ranged from moderate to high. Level of evidence in the included studies ranged from 2b to 4 and all studies had an overall low risk of bias. Conclusion: While an osteotome technique may increase the risk of TM perforation, drill canaloplasty may increase the risk of SNHL and postoperative stenosis in EAC exostectomy. The exact quantity of hearing loss could not be definitively evaluated. Additional research with participant randomization is needed to assess clinical efficacy. Level of Evidence: Level 8 [ABSTRACT FROM AUTHOR]

Purpose: To determine whether preeclampsia and gestational diabetes mellitus is a risk factor for cochlear damage and sensorineural hearing impairment in infants. Materials and Methods: Longitudinal study was conducted in 2 tertiary referral centers. 1068 neonates were included, who were born to preeclampsia, gestational diabetes mellitus, and healthy mothers. The hearing evaluation was done using DPOAE on day 2 and for those who failed the initial DPOAE on day 2, underwent repeat DPOAE on day 15, ABR was done on day 30 if repeat DPOAE was Refer. The results were compared between the groups and analyzed. Results: On initial DPOAE, bilateral ear absent DPOAE rates were 19.5%, 15.8%, and 3.5% among preeclampsia, Gestational Diabetes Mellitus (GDM), control groups respectively. The difference was statistically significant (P <.001). Also it was noted that absent DPOAE was significantly high at low and mid frequencies (1000, 2000, 3000, and 4000 Hz) in bilateral ear. However the difference in repeat DPOAE among the groups were not significant (Right ear P =.17, Left ear P =.31). Infants who failed repeat DPOAE test underwent ABR test in which 3 of GDM group, 2 infants of preeclampsia group and 1 infant of control group had absent ABR test. Conclusion: This study reveals that GDM and preeclampsia showed remarkable association of hearing loss at lower and mid frequencies which was transient. The prevalence of absent DPOAE was corresponding to the severity of the maternal conditions under the study. [ABSTRACT FROM AUTHOR]

Objective: To investigate how dexamethasone dosage impacts intratympanic steroid therapy (IST) for treatment of sudden sensorineural hearing loss (SSNHL). Study Design: Retrospective review. Methods: Inclusion criteria identified subjects who received IST between January 1, 2010 and June 1, 2020 for iSSNHL. Subjects with Meniere's disease, autoimmune inner ear disease, subjects who received injections of non-dexamethasone steroid formulations, and subjects with missing audiogram data were excluded. Subjects were stratified by dexamethasone dosage: low-dose (10 mg/ml) versus high-dose (24 mg/ml), time-to-treatment and oral corticosteroid use. Outcome measures included post-treatment improvement in 4-frequency pure tone average (4F-PTA [500, 1000, 2000,4000 Hz]), low- and high-frequency PTA (250-1000 Hz and 2000-8000 Hz, respectively). Results: Of the 55 included subjects (50.9% male, mean age 48.9 ± 16.4 years), 31 received high-dose while 24 received low-dose injections. 90.9% of subjects were treated with oral steroids prior to or during IST. No significant differences in hearing outcomes were observed between low- and high-dose cohorts or when stratifying by oral steroid use. Time-to-treatment analysis comparing ≤1 month (67.3%) versus >1 month (32.7%) groups demonstrated a significant improvement in post-treatment 4F-PTA (P =.01) in the ≤1 month group. Conclusions: Hearing recovery was not significantly different between the 10 mg/ml versus 24 mg/ml doses of intratympanic dexamethasone, suggesting that steroid dose may not impact the efficacy of IST. A shorter time-to-treatment was observed to be favorable for hearing improvement. [ABSTRACT FROM AUTHOR]

Background: About 80% of congenital hearing loss cases have genetic causes, often autosomal recessive and non-syndromic. Autosomal Recessive Non-syndromic hearing loss is characterized by extreme genetic heterogeneity. Objectives: To report a case of congenital hearing loss with novel homozygous deletion in GRXCR1 gene. Methods: Case reports and review of literatures. Results: In this study, the proband was a 32-year-old woman seeking pre-marriage genetic counseling with non-syndromic congenital hearing loss. An owing negative test for GJB2 mutations, she underwent exome sequencing, unveiling a novel homozygous exon 2 deletion of the GRXCR1 gene. This mutation was confirmed in her affected mother and sibling by PCR and Quantitative Real-Time PCR. Conclusion: We identified a novel GRXCR1 gene mutation related to congenital hearing loss in a family. Our study highlights the efficiency of exome sequencing in discovering gene mutations in cases of diseases with genetic heterogeneity. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the correlation between clinical features and endolymphatic hydrops (EH) in children with sudden sensorineural hearing loss (SSNHL). Methods: We collected 30 SSNHL children aged ≤17 years old, all of whom underwent intravenous gadolinium injection. After 4 hours, inner ear 3-dimensional inversion recovery sequence with real reconstruction (3D-real IR) magnetic resonance imaging was performed. Combined with their medical history such as gender, age, disease course, hearing loss, and so on, the results were analyzed. Results: Different degrees of EH were shown in the vestibule or different turns of cochlea in the affected ears of SSNHL children, and 12 (40%) of 30 children showed positive EH. Age, low and middle frequency hearing loss, and other clinical symptoms such as dizziness and ear fullness have been shown to be related to a certain degree of EH in vestibule or cochlea, whereas no relationship was found between EH and other clinical features such as high-frequency hearing loss, gender, affected side, and tinnitus. Conclusions: Endolymphatic hydrops may not reflect the trend of disease progression over time in children with SSNHL, but the age of onset may be an important factor in the presence or absence of EH. Endolymphatic hydrops may be one of the causes of dizziness and ear fullness but has no obvious connection with the occurrence of tinnitus. Older SSNHL children with dizziness and ear fullness should maintain long-term follow-up to dynamically monitor the changes in EH. [ABSTRACT FROM AUTHOR]

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P =.005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely. [ABSTRACT FROM AUTHOR]

Introduction: Childhood cancers are usually treated with chemotherapy and radiation. Therefore, understanding the late side effects of such treatments is important to improve the quality of life in childhood cancer survivors. The present study aimed to investigate the late complications of treatments in childhood cancer survivors. Methods: This study is a retrospective descriptive study. A total number of 93 cases were enrolled in this study. These cases had a history of childhood cancer documented in their medical records at the Shafa Hospital, Ahvaz, Iran. The age range was 5.9–21.3 years and included 62 males and 31 female patients. Results: Many of the patients at this hospital with childhood cancer had experienced chemotherapy side effects as well as late effects of cancer therapy. Hypothyroidism is a late complication of therapy in thoracic cancers and head/neck tumors with relative frequencies of 23.1% and 12.5%, respectively. Scoliosis was observed in the patients undergoing the ABVD + COPP and 8/1 regimens with relative frequencies of 4% and 50%, respectively. Lower growth percentiles were also late side effects of cancer therapy. The highest relative frequency of growth retardation was observed in the <5 age group (46.7%). Restrictive lung changes had an overall relative frequency of 6.5% in male patients with all types of tumors. Sensorineural hearing loss was observed in patients with leukemia and Hodgkin lymphoma with relative frequencies of 8.7% and 24.0, respectively. Conclusion: The occurrence of most side effects could be decreased through early diagnosis, dose adjustment of some drugs, and preventative measures. [ABSTRACT FROM AUTHOR]

Objectives: The neurotropic and neuroinvasive properties of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) have been described. It remains unknown how SARS-CoV-2 affects the audiovestibular system when it causes mild or severe disease. In this study, the sequelae effect of SARS-CoV-2 on the audiovestibular systems of different patient groups was investigated using objective and subjective test batteries. Methods: In this present study, we evaluated vestibulocochlear functions of patients who previously had Coronavirus Disease-2019 (COVID-19) with pure tone audiometry, ocular vestibular-evoked myogenic potential (o-VEMP), and cervical vestibular-evoked myogenic potential (c-VEMP) tests to identify possible sequelae by comparing them with the control group. Results: We found that the amplitude of p13-n23 was lower in both groups of patients than in the control group (p < 0.001). In the results of the left ear c-VEMP, the amplitude of p13-n23 was statistically different between the outpatient, inpatient, and control groups. The amplitude of p13-n23 was lower in both groups of patients than in the control group (p < 0.001). In the evaluation of the o-VEMP in the left ear, we observed a statistically significant difference in the latency of n10 (p = 0.006) and the amplitude of n10-p15 (p < 0.001) between the groups. The n10 latency was prolonged in both groups of patients compared to the control group and there was no statistically significant difference between groups of patients. Furthermore, the amplitude of n10-p15 was lower in both groups of patients compared to the control group and there were no statistically demonstrable differences between the groups of patients. Conclusions: In conclusion, our results suggest that SARS-CoV-2 may affect the vestibulocochlear system. But we could not find a direct relationship according to the severity of the disease. [ABSTRACT FROM AUTHOR]

Objective: The aim of this meta-analysis was to critically assess the effect of cochlear implantation on auditory and speech performance outcomes of children with auditory neuropathy spectrum disorder (ANSD). Material and methods: A systematic literature search was conducted on PubMed, EMbase, and Web of Science. The outcomes included speech recognition score, Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR) score, and open-set speech perception. Results were expressed as standardized mean difference (SMD) or risk ratio (RR) with a 95% confidence interval (95% CI). Results: A total of 15 studies was included in this meta-analysis. Pooled data showed that, there were no significant differences between ANSD and sensorineural hearing loss (SNHL) groups in terms of speech recognition score (SMD = 0.01, 95% CI: −0.45, 0.47; P =.959),CAP (SMD = 0.71, 95% CI: −0.13, 1.54; P =.098), SIR score (SMD = −0.09, 95% CI: −0.49, 0.32; P =.667), and open-set speech perception (RR = 0.85, 95% CI: 0.69, 1.05; P =.142). Sensitivity analysis by removing individual studies one at a time showed that the overall estimate and level of heterogeneity did not change substantially. Conclusion: The current evidence suggested that children with ANSD who underwent cochlear implants achieved comparable effects in auditory and speech performance as children with non-ANSD SNHL. [ABSTRACT FROM AUTHOR]

Objective: To investigate the results of cochlear implantation in subjects with neurofibromatosis type 2 (NF2) and bilateral vestibular schwannomas (VS). Study Design: Retrospective case series. Setting: University-based tertiary referral center. Subjects: Five subjects with NF2 and severe-to-profound sensorineural hearing loss. Intervention: Cochlear implantation. Main Outcome Measure: Surgical outcomes and audiometric performance after cochlear implantation. Results: Five subjects (3 female, 2 male) were included in the study. The mean age at the time of implantation was 54 years old (range 35-78 years). Follow-up after cochlear implantation averaged 38 months (range 21-106 months). In the 5 implanted ears, 2 had no prior treatment, 1 had undergone prior radiation therapy, 1 underwent prior microsurgical removal, and 1 underwent prior microsurgical removal with adjuvant radiation therapy. The mean ipsilateral VS dimensions at time of implantation were 14 mm × 7.2 mm × 6.1 mm (mediolateral × anteroposterior × craniocaudal). Following cochlear implant activation, all 5 subjects achieved sound awareness, open set speech recognition, and 4 continue to be daily users of the devices. Conclusion: Cochlear implantation is a viable hearing rehabilitation option for subjects with NF2 and severe-to-profound sensorineural hearing loss. All subjects reported benefit with their cochlear implant, including open set speech recognition, enhanced lip-reading skills and environmental awareness of sound. Four subjects continued to demonstrate improved open-set speech recognition at the time of their last evaluations. [ABSTRACT FROM AUTHOR]

Background: The glucocorticoid receptor gene (NR3C1) encodes the receptor to which cortisol and other glucocorticoids bind. Steroids in either oral, intratympanic, or intravascular forms are the treatment of choice for sudden sensorineural hearing loss (SSNHL), but the outcome varies. The outcomes of SSNHL have been investigated for related factors, including age, initial hearing loss severity and pattern, vertigo, genetic variations, and the time between onset and treatment. The objective of the present study was to analyze the association of genetic polymorphisms of NR3C1 with the outcomes of SSNHL. Materials and methods: We conducted a comparison study of 93 cases with a poor outcome (control) and 100 cases with a good outcome (case) in SSNHL patients. Six single nucleotide polymorphisms (SNPs) were selected. The genotypes were determined using TaqMan technology. Results: The heterozygous AT genotype of rs17100289 was associated with a poor outcome in comparison with the major homozygous AA genotype after adjustments for age and sex (OR = 0.50; 95% CI 0.26–0.95; P = 0.035) in SSNHL patients. The CT genotype of rs4912912 was also associated with a poor outcome compared with the major homozygous TT genotype after the adjustments (OR = 0.47; 95% CI 0.24–0.92; P = 0.026). Conclusion: These results suggest that NR3C1 genetic polymorphisms may influence the outcomes of SSNHL. [ABSTRACT FROM AUTHOR]

Objective: A retrospective study was conducted to explore the immune-inflammatory responses in sudden sensorineural hearing Loss (SSNHL) patients with different audiogram shapes. Methods: One hundred and ten inpatients with SSNHL were assigned to 4 subgroups according to the audiogram shape and treated with systemic methylprednisolone. The numbers of white blood cells, neutrophils, lymphocytes, platelets, and monocytes were counted. The neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and the monocyte-to-lymphocyte ratio (MLR) were calculated and statistically analyzed. Results: Neutrophil-to-lymphocyte ratio, MLR, and systemic immune index (SII) of SSNHL patients were significantly higher than the control group, while PLR was not statistically significant. There were no statistical differences in NLR, PLR, MLR, and SII among the 4 subgroups. Conclusion: Immune-inflammatory response may be a common pathogenesis in all SSNHL patients with different audiogram shapes. The predictive value of these hematologic markers needs further research in the future. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to create a nomogram for accurately predicting the prognosis of idiopathic sudden sensorineural hearing loss (ISSNHL) and provide a reference for clinical treatment. Methods: Three hundred and twenty-three patients with ISSNHL were admitted from September 2014 to November 2020. The clinical data were retrospectively reviewed. Prognostic factors for ISSNHL were assessed based on univariate and multivariate logistic regression analysis and used to create a nomogram. Nomogram performance in terms of predictive and discriminatory ability was evaluated by calculating the concordance index (C-index) and generating calibration plots. Results: The overall hearing improvement rate was 41.4%, comprising complete recovery (13.3%), marked recovery (17.0%), and slight recovery (11.1%). Multivariate logistic regression analysis showed that age, symptoms of vertigo, interval between onset and treatment, low-density lipoprotein, and type of hearing loss were independent predictors of ISSNHL. A nomogram based on these 5 factors had a C index of 0.798 (95% confidence interval 0.750-0.845). Conclusions: Age, vertigo, interval between onset and treatment, low-density lipoprotein level, and type of hearing loss are closely associated with hearing recovery. The nomogram may enable prediction of the prognosis of ISSNHL and facilitate clinical decision-making. [ABSTRACT FROM AUTHOR]

Objectives: Sudden sensorineural hearing loss (SSNHL) has recently been investigated in terms of chronic inflammation. In this study, the associations of immunologic inflammatory factors (C-reactive protein [CRP] and erythrocyte sedimentation rate [ESR]) and hematologic inflammatory factors (white blood cell [WBC] count, neutrophil percentage, lymphocyte percentage, neutrophil-to-lymphocyte ratio [NLR], and platelet-to-lymphocyte ratio [PLR]) with prognosis of SSNHL were evaluated. Methods: Fifteen patients with SSNHL were prospectively enrolled and followed. For the analysis of the prognostic factors for SSNHL, the patients were divided into two groups according to the final hearing threshold of the affected ear: 'complete or partial recovery group' and 'slight improvement or no recovery group'. The CRP, ESR, WBC count, neutrophil percentage, lymphocyte percentage, NLR, and PLR obtained at the initial visit were compared. Results There were no significant differences in CRP, ESR, WBC count, and PLR between the two groups. In contrast, neutrophil percentage and NLR were significantly lower in the complete or partial recovery group than in the slight improvement or no recovery group (p = 0.005 and 0.013, respectively). Lymphocyte percentage was significantly higher in the complete or partial recovery group than in the slight improvement or no recovery group (p = 0.019). Conclusion NLR may be a useful prognostic inflammatory biomarker of SSNHL. Further evaluation of the association of immunologic and hematologic inflammatory factors with the prognosis of SSNHL is necessary with respect to inflammation to reveal additional significant biomarkers because inflammation is related to SSNHL. [ABSTRACT FROM AUTHOR]

Objectives: Celiac disease is a common multisystemic autoimmune disorder. It is now increasingly recognized that it may present with extraintestinal manifestations which contribute to the difficulty in its diagnosis. The objective of this scholarly review was to examine the extraintestinal ENT manifestations of celiac disease and its pathophysiology and management, in order to highlight that some patients with celiac disease may present initially to the otolaryngologist. Improving awareness of celiac disease among otolaryngologists may aid in the correct diagnosis and correct management plan. Methods: A literature review was conducted using the PubMed database to identify original articles related to celiac disease and ENT manifestations between the years 2000 and 2020. The search was performed using the search string: ("coeliac disease" OR "celiac disease") AND ("ENT manifestations" OR "hearing loss" OR "epistaxis" OR "nasal septal perforation" OR "obstructive sleep apnoea" OR "vertigo" OR "tonsillitis" OR "sinusitis"). Only articles written in English were reviewed. Results: A total of 17 papers met the inclusion criteria. Extraintestinal ENT manifestations of celiac disease include sensorineural hearing loss, obstructive sleep apnea, nasal septal perforation, epistaxis, and vertigo with nystagmus. Sensorineural hearing loss, obstructive sleep apnea, nasal septal perforation, vertigo, and nystagmus are thought to result from immunologically mediated mechanisms, with intestinal malabsorption resulting in epistaxis. Conclusions: Celiac disease can cause extraintestinal ENT manifestations and requires a high index of suspicion from the otolaryngologist to diagnose and suitably manage. A gluten-free diet may result in sufficient symptom resolution for most manifestations. Sensorineural hearing loss due to celiac disease appears to be progressive and permanent and may require frequent audiological monitoring. [ABSTRACT FROM AUTHOR]

Cochlear implants improve the quality of life of patients with bilateral severe sensorineural hearing loss. Normally, patients with cochlear implants can continue to use the devices for years without any complications. However, equipment failure or infection at the implant site could develop in some patients, and this might often necessitate implant replacement. Although cochlear implant replacement surgery itself is not a major risk in most cases, extensive tissue resection will be required in cases involving infection, and the insertion site of the temporal bone implant will need to be changed. We encountered a case of skin necrosis at the temporal bone implant site caused by constant external irritation from the temple of an eyeglass frame. The patient underwent cochlear implant replacement surgery involving full-thickness skin grafting from the abdomen. Thereafter, the patient's condition improved. Full-thickness skin grafting can be useful in cases of extensive skin defects encountered during cochlear implant replacement. [ABSTRACT FROM AUTHOR]

Viruses are often implicated as a cause of sensorineural hearing loss (SNHL), particularly sudden cases, including COVID-19. Determining the viral mechanism that leads to hearing loss is necessary for its future prevention and treatment. The 47-year-old woman who is the subject of this case study presented with sudden SNHL following multiple infections of COVID-19. Following a trial of a contralateral routing of sound device, she received a right cochlear implant (CI). Following a period of high performance, additional cases of COVID-19 infection and device failure issues resulted in the explant/reimplant of 1 ear and implantation of the contralateral ear. Despite extensive rehabilitation after these events, the patient continues to experience difficulties in speech understanding, not reaching her initial high levels of right ear performance. Further research is needed to determine the implications of COVID-19 as it relates to SNHL. This case study aimed to highlight the course of treatment and provide insight into the impact of COVID-19 on sudden hearing loss and its relationship to CI performance. [ABSTRACT FROM AUTHOR]

Objectives: In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies. Methods: In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes. Results: Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered. Conclusion: middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

Objective: To characterize the prevalence, imaging characteristics, and cochlear implant candidacy of pediatric patients with single-sided deafness (SSD). Methods: An audiometric database of patients evaluated at a large tertiary academic medical center was retrospectively queried to identify pediatric patients (<18 years old) with SSD, defined as severe to profound sensorineural hearing loss in one ear and normal hearing in the other. Medical records of identified patients were reviewed to characterize the prevalence, etiology, and cochlear implant candidacy of pediatric patients with SSD. Results: We reviewed audiometric data obtained from 1993 to 2018 for 52,878 children at our institution. 191 (0.36%) had the diagnosis of SSD. Cochlear nerve deficiency (either hypoplasia or aplasia) diagnosed on MRI and/or CT was the most common etiology of SSD and was present in 22 of 88 (25%) pediatric SSD patients with available imaging data. 70 of 106 (66%) pediatric SSD patients with available imaging had anatomy amenable to cochlear implantation. Conclusions: Pediatric SSD is a rare condition and the most common etiology based on radiology is cochlear nerve deficiency. High resolution imaging of the temporal bone is essential to determine cochlear nerve morphology prior to consideration of cochlear implantation. [ABSTRACT FROM AUTHOR]

Objectives: To assess the clinical value of periventricular white matter hyperintensity (PWMH) found on brain magnetic resonance imaging (MRI) in patients with sudden sensorineural hearing loss (SSNHL). Methods: In this prospective study, 115 patients who were diagnosed with SSNHL aged between 55 and 75 years were analyzed. All subjects underwent brain MRI and were divided into a PWMH and control groups, depending on the presence of PWMH on MRI. PWMH was subdivided into 3 groups according to severity. Pure-tone average results and hearing gain were compared between the 2 groups before treatment and 2 months after treatment. Hearing improvement was assessed using Sigel's criteria. Results: A total of 106 patients (43 in the PWMH group and 63 in the control group) finally completed the 2-month follow-up. Average hearing gain in the PWMH group was significantly higher than in the control group (34.8 ± 20.3 and 25.9 ± 20.3, respectively, P =.029). PWMH score 1 showed significantly better hearing levels and hearing gain compared to PWMH score 3 and the control group. Multivariate analysis revealed that younger age, better initial hearing level, and the presence of PVWM score 1 were associated with good recovery. Conclusions: The presence of PWMH score 1 on brain MRI in patients with SSNHL was associated with better treatment response and was a good prognostic factor in a multivariate analysis while the hearing recovery in more severe PWMH (scores 2, 3) was not different from the control group. [ABSTRACT FROM AUTHOR]

Objectives: Sudden sensorineural hearing loss (SSNHL) has recently been investigated in terms of chronic inflammation. In this study, the associations of immunologic inflammatory factors (C-reactive protein [CRP] and erythrocyte sedimentation rate [ESR]) and hematologic inflammatory factors (white blood cell [WBC] count, neutrophil percentage, lymphocyte percentage, neutrophil-to-lymphocyte ratio [NLR], and platelet-to-lymphocyte ratio [PLR]) with prognosis of SSNHL were evaluated. Methods: Fifteen patients with SSNHL were prospectively enrolled and followed. For the analysis of the prognostic factors for SSNHL, the patients were divided into two groups according to the final hearing threshold of the affected ear: 'complete or partial recovery group' and 'slight improvement or no recovery group'. The CRP, ESR, WBC count, neutrophil percentage, lymphocyte percentage, NLR, and PLR obtained at the initial visit were compared. Results There were no significant differences in CRP, ESR, WBC count, and PLR between the two groups. In contrast, neutrophil percentage and NLR were significantly lower in the complete or partial recovery group than in the slight improvement or no recovery group (p = 0.005 and 0.013, respectively). Lymphocyte percentage was significantly higher in the complete or partial recovery group than in the slight improvement or no recovery group (p = 0.019). Conclusion NLR may be a useful prognostic inflammatory biomarker of SSNHL. Further evaluation of the association of immunologic and hematologic inflammatory factors with the prognosis of SSNHL is necessary with respect to inflammation to reveal additional significant biomarkers because inflammation is related to SSNHL. [ABSTRACT FROM AUTHOR]

Background: Sudden sensorineural hearing loss (SSNHL) is a disease with an unknown etiology; damage to the auditory nerve from inflammation due to viral infection or vascular incidents has been implicated. According to several studies, cytokines, including interleukins, are associated with SSNHL in terms of serum expression and genetic polymorphisms. Interleukin-1 (IL-1) plays a key role in inflammation and may be associated with SSNHL. This study analyzed the association of single nucleotide polymorphisms (SNPs) of IL-1 receptor (IL-1R) genes with SSNHL in Taiwan. Methods: We conducted a case–control study involving 401 patients with SSNHL and 730 healthy controls. Four SNPs (IL-1R type 1 gene [IL1R1] [rs3917225 and rs2234650] and IL-1R type 2 gene [IL1R2] [rs4141134 and rs2071008]) were selected. The genotypes were determined using the TaqMan assay. The Hardy–Weinberg equilibrium (HWE) was tested for each SNP, and genetic effects were evaluated. Results: The TT genotype of rs2234650 had an adjusted odds ratio (OR) of 2.988 (95% confidence interval [95% CI] 1.27–6.82) (P = 0.012) compared with the CC genotype in patients with SSNHL. The SNP rs2234650 was associated with SSNHL in the recessive model (TT vs. CC + CT, P = 0.0206, OR = 2.681). The CT genotype of rs4141134 had an adjusted OR of 3.860 (95% CI 2.01–7.44; P < 0.0001) compared with the TT genotype, in patients with SSNHL. The SNP rs4141134 was associated with SSNHL under the dominant model (CC + CT vs. TT, P < 0.0001, OR = 4.087). Conclusion: These findings suggest that IL1R1 and IL1R2 gene polymorphisms may contribute to an increased risk of SSNHL in Taiwan. [ABSTRACT FROM AUTHOR]

Background: Sudden sensorineural hearing loss (SSNHL) is a disease with an unknown etiology; damage to the auditory nerve from inflammation due to viral infection or vascular incidents has been implicated. According to several studies, cytokines, including interleukins, are associated with SSNHL in terms of serum expression and genetic polymorphisms. Interleukin-1 (IL-1) plays a key role in inflammation and may be associated with SSNHL. This study analyzed the association of single nucleotide polymorphisms (SNPs) of IL-1 receptor (IL-1R) genes with SSNHL in Taiwan. Methods: We conducted a case–control study involving 401 patients with SSNHL and 730 healthy controls. Four SNPs (IL-1R type 1 gene [IL1R1] [rs3917225 and rs2234650] and IL-1R type 2 gene [IL1R2] [rs4141134 and rs2071008]) were selected. The genotypes were determined using the TaqMan assay. The Hardy–Weinberg equilibrium (HWE) was tested for each SNP, and genetic effects were evaluated. Results: The TT genotype of rs2234650 had an adjusted odds ratio (OR) of 2.988 (95% confidence interval [95% CI] 1.27–6.82) (P = 0.012) compared with the CC genotype in patients with SSNHL. The SNP rs2234650 was associated with SSNHL in the recessive model (TT vs. CC + CT, P = 0.0206, OR = 2.681). The CT genotype of rs4141134 had an adjusted OR of 3.860 (95% CI 2.01–7.44; P < 0.0001) compared with the TT genotype, in patients with SSNHL. The SNP rs4141134 was associated with SSNHL under the dominant model (CC + CT vs. TT, P < 0.0001, OR = 4.087). Conclusion: These findings suggest that IL1R1 and IL1R2 gene polymorphisms may contribute to an increased risk of SSNHL in Taiwan. [ABSTRACT FROM AUTHOR]

Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review. Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds. [ABSTRACT FROM AUTHOR]

The pandemic of "Corona Virus Disease 2019" (COVID-19) has changed the lives of people. There have been changes in common outpatient and emergency cases in otolaryngology, so an analysis of data pertaining to this was completed. This study is to evaluate the impact of viral infection disease in otolaryngological common disease. This study uses the data of common diseases in the outpatient and emergency department during the "COVID-19" pandemic (from February to April 2020) and the same period in the past 3 years from the Department of Otolaryngology. During the "COVID-19" period compared with the same period last year, the ranking of cases by diseases has changed. Diseases such as chronic pharyngitis, allergic rhinitis, sudden deafness, and tinnitus increased, meanwhile acute pharyngitis and acute laryngopharyngitis decreased (p < 0.05). The viral infection has impacted the mental behaviors of people, therefore mental-related disease cases of the department of Otolaryngology have increased indirectly. This study provides real data to illustrate mental-related diseases. It also provides experience and shows the importance of keeping and maintaining mental health. [ABSTRACT FROM AUTHOR]

Objectives: The objective of this study was to begin to measure the prevalence of elevated hearing thresholds in Ethiopia, with audiometric equipment, including bone oscillators and tympanometers. To that end Ethiopian nationals were trained in audiometric techniques to obtain sensorineural hearing loss (SNHL) and conductive hearing loss (CHL) rates in school age children. Methods: Five mainstreamed public grade schools in northern Ethiopia were the sites for audiometry campaigns examining 1351 children and testing them with behavioral audiometry. Results: Seven percent of students had SNHL > 20 dB in at least 1 frequency in at least 1 ear, and 0.6% of ears had SNHL > 50 dB. Eighteen percent8% of students had CHL > 20 dB in 1 or both ears. The frequency with the highest incidence of elevated pure tone thresholds was at 8000 Hz. Seven percent of students had an air bone gap at 2000 and/or 4000 Hz of at least 15 dB. Air bone gap is not obtained at 8000 Hz. There were 22 perforated tympanic membranes in 17 children (0.8% of tympanic membranes, 1% of students). Conclusion: This study gives a baseline for the prevalence of hearing loss in school age children in mainstreamed northern Ethiopian schools. It is the first of its kind and perhaps can help in estimated hearing health needs there. Level of Evidence: 2 [ABSTRACT FROM AUTHOR]

Background: Sudden Sensorineural Hearing Loss (SSNHL) is a medical emergency requiring immediate attention as delayed treatment can lead to permanent and devastating consequences. Primary care physicians are likely the first to be presented with SSNHL and therefore have the crucial role of recognizing it and initiating timely and appropriate management. The aim of this study was to gain insight into the current knowledge and practice trends pertaining to the diagnosis and management of SSNHL among family physicians in Canada. Methods: An 18-question survey targeting Canadian family physicians was marketed through two, physician-only discussion groups on the social media platform Facebook. Responses were collected between August 1st and December 22nd 2019 then aggregated and quantified. Results: 52 family physicians submitted responses. 94.2% (n = 49) reported that in their practice, unilateral SSNHL warrants urgent referral to otolaryngology and 84.6% (n = 44) reported that unilateral sudden-onset hearing loss warrants urgent referral for audiological testing. 73.1% of participants (n = 38) reported that they would attempt to differentiate between conductive and sensorineural hearing loss if presented with unilateral, acute or sudden-onset hearing loss. 61.5% (n = 32) would rely on tuning fork tests to inform management decisions, as compared to 94.2% (n = 49) relying on case history and 88.5% (n = 46) on otoscopy. 76.9% (n = 40) would prescribe corticosteroids if presented with confirmed, unilateral SSNHL. Conclusion: The majority of family physicians in the study would make appropriate referral and treatment decisions in the management of SSNHL, understanding it is a medical emergency. Tuning fork tests are under-utilized for informing management decisions compared to other means of differentiating conductive and sensorineural hearing loss. Further research is needed to understand why some family physicians do not prescribe corticosteroids for treatment of SSNHL, which may then identify any gaps in knowledge or inform improvements in clinical protocol. [ABSTRACT FROM AUTHOR]

To date, no specific biomarkers for idiopathic sudden sensorineural hearing loss (ISSHL) have been used. The aim of this study is to investigate whether prestin, the motor protein of cochlear outer hair cells, could be used as a biomarker candidate for the diagnosis and prognosis judgement of ISSHL. Blood samples of 14 ISSHL patients and 28 control patients without history of hearing loss were collected. Plasma prestin concentration was measured using Human Prestin (SLC26A5) ELISA Kit. The results showed that prestin was detectable in the plasma of all patients and the concentration of prestin was significantly higher in ISSHL patients with about half being above the average range of control patients. Moreover, in treatment responsive group, 6 of 10 patients had decreased prestin levels after treatment compared to those of before treatment, while the prestin levels of all the 4 patients in treatment unresponsive group increased in varying degrees. Our promising preliminary results suggest that prestin has the potential to serve as a biomarker to assist diagnosis and judge response to pharmacological treatments. [ABSTRACT FROM AUTHOR]

Objectives: Recent studies suggest that elevated neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) are poor prognostic factors in sudden sensorineural hearing loss (SSNHL). We aimed to investigate the accuracy of this hypothesis by taking into account the effect of cardiovascular risk (CVR) factors. Methods: Medical records of 122 patients with SSNHL were reviewed retrospectively and grouped into 2 as; patients without CVR (group 1; n = 68) and patients having CVR (group 2; n = 54). Moreover, 60 control cases who did not have SSNHL were also included and grouped into 2 as; group 3 (n = 30) with CVR and group 4 (n = 30) healthy controls without having SSNHL or CVRs. Neutrophil (N), lymphocyte (L), platelet (Plt), NLR, and PLR between the groups and their relationship with the severity of hearing loss, recovery rates, and audiogram configurations were analyzed. Results: The highest N and NLR values were in group 1 and were significantly higher than the values of group 4 (P <.05, P <.01). There was no significant relationship between the groups in terms of L, Plt, or PLR values. The highest NLR and PLR values were determined in SSNHL patients with mild hearing loss, complete recovery, and up-sloping audiogram configuration (P >.05). Conclusions: Elevated levels of N and NLR may be considered as strong laboratory findings showing an inflammatory response in the diagnosis of SSNHL, but the presence of CVR factors does not seem to increase the inflammatory response in SSNHL as expected. In patients with SSNHL, NLR and PLR elevation may indicate better prognosis. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to investigate the serum renin levels of patients with idiopathic sudden sensorineural hearing loss (ISSNHL). Material and Methods: Twenty-four patients with ISSNHL and 24 asymptomatic healthy volunteers were included in the study. Subjects underwent pure-tone audiometry and serum renin levels were measured. Results: There were 14 women (mean age:42.35 ± 9.53) and 10 men (mean age:43.8 ± 6.87) in the patient group. There were 14 women (mean age:42.4 ± 4.7) and 10 men (mean age:41.4 ± 4.59) in the control group. ISSNHL was detected on the right side in 13 patients and on the left side in 11 patients. Serum renin levels of the patients and controls were 788.01 ± 327.8 and 282.37 ± 107.73 pg/mL, respectively. The serum renin levels were found to be significantly higher in the patient group compared to the control group (P ≤.001). There was a statistically significant strong positive correlation between serum renin level and the severity of hearing loss (r = 0.77; P =.001). Conclusion: Serum renin levels of patients with ISSNHL were higher than controls. There was a statistically significant strong positive correlation between serum renin level and the severity of hearing loss. [ABSTRACT FROM AUTHOR]

Objectives: West Nile virus (WNV) has been spreading over the last 20 years. Human infection is asymptomatic in most cases. When the disease becomes clinically manifest, it may involve a range of issues, from a mild infection with flu-like symptoms to a neuroinvasive disease. Albeit rarely, WNV-associated sensorineural hearing loss (SNHL) has also been reported. Here we describe two new cases of SNHL and balance impairment caused by WNV infection. Methods: The patients were investigated with repeated audiometric tests and, for the first time, videonystagmography was also used. Results: Unlike findings in the few other published cases, an improvement in audiometric thresholds and vestibular function was documented in both of our patients. Conclusions: In the light of our findings, a prospective study would be warranted on a large series of patients with WNV infection in order: (i) to better define the epidemiology of the related cochlear-vestibular involvement; and (ii) to elucidate the virus-related changes to peripheral and central auditory and vestibular functions. [ABSTRACT FROM AUTHOR]