Predictors of computed tomography imaging in patients presenting with sudden hearing loss.

Objective: Sudden sensorineural hearing loss (SSNHL) is a rare presentation requiring timely diagnosis and treatment. Despite recommendations against obtaining computed tomography (CT) imaging of the head in clinical practice guidelines, this investigation is often completed in patients with sudden hearing loss. The aim of this study was to determine the proportion of patients undergoing CT imaging of the head for SSNHL at our center and identify predictive factors for the use of CT imaging. Methods: Retrospective chart review of adult patients referred for SSNHL to two academic otology/neurotology practices between January 2018 and May 2021. Patient demographics, comorbid medical conditions, associated symptoms, location of initial presentation, audiologic results, and completed imaging studies were collected. Statistical analysis was performed with SPSS software. Results: Ninety-eight patients with audiologically confirmed SSNHL were included. Twenty-two patients (22.4%) underwent CT imaging as an investigation for SSNHL. The presence of vertigo (odds ratio 6.90; 95% confidence interval 2.43, 19.56) and presentation to the emergency room (odds ratio 8.71; 95% confidence interval 3.02, 25.16) were significantly associated with undergoing CT imaging. These two variables were statistically significant independent predictors of CT imaging on multivariate regression analysis (p = .01, p = .001, respectively). Conclusion: A significant proportion of patients with SSNHL undergo low-yield CT imaging of the head, particularly patients presenting to the emergency room with vertigo. These results highlight an opportunity for focused education and quality improvement initiatives.Level of evidence: 4.

Assessment of Peripheral and Central Auditory Processing after Treatment for Idiopathic Sudden Sensorineural Hearing Loss.

Introduction When cases of idiopathic sudden sensorineural hearing loss (SSNHL) are treated successfully, most clinicians assume the normality and symmetry of the auditory processing. This assumption is based on the recovery of the detection ability on the part of the patients, but the auditory processing involves much more than detection alone. Since certain studies have suggested a possible involvement of the central auditory system during the acute phase of sudden hearing loss, the present study hypothesized that auditory processing would be asymmetric in people who have experienced sudden hearing loss. Objective To assess the physiologic and electrophysiological conditions of the cochlea and central auditory system, as well as behavioral discrimination, of three primary aspects of sound (intensity, frequency, and time) in subjects with normal ears and ears treated successfully for SSNHL. Methods The study included 19 SSNHL patients whose normal and treated ears were assessed for otoacoustic emissions, speech auditory brainstem response, intensity and pitch discrimination, and temporal resolution in a within-subject design. Results The otoacoustic emissions were poorer in the treated ears compared to the normal ears. Ear- and sex-dependent differences were observed regarding otoacoustic emissions and pitch discrimination. Conclusion The asymmetrical processing observed in the present study was not consistent with the hearing threshold values, which might suggest that the central auditory system would be affected regardless of the status of the peripheral hearing. Further experiments with larger samples, different recovery scenarios after treatment, and other assessments are required.

MRI-Based Inner Ear Assessment and Cochlin Tomoprotein-Based Evaluation of Perilymphatic Fistula in Patients with Sudden Hearing Loss.

OBJECTIVES: To study the correlation between positive cochlin tomoprotein testing (CTP), magnetic resonance (MR) imaging, and the auditory and vestibular function amongst patients with sudden hearing loss. STUDY DESIGN: Prospective case series. METHODS: We prospectively examined eight patients who presented with sudden hearing loss (>60 dB) with or without vertigo or tinnitus. We performed an ELISA-based CTP detection test using middle ear lavage samples. In addition to the CTP examination, a magnetic resonance imaging (MRI) examination was performed using different sequences (T1 and a T1 sequence with a contrast medium (CM), a T2 sequence, 4 h delayed intravenous gadolinium-enhanced three-dimensional fluid-attenuated inversion recovery (3D FLAIR)). RESULTS: All patients with sudden hearing loss (>60 dB) presented a non-specific contrast enhancement in the cochlea and vestibulum on the affected side on delayed 3D-FLAIR MRI. Four patients had a positive CTP test, suggesting a perilymphatic fistula (PLF). However, no specific MRI signal for a PLF was observed. CONCLUSIONS: Using multimodal diagnostic measures, such as CTP testing and different MRI sequences, no correlation could be found in patients with a PLF.

Clinical characteristics of patients diagnosed with bilateral sudden sensorineural hearing loss.

This study investigated the etiology, clinical features, and prognosis of patients diagnosed with bilateral sudden sensorineural hearing loss (BSSNHL). The clinical data of 100 patients with bilateral sudden hearing loss as a chief complaint treated at Xiangya Second Hospital of Central South University between January 2010 and August 2022, including clinical characteristics, audiometric data, and prognosis, were retrospectively analyzed. These 100 cases accounted for 8.09% (100/1235) of all patients admitted for sudden sensorineural hearing loss (SSNHL) during the same period. Of these, 71 were simultaneous cases and 29 were sequential cases of BSSNHL. Among the 200 ears analyzed in this study, 13, 36, 57, and 94 had mild, moderate, severe, and profound sensorineural hearing loss, respectively. The overall effective rate after comprehensive treatment was 32%, with significant differences in efficacy and prognosis among different degrees of hearing loss (p < 0.05). Comorbidities of hypertension (24 cases), diabetes (14 cases), and coronary heart disease (9 cases) significantly impacted therapeutic efficacy and prognosis in patients with BSSNHL (p < 0.05). Compared to unilateral SSNHL, BSSNHL exhibits distinctive characteristics.

Access to Sudden Hearing Loss Care at Urgent Care Centers.

OBJECTIVES: To compare patient access to urgent care centers (UCCs) with a diagnosis of sudden hearing loss based on insurance. METHODS: One hundred twenty-five random UCCs in states with Medicaid expansion and 125 random UCCs in states without Medicaid expansion were contacted by a research assistant posing as a family member seeking care on behalf of a patient with a one-week history of sudden, unilateral hearing loss. Each clinic was called once as a Medicaid patient and once as a private insurance (PI) patient for 500 total calls. Each phone encounter was evaluated for insurance acceptance and self-pay price. Secondary outcomes included other measures of timely/accessible care. Chi-square/McNemar's tests and independent/paired sample t-tests were performed to determine whether there were statistically significant differences between expansion status and insurance type. Calls ended before answering questions were not included in the analysis. RESULTS: Medicaid acceptance rate was significantly lower than PI (68.1% vs. 98.4%, p < 0.001). UCCs in Medicaid expansion states were significantly more likely to accept Medicaid (76.8% vs. 59.2%, p = 0.003). The mean wage-adjusted self-pay price was significantly greater in states with Medicaid expansion at $169.84 than in states without at $145.34 when called as a Medicaid patient (mean difference: $24.50, 95% Confidence Interval: $0.45-$48.54, p = 0.046). The rates of referral to an emergency department and self-pay price nondisclosure rates were greater for Medicaid calls than for private insurance calls (8.2% vs. 0.4% and 17.4% vs. 5.8%; p < 0.001 for both). CONCLUSION: Medicaid patients with otologic emergencies face reduced access to care at UCCs. LEVEL OF EVIDENCE: NA Laryngoscope, 2024.

Effect of pre-incident antiplatelet therapy on sudden sensorineural hearing loss.

OBJECTIVE: Identify if pre-incident aspirin influences severity and outcome of idiopathic sudden sensorineural hearing loss (SSNHL). STUDY DESIGN: Retrospective review. SETTING: Tertiary care center. METHODS: Patients with idiopathic SSNHL were identified and separated into aspirin and non-aspirin groups. Variables, including demographics, comorbid conditions, audiologic outcomes were identified and compared between groups. RESULTS: One hundred forty-eight patients were included that met inclusion criteria. There were 38 patients who were on pre-incident aspirin therapy and 110 patients not on aspirin prior to the onset of SSNHL. Pre- and post-treatment audiologic status was worsened in the aspirin group. Other comorbid conditions, including hyperlipidemia, coronary artery disease (CAD), cerebrovascular accident (CVA), and vertigo symptoms had an effect as well. With multivariate analysis, CAD, CVA, and vertigo symptoms appeared to have an effect more than aspirin. CONCLUSIONS: Patients on aspirin have a worsened pre- and post-treatment audiologic status. This appears to be more due to the underlying CAD or history of CVA rather than aspirin use itself.

Could the thick of retinal nerve fiber layer be a potential measure of axonal loss in hearing loss?

OBJECTIVE: The objective of the study is to compare the optic coherence tomography (OCT) parameters of the healthy and affected sides of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) and to investigate the relationships between these and the improvement in hearing levels. METHODS: A bilateral eye evaluation of patients diagnosed with ISSNHL was performed with OCT. The ganglion cell complex (GCC) and retina nerve fiber layer (RNFL) thickness values were recorded and the differences between the two eyes were examined. RESULTS: An evaluation was made of 39 patients with a mean age of 44.82 ± 14.90 years. The RNFL thickness of the eyes was determined to be mean 89.87 ± 3.65 µm on the affected side and 103.87 ± 3.98 µm on the healthy control side (p = 0.0001). The mean GCC was determined to be mean 90.46 ± 3.49 µm on the affected side and 103.77 ± 3.96 µm on the healthy control side (p = 0.0001). CONCLUSIONS: A statistically significant difference was observed between the healthy and affected eyes of patients with ISSNHL with respect to mean GCC and mean RNFL thickness. OCT could be a useful technique for measuring this neural degeneration.

OBJETIVO: Comparar e investigar los parámetros de la tomografía de coherencia óptica (OCT) de los lados sanos y afectados de pacientes con pérdida auditiva neurosensorial súbita idiopática (PANSI). MÉTODO: La evaluación ocular bilateral de los pacientes diagnosticados con PANSI se realizó con OCT. Se registraron los valores de espesor del complejo de células ganglionares (CCG) y de la capa de fibras nerviosas de la retina (CFNR), y se examinaron las diferencias entre los dos ojos. RESULTADOS: Se evaluaron 39 pacientes, con una edad media de 44.82 ± 14.90 años. Se determinó que el grosor de la CFNR de los ojos era una media de 89.87 ± 3.65 µm en el lado afectado y 103.87 ± 3.98 µm en el lado de control sano (p = 0.0001). Se determinó que el CCG medio era 90.46 ± 3.49 µm en el lado afectado y 103.77 ± 3.96 µm en el lado de control sano (p = 0.0001). CONCLUSIONES: Se encontró una diferencia estadísticamente significativa entre los ojos sanos y afectados de pacientes con PANSI con respecto al CCG medio y al espesor medio de la CFNR. La OCT podría ser una técnica útil para medir esta degeneración neuronal.

Effect of acupuncture intervention time on the therapeutic effect in patients with sudden hearing loss. / é’ˆåˆºä»‹å ¥æ—¶æœºå¯¹çªå‘æ€§è‹æ‚£è€ ç–—æ•ˆçš„å½±å“.

OBJECTIVES: To observe the clinical efficacy of acupuncture intervention at different time for patients with sudden hearing loss. METHODS: According to the timing of acupuncture intervention, 86 patients were divided into early exposure group (n=43) and late exposure group (n=43) . The early exposure group was given acupuncture treatment within 14 days of onset, and the late exposure group was given acupuncture treatment after 14 days of onset. After propensity score matching (PSM, a statistical matching technique for observational data) processing by using SPSS26.0 software, outcomes of 30 cases in the early exposure group and 30 cases in the late exposure group were analyzed. In addition to receiving basic treatment with drugs for vascular dilatation, thrombolysis, nourishing nerve, etc., all patients of the two groups were treated with neck acupuncture ("Neck Seven Meridian Lines" acupuncture), once every other day except Sundays, for a total of 12 time. Before, after the treatment and 3 months after the treatment, the total score of the Tinnitus Handicap Inventory (THI, 0, 2 and 4 points for each of the 25 items, total scores = 100 points) scale was used to evaluate the improvement of tinnitus symptoms caused by hearing loss. The clinical therapeutic effect was evaluated according to the efficacy grading criteria in the Guidelines for Diagnosis and Treatment of Sudden Deafness (2015) and the changes of pure tone audiometry curve. Multivariate Logistic regression was used to analyze the effect of factors that might influence efficacy before propensity score matching. RESULTS: The THI scores of patients in both groups decreased strikingly after the treatment and 3 months' follow-up (P<0.05). Compared with the same time-points of the late exposure group, the total THI scores of post-treatment and 3 months' follow-up were evidently lower in the early exposure group (P<0.05). The effective rate of the early exposure group (22/30, 80.00%) was significantly higher (P<0.05) than that of the late exposure group (16/30, 53.33%). The classification of sudden deafness and the application of traditional Chinese medicine in this study were not independent factors affecting the total effective rate. CONCLUSIONS: The time point of acupuncture intervention is an important factor affecting the effect on hearing and tinnitus disability of patients with sudden deafness. The earlier acupuncture treatment is accepted, the better the therapeutic effect is.

Sound Exposure Promotes Intratympanic Drug Delivery to the Inner Ear.

OBJECTIVE: To investigate the impact of sound exposure, with the resultant windows vibration on perilymphatic concentrations following intratympanic (IT) dexamethasone and gentamicin in an animal model. STUDY DESIGN: Animal model blinded study. SETTING: Animal facility of a tertiary medical center. METHODS: Bilateral IT dexamethasone or gentamicin was applied to 15 tested rats. Following injections, each rat was exposed for 3 minutes to free field 30 dB sound pressure level (SPL), 512 vHz noise, with 1 external auditory canal plugged (contralateral control). Following noise exposure, perilymph was obtained from both ears. Drug concentrations were measured using ultrahigh-performance liquid chromatography-mass spectrometer. RESULTS: For dexamethasone, the average (±SD) perilymphatic steroidal concentration was 0.417 µg/mL (±0.549) in the control ears versus 0.487 µg/mL (±0.636) in the sound-exposed ears (P = .008). The average (±SD) gentamicin perilymphatic concentration was 8.628 µg/mL (±2.549) in the sound-exposed ears, compared to 4.930 µg/mL (±0.668) in the contralateral control (nonsound exposed) ears. Sound exposure promoted steroidal and gentamicin diffusion to the inner ear by an averaged (±SD) factor of 1.431 and 1.730 (±0.291 and 0.339), respectively. CONCLUSION: Low-intensity noise (30 dB SPL) was found to enhance dexamethasone phosphate and gentamicin diffusion to the inner ear (by an averaged factor of ∼1.4 and 1.7, respectively) in a murine model.

Bilateral Sudden Hearing Loss in Iron Deficiency Anemia.

The present study describes an unusual case of bilateral sudden hearing loss associated with iron deficiency anemia. Although hematologic disorders such as anemia or leukemia have been reported to be associated with sudden hearing loss, bilateral sudden hearing loss, which was presented as the first manifestation of iron deficiency anemia, has not been reported. A 74-year-old man presented with simultaneous bilateral sudden hearing loss without vertigo. A complete blood count test revealed a hemoglobin level of 6.4 g/dL and a ferritin level of 14.5 mg/mL, indicating iron deficiency anemia. Postcontrast 3D FLAIR MRI showed enhancement of the bilateral cochlea, vestibules, and lateral semicircular and posterior semicircular canals. After treatment, the patient's hearing loss partially improved.

Asociación del resultado auditivo final, en pacientes con hipoacusia súbita, a la respuesta a glucocorticoides in vitro

Introducción: La hipoacusia súbita (HS) es poco frecuente y su etiopatogenia no está definida. La terapia con corticoides es de elección en base a recomendaciones de expertos por sus efectos teóricos y no en base a utilidad clínica demostrada. Objetivo: Evaluar si existe correlación entre el resultado auditivo final, de pacientes con HS tratados con corticoides, y la respuesta in vitro de sus leucocitos a corticoides, medida como diferencias en la expresión relativa de genes blanco del receptor de glucocorticoides. Material y Método: Estudio de casos (recuperación total) y controles (no recuperados) de pacientes con HS tratados con corticoides en el Hospital Clínico de la Universidad de Chile, durante 2017-2019. Se obtuvo DNA que fue almacenado en el Biobanco de Tejidos y Fluidos de la Universidad de Chile (BTUCH). Se purificaron y cultivaron leucocitos mononucleares de sangre periférica, expuestos in vitro a hidrocortisona. Se determinó la diferencia en la expresión relativa de genes blanco (IGFBP1, CAT, HSD17B12, APOA2), por Q-RTPCR, entre ambos grupos. Resultados: Se reclutaron 35 pacientes; se incluyeron para análisis 23: 11 casos y 12 controles, con edad promedio de 54,9 y 50,8 años respectivamente, distribución homogénea de sexo. No hubo diferencia estadísticamente significativa en la expresión relativa de los genes blanco, a la exposición in vitro a corticoides, entre ambos grupos. Conclusión: En nuestro estudio, modelo, y sistema de evaluación no se evidenciaron efectos de los corticoides. No podemos descartar que, con un número mayor de pacientes, otros genes blanco u otros protocolos de estudio podrían detectarse diferencias.

Introduction: Sudden hearing loss (SHL) is rare and its etiopathogenesis is still not clear. Corticosteroid therapy is of choice based on expert recommendations due to its theoretical effects and no based on proved clinical efficacy. Objectives: To assess whether there is a correlation between the final auditory outcome of patients with SHL treated with corticosteroids and the in vitro response of their leukocytes to corticosteroids, measured as differences in the relative expression of glucocorticoid receptor target genes. Material and Method: Case-control (total recovery and not recovered respectively) study of patients with SHL treated with corticosteroids at Clinical Hospital Universidad de Chile between 2017 and 2019. DNA was obtained and stored in the Biobanco de Tejidos y Fluidos de la Universidad de Chile (BTUCH). Peripheral blood mononuclear leukocytes were purified and cultured and then exposed to hydrocortisone. The difference in the relative expression of target genes (GFBP1, CAT, HSD17B12, APOA2), by Q-RTPCR was determined. Results: Thirty-five patients were recruited, 24 were included for the analysis: 11 cases and 12 controls, with and average age of 54.9 and 50.,8 years respectively, homogeneous sex distribution. There was no statistically significant difference in the relative expression of the target genes, upon in vitro exposure to corticosteroids, between both groups. Conclusion: In our study, model and evaluation system, no effects of corticosteroids were evidenced. With a larger number of patients, other target genes or other study protocols, we cannot rule out that differences could be detected.

[Management of ear, nose and throat emergencies]. / Management von Hals-Nasen-Ohren-Notfällen.

Emergency situations involving the ears, nose, and throat (ENT) area can pose considerable challenges for clinicians and often require an interdisciplinary approach due to the involvement of different organ systems. To avoid damage to highly relevant sensory and perception organs and life-threatening bleeding or respiratory complications, strategies that are as quick and targeted as possible are necessary. This article aims to provide an overview of ENT emergency management strategies. The entire spectrum from simple conservative to highly complex surgical measures plays a role here, both diagnostically and therapeutically. Aspects such as bleeding, respiratory problems, inflammation, hearing disorders, vertigo, facial palsy and injuries to the head and neck area are discussed. In addition, important topics such as preventive measures and possible complications are also addressed to ensure optimal patient care.

Sudden hearing loss secondary to syphilis.

BACKGROUND: Syphilis is a sexually transmitted disease caused by the spirochete Treponema pallidum, whose incidence has increased significantly in recent years. Some patients may develop sudden hearing loss (SHL) against the background of otosyphilis. OBJECTIVES: The objective of our study was to determine whether routine lues serology is useful in patients presenting with sudden hearing loss. METHODS: For this purpose, all cases of SHL treated in our hospital during a period of 6 years were propectively collected. The frequency of positivity for syphilis in these patients, the treatment received, and their evolution were determined. RESULTS: Of the total number of patients evaluated during that period, 71 underwent serological screening for syphilis, of whom 2 (2.8 %) presented positive screening antibodies. In one of them, the RPR was normal and had been treated with lues a few years before. After treatment there was no improvement. The other patient, diagnosed with otosyphilis with unconfirmed suspected neurological disease, showed normalization of hearing after specific treatment. CONCLUSIONS: Since it is a potentially curable disease, despite the low overall frequency of syphilis in patients with SHL it is advisable to perform serological screening for syphilis in high risk patients (e.g., incarceration, multiple recent sexual partners, men who have sex with men) or atypical clinical presentation (e.g., concurrent neuropathies).

[A case of sudden hearing loss combined with familial hyperlipidemia].

Hyperlipidemia is characterized by elevated levels of blood lipids. The clinical manifestations are mainly atherosclerosis caused by the deposition of lipids in the vascular endothelium. The link between abnormal lipid metabolism and sudden hearing loss remains unclear. This article presents a case study of sudden hearing loss accompanied by familial hyperlipidemia. Pure tone audiometry indicated intermediate frequency hearing loss in one ear. Laboratory tests showed abnormal lipid metabolism, and genetic examination identified a heterozygous mutation in theAPOA5 gene. Diagnosis: Sudden hearing loss; hypercholesterolemia. The patient responded well to pharmacological treatment. This paper aims to analyze and discuss thepotential connection between abnormal lipid metabolism and sudden hearing loss.

Sudden Secondary Sensorineural Hearing Loss Following Hand, Foot, and Mouth Disease in an Adult Patient: A Case Report.

Introduction: Sudden sensorineural hearing loss (SSNHL) can present as sudden hearing loss within 72 hours. Studies have shown that viral infection, including direct invasion, indirect reactions, stress responses, and immune-mediated hearing loss, is the main cause of SSNHL. Viral infection may play an important role in SSNHL by causing injury to the inner ear through blood or cerebrospinal fluid. In this article, we describe an adult case of SSNHL following hand, foot, and mouth disease (HFMD). Case Report: A 34-year-old man presented with sudden hearing loss in the right ear 4 days after HFMD onset. Tinnitus, ear fullness, and a slightly heavy head appeared synchronously as accompanying symptoms. Before 6 days, he had a fever for 2 days (the highest temperature was 39.4°C), followed by vesicles in the oral mucosa and papules on the hands and feet after cohabitation with his child diagnosed with HFMD.

The role of a mobile hearing application in secondary care ENT (HAppENT).

OBJECTIVE: Sudden hearing loss is a common presentation to ENT. In the authors' practice, patients often wait many weeks for formal hearing testing. This study aimed to assess whether a tablet-based hearing test, hearTest, could aid clinical decision-making within secondary care ENT. METHOD: This was a multi-centre, prospective, non-randomised study to assess the feasibility, usability and accuracy of hearTest. RESULTS: In the sample, hearTest was shown to be an acceptable method of testing for hearing loss by both patients and clinicians. The 0.5-4 kHz range had an average clinical agreement rate of 95.1 per cent when compared with formal pure tone audiometry, deeming it an accurate test to diagnose hearing loss. CONCLUSION: The authors propose that hearTest can be used within ENT as a clinical decision support tool when manual audiometry is not immediately available. Within the authors' practice, hearTest is used to aid diagnosis and management of sudden sensorineural hearing loss.

Contemporary Review of Idiopathic Sudden Sensorineural Hearing Loss: Management and Prognosis.

Sudden sensorineural hearing loss (SSNHL) is a rapid decline in auditory function that needs urgent medical management. Although etiologic factors, including viral infections, autoimmune diseases, and vascular issues, contribute to the understanding of SSNHL, the condition remains unclear in most cases. Systemic steroids are often used as the first-line treatment because they reduce inner ear inflammation; however, there remains numerous discussions about the effectiveness of alternative treatments. To predict hearing recovery is crucial in patients' counseling with factors, including delayed treatment, vertigo, and other health conditions, which indicate poor prognosis. Herein, we review contemporary research on the treatment approaches and outcome predictions of SSNHL to establish important guidelines for physicians in evaluating and treating patients with SSNHL.

Relevant Research of Inflammatory Cytokines Spectrum in Peripheral Blood of Sudden Hearing Loss.

OBJECTIVE: To investigate whether there is a correlation between the inflammatory state and the pathogenesis and clinical features of sudden hearing loss (SHL) by studying the expression of inflammation-related cytokines in the peripheral blood of patients with SHL. METHODS: In this work, we analyzed the cytokine profiles of 48 analytes in 38 patients with SHL compared to 38 healthy donors using a multiplex immunoassay. This study used appropriate statistical methods to screen for inflammatory cytokines associated with the pathogenesis of SHL, to analyze their network correlation, and to analyze the relationship between clinical features of SHL and inflammatory cytokines. RESULTS: Several cytokines, including CTACK, Eotaxin, HGF, INF-α2, IFN-ß, IL-1ß, IL-1ra, IL-2Rα, IL-4, IL-7, IL-8, IL-9, IL-10, IL-12(p40), IL-13, MIG, ß-NGF, SCF, and TNF-α, exhibited significantly higher levels in the peripheral blood of the SHL group compared to the control group. An inflammatory network composed of multiple cytokines, including IL-1ß, is a risk factor for the development of SHL. CONCLUSION: This study identified several inflammatory cytokines with elevated expression, which may be linked with the onset of SHL. The results of this study also provide a basis for the theoretical hypothesis of inflammation in SHL. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3293-3301, 2024.

A Systematic Review on Heritability of Sudden Sensorineural Hearing Loss.

OBJECTIVE: To assess the evidence supporting the heritability and genetic basis of sudden sensorineural hearing loss (SSNHL). DATA SOURCE: Records were extracted from PubMed, Scopus, and Cochrane databases. REVIEW METHODS: The protocol was registered on PROSPERO (CRD42022357389) and includes a systematic review on the genetic contribution to SSNHL. The search strategy yielded 1.483 articles from electronic databases. After quality assessment, 34 records were selected, including 369.650 patients with SSNHL from nine prevalence studies, two familial aggregation studies, one twin study, and 22 genetic studies. The prevalence of SSNHL was calculated from data on its incidence from population-based studies (period prevalence). To evaluate the heritability of SSNHL, the sibling recurrence risk ratio (λs) was calculated, by comparing the prevalence of SSNHL among siblings within the same generation to the estimated prevalence in the overall population. Genetic variants were grouped, based on the pathological mechanism related to SSNHL. RESULTS: The prevalence of SSNHL ranged from 0.1% to 0.0003% in America to 0.12%-0.0093% in Asia. The estimated sibling recurrence risk ratio for SSNHL (λs = 20.8-83.3) supports a significant familial aggregation. Although several genetic variants were reported to be associated with SSHL in controlled studies, neither was replicated in an independent cohort. CONCLUSIONS: Evidence supporting heritability of SSNHL is limited to epidemiological studies showing prevalence differences across different populations and familial aggregation. Genetic studies are of low quality and they lack replication cohort to confirm their findings. According to its low prevalence, exome or genome sequencing familial-based studies are needed to identify rare genetic variants in SSNHL. LEVEL OF EVIDENCE: NA Laryngoscope, 134:3447-3457, 2024.

Association of Autonomic Symptom Burden with Sudden Sensorineural Hearing Loss.

OBJECTIVE: To investigate the autonomic symptom burden in patients with sudden sensorineural hearing loss (SSNHL) and its association with the severity and prognosis. STUDY DESIGN: Observational prospective study. SETTING: Tertiary academic medical center. METHODS: Patients diagnosed with SSNHL at a single medical center completed the COMPASS 31 questionnaire, which assesses dysautonomia across 6 domains with 31 questions. A total COMPASS 31 score was calculated by summing the scores from each weighted domain. The treatment outcome was evaluated by the percentage of recovery, calculated as the hearing gain in pure tone average (PTA) after treatment divided by the pretreatment PTA difference between the 2 ears. We defined poor recovery as a percentage of recovery <80%. RESULTS: A total of 63 SSNHL patients were included. The mean COMPASS 31 score was 23.4 (SD 14). Patients with poor recovery had significantly higher COMPASS 31 scores than those with good recovery (mean 26.4 [SD 14.4] vs 16.9 [SD 10.4]; 95% confidence interval [CI] 2-17). There was a negative association between COMPASS 31 score and both hearing gain (r = -.323, 95% CI -0.082 to -0.529) and percentage of recovery (r = -.365, 95% CI -0.129 to -0.562). Multivariate analyses of independent factors indicate that patients with higher COMPASS 31 scores had a greater risk for poor recovery (OR 1.06 [95% CI 1.003-1.117]). CONCLUSION: This study highlights the association between autonomic symptom burden and poor hearing outcomes in SSNHL patients. The findings underscore the importance of evaluating autonomic function during the treatment of SSNHL.