Article

Sudden Sensori-Neural Hearing Impairment: A Report of 1,220 Cases

Wiley
The Laryngoscope
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Abstract

We reviewed the findings in 1,220 cases of sudden sensori-neural hearing impairment encountered over a nine-year period at the Otologic Medical Group. A thorough neuro-otologic evaluation is indicated in each case. Ten patients had an acoustic tumor. A vasodilator regimen was prescribed in 380 cases and resulted in hearing improvement in 40 percent. Favorable prognostic findings were a low tone loss and absence of vertigo.

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... Sudden sensorineural hearing loss (SSNHL) is an audiologic emergency with an annual incidence of 5-20 per 100000. SSNHL is idiopathic most of the times however about 10-15% of cases are due to identifiable causes like Meniere's disease, trauma, autoimmune disease, infectious causes etc. [1][2][3][4] The most obvious perceptual consequence of sensorineural hearing loss is insensitivity to weak sounds. Other adverse perceptual consequences include loudness recruitment, poor perception of pitch and auditory space, and difficulty understanding speech, particularly in the presence of background noise. ...
... [8] Most of the SSNHL cases are idiopathic but viral infections, autoimmune disorders, blood vessels disorders, inner ear membrane rupture are among the few causes isolated. [1,3,8,9] SSNHL can occur over a wide age range but it frequently occurs in people between 30 to 50 years of age. Usually both the sex are affected equally. ...
... The condition is usually unilateral with bilateral cases reported in less than 1.7% cases. [3] There was no case with bilateral involvement in our series and the right ear was involved more (75.2%) than the left. ...
... Para explorar los procesos fisiopatológicos que subyacen en los pacientes con SSI+SV, se han aplicado diferentes pruebas clínicas: tradicionalmente la prueba calórica registrada con videonistagmografía (VNG) y más recientemente, los potenciales evocados miogénicos vestibulares (VEMPs cervicales y/u oculares) y la prueba de impulso cefálico (vHIT) [12,19,[21][22][23]. En el 39-74% de casos de SSI+SV, se ha encontrado alterada la prueba térmica [24][25] y en un 40% los VEMPs [26]. La afectación vestibular descrita como más frecuente es la del CSP [27][28], seguida por el CSH [27]. ...
... La afectación vestibular en la SSI fue descrita inicialmente por Rasmusen en 1949 [34] y desde entonces se estudia la influencia del vértigo en las manifestaciones clínicas y el pronóstico de la SSI [24][25]30]. ...
... La mutua influencia entre el vértigo y la sordera profunda, hace suponer que la evaluación de los patrones de disfunción vestibular involucrados en la SSI, podría predecir el pronóstico de la pérdida auditiva [25,18]. Pero la literatura ofrece resultados contradictorios, donde algunos afirman que Ediciones Universidad de Salamanca / [ 13 ] Rev. ORL, 2024, 15, e32020, 1-26 [Publicación continua] la paresia vestibular supone un valor pronóstico negativo [11-12, 24, 37], mientras otros consideran que la afectación vestibular "no" tiene valor predictivo [7,18,30]. ...
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Introducción y objetivos: Evaluar la relación entre la clínica y la función cócleo-vestibular en los pacientes diagnosticados de sordera súbita idiopática (SSI) asociada a vértigo (SV). Con este estudio pretendemos investigar la prevalencia de las lesiones vestíbulo-cocleares encontradas en los pacientes con SSI+V y la correlación del resultado de las pruebas de función vestibular con la severidad y el pronóstico de la hipoacusia. Material y método: Estudio retrospectivo/prospectivo de 42 pacientes diagnosticados de SSI+SV mediante audiometría, videonistagmografía, potenciales evocados miogénicos vestibulares cervicales y vHIT. Tuvimos en cuenta datos epidemiológicos y clínicos, complementándolos con un examen de resonancia magnética y un amplio estudio analítico. Finalmente evaluamos estadísticamente los datos obtenidos. Resultados: El deterioro cócleo vestibular varía en función del tiempo transcurrido desde el evento. Hay una estrecha correlación entre el grado de hipoacusia y la disfunción vestibular. El daño vestibular es mayor en caso de SSI profunda. Los receptores vestibulares más afectados fueron el sáculo y el conducto semicircular posterior y el menos afectado fue el anterior. La función del conducto semicircular horizontal es fundamental en la predicción de la hipoacusia. Discusión: El pronóstico de una paciente con SSI+SV, es un desafío que se va superando a medida que reconocemos los factores que determinan su evolución. Conclusiones: En el síndrome de SSI+SV, la severidad de la hipoacusia depende de la extensión del daño vestibular, pero también está influido por otros factores acompañantes.
... Nonetheless, whether ALFHL is an independent disease is yet controversial. Some studies considered ALFHL as a subtype of SSNHL, which has a better prognosis than other types (Shaia and Sheehy, 1976;Mattox and Simmons, 1977). In contrast, because of the good prognosis, high recurrence rate, ease of development into MD, and some other clinical characteristics, some studies classified it as a type of disease independent of SSNHL. ...
... Consistent with these findings, the current study showed that PTA at admission and delay from onset-therapy were independent factors related to the prognosis of ascending SSNHL. Many studies speculated that the earlier the patient receives treatment, the better the prognosis (Shaia and Sheehy, 1976;Byl, 1984). Byl (1984) demonstrated that when patients receive treatment within 7 days from onset, the recovery rate is 56%, while the recovery rate of patients who received treatment for >30 days after onset is only 27%. ...
... Byl (1984) demonstrated that when patients receive treatment within 7 days from onset, the recovery rate is 56%, while the recovery rate of patients who received treatment for >30 days after onset is only 27%. Shaia and Sheehy (1976) also proposed that the prognosis is improved when patients are treated within 30 days from onset. Moreover, the severity of hearing loss was a major prognostic factor. ...
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Objective The present study aimed to explore the pathogenesis of the ascending sudden sensorineural hearing (SSNHL) loss by comparing the clinical characteristics and prognosis of acute low-frequency hearing loss (ALFHL) and ascending SSNHL. Methods A total of 43 patients with ALFHL and 122 patients with ascending SSNHL were enrolled in this study. First, the prognosis of patients with ALFHL and ascending SSNHL were compared, and the prognostic factors of AFHL and ascending SSNHL were analyzed. Results Acute low-frequency hearing loss and ascending SSNHL have no remarkable difference in complete recovery rate. Compared to ascending SSNHL, ALFHL has younger onset age, female prevalence, lower hearing threshold, shorter time from onset to recovery, and a lower proportion of combined tinnitus. The PTA at admission and delay from onset to therapy were significantly related to the prognosis of patients with ascending SSNHL, while only delay from onset to therapy was significantly related to the prognosis of patients with ALFHL. The majority of patients with ascending SSNHL and ALFHL recovered completely within 10 days from onset. Conclusion Audiogram shape plays a critical role in the prognosis of SSNHL. Ascending SSNHL and ALFHL may share a common pathological mechanism.
... Sudden sensorineural hearing loss (SSNHL) is a condition in which hearing loss ≥30 dB occurs at three or more consecutive frequencies within 3 days. 1) It is accompanied by vertigo in about 30% of cases, and in clinical settings, 2) it is defined as SSNHL with vertigo or labyrinthitis. 3,4) However, if direction-changing spontaneous nystagmus or direction-changing gaze-evoked nystagmus is observed, additional evaluations should be performed to differentiate central causes such as anterior inferior cerebellar artery (AICA) infarction and vestibular schwannoma. ...
... In severe cases, dysfunction may occur in both the cochlear and vestibular systems, leading to vertigo in 30%-40% of cases. 2,6) On the other hand, SSNHL accompanied by vertigo can occur due to lesions in the central nervous system, with AICA infarction and acoustic neuromas as major causes. In more than 90% of the cases, the labyrinthine artery originates from the AICA, and AICA infarction causes ischemic damage to the inner ear, leading to acute vertigo and hearing loss. ...
Article
If direction-changing gaze-evoked nystagmus accompanies sudden hearing loss, central lesions should be considered as the cause. A 33-year-old female presented at our hospital with sudden hearing loss and dizziness. A series of vestibular function tests confirmed a visual fixation disorder with central causes; however, no specific findings were found on brain MRI. Subsequent smooth pursuit and optokinetic nystagmus tests confirmed bidirectional saccadic pursuit and reversed optokinetic nystagmus, suggesting congenital nystagmus. A history of abnormal eye tremors was rechecked, and labyrinthitis occurring in undiagnosed congenital nystagmus was diagnosed. The diagnosis can be challenging when vestibulocochlear disease occurs in patients with undiagnosed congenital nystagmus. In the absence of specific findings on MRI, vestibular function tests, including the optokinetic nystagmus and smooth pursuit tests, should be conducted. In particular, it is crucial to investigate the history of abnormal eye tremors.
... Idiopathic sudden sensorineural hearing loss (SSNHL) usually arises unilaterally and indicates rapid dysfunction of the hearing sense organs; tinnitus usually presents in about 85% of cases whereas vertigo presents in up to 30%. Unilateral hearing loss impairs the localization of sound and the comprehension of spoken language [1][2][3][4][5]. ...
... The total duration of therapy was 30 days [18]. (2) In the second group [intratympanic methylprednisolone acetate (IT-MPA)], 23 patients received IT-MPA (40 mg/ml) with a 21-G needle, four times, over 30 days [19]. ...
Article
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Background The etiology of sudden sensorineural hearing loss is diverse; viral, vascular, immunologic, and abnormal cell stress responses have been proposed, the presentation of the disorder is abrupt, and hearing loss is progressive over a very short period. Steroids remain the treatment of choice irrespective of the etiology of hearing loss. Intratympanic corticosteroid injections have been widely used to deliver corticosteroids directly into the inner ear for those whom systemic steroids have not been successful. Complications such as perforations of the tympanic membrane, myringitis, and otitis media have been reported rarely. Objective To compare the hearing recovery results in patients with sudden sensorineural hearing loss receiving systemic versus intratympanic methylprednisolone acetate. Methods A randomized clinical trial was carried out over 4 years that included 46 patients randomly assigned to two groups of 23 patients each. Pretreatment hearing levels were compared with post-treatment audiograms up to 5 weeks following initial therapy. A 20-dB gain in pure-tone audiometry or a 20% improvement in speech discrimination score was considered a significant improvement. The results of both groups were compared and tested for statistical significance. Results The recovery rate in the systemic group was 65%, whereas the recovery rate in the intratympanic group was 56%; the overall results were comparable over different frequencies. Failure to improve was observed equally in both groups in 21% of patients. Conclusion No statistically significant difference was observed between both the groups. Intratympanic steroid injection as a primary treatment of idiopathic sensorineural hearing loss is an effective alternative to systemic therapy.
... The patients' hearing loss was on the right side in 22 (44%) patients and on the left side in 28 (56%) patients. The absence of side dominance in the studies by Shaia and Sheehy, and Van Dishoeck and Bierman are consistent with the data in our study [20]. ...
... The amount of studies investigating the effect of the degree of hearing loss on the prognosis is quite limited in the literature [7][8][9][10]. In the study conducted by Wilson et al., no complete recovery was achieved in patients with a hearing loss ≥90 dB; the authors concluded that patients with severe hearing loss had a low probability of recovery independent of treatment [20]. ...
Article
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Idiopathic sudden hearing loss (ISHL) is associated with vestibular complaints in 30% of cases. In this study, we aimed to determine the change in caloric test responses in ISHL, determine the degree of influence on the vestibular nerve, investigate the correlation between vestibular nerve damage and vestibular complaints during admission, and evaluate the effect of vestibular nerve influence on the recovery process. Patients with ISHL were divided into groups with and without vestibular complaints and classified according to their hearing level. Pure sound thresholds of 250 to 8000 Hz and caloric responses with air stimulus were recorded via videonystamography. Audiometry and videonistagmography were repeated three months after the end of systemic corticosteroid therapy. The difference between the audiometric and caloric test data according to the level of hearing loss at the time of diagnosis and the difference between the patient groups were examined. 50 patients with idiopathic ISHL were included. The hearing threshold at 8000 Hz frequency in patients with idiopathic hearing loss who had vestibular complaints was found to be more affected. Unilateral weakness in the caloric test was significantly higher in patients with vestibular complaints. In patients with ISHL accompanied by vestibular complaints, higher frequency hearing thresholds are affected to a higher degree. The presence of vestibular complaints suggests that vestibular function is also affected by ISHL. Improvement in high-frequency hearing thresholds is seen at a lower degree in patients with vestibular complaints.
... Various postulated etiological theories have been proposed in the literature, including viral infection, vascular embolism, and metabolic abnormalities (4)(5)(6)(7). Nearly 40%−55% of patients with SSNHL show vestibular symptoms such as dizziness and instability, which can be delayed or occur at the same time with sudden hearing loss (8)(9)(10). This suggests that cochlear impairment and vestibular dysfunction can accompany each other (8,9,(11)(12)(13). ...
... Nearly 40%−55% of patients with SSNHL show vestibular symptoms such as dizziness and instability, which can be delayed or occur at the same time with sudden hearing loss (8)(9)(10). This suggests that cochlear impairment and vestibular dysfunction can accompany each other (8,9,(11)(12)(13). ...
Article
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Objective This study investigates the association between vestibular function and prognosis in patients with unilateral idiopathic sudden sensorineural hearing loss (UISSNHL). Design A retrospective analysis of 64 patients with UISSNHL was performed. Pure tone audiometry and vestibular function tests for otoliths and semicircular canals were performed to assess the influence of vestibular functional status on the outcome of patients with UISSNHL. Results Patients with abnormal cervical vestibular evoked myogenic potential (cVEMP) or ocular vestibular evoked myogenic potential (oVEMP) responded less favorably to treatment. In the ineffective group, cVEMP was normal in four patients (6.3%) and oVEMPs in three (4.7%). Meanwhile, cVEMP was abnormal in 32 patients (50.0%) and oVEMP in 33 (51.6%). Better hearing recovery occurred in those with normal cVEMP (33.76 ± 15.07 dB HL improvement) or oVEMP (32.55 ± 19.56 dB HL improvement), but this was not the case in those with normal caloric tests. Patients with abnormalities in both cVEMP and oVEMP were less responsive to treatment and had worse hearing recovery than those with normal results in only one of the two tests. Conclusion Abnormal oVEMP and/or cVEMP results indicate poor auditory outcomes in patients with UISSNHL. Patients with impaired otolith organ function are likely to have a larger and more severe pathological change in their inner ear.
... The male/female 5 distribution is essentially equal. Bilateral involvement is rare, 6 and simultaneous bilateral involvement is very rare. In general, the causes of SSNHL can be conveniently broken down into infectious, neoplastic, traumatic, ototoxic, immunologic, vascular, developmental, psychogenic, and idiopathic etiologies. ...
... It is difcult to pinpoint the exact cause of SNHL. Contemporary to our study, numerous authors have reported that majority of individuals who come up with sudden SNHL have idiopathic hearing loss because the cause 4,6,14,16 of their hearing loss is unclear. ...
Article
Background: Sudden sensorineural hearing loss (SSNHL) is dened as sensorineural hearing loss of 30 dB or more over at least three consecutive audiometric frequencies that occurs within a 72-hour period. Even though there are several potential causes of SSNHL, however, the majority of patients do not have well established etiology. Methods: The present prospective observational study was conducted in the Post Graduate Department of Otorhinolaryngology and Head and Neck surgery (ORL AND HNS), Government SMHS Hospital Srinagar, which is the associated Hospital of Government Medical College Srinagar & caters patients from whole of the Kashmir division. A total of 115 patients who gave the consent & fullled the inclusion criteria were taken into the study. Results: When the subjects were stratied based on severity of the disorder, ~70% subjects reported mild to moderate SSNHL, while as the rest reported severe forms of SSNHL with ~10% reporting profound SSNHL. Most of cases were idiopathic (57%). Nearly 11% subjects had diabetes mellitus, ~8% had hypertension and an even less number had infectious cause. History of Meinere's Disease was reported in ~4% of subjects and history of fractured temporal bone was presented in ~2% of subjects. Conclusion: The etiology of SSHNL is heterogenic, with majority of cases as idiopathic. For practitioner pinpointing the exact etiology is equally all the more important for targeted treatments. We recommend large sample comprehensive extended studies to further investigate the possible cases associated with this disease.
... The mean age of presentation of our study is similar to other studies. In Shaia and Sheehy study [19] which included 1220 cases three quarter of patients were over the age of 40yrs. The age of onset of symptoms are as follows <30yrs-13%, 30-39yrs-13%, 40-49yrs-21%, 50-59yrs-22%, 60-69yrs-18%, >70yrs-13%. ...
... In Tiong TS et al. [17] study out of 50 patient, 16 patients were found to have associated conditions of diabetes mellitus, seven with hypertension, and 12 with hyperlipidaemia. Chamyal P C et al. [19] found hearing loss mild to moderate and was detected in 40% of diabetes. In our study of the 50 patients, 14 patient had diabetes mellitus of which 2 had hypertension and one was post renal transplant patient. ...
... The hearing loss can develop either instantaneously or over the course of several hours within this time period [2]. Patients almost always report aural fullness and tinnitus as accompanying symptoms, with some experiencing vertigo as well [3][4][5][6][7][8]. SSNHL is typically unilateral, with bilateral involvement occurring less than 2% of the time [9]. ...
... Some have even demonstrated that patients who begin treatment within seven days of onset, have significantly better hearing outcomes than those who start later [2,47]. The importance of treating SSNHL in a timely manner can be appreciated when considering the challenges associated with unilateral hearing loss, such as poor speech understanding in background noise and compromised sound localization, as well as the psychosocial impact on affected individuals [7,43,48]. In addition to hearing recovery, successful treatment of SSNHL usually results in an improvement of the concomitant tinnitus [5,43]. ...
Article
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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. Graphical abstract
... Vestibular schwannoma has been reported as the cause of SSNHL in 1% of patients. 7 However, a study in South Korea by Lee et al 8 observed VS in 4% of patients with SSNHL, a difference that was attributed to the widespread use of MRI. In South Korea, national health insurance covers MRI for SSNHL patients with moderate to severe hearing loss, allowing them to pay approximately US $200 for an MRI, making it a cost-effective modality. ...
Article
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Background Gadolinium-enhanced magnetic resonance imaging (MRI) is the gold standard for diagnosing vestibular schwannoma (VS). This study aimed to compare the incidence of VS among patients with low-frequency, high-frequency, and flat-type sudden sensorineural hearing loss (SSNHL) and to assess the effectiveness of MRI for each SSNHL type. Methods We analyzed 755 patients diagnosed with SSNHL at a single tertiary center between January 2014 and March 2020. All underwent temporal bone MRI. Logistic regression was used to determine the associations of SSNHL types with VS. Additionally, we conducted a correlation analysis to examine the relationship between word recognition scores (WRS) and the size of tumors measured on MRI at the time of diagnosis. Results Magnetic resonance imaging identified VS in 23 of 755 cases (3.0%). Of the patients, 123 (16.3%) had low-frequency SSNHL; none of these patients had VS. The incidence of VS differed significantly between the low-frequency SSNHL group and other SSNHL types (P < .001). A significant negative correlation was found between initial tumor size and WRS (r² = 0.2905, P = .008). Conclusion The prevalence of VS was rare in patients with low-frequency SSNHL. The pattern of hearing loss may provide insights into the likelihood of tumor presence, thereby aiding in more effective audiometric screening and decision-making regarding MRI for detecting VS.
... Sudden sensorineural hearing loss (SSNHL) is defined as a decrease in sensory hearing of 30 dB or more across three contiguous frequencies within 3 days [1]. Due to the close anatomical proximity of the cochlea and vestibule, 28.8%-40% of patients exhibit concomitant vestibular dysfunction, which includes symptoms such as vertigo, dizziness, or unsteadiness [2,3]. In cases of SSNHL with vertigo (SSNHLV), 11.2% of patients report vestibular symptoms that persist for more than 2 months, indicative of labyrinthitis, which significantly affects their quality of life [4][5][6]. ...
Article
Objectives. Our study aimed to explore the role of the potassium channel KCNK1 in head and neck squamous cell carcinoma, focusing on its impact on tumor growth, invasion, and metastasis. We also investigated the therapeutic potential of quinidine, a known KCNK1 inhibitor, in both in vitro cell lines and a zebrafish patient-derived xenograft (PDX) model.Methods. We established primary cell cultures from head and neck cancer tissues and employed the FaDu cell line for in vitro studies, modulating KCNK1 expression through overexpression and knockdown techniques. We evaluated cell migration, invasion, and proliferation. Additionally, we developed a zebrafish PDX model to assess the impact of quinidine on tumor growth and metastasis in vivo. RNA sequencing and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analyses were conducted to elucidate the molecular mechanisms underlying the role of KCNK1 in cancer progression.Results. Overexpression of KCNK1 in FaDu cells resulted in enhanced cell migration and invasion, whereas its knockdown diminished these processes. In the zebrafish PDX model, quinidine markedly inhibited tumor growth and metastasis, demonstrating a significant reduction in tumor volume and micrometastasis rates compared to the control groups. The molecular analyses indicated that KCNK1 plays a role in critical signaling pathways associated with tumor growth, such as the Ras and MAPK pathways.Conclusion. Our findings highlight the critical role of KCNK1 in promoting tumor growth and metastasis in head and neck cancer. The inhibitory effect of quinidine on tumor progression in the zebrafish PDX model highlights the therapeutic potential of targeting KCNK1. These results suggest that KCNK1 could serve as a valuable therapeutic target for head and neck cancer, warranting further investigation into treatments that target KCNK1.
... Sudden sensorineural hearing loss (SSNHL) is defined as a hearing loss of at least 30 dB in a minimum of three contiguous frequencies on a pure-tone audiogram over less than 72 h. 1 Idiopathic sudden sensorineural hearing loss (ISSNHL) has an estimated incidence of 5-20 per 100,000 persons per year 2-4 and occurs with equal incidence in both men and women. 2,3,5,6 However, these data may be inaccurate and underestimate the true incidence of ISSNHL as some affected individuals recover before seeking medical attention. 2,7 Possible etiologies for sudden hearing loss include neoplastic, infectious, autoimmune, neurologic, otologic, metabolic, and vascular causes in addition to ototoxic drugs and trauma, although only 30% of cases have an identified cause. ...
Article
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Objective Although corticosteroids and other treatments for idiopathic sudden sensorineural hearing loss (ISSNHL) have been described, understanding its prognosis without intervention provides valuable information for patient management. The objective of this study is to provide a comprehensive, quantitative statistical analysis of the natural history of untreated idiopathic sudden sensorineural hearing loss (ISSNHL). Study Type and Design A systematic review and meta‐analyses. Methods Two independent searches of PubMed, Scopus, Web of Science, and Cochrane Library databases up to June 30, 2022, were performed. Adults with idiopathic sudden sensorineural hearing loss who received placebo or were untreated and had audiometric outcome measures in all study types were reviewed. These data indicative of the natural history of ISSNHL were analyzed, as were study characteristics related to risk of bias. Heterogeneity as assessed via I² and random effects analyses were performed. Results Six studies meeting the inclusion criteria yielded 319 untreated patients whose natural history could be assessed. Heterogeneity among studies was moderate, with a variety of reported outcomes. A hearing improvement of at least 30 dB HL was observed in 36% (95% CI 0.28–0.44) of untreated patients, and of at least 10 dB HL was observed in 70% (95% CI 0.57–0.82) of untreated patients at 3 months. The mean hearing gain among untreated patients was 24.0 dB HL (95% CI 2.65–45.37) at 2–3 months. Conclusions The observed natural history of ISSNHL suggests that patients can regain some hearing without active treatment. In the absence of future studies collecting prospective natural history data from untreated or placebo‐treated ISSNHL patients, the data presented here provide the best available historical control data for reconsideration of results in past ISSNHL studies, as well as a roadmap for design and interpretation of future ISSNHL treatment clinical trials. Furthermore, knowing there is a statistically significant mean hearing gain of 24.0 dB HL in the untreated/placebo group provides an ethical basis for future placebo study of ISSNHL. The current status on ISSNHL management calls for a multi‐institutional, randomized, double‐blind placebo‐controlled trial with validated outcome measures to provide science‐based treatment guidance. Laryngoscope, 134:S1–S15, 2024
... This study is, to the best of our knowledge, the first report of bilateral sudden hearing loss associated with iron deficiency anemia. Bilateral cases account for approximately 1%-5% of all patients with sudden hearing loss [3,[5][6][7][8], and bilateral involvement has been regarded as a "red flag" sign for a more severe underlying condition. The etiology of unilateral sudden hearing loss has not yet been elucidated, and inner ear vascular compromise, viral infection, autoimmune disease, or rupture of Reissner's membrane has been proposed as a possible etiology [1]. ...
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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.
... Byl [2] reported a complete recovery rate of 56% when patients started treatment within 7 days, but only 27% showed a complete recovery rate when patients started treatment after more than 30 days. Shaia and Sheehy [16] reported that the prognosis was better when patients received treatment within 1 month. The rationale for early intervention lies in the fact that the pathological changes responsible for causing hearing loss can progress to irreversible states over time. ...
Article
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.
... While its incidence is low in the 20-30 year age group, its incidence sees an increase in the 50-60 year age group (14,5,6). Various studies have reported similar sex distributions (7)(8)(9)(10)(11)(12). ...
... In fact, the rate of hearing recovery following audiogram within the first few days of onset is 87%, with a week, 87%, 2 weeks 52%, and 10% or less after 3 months. [30][31][32][33][34][35][36] Many of the office administrative assistants who were contacted as a part of this study appeared to be unaware of the urgency behind a sudden hearing loss diagnosis. Many of those who did urged us to visit the emergency room due to lack of scheduling availability, or attempted to schedule us with a physician extender. ...
Article
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Objective The objective of this study was to quantify the wait times that patients may encounter for common clinical diagnoses when seeking otolaryngology care, while determining whether a wait time disparity exists based on geographic location within Illinois. Methods In November 2022, a list of Illinois otolaryngologists was obtained from www.entnet.org. Using a uniform script, each of the 291 otolaryngologists were contacted. The caller posed as a new patient with either sudden sensorineural hearing loss, a neck mass, or chronic sinusitis. Each clinic was called 3 times and wait times were recorded. One hundred fifty‐eight otolaryngologists were included in the analysis. Results The average statewide wait time for a new patient presenting with sudden unilateral hearing loss, a neck mass, and chronic sinusitis was 18.0, 22.6, and 25.5 days, respectively. There was no statistically significant difference between urban and rural wait times. Discussion Although wait time differences were noted, the lack of urban versus rural p value significance may be attributed to the small sample size (n = 11) of rural otolaryngologists in Illinois. However, the overall wait times in this study were longer compared to those reported in other studies, suggesting that the current number of otolaryngologists in Illinois is inadequate to meet the public need. Implications for Practice We have demonstrated that the current demand for otolaryngology care is outstripping the existing supply in Illinois. This suggests that an emphasis should be placed on training more otolaryngologists, or increasing the use of physician extenders, while incentivizing otolaryngologists to practice in rural areas. Level of Evidence 5
... Normal or complete recovery to functional hearing occurred in 45-65% of patients [30,32]. Identifiable etiologies are noted among 7% to 45% of individuals [3,29,30,[32][33][34][35]; therefore, the majority of cases are idiopathic. Several studies have proposed etiologies for SSNHL that include vascular compromise, cochlear membrane rupture, and viral infection [29,36,37]. ...
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Viral infection serves as the crucial etiology for the development of sudden sensorineural hearing loss (SSNHL). We aimed to investigate whether there is an association between concurrent Epstein–Barr virus (EBV) infection and SSNHL in an East Asian population. Patients who were older than 18 years of age and met the criteria of sudden hearing loss without an identifiable etiology were enrolled from July 2021 until June 2022, followed by the serological testing of IgA antibody responses against EBV-specific early antigen (EA) and viral capsid antigen (VCA) with an indirect hemagglutination assay (IHA) and real-time quantitative polymerase chain reaction (qPCR) of EBV DNA in serum before the treatment was initiated. After the treatment for SSNHL, post-treatment audiometry was performed to record the treatment response and degree of recovery. Among the 29 patients included during enrollment, 3 (10.3%) had a positive qPCR result for EBV. In addition, a trend of poor recovery of hearing thresholds was noted for those patients with a higher viral PCR titer. This is the first study to use real-time PCR to detect possible concurrent EBV infection in SSNHL. Our study demonstrated that approximately one-tenth of the enrolled SSNHL patients had evidence of concurrent EBV infection, as reflected by the positive qPCR test results, and a negative trend between hearing gain and the viral DNA PCR level was found within the affected cohort after steroid therapy. These findings indicate a possible role for EBV infection in East Asian patients with SSNHL. Further larger-scale research is needed to better understand the potential role and underlying mechanism of viral infection in the etiology of SSNHL.
... 4 Vestibular involvement in idiopathic sudden SNHL was first reported in 1949, and its incidence was estimated as being between 30 and 40 per cent. [5][6][7] A significant percentage of patients diagnosed with idiopathic sudden SNHL complain of mild-to-moderate instability, 8 but no evidence of true vertigo is detected by the physical examination for vestibular function in most cases. Although vestibular dysfunction in idiopathic sudden SNHL is hypothesised to be an extension of the condition from the cochlea to the vestibular organs given their anatomical proximity, the relationship and pathogenesis of cochlear dysfunction and vestibular involvement in this setting remain controversial. ...
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Objective Idiopathic sudden sensorineural hearing loss may be accompanied by dizziness without true vertigo. This study used the video head impulse test to evaluate vestibular function in idiopathic sudden sensorineural hearing loss patients who described experiencing dizziness and not true vertigo. Methods A prospective study was conducted of 30 consecutive patients diagnosed with idiopathic sudden sensorineural hearing loss with dizziness without true vertigo. A comparison of the video head impulse test results of the patients who complained of dizziness (symptomatic group) with a group of patients with idiopathic sudden sensorineural hearing loss and no dizziness (asymptomatic) was performed. Results Nine patients (30 per cent) were symptomatic. Two of those patients had abnormal video head impulse test findings. Seven patients in the asymptomatic group (7 out of 21, 33 per cent) presented with abnormal video head impulse test results. No significant difference in vestibular function between the two groups was detected by the video head impulse test. Conclusion The site of insult in patients with idiopathic sudden sensorineural hearing loss without true vertigo is usually limited to the cochlea or the cochlear nerve.
... Furthermore, vertigo develops in approximately 20% to 60% of the patients with ISSNHL (Moskowitz et al., 1984;Park et al., 2001;Rauch, 2008;Pogson et al., 2016;Chang et al., 2018). Vertigo indicated a poor prognosis for hearing recovery as the incidence of severe or profound hearing loss in ISSNHL patients with vertigo was increased (Shaia and Sheehy, 1976;Wang et al., 2009;Kim et al., 2018;Zhou et al., 2018). Chang et al. concluded that sudden hearing loss with vertigo portended greater stroke risk than sudden hearing loss or vertigo alone (Chang et al., 2018). ...
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The underlying pathophysiology of idiopathic sudden sensorineural hearing loss (ISSNHL) with vertigo has yet to be identified. The aims of the current study were (1) to elucidate whether there are functional changes of the intrinsic brain activity in the auditory and vestibular cortices of the ISSNHL patients with vertigo using resting-state functional magnetic resonance imaging (rs-fMRI) and (2) whether the connectivity alterations are related to the clinical performance associated with ISSNHL with vertigo. Twelve ISSNHL patients with vertigo, eleven ISSNHL patients without vertigo and eleven healthy subjects were enrolled in this study. Rs-fMRI data of auditory and vestibular cortices was extracted and regional homogeneity (ReHo) and seed-based functional connectivity (FC) were evaluated; the chi-square test, the ANOVA and the Bonferroni multiple comparison tests were performed. Significantly decreased ReHo in the ipsilateral auditory cortex, as well as increased FC between the inferior parietal gyrus and the auditory cortex were found in the ISSNHL with vertigo groups. These findings contribute to a characterization of early plastic changes in ISSNHL patients with vertigo and cultivate new insights for the etiology research.
... Moreover, vasodilators were shown to be useful in treating blood circulatory disturbances in the internal ears, with regulation of blood flow in the internal ears and cerebrovascular automatic nerve regulation being related to variations in blood pressure. In a double-blind trial of 50 patients with tinnitus due to various causes, no difference was observed between flunarizine and the placebo with respect to the suppressive effect on tinnitus [71][72][73]. ...
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Various medications are currently used in the treatment of tinnitus, including anesthetics, antiarrhythmics, anticonvulsants, antidepressants, antihistamines, antipsychotics, anxiolytics, calcium channel blockers, cholinergic antagonists, NMDA antagonists, muscle relaxants, vasodilators, and vitamins. To date, however, no medications have been specifically approved to treat tinnitus by the US Food and Drug Administration (FDA). In addition, medicines used to treat other diseases, as well as foods and other ingested materials, can result in unwanted tinnitus. These include alcohol, antineoplastic chemotherapeutic agents and heavy metals, antimetabolites, antitumor agents, antibiotics, caffeine, cocaine, marijuana, nonnarcotic analgesics and antipyretics, ototoxic antibiotics and diuretics, oral contraceptives, quinine and chloroquine, and salicylates. This review, therefore, describes the medications currently used to treat tinnitus, including their mechanisms of action, therapeutic effects, dosages, and side-effects. In addition, this review describes the medications, foods, and other ingested agents that can induce unwanted tinnitus, as well as their mechanisms of action.
... The incidence of SSNHL is higher in individuals aged 45-60 years compared with other age groups. The mean age at which individuals develop SSNHL is 43-53 years according to studies on 7,500 patients in Europe, Japan, and the United States [12][13][14] . In this study, the mean age of the patients with SSNHL was 50.0 (±15.9) ...
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Objectives: This study aimed to evaluate the prognostic value of neutrophil/lymphocyte (N/L) and platelet/lymphocyte (P/L) ratios in sudden sensorineural hearing loss (SSNHL) and investigate the effect of combined corticosteroid medical treatment and/or hyperbaric oxygen (HBO) therapy on these values. Materials and methods: In this study, patients with SSHL at our tertiary center were examined retrospectively. A total of 60 patients with SSNHL and 30 healthy individuals as the control group were included. The patient and control groups were compared in terms of N/L and P/L rates. Furthermore, 60 patients were divided into 2 equal groups (n=30) on the basis of whether they received HBO in addition to combined corticosteroid treatment. Results: The N/L and P/L rates were significantly higher in the patient groups than in the control group at the time of diagnosis and significantly decreased after treatment in the patient groups (p<0.05). It was observed that HBO therapy lowered the N/L and P/L rates more than the HBO-free group, but the statistically significant decrease was only in N/L ratio (p<0.05). Conclusion: The N/L and P/L rates were higher in the patient groups than in the control group, and there was also a significant decrease in the 2 values after treatment. This was an important finding showing that SSNHL has a possible underlying inflammatory and vascular (ischemic) condition. We also found that the higher the P/L ratio, the lower the recovery rate from hearing loss. This finding suggests that the P/L ratio can be an important prognostic indicator in patients with SSNHL.
... Puretone audiometry and MRI should be considered to rule out vestibular schwannoma or other intracranial causes such as multiple sclerosis and small vessel ischemic changes. The prevalence of acoustic neuroma among patients with sudden SNHL is less clear, although estimates range from 0.8% [11]. ...
Chapter
This chapter covers sensorineural hearing loss disorders. Sensorineural hearing loss is considered one of the major causes of hearing impairment that affects the quality of life and ability to communicate with others which can affect human well-being and performance. The clinical and audiological features of sensorineural hearing loss were discussed in this chapter. The etiology of sensorineural hearing impairment is divided into two major categories: congenital (hereditary, non-hereditary, and idiopathic) and acquired. The most common cause of hereditary congenital sensorineural hearing loss is non-syndromic autosomal-recessive hearing loss. The most common acquired cause of sensorineural hearing loss is presbycusis, and it has four types which have been reviewed in this chapter. Ototoxicity is one of the acquired causes as well, and the physicians should be aware of these ototoxic medications to avoid their harmful hearing loss effect which can be irreversible. Other acquired etiologies including autoimmune inner ear disease and sudden sensorineural hearing loss are discussed in detail. At the end of this chapter, auditory rehabilitation with hearing aids is covered along with its different types.
Article
Background Tinnitus, a common and troublesome symptom of idiopathic sudden sensorineural hearing loss (ISSNHL), is the perception of sound without any external or internal sound source. The etiology of ISSNHL accompanied by tinnitus remains unclear. Tinnitus often occurs alongside or before hearing loss and can severely impact patients’ quality of life, leading to psychological stress and emotional distress. Current treatments primarily focus on improving hearing loss while neglecting tinnitus treatment. Electroacupuncture (EA), a technique widely applied in China for tinnitus management, may become a promising intervention by addressing both auditory and psychological symptoms. Objective This study aims to conduct a double-center, assessor-blinded, prospective randomized pilot clinical trial to explore the potential effects and feasibility of EA combined with medication for patients with ISSNHL accompanied by tinnitus, and to generate preliminary data to inform future large-scale trials of EA as an adjunctive therapy for this condition. Methods In this double-center, assessor-blinded, randomized controlled trial, 60 patients with ISSNHL accompanied by tinnitus will be randomly allocated to either the EA group, receiving EA combined with medication, or the control group, receiving standard medication treatment, for a total treatment duration of 4 weeks. Follow-up assessments will be conducted at weeks 2 and 4, and at 1, 2, and 3 months after treatment. The feasibility primary outcomes are recruitment rate, adherence rate, data completeness, and safety outcomes. The primary outcome measures will include pure tone audiometry, tinnitus matching, and the Tinnitus Handicap Inventory, while the secondary outcome measures will comprise speech-recognition thresholds in noise, the Self-Rating Anxiety Scale, and the 36-item Short Form Health Survey Questionnaire. Results This study began recruitment on July 15, 2024, and is scheduled to conclude on August 31, 2025. To date, 42 participants have been enrolled, with 35 having completed the intervention and posttreatment assessments (control group: n=21; EA group: n=14). Following protocol publication, data compilation and analysis will be conducted, with results anticipated to be published in a relevant journal in 2026. Conclusions This pilot trial is expected to provide critical insights into the feasibility of integrating EA with standard medication for managing ISSNHL with tinnitus. By generating preliminary evidence on its practicality and potential benefits, this study aims to inform the design and sample size estimation of future multicenter trials, potentially advancing nonpharmacological treatment options for this challenging condition. Trial Registration Chinese Clinical Trial Registry ChiCTR2400086802; https://tinyurl.com/mtxthpzj International Registered Report Identifier (IRRID) DERR1-10.2196/69163
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Two hundred and four patients with audiometric and vestibular findings suggestive of a retrocochlear lesion were studied prospectively by computed tomographic scan with air contrast of the internal auditory canal. With this technique tumor limits are clearly defined. In the absence of disease the internal auditory canal has been completely air-filled in over 97% of cases. Soft tissue masses occluding the internal auditory canal were demonstrated in 11 patients. Four studies were inconclusive and there was one false positive. Headaches have been the only side effect and there have been no complications. In reporting their results the authors examine the indications, accuracy and limitations of this technique. This study demonstrates that computed tomographic scan with air contrast of the internal auditory canal should be considered as the radiologic procedure of choice for the evaluation of small acoustic tumors.
Chapter
Sudden sensorineural hearing loss is considered an otologic emergency in which a hearing loss of 30 dB or more is observed at three consecutive frequencies, usually in one ear, over a period of 72 hours or less. Most cases are idiopathic. The hearing loss may be accompanied by a sensation of fullness, tinnitus, and dizziness. The male/female distribution is similar, and although it may occur in any age group, the highest incidence has been reported in the sixth decade of life. Because of the high rate of spontaneous recovery, the true incidence may be higher than estimated. The rate of spontaneous recovery varies from 32% to 65%. Recovery is usually observed within the first 2 weeks after the onset of hearing loss, and if there is no complete recovery by the third month, the probability of recovery is extremely low.
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The sudden sensory neural hearing loss is a condition with considerable incidence and confers some risk factors behind. Use of systemic steroids is effective for the condition but with several adverse effects, so; use of local intratympanic injection of steroid is safer option warrants efficacy confirmation. This trial included 60 patients with the condition allocated into three arms according to steroid treatment; oral, local and combined. The patients were compared before and after and were assessed with pure tone audiometry. The result show statistically significant difference for each arm in before and after testing but not inter-groups comparison. This study adds evidence to enhance the use of steroid as an intra-tympanic topical therapy in managing sudden sensory neural hearing loss.
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Introdução: A surdez neuro-sensorial súbita está associada a acufenos em até 90% dos casos. Os acufenos constituem uma queixa subjetiva, tornando a sua avaliação difícil. A oxigenoterapia hiperbárica (OHB) é utilizada como terapêutica adjuvante nos casos refratários ou com resposta parcial a corticoterapia sistémica, mas o seu efeito no acufeno acompanhante está pouco estudado. Objectivos: Avaliar o efeito da OHB na evolução dos acufenos e dos limiares auditivos em doentes com surdez neuro-sensorial súbita e acufeno associado, refratários à corticoterapia sistémica. Métodos: Foi realizado um estudo prospetivo não controlado de forma a avaliar o efeito da OHB na evolução dos acufenos, aplicando o Tinnitus Handicap Inventory (THI) antes e após o tratamento com OHB. O estudo decorreu no Centro de Medicina Subaquática e Hiperbárica da Marinha Portuguesa, entre Maio e Dezembro de 2018. O protocolo de OHB incluiu em média 20 sessões a 2.5ATM durante 90 minutos. O trabalho de análise estatística foi realizado com recurso ao software IBM SPSS Statistics 26. Resultados: Foram incluídos no estudo 57 doentes, com idade entre 25-78 anos (média 51A), 50,8% do sexo feminino. O intervalo de tempo médio entre o aparecimento dos sintomas e início de OHB foi de 34,9 dias. Foi possível estabelecer uma relação inversa estatisticamente significativa entre o intervalo sintomas-tratamento e a melhoria auditiva (p=0,045). Verificou-se uma melhoria média de 17 dB na acuidade auditiva. Segundo os critérios de recuperação auditiva de Spiegel, verificou-se melhoria completa em 19% dos doentes, melhoria parcial em 23%, melhoria ligeira em 33% e ausência de melhoria em 25% dos doentes. Quanto ao acufeno, a maioria dos doentes (71%) obteve classificação 3 ou 4 antes do tratamento. Após tratamento, 77% dos doentes apresentaram THI de grau inferior a 3. Observou-se melhoria da classificação THI mesmo em doentes sem melhoria audiométrica. Conclusão: Estes dados permitem concluir que, além da melhoria na acuidade auditiva, a oxigenoterapia hiperbárica parece ter um efeito positivo na melhoria do impacto do acufeno na qualidade de vida dos doentes. Os doentes devem ser referenciados a tratamento com OHB assim que possível, uma vez que o intervalo entre sintomas e início de tratamento tem um efeito significativo na melhoria audiométrica.
Article
Objective This study evaluates intratympanic lidocaine's efficacy and safety for tinnitus relief in sudden sensorineural hearing loss (SSNHL) patients. Methods In a double-blind randomized controlled trial, 100 SSNHL patients with unilateral tinnitus received either intratympanic lidocaine or saline plus usual care. Treatment impact was assessed at 1 and 3 months using the Tinnitus Handicap Inventory, subjective visual analog scale, pure-tone audiometry. Results The lidocaine group demonstrated significant tinnitus relief according to the Tinnitus Handicap Inventory and visual analog scale, without pure-tone audiometry improvement or serious adverse events throughout the study period. Conclusion Intratympanic lidocaine provides a safe, efficacious treatment option for SSNHL tinnitus. Further studies should refine the dosage and delivery parameters because of SSNHL's heterogenous nature.
Article
Abstract Background Intratympanic steroids (ITS) in treating sudden sensorineural hearing loss (SSNHL) have become more widespread. Aim This study investigates whether ITS treatment provides additional benefits when combined with systemic steroids (SS) in patients with severe and profound SSNHL. Materials and methods Patients diagnosed with severe and profound SSNHL were divided into two groups: SS group and SS combined with ITS group. Pure-tone audiometry was performed before and three months after treatment completion. The pure-tone average (PTA), frequency-specific hearing gains, and average values were compared between the groups. Results The study included 57 patients (27 SS, 30 ITS), with a mean age of 50.09 ± 15.56. Before treatment: SS PTA 84.40 ± 15 dB HL, ITS 87.50 ± 9.38 dB HL (p = 0.36). After treatment: SS 62.2 ± 23.13 dB HL, ITS 65.17 ± 12.19 dB HL (p = 0.55). Average hearing gain: SS 22.19 ± 13.81 dB HL, ITS 22.33 ± 12.24 dB HL (p = 0.96). Frequency-specific gains were similar (p > 0.05). SS group: 12 slight improvement, 10 no improvement, 3 partial, 2 complete recovery. ITS group: 23 slight improvement, 6 no improvement, 1 partial. Conclusion In our study, combining ITS with SS treatment did not provide additional benefits in treating severe and profound SSNHL.
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Artigo recebido a 7 de Abril 2022. Aceite para publicação a 27 de Outubro de 2022. Autores Introdução: A surdez neuro-sensorial súbita está associada a acufenos em até 90% dos casos. Os acufenos constituem uma queixa subjetiva, tornando a sua avaliação difícil. A oxigenoterapia hiperbárica (OHB) é utilizada como terapêutica adjuvante nos casos refratários ou com resposta parcial a corticoterapia sistémica, mas o seu efeito no acufeno acompanhante está pouco estudado. Objectivos: Avaliar o efeito da OHB na evolução dos acufenos e dos limiares auditivos em doentes com surdez neuro-sensorial súbita e acufeno associado, refratários à corticoterapia sistémica. Métodos: Foi realizado um estudo prospetivo não controlado de forma a avaliar o efeito da OHB na evolução dos acufenos, aplicando o Tinnitus Handicap Inventory (THI) antes e após o tratamento com OHB. O estudo decorreu no Centro de Medicina Subaquática e Hiperbárica da Marinha Portuguesa, entre Maio e Dezembro de 2018. O protocolo de OHB incluiu em média 20 sessões a 2.5ATM durante 90 minutos. O trabalho de análise estatística foi realizado com recurso ao software IBM SPSS Statistics 26. Resultados: Foram incluídos no estudo 57 doentes, com idade entre 25-78 anos (média 51A), 50,8% do sexo feminino. O intervalo de tempo médio entre o aparecimento dos sintomas e início de OHB foi de 34,9 dias. Foi possível estabelecer uma relação inversa estatisticamente significativa entre o intervalo sintomas-tratamento e a melhoria auditiva (p=0,045). Verificou-se uma melhoria média de 17 dB na acuidade auditiva. Segundo os critérios de recuperação auditiva de Spiegel, verificou-se melhoria completa em 19% dos doentes, melhoria parcial em 23%, melhoria ligeira em 33% e ausência de melhoria em 25% dos doentes. Quanto ao acufeno, a maioria dos doentes (71%) obteve classificação 3 ou 4 antes do tratamento. Após tratamento, 77% dos doentes apresentaram THI de grau inferior a 3. Observou-se melhoria da classificação THI mesmo em doentes sem melhoria audiométrica. Conclusão: Estes dados permitem concluir que, além da melhoria na acuidade auditiva, a oxigenoterapia hiperbárica parece ter um efeito positivo na melhoria do impacto do acufeno Resumo
Article
Objectives: To review the historical circumstances that led to the emergence of corticosteroid therapy for idiopathic sudden sensorineural hearing loss (ISSNHL) and to discuss how this history has influenced current perspectives on the condition. Methods: PubMed and Google scholar were used to identify articles of ISSNHL and oral corticoid steroid use. Historical articles accessed through our institutional medical library were also reviewed. Results: The use oral corticosteroids as a treatment for ISSNHL was seemingly influenced by three key historical circumstances that, together, provided the substrate for the treatment's use in ISSNHL. First, ISSNHL was a frustrating condition with uncertainty regarding its etiology and few reliable treatment options. Second, the discovery of corticosteroids was awarded the Nobel Prize in 1950, which led to widespread application of this therapy. Third historical circumstance was the evolution and emergence of more rigorous methodological study designs in clinical research. In 1980, these events culminated in a double-blind study evaluating the effectiveness of oral steroids for treatment of ISSNHL. Interestingly, this study is often misrepresented as a randomized controlled trial, which ultimately contributed to adoption of a new standard for treatment in ISSNHL. Research subsequent to these historical events has challenged the notion of corticosteroids as a gold standard but has not altered the historically established paradigm of corticosteroid treatment. Conclusions: The use of steroids as a treatment for ISSNHL evolved from our specialty's need to address a complex condition, a novel therapeutic discovery, and a landmark study that met emerging methodological standards. Despite these strong historical foundations, ISSNHL remains a condition with an unknown etiology and the therapeutic value of corticosteroids remains unpredictable despite their gold standard label.
Article
Background and Objectives:From time to time, we encounter patients with sudden sensorineural hearing loss (SSNHL) whose hearing loss is aggravated during treatment. We analyzed the clinical characteristics and treatment outcome of this particular group of patients. Subjects and Method:Among 194 patients who were diagnosed with sudden sensorineural hearing loss from Jan. 2002 through Jul. 2007, we focused on 17 patients who had an additional 15 dB or more hearing loss within the 7 th day of systemic steroid treatment. We enrolled this group of patients in the ADT (aggravation during treatment) group. The remainder of 175 patients served as a control (non-ADT group). We compared the demographic details, type of audiogram, degree of hearing loss, treatment outcome between the ADT group and the non-ADT group. Results:There was no significant demographic difference between the ADT group and non-ADT group. However, they revealed a difference in the type of audio-gram. While the ADT group showed a predominance of ascending type audiogram, the non-ADT group showed a predominance of flat type audiogram. Degree of hearing loss was also significantly different between the two groups:the ADT group revealed relative mild hearing loss while non-ADT group showed relative severe hearing loss. The treatment outcome was significantly better in the non-ADT group compared to the ADT group. In addition, the possibility of later development of Meniere's disease was significantly higher in the ADT group. Conclusion:The clinical characteristics and treatment outcome was quite different in the ADT groups compared to the non-ADT group. This may imply that ADT group is related with a slightly different patho-physiology over against the conventional SSNHL and that SSNHL may not be a single disease entity. (Korean J Otorhinolaryngol
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Idiopathic sudden-sensorineural hearing loss (ISSHL) is an emergent otological condition needing prompt treatment for better recovery. In our study we aimed to determine the efficacy of intra-tympanic dexamethasone therapy after inserting a grommet in postero-inferior quadrant of the tympanic membrane for dexamethasone instillation. This is a prospective cohort study of 31 patients of ISSHL, in whom grommet was inserted and dexamethasone drops were instilled for 5 days. Several factors such as time of initiation of therapy, age of patient was considered, and inferences were drawn. Auditory outcomes were divided into low, mid and high frequency and the results were tabulated. Paired t tested was applied for all frequencies for both pre-test and post-test. P-value was (<0.05) in all the three ranges of frequencies. Also, statistical significance was found between early treatment from onset of disease and auditory outcome. The earlier the therapy was initiated, better were the results. Key-words: Intratympanic steroid, Dexamethasone, Sudden-sensorineural hearing loss, Grommet
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Corticosteroids, oral or transtympanic, remain the mainstay for inner ear diseases characterized by hearing fluctuation or sudden changes in hearing, including sudden sensorineural hearing loss (SSNHL), Meniere’s disease (MD), and autoimmune inner ear disease (AIED). Despite their use across these diseases, the rate of complete recovery remains low, and results across the literature demonstrates significant heterogeneity with respect to the effect of corticosteroids, suggesting a need to identify more efficacious treatment options. Previously, our group has cross-referenced steroid-responsive genes in the cochlea with published single-cell and single-nucleus transcriptome datasets to demonstrate that steroid-responsive differentially regulated genes are expressed in spiral ganglion neurons (SGN) and stria vascularis (SV) cell types. These differentially regulated genes represent potential druggable gene targets. We utilized multiple gene target databases (DrugBank, Pharos, and LINCS) to identify orally administered, FDA approved medications that potentially target these genes. We identified 42 candidate drugs that have been shown to interact with these genes, with an emphasis on safety profile, and tolerability. This study utilizes multiple databases to identify drugs that can target a number of druggable genes in otologic disorders that are commonly treated with steroids, providing a basis for establishing novel repurposing treatment trials.
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Patients with acoustic neuroma may have sudden sensorineural hearing loss. Most patients with sudden hearing loss seek medical attention promptly, but the diagnosis of an acoustic neuroma may be delayed for months or years because sudden hearing loss is an unusual initial symptom of an acoustic neuroma. In a retrospective review of 836 cases of sudden hearing loss, we found 13 patients with acoustic neuromas. The prevalence of acoustic neuromas for those screened with auditory brain stem response or magnetic resonance imaging was 2.5%. In addition to these 13 patients, 79 acoustic neuroma patients treated in our clinic had well-documented sudden hearing loss as the initial symptom. Hearing loss in these 92 patients ranged from mild to profound. Associated symptoms of pain, facial paresthesia, or unilateral tinnitus preceding the sudden hearing loss were suggestive of an acoustic neuroma, as was a midfrequency (U-shaped) hearing loss. A history of other diseases or events that might explain the sudden hearing loss, a normal electronystagmogram, or recovery of hearing does not eliminate the possibility of a tumor. Because there are no clinical findings that clearly distinguish those patients with acoustic neuromas from other patients with sudden hearing loss, we recommend either an evaluation with auditory brain stem response or gadolinium-enhanced magnetic resonance imaging for any patient with sudden hearing loss.
Article
Ideally, clinicians recommend diagnostic tests when the patient's risk of disease is sufficient to justify putting numerous similar patients through the morbidity required to diagnose disease in one patient. In the case of acoustic tumor diagnosis, there are few published data available to the clinician to help assess risk in an individual patient. The purpose of this study was to obtain information by an opinion poll of a group of experts. We used the Delphi method to poll clinicians trained at the House Ear Clinic. We asked these experts 20 questions related to acoustic tumor diagnosis. Some of the expert opinion presented herein is the only data related to acoustic tumor diagnosis available to clinicians. These data are a first step in elevation of decision-making for tumor diagnosis above the level of speculation. However, the experts, responses displayed a pattern of inaccuracy that limits the clinical application of their opinion. Exposing this pattern was instructive for identifying desirable features of protocols for diagnosing tumors. We recommend that protocols not depend on clinicians estimating probability of tumor. Instead, protocols may list specific findings, such as unilateral distortion on the telephone, to indicate, when present, that the risk of tumor is sufficient to order a diagnostic test.
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It has long been recognized that sudden hearing loss (SHL) may be a harbinger of vestibular schwannoma (VS). Among 192 VS patients who underwent operation in the Gruppo Otologico, Piacenza, Italy, from April 1987 to October 1995, the charts of 14 (7.3%) cases with a history of SHL were examined. SHL was the first symptom in 8 (4.2%) patients. Eight (57.1%) of 14 VS cases with SHL anamnesis had reported recovery of their previous hearing either totally or partially before establishment of tumor diagnosis. Five (35.7%) cases had recurrent bouts of SHL. SHL was observed less frequently in cases with large tumors (<3 cm). However, the frequency of SHL in patients with small tumors did not differ from that of medium-sized tumors. Awareness about coexistence of SHL and VS, as well as concomitant use of auditory brain stem response and magnetic resonance imaging, is crucial to rule out the diagnosis of VS in a patient with SHL.
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Purpose We evaluated the clinical significance of magnetic resonance imaging (MRI) findings and their prognostic value for initial hearing loss and recovery in patients with sudden sensorineural hearing loss (SSNHL). Materials and Methods This retrospective study included consecutive adult patients with unilateral SSNHL, contrast‐enhanced MRI and audiometric testing evaluated in our institution between 2005 and 2017. MRI reports, patient data, treatment, and audiometric tests were reviewed, with the relationship between MRI findings and hearing loss/recovery analyzed. Results Overall, 266 patients were included. Additional symptoms comprised tinnitus (114/266; 43%), vertigo (45/266; 17%), ear pain (26/266; 10%), and ear pressure (6/266; 2%). At least one cardiovascular risk factor (hypertension, diabetes, hypercholesterolemia, cardiopathy, and active smoking) existed in 167/266 (63%) patients. Corticosteroid treatment was followed by 198/266 (74%) patients while contraindications/refusal/compliance precluded treatment in 68/266(26%). Complete, partial or slight hearing recovery occurred in 167/266 (63%) patients. Three MRI patient groups were identified: a group with normal MRI examinations or incidentalomas (128/266; 48%), a group with peripheral auditory system (PAS) lesions (95/266; 36%), and a group with central nervous system (CNS) lesions (43/266; 16%). PAS lesions included lesions from the cochlea to the brain stem (e.g., schwannoma, meningioma, labyrinthitis, intracochlear hemorrhage, vestibulocochlear neuritis), whereas CNS lesions corresponded in 42/43(98%) of cases to leukoaraiosis and other vascular lesions (e.g., stroke, hemorrhage, aneurysm, venous sinus thrombosis, and cavernoma). Belonging to one of the three MRI groups did not influence the degree of initial hearing loss, affected frequencies or treatment, p > .05. Gender and cardiovascular risk factors did neither affect initial hearing loss nor recovery. However, age > 70 years negatively affected initial hearing loss in all frequencies, as well as recovery in all frequencies except 1000 Hz. Also, poor recovery of initial high‐frequency hearing loss (>1000 Hz) was significantly associated with CNS lesions. Conclusion Age > 70 years and CNS lesions depicted by MRI independently predicted poor auditory recovery, albeit in different frequencies. Lay Summary In patients with sudden hearing loss, older age (above 70 years) predicts poorer hearing recovery than in younger patients in most hearing frequencies. In addition, abnormalities of brain tissue revealed by MRI predict poorer hearing recovery at high frequencies. Level of Evidence Level III.
Chapter
Sudden sensorineural hearing loss (SSNHL) is defined as a hearing loss of at least 30 dB that occurs within 72 h at three contiguous frequencies. Although this is the general acceptance, there is no consensus regarding the degree of hearing loss and the duration of its occurrence [1]. Bilateral involvement is rare. Accompanying symptoms may include fullness in the affected ear, varying degrees of tinnitus, dizziness, and impaired balance [2, 3]KeywordsSudden hearing lossInner earOtolaryngologyPregnancyPostpartum period
Article
Purpose: To evaluate the effect of hyperbaric oxygen therapy (HBOT) for idiopathic sudden sensorineural hearing loss (ISSNHL) on central macular thickness (CMT) and choroidal thickness (CT). Materials and methods: The study included 42 healthy eyes of 21 patients with ISSNHL (aged 24-61 years) who started HBOT within the first three days of the onset of hearing loss. Duration and severity of hearing loss were noted before starting HBOT. Central macular thickness (CMT), choroidal thickness (CT) 1500 µm nasal and temporal of the fovea, and subfoveal CT were measured by spectral domain optic coherence tomography before the first session of HBOT and after sessions 10 and 20. measurements obtained before and after HBOT were compared. Results: Eleven patients (52.4%) were men and 10 (47.6%) were women. The mean age was 44.67 ± 10.1 years. The mean duration of sudden hearing loss before HBOT was 2.05 ± 1 day. Hearing loss was mild in 5 patients, moderate in 5 patients, moderate to severe in 2 patients, severe in 4 patients and profound in 5 patients. Comparison of measurements obtained before HBOT and after 10 and 20 sessions of HBOT revealed no significant differences in CMT (219.17 ± 22.91, 220.33 ± 19.66, and 220.21 ± 19.3 µm), subfoveal CT (347.71 ± 66.82, 348.38 ± 74.55, and 345.45 ± 75.39 µm), nasal CT (328.64 ± 82.31, 316.02 ± 79.32, and 313.52 ± 89.92 µm), or temporal CT (321.76 ± 71.29, 317.05 ± 73.94, and 314.05 ± 74.61 µm, respectively) (p > 0.05). Conclusions: HBOT for the treatment of ISSNHL had no significant effect on CMT or CT in healthy eyes.
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Objective: Sudden idiopathic sensorineural hearing loss (SISHL) is defined as an audiological emergency and although many studies have been conducted on the factors affecting prognosis, there is no consensus yet. The aim of this study is to analyze the prognostic value of clinical and audiological factors in patients with SISHL. Patients and Methods: The data of 210 patients, 118 male and 92 female, who were treated for SISHL, were analyzed retrospectively. Demographic data, audiometry findings, additional symptoms and diseases of the patients were recorded. Degree of hearing loss; were classified as mild (26-40 dB), moderate (41-55 dB), moderate-severe (56-70 dB), severe (71-90 dB) and deep (> 90 dB) according to pure tone audiometry test. The type of hearing loss was determined as descending type, ascending type, midfrequency type and flat type. The patients were divided into three groups as those who started treatment in the first 3 days, between 3-10 days and after 10 days. The effects of age, gender, audiometric findings, additional symptoms and diseases on pre-treatment hearing level, post-treatment hearing level and hearing gain levels were evaluated. Results: The median age of the patients was 46.0 (18.0) years. The most common flat audiogram (54.8%) was seen in the patients. It was observed that patients with flat type audiograms had more hearing loss and less hearing gain. It was determined that hearing gain was higher in patients with severe hearing loss (p < 0.05). There was no significant difference in the pre-treatment and post-treatment hearing levels (p; 0.051 and 0.409, respectively) according to the treatment initiation time, but there was a significant difference in the hearing gain levels (p = 0.005). In patients who started treatment in the first 3 days, the gain was higher than those who started after 4-10 days and 10 days. It was observed that there was no significant difference in pre-treatment and post-treatment hearing levels and hearing gain levels according to gender, affected ear direction, additional findings and presence of diseases. Conclusion: Early initiation of treatment was found to be the most important prognostic factor in SISHL. Raising public awareness for early diagnosis and treatment will reduce the sequelae that may occur due to SISHL.
Article
Objectives: This study was conducted to explore the effectiveness of hyperbaric oxygen (HBO) in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL) and recommend the appropriate course of treatment. Methods: 102 patients (105 diseased ears) with ISSNHL were recruited from the Department of Neurology and Otorhinolaryngology, West China Fourth Hospital, Sichuan University, between January 2018 and September 2020. Of them, 45 patients (group A) received intravenous steroid (IVS), and the remaining patients (group B) received IVS and HBO therapy (HBOT). Pure-tone audiometry (PTA) was performed twice at baseline and 10 days after treatment. Patients in group B were subdivided into group 1 (≤10 sessions) and group 2 (>11 sessions) to verify the correlation between the efficacy and course of HBOT, at the follow-up endpoint, the PTA was performed again. The multivariate logistical regression model was used to analyze the related factors of prognosis. Results: Compared with the control group, significantly larger hearing gains and better hearing recovery rate were observed in the IVS + HBOT group (p < 0.05). The time of treatment and course of HBOT were significantly correlated with the hearing threshold after treatment (p < 0.05) and had no significant relationship with tinnitus and age (p > 0.05). Conclusion: HBOT + IVS is an effective method for ISSNHL, especially for the recovery of low-frequency hearing and initial hearing levels of severe and profound. Tinnitus is the most common concomitant symptom of ISSNHL, and prolonging the course of HBOT did not significantly improve it. Initiating HBOT within 7 days for 10-25 sessions of treatment was more beneficial.
Article
Objective: Idiopathic sudden sensorineural hearing loss (ISSNHL) may not only impair cochlear function but also damage vestibular structures, including the saccule, utricle, semicircular canals, and vestibular afferents. Poor recovery often occurs in patient with serious ISSNHL and accompanying vertigo. To define the severity of the disease and to predict prognosis, galvanic vestibular-evoked myogenic potentials (VEMPs) are added to the inner ear test battery. Study design: Retrospective case review. Setting: Tertiary referral center. Patients: Thirty patients with unilateral severe to profound ISSNHL and accompanying vertigo were enrolled in this retrospective study. Intervention: Diagnostic. Main outcome measures: All subjects underwent pure tone audiometry, cervical and ocular VEMPs, and caloric tests before the initiation of treatment. The treatment outcome, rates of abnormal responses in the tests, and the characteristic parameters of VEMPs such as latencies and amplitudes, were analyzed. Results: In affected ears, the rates of abnormal acoustic cVEMPs, vibratory oVEMPs, galvanic cVEMPs, and galvanic oVEMPs were 60, 47, 37, and 20%, respectively. The improvement in the hearing of the affected ear was specified as good recovery or poor recovery. The normal galvanic VEMP group had a significant higher rate of good recovery than abnormal galvanic VEMP group (87% versus 27%; p = 0.003). Conclusions: Patients with unilateral severe to profound ISSNHL and accompanying vertigo who have normal galvanic VEMPs have a higher likelihood of hearing recovery than those who have abnormal galvanic VEMPs.
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EIGHTH nerve symptoms, with rare exceptions, are the first indication of a developing acoustic neuroma. A high index of suspicion combined with a thorough neuro-otologic evaluation should make it possible to diagnose this tumor when it is relatively small; hopefully, when still localized to the internal auditory canal.The purpose of this section of the monograph is to describe the neuro-otologic evaluation and discuss the symptoms produced by this histologically benign but clinically malignant tumor.Symptomatology An acoustic tumor produces symptoms and findings by pressure on both blood vessels and nerve tissue and, when quite large, by blocking the cerebrospinal fluid (CSF) absorption mechanism. When resulting from interference with blood supply, these symptoms and findings may be of dramatically sudden onset, simulating a cochlear or inner ear problem, or basilar vertebral insufficiency. As such, they may fluctuate or apparently respond to medical treatment.The symptoms resulting from pressure on neural
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Idiopathic sudden deafness, generally unilateral and always sensorineural, is more common than generally recognized. The site of the lesion producing deafness may be either cochlear or neural (retrocochlear), and it is important to attempt to try to distinguish, by means of audiometric examination, one locus from the other. The causes generally are believed to be either vascular or viral. Recently there is evidence to suggest that the two causes need not be separate or mutually exclusive but, in fact, a viral etiology may cause certain vascular changes that result in a reduced blood flow to the cochlea. There is no consensus concerning proper treatment for sudden deafness and about one-third of the otolaryngologists replying to a questionnaire said they were not certain that any treatment helps; but because the only alternative is no treatment, most physicians will want to offer treatment. A brief plan is given using the site of the lesion as a prognostic and therapeutic guide.
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Reexamination of accepted techniques of histamine therapy for episodic vertigos in the light of clinical experience and recent laboratory studies suggests than an optimum dosage of exogenous histamine may be of therapeutic value in selected cases. Patients surmised to be capable of manifesting an abnormal response to stress by local microcirculatory dysfunction are, theoretically, favorable candidates for such therapy. Microvascular dysfunction of the cochlear or vestibular labyrinth may or may not contraindicate intravenous histamine therapy.
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In addition to the CLASSIC-CROS hearing aid, originally intended only for persons with unilateral hearing loss, there are now ten versions: MINI-CROS, HI-CROS, FOCAL-CROS, POWER-CROS, BICROS, OPEN-BICROS, UNI-CROS, MULTI-CROS, IROS, and FROS. As a consequence, a greater variety of hearing impairment can be accommodated with wearable amplification. Thus, today more hearing-impaired persons are able to use a hearing aid who otherwise might not, and still others are achieving greater satisfaction from wearable amplification. Most noticeable are persons with asymmetrical hearing loss and those with bilateral high-frequency hearing impairment. We suggest certain guidelines for selecting an appropriate form of amplification on the basis of audiometric configuration and the difference in hearing between the two ears.
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To the 28 cases of rupture of the inner ear windows reported during the past five years, 19 cases are now added. Many have resulted from accidental trauma or barotrauma, with initial manifestation of gait disturbance, ataxia, episodic vertigo, and sensorineural hearing loss of varying severity. The finding of positional nystagmus, a positive Romberg or fistula sign with a sensory hearing loss makes the diagnosis of an inner ear window rupture most probable. Surgical exploration of the middle ear is necessary to identify and close the fistula.