Demographic analysis of hearing impairment based on various parameters in patients with cochlear implant.

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.

Diagnóstico genético de la hipoacusia neurosensorial infantil / Genetic diagnosis of childhood sensorineural hearing loss

Introducción: La hipoacusia neurosensorial (HNS) congénita o de inicio precoz es una de las enfermedades hereditarias más frecuentes en nuestro medio y es la deficiencia sensorial más frecuente. Es importante realizar un estudio etiológico de la hipoacusia y el estudio genético mediante la secuenciación de nueva generación (NGS) es la prueba con mayor rendimiento diagnóstico. Nuestro estudio muestra los resultados genéticos obtenidos en una serie de pacientes con HNS congénita/de inicio precoz bilateral. Material y método: Se incluyeron 105 niños diagnosticados de HNS bilateral a los que se les realizó un estudio genético entre los años 2019 y 2022. El estudio genético consistió en una secuenciación masiva del exoma completo, filtrando el análisis para los genes incluidos en un panel virtual de hipoacusia con 244 genes. Resultados: Se obtuvo un diagnóstico genético en 48% (50/105) de los pacientes. Se detectaron variantes patogénicas y probablemente patogénicas en 26 genes diferentes, siendo los genes más frecuentemente afectados el gen GJB2, USH2A y STRC. De las variantes detectadas 52% (26/50) se asociaron a una hipoacusia no sindrómica, 40% (20/50) una hipoacusia sindrómica y 8% restante (4/50) se podían asociar tanto a una hipoacusia sindrómica como no sindrómica. Conclusiones: El estudio genético constituye una parte fundamental del diagnóstico etiológico de la HNS bilateral. Nuestra serie muestra que el estudio genético de la hipoacusia mediante NGS tiene un alto rendimiento diagnóstico y nos proporciona información de gran utilidad en la práctica clínica.(AU)

Introduction: Congenital/early-onset sensorineural hearing loss (SNHL) is one of the most common hereditary disorders in our environment. There is increasing awareness of the importance of an etiologic diagnosis, and genetic testing with next-generation sequencing (NGS) has the highest diagnostic yield. Our study shows the genetic results obtained in a cohort of patients with bilateral congenital/early-onset SNHL. Materials and methods: We included 105 children with bilateral SNHL that received genetic testing between 2019 and 2022. Genetic tests were performed with whole exome sequencing, analyzing genes related to hearing loss (virtual panel with 244 genes). Results: 48% (50/105) of patients were genetically diagnosed. We identified pathogenic and likely pathogenic variants in 26 different genes, and the most frequently mutated genes were GJB2, USH2A and STRC. 52% (26/50) of variants identified produced non-syndromic hearing loss, 40% (20/50) produced syndromic hearing loss, and the resting 8% (4/50) could produce both non-syndromic and syndromic hearing loss. Conclusions: Genetic testing plays a vital role in the etiologic diagnosis of bilateral SNHL. Our cohort shows that genetic testing with NGS has a high diagnostic yield and can provide useful information for the clinical workup of patients.(AU)

The Auditory Pathway in Congenitally Cytomegalovirus-Infected Human Fetuses.

Congenital cytomegalovirus (CMV) infection is the main cause of non-hereditary sensorineural hearing loss (SNHL). In order to shed light on SNHL pathophysiology, we examined the auditory pathway in CMV-infected fetuses; the temporal lobe, in particular the auditory cortex, and the inner ear. We investigated both inner ears and temporal lobes of 20 human CMV-infected fetuses at 21 weeks of gestation. As a negative group, five fetuses from spontaneous miscarriages without CMV infection were studied. Inner ears and temporal lobes were histologically examined, immunohistochemistry for CMV and CMV-PCR were performed. On the auditory cortex, we evaluated the local microglial reaction to the infection. CMV-positive cells were found in 14/20 brains and the damage was classified as severe, moderate, or mild, according to histological features. Fetuses with severe brain damage had a statistically higher temporal lobe viral load and a higher number of activated microglial cells in the auditory cortex compared to fetuses with mild brain damage (p: 0.01; p: 0.01). In the inner ears, the marginal cells of the stria vascularis were the most CMV positive. In our study, CMV affected the auditory pathway, suggesting a tropism for this route. In addition, in the auditory cortex, microglial activation may favor further tissue damage contributing to hearing loss.

[Endothelial dysfunction as a pathogenetic factor of sensorineural hearing loss]. / Endotelial'naya disfunktsiya kak patogeneticheskii faktor sensonevral'noi tugoukhosti.

Chronic sensorineural hearing loss (SNHL) is a common disease that leads to disability of the population. Despite the many reports devoted to SNHL, the question of the pathogenesis of the disease is still open. Many researchers consider the development of SNHL as a manifestation of microangiopathy. The mechanism of development of microangiopathy in SNHL is multifactorial, but most researchers agree that endothelial dysfunction (ED) triggers a complex of pathological changes in the vessels of the inner ear. OBJECTIVE: Review of the results of scientific research in recent years on the problem of etiopathogenesis of sensorineural hearing loss from the perspective of endothelial dysfunction in the formation of auditory disorders.

[Role of intratympanic glucocorticoid treatment in sudden hearing loss]. / Stellenwert der intratympanalen Glukokortikoidtherapie in der Behandlung des Hörsturzes.

Idiopathic sudden sensorineural hearing loss (ISSNHL) is one of the most common diseases in otolaryngology. Its etiology remains unknown. Furthermore, there is only a low level of evidence for the efficacy of established treatment modalities. In addition to systemic glucocorticoids, intratympanic corticosteroid treatment (ICT) has become increasingly important for treatment of ISSNHL. Different application strategies and treatment regimens have been described; however, uniform standards do not yet exist. ICT may be used for primary treatment as well as salvage therapy. Current data from meta-analyses show no benefit of intratympanic versus systemic primary therapy for sudden hearing loss (moderate evidence) but suggest a benefit of intratympanic secondary treatment over no treatment or placebo (high effect size, low evidence). Regarding combination of systemic and local glucocorticoid therapy in primary treatment of hearing loss, there may be a small benefit over systemic treatment alone (low effect size, low evidence).

Increased risk of sudden sensorineural hearing loss in patients with cervical spondylosis.

Whether cervical spondylosis (CS) is a risk factor for sudden sensorineural hearing loss (SSNHL) remains unclear. This study used national population-based data to investigate the risk of SSNHL in patients with CS in Taiwan of different ages and sexes. This study used data covering 2 million people in Taiwan, which were obtained from the National Health Insurance Research Database. The data that support the findings of this study are available from National Health Insurance Research Database but restrictions apply to the availability of these data, which were used under license for the current study, and so are not publicly available. Data are however available from the corresponding authors upon reasonable request and with permission of National Health Insurance Research Database. This retrospective cohort study enrolled 91,587 patients with a newly diagnosed CS between January 2000 and December 2018. Case and control cohorts were matched 1:1 according to age, sex, and comorbidities. SSNHL incidence rate and risk were compared between the groups. Cox regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). The mean follow-up period was 8.80 (SD = 4.12) and 8.24 (SD = 4.09) years in the CS and control cohorts, respectively. The incidence rate of SSNHL in the CS cohort (85.28 per 100 000 person-years) was 1.49-fold significantly higher than that in the non-CS cohort (57.13 per 100,000 person-years) (95% CI 1.32-1.68, P < .001). After age, sex, and selected comorbidities were adjusted for, CS exhibited an independent risk factor for SSNHL (adjusted HR = 1.52; 95% CI 1.34-1.71, P < .001). An age-stratified analysis in this study demonstrated a strong and highly significant association between CS and SSNHL in patients aged < 35 years (IRR = 2.28, 95% CI 1.18-4.39, P = .013). This large-scale Taiwanese-population-based retrospective study found that CS was associated with an increased risk of SSNHL. Acute hearing loss in patients with CS, particularly at a young age, should be carefully evaluated, and prompt treatment for SSNHL should be initiated.

Sensorineural hearing loss as an atypical presentation of typhoid fever: A case report.

Typhoid fever, also known as enteric fever, is a multisystemic infection primarily caused by Salmonella enterica serotype Typhi, and less commonly by Salmonella enterica serotypes Paratyphi A, B, and C. The classic presentation includes fever, malaise, diffuse abdominal pain, and constipation. If left untreated, typhoid fever can progress to delirium, obtundation, intestinal haemorrhage, bowel perforation, and death within a month of onset. However, the clinical course can deviate from the classic stepladder fever pattern, which now occurs in as few as 12% of cases.1 In this report, we describe an atypical presentation as sensorineural hearing loss in an otherwise healthy young male.

Normal Weight Central Obesity is a Poor Prognostic Factor for Sudden Sensorineural Hearing Loss.

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.

Dynamic Molecular Markers of Otosclerosis in the Human Cochlea.

OBJECTIVE: To investigate the role and distribution of various molecular markers using immunohistochemistry and immunofluorescence to further elucidate and understand the pathogenesis of otosclerosis. METHODS: Archival celloidin formalin-fixed 20-micron thick histologic sections from 7 patients diagnosed with otosclerosis were studied and compared to controls. Sections in the mid-modiolar region were immunoreacted with rabbit polyclonal antibodies against nidogen-1, ß2-laminin, collagen-IX, BSP, and monoclonal antibodies against TGF ß-1 and ubiquitin. Digital images were acquired using a high-resolution light and laser confocal microscope. RESULTS: Nidogen-1, BSP, and collagen-IX were expressed in the otospongiotic regions, and to lesser extent, in the otosclerotic regions, the latter previously believed to be inactive. ß2-laminin and ubiquitin were uniformly expressed in both otospongiotic and otosclerotic regions. There was a basal level of expression of all of these markers in the normal hearing and sensorineural hearing loss specimens utilized as control. TGF ß -1, however, though present in the otosclerosis bones, was absent in the normal hearing and sensorineural hearing loss controls. CONCLUSIONS: Our results propose that the activity and function of TGF-1 may play a key role in the development and pathogenesis of otosclerosis. Further studies utilizing a higher number of temporal bone specimens will be helpful for future analysis and to help decipher its role as a potential target in therapeutic interventions.

Clinical efficacy of intratympanic steroid injection for treating idiopathic sudden sensorineural hearing loss.

BACKGROUND: Idiopathic sudden sensorineural hearing loss (ISSNHL) is an emergency that causes permanent hearing loss if timely treatment is not provided. However, the evidence supporting the effect of intratympanic steroid injection (ITSI) starting time on hearing outcome is limited. METHODS: We retrospectively enrolled 582 patients with ISSNHL who were treated with ITSIs and reviewed their clinical and audiological variables. The relationship between ITSI starting time and hearing recovery was analyzed. RESULTS: The mean starting time of ITSI was 13.17 ± 16.53 days. The overall hearing recovery rate was 55.15% (recovery = mean hearing level gain of ≥10 dB). The recovery rates were 79.2%, 67.4%, 50%, 36.6%, and 17.8% for the ITSI starting times of 1 to 3, 4 to 7, 8 to 14, 15 to 28, and ≥29 days, respectively. A multivariate analysis revealed that ITST starting time (odds ratio [OR] = 0.94, 95% CI, 0.92-0.96, p < 0.001) and salvage therapy (OR = 0.55, 95% CI, 0.35-0.86, p = 0.009) were independent poor prognostic factors for patients with ISSNHL. CONCLUSION: Earlier ITSI treatment is associated with a higher hearing recovery rate. Comorbidities and post-ITSI complications were nonsignificant independent risk factors.

Does a patient with acquired arbovirus infection have a hearing impairment? A scoping review of hearing changes in an adult with Dengue, Chikungunya, and Zika.

OBJECTIVES: To identify and understand the evidence regarding hearing changes related to acquired Dengue, Chikungunya, and Zika virus infection in adult individuals. METHODS: A scoping review was performed according to the recommendations of The Joanna Briggs Institute and guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews in the Embase, PubMed/Medline, ScienceDirect, Scopus, and Web of Science databases without restriction on language and year of publication. Case studies, observational studies, and clinical trials reporting hearing loss in adult subjects (>18-60 years of age) of both sexes with DENV, CHIKV, or ZIKV diagnosed by positive molecular/serological examination by RT-PCR or IgM/IgG by ELISA method were included. RESULTS: Thirteen studies met the inclusion criteria and were selected for review. The occurrence of auditory symptoms caused by arboviroses and the presence of permanent or transient sensorineural hearing loss was variable in adults. CONCLUSIONS: Dengue, Chikungunya, and Zika infections in adults are associated with a variety of auditory symptoms. The frequency of permanent or transient sensorineural hearing loss is low but not negligible.

Risk factors and outcomes in patients with cochlear nerve deficiency.

INTRODUCTION: Cochlear nerve deficiency (CND) is a cause of sensorineural hearing loss made by radiologic criteria. There is sparse literature involving audiological outcomes and cochlear implantation (CI) success in patients with CND. METHODS: A retrospective chart review of all patients with sensorineural hearing loss at a tertiary children's hospital from 2000 to 2020 was conducted. Patients with CND on radiographic imaging were included and categorized as hypoplastic, aplastic, or indeterminate. RESULTS: In this study, 53 patients were identified with CND, totaling 70 ears. Of the 53 patients, 30 (56.6 %) were male, 8 (16.0 %) had a family history of childhood hearing loss, 6 (11.3 %) were born preterm, and 11 (23.4 %) required neonatal intensive care admission. The median maternal age was 29 years old [IQR: 27, 35], and 8 (15 %) patients were born to mothers with diabetes. Of the 70 ears, 49 (70 %) utilized conventional hearing aids, 12 (17.1 %) utilized a bone-anchored hearing aid, and 10 (14.3 %) underwent CI. Of the 10 ears implanted, 4 (40 %) ears had nerves classified as hypoplastic, 3 (30 %) as aplastic, and 3 (30 %) as indeterminate. Improvement in pure tone averages compared to preoperative testing was demonstrated in 8 (80 %), and 6 (60 %) displayed improved speech awareness thresholds. CONCLUSION: This study demonstrates that there may be an association between CND and maternal diabetes and NICU admission. There are variable results with hearing amplification options in patients with CND, and further research is needed to better describe the role of CI, bone-anchored hearing aids and conventional hearing aids in patients with CND.

Sensorineural hearing loss among children at risk: A 16-year audiological records review in a tertiary referral center.

OBJECTIVES: Hearing loss (HL) constitutes an increasing worldwide health problem. Neonatal hearing screening improved early detection and management to alleviate HL detriments on the person and society. Still, HL in childhood, beyond infancy, is under-investigated, especially in developing countries. This study aimed to explore the prevalence of HL in childhood amongst Jordanian children with HL risk factors and investigate the associated risk factors. METHODS: Retrospective cross-sectional review of audiological records in a tertiary public and teaching hospital. The data of 1307 children aged 0-15 years who underwent audiological assessment from 2000 to 2016 were included. A review of diagnostic audiological and medical records was conducted to investigate the prevalence of sensorineural HL in high-risk (HR) children and the most contributing risk factors. RESULTS: Descriptive statistical analysis showed that the prevalence of sensorineural HL was 29.2% in the study sample. The HL was bilateral in 95% and mild to moderate HL in 73%. The mean age at the diagnosis was around 4.5 years. The most common risk factors were parental concern about their child's hearing, ototoxic drug use, and developmental and speech delay. The Chi-squared test showed that parental concern and ototoxic drug use were associated with an increased probability of having HL. CONCLUSION: The prevalence of HL amongst at-risk children in Jordan is relatively high, and the diagnosis is delayed. The results highlight the importance of implementing a hearing screening program in at-risk children. This needs to start from birth and include a serial follow-up to detect cases of delayed-onset HL.

The potential relationship between uric acid and the recovery in sudden sensorineural hearing loss.

OBJECTIVE: Serum uric acid is proven to be associated with chronic hearing loss, but its effect on Sudden Sensorineural Hearing Loss (SSNHL) is unclear. This study aims to evaluate the prognostic values of serum uric acid levels in SSNHL patients. METHODS: The clinical records of SSNHL patients were retrospectively reviewed. Patients were divided into different groups based on hearing recovery and audiogram type, and uric acid levels were compared. Based on uric acid levels, patients were categorized into normouricemia and hyperuricemia groups, and clinical features and hearing recovery were evaluated. Univariate and multivariate analyses were performed to identify prognostic factors. RESULTS: In total, 520 SSNHL patients were included in this study, including 226 females and 294 males. In female patients, 186 patients were included in the normouricemia group, and 40 patients were enrolled in the hyperuricemia group. Significant differences were observed in uric acid levels, Total Cholesterol (TC), rate of complete recovery, and slight recovery between the two groups. In male patients, 237 subjects were categorized into the normouricemia group, and 57 patients were included in the hyperuricemia group. The rate of complete recovery and slight recovery was lower in the hyperuricemia group compared to the normouricemia group. All patients were further divided into good recovery and poor recovery groups based on hearing outcomes. The uric acid levels, initial hearing threshold, rate of hyperuricemia, and TC were lower in the good recovery group than the poor recovery group both in female and male patients. Binary logistic regression results showed that uric acid levels, initial hearing threshold, and hyperuricemia were associated with hearing recovery. CONCLUSION: Hyperuricemia might be an independent risk factor for hearing recovery in SSNHL patients. Serum uric acid and initial hearing threshold possibly affected the hearing outcome in males and females with SSNHL. LEVEL OF EVIDENCE: Level 4.

The value of gadolinium-enhanced MRI in predicting the development of sudden hearing loss into Ménière's disease.

OBJECTIVE: To compare the clinical features of sudden hearing loss (SHL) in patients with and without endolymphatic hydrops (EH), and to investigate the association between SHL with EH and Ménière's disease (MD). METHODS: The clinical data of 63 SHL patients with first symptoms were evaluated retrospectively. Patients were separated into two groups based on the results of gadolinium-enhanced magnetic resonance imaging: EH and non-EH groups. Independent sample t-test and U-test were used to compare groups for continuous variables, and the chi-squared test, corrected chi-squared test and Bonferroni correction test were used to compare groups for binary and ordinal variables. The binary logistic regression model was utilised for univariate and multivariate analysis of follow-up patient prognosis. RESULTS: The EH and non-EH groups contained 32 and 31 patients, respectively. The EH group had a higher prevalence of low-tone descending hearing loss. Fifty-one patients were followed for more than 2 years. In the EH group, 11 and 15 patients were diagnosed with sudden sensorineural hearing loss (SSNHL) and MD, respectively, while in the non-EH group, 24 patients were diagnosed with SSNHL and only one with MD. EH, low-tone descending hearing loss and vertigo were risk factors for the diagnosis of MD in a subgroup univariate regression analysis of patients experiencing SHL. EH was found to be a risk factor for the progression of SHL into MD in a multifactor regression analysis. CONCLUSIONS: Patients with SHL who have EH are more likely to present with low-tone descending hearing loss. EH is a risk factor for the subsequent development of MD.

Case report of scrub typhus with bilateral acute sensorineural hearing loss and cerebral salt-wasting disease.

Scrub typhus is a re-emerging and endemic disease in the Asia Pacific region caused by Orientia tsutsugamushi. We present a 65-year-old male from Sri Lanka who presented with fever, bilateral acute sensorineural hearing loss, and confusion. On examination, he was dehydrated. Significant orthostatic hypotension and an eschar were noted. Investigations revealed hyponatraemia with elevated urine sodium, reduced serum osmolality, and normal urine osmolality suggestive of cerebral salt wasting. After initial hydration with 0.9% NaCl, hyponatraemia was corrected with 3% NaCl. Oral doxycycline was prescribed, and he showed dramatic clinical improvement. A diagnosis of typhus must be considered in a patient presenting with a febrile illness and acute hearing loss. Cerebral salt-wasting disease should be considered in a patient with typhus who develops hyponatraemia with dehydration. Furthermore, acute sensorineural hearing loss in both ears is an important manifestation of the disease.

Preoperative EABR evaluation of auditory pathway integrity in patients with different etiology and postoperative effect estimation.

OBJECTIVES: To evaluate the effect of electrical auditory brainstem response (EABR) on the integrity evaluation of auditory pathway and the prediction of postoperative effect of cochlear implantation in patients with different etiology of hearing loss. METHODS: A total of 580 patients with neuropathic deafness who underwent cochlear implantation surgery from August 2011 to December 2020 were selected for EABR test. The preoperative EABR waveform was analyzed, and parameters such as V wave amplitude, threshold, latency and interval of each wave, and slope of V wave I/O curve were measured. Neural response telemetry (NRT) test was performed during MAP 1 month after operation, and C and T values of the machine were recorded. RESULTS: The total EABR extraction rate was 98.45% among 580 patients, including 100% for the normal structure group and enlarged vestibular aqueduct group (LVAS), 92.44% for other malformed group. The average threshold of V wave in patients with normal cochlear structure was significantly better than the malformation groups (p < 0.05). The total extraction rate of NRT was 78.62%, including 99.72% in the group with normal structure, 95.65% in the LVAS group, 1.85-88.24% in the group with other malformations, and 0% in the cochlear ossification group. The correlation analysis showed a statistically significant correlation between the average preoperative EABR threshold and the C value of NRT. CONCLUSIONS: Preoperative EABR could evaluate the integrity of auditory conduction pathway of patients with cochlear implantation and predict the postoperative hearing rehabilitation effect.

A Nationwide Population-Based Study for the Recurrence and Comorbidities in Sudden Sensorineural Hearing Loss.

OBJECTIVES: The aims of this study were to investigate the cumulative recurrence rate of SSNHL and to determine association between comorbidities and recurrence of SSNHL by comparing patients with and without recurrence. METHODS: Using the National Health Insurance Claims Database (NHICD) from 2009 to 2020, we conducted a population-based study. We only enrolled patients whose records showed a prescription for steroid and audiometry findings as well as an appropriate diagnostic code. Recurrence of SSNHL was defined as an episode of SSNHL greater than or equal to 3 months after the first episode of SSNHL. We compared the recurrence rate of SSNHL according to age and number of SSNHL recurrences. We also explored comorbidities including autoimmune, metabolic, chronic renal diseases, cancer, and migraine associated with recurrent SSNHL. RESULTS: A total of 257,123 patients were identified. We found that 6.7% (17,270/257,123) of the patients had at least one recurrence of SSNHL. The recurrence rate increases with the number of recurrences and over time. The incidence per 100,000 people tended to increase with age, and the recurrence rate appeared to decrease with age. We found an increase in the incidence of ankylosing spondylitis (AS) and a decrease in the incidence of type 2 diabetes mellitus (T2DM), myocardial infarction (MI), and hemorrhagic stroke in patients with recurrence. CONCLUSION: For patients with recurrence or AS, considerable efforts should be made to prevent recurrence. As SSNHL is an emergent otologic condition, when symptoms occur, they should receive immediate treatment. Additional well-designed population-based studies are required to generalize our results. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1417-1425, 2024.

Cochlear implantation in Bjornstad syndrome: a case series with literature review.

BACKGROUND: To report the presentation, diagnostic process, management and results of cochlear implantation of patients diagnosed with Bjornstad syndrome with profound sensorineural hearing loss (SNHL). CASE PRESENTATION AND MANAGEMENT: A retrospective report of two siblings with Bjornstad syndrome suffering profound SNHL unresponsive to conventional hearing aids treated with bilateral simultaneous cochlear implantation. SETTING: Tertiary-referral center. RESULTS: Cochlear implant surgeries of two siblings (four ears) with profound SNHL and bilateral inner ear anomaly (incomplete partition type 1) were performed without complications. Postoperative audiometric measurements showed a significiant improvement in pure-tone threshold and a word recognition score. In the literature review, no previous case of Bjornstad syndrome treated with cochlear implantation has been reported. CONCLUSIONS: Cochlear implantation is an effective, safe, and ultimate treatment option for Bjornstad syndrome with profound SNHL not responding to hearing aids.

Efficacy of high dose systemic versus combined (systemic and intratympanic) corticosteroid therapy in idiopathic sudden sensorineural hearing loss: A prospective randomized trial and risk factor analysis.

The pathophysiology and the proper treatment of idiopathic sudden sensorineural hearing loss (ISSNHL) are an ongoing subject of debate. Locally or systemic administered corticosteroids are the most accepted drugs of treatment in reference to ISSNHL (idiopathic sudden sensorineural hearing loss), however, no strong evidence nor guidelines regarding their effectiveness yet exists. In our prospective, randomized, controlled trial 78 participants were enrolled. Patients were randomly assigned based on the day of admission to two groups according to treatment: group SS (n = 43) received intravenous systemic methylprednisolone alone, and group CT (n = 35) received intratympanic dexamethasone + systemic methylprednisolone. The primary outcome was to compare the hearing outcomes between the treatment groups based on different, widely accepted categories (Siegel, Kanzaki, modified Siegel and PTA4 gain). In consideration of the secondary outcome, we examined the effect of the various risk factors on the hearing improvement. No differences were detected regarding hearing improvement between the two groups, based on any criteria [Siegel's criteria (p = 0.604); Kanzaki's criteria (p = 0.720); modified Siegel's criteria (p = 0.524) and PTA 4 gain (p = 0.569)]. However, several clinical factors such as vertigo (p = 0.039), or cardiovascular comorbidity (p = 0.02) and the severity of initial hearing loss (p = 0.033) were found to bear a significant impact upon the hearing outcome. To the best of our knowledge, this is the first randomized controlled trial comparing high dose systemic and combination corticosteroid therapy in ISSNHL patients. Our findings suggest coexisting cardiovascular comorbidity, vertigo and severity of the initial hearing loss may bear a significantly higher impact upon hearing improvement, than the additional intratympanic steroid administration. The presented trial was registered in the European Union Drug Regulating Authorities Clinical Trials Database (name: Combinated systemic and intratympanic steroid therapy in idiopathic sudden sensorineural hearing loss, No.: 2017-000658-20) and with the ethical approval of The National Institute of Pharmacy and Nutrition (OGYÉI) (protocol No.: 7621, on 2017.02.16.).