Evaluation of super-high-dose steroid therapy for sudden sensorineural hearing loss.

OBJECTIVE: To determine the efficacy of a high-dose steroid regimen for patients with sudden sensorineural hearing loss (SSNHL) and to evaluate the relationship between outcome and initial steroid dose. STUDY DESIGN AND SETTING: We conducted a retrospective study of 112 patients presenting to Saitama Medical Center. Patients received tapering courses of hydrocortisone (HC) with an initial dose of either 1200 mg or 600 mg. RESULTS: More than 87% of patients had recovery of hearing. When hearing stabilized, there were no significant differences between the 1200 mg and 600 mg groups in terms of hearing outcome (P>0.05). However, at completion of treatment, the 1200 mg group exhibited significantly superior complete recovery rate and improvement rate (P<0.05). CONCLUSION: Application of our treatment protocol of a tapered course of 1200 mg HC significantly and rapidly improves recovery outcomes in patients of SSNHL. EBM rating: C-4.     

Transtympanic steroids for treatment of sudden hearing loss.

OBJECTIVES: To determine whether transtympanic steroid administration may be an effective treatment for sudden onset sensorineural hearing loss (SSNHL) in patients for whom systemic steroid treatment has failed or who were not candidates for systemic steroids. METHODS: The standard medical regimen for SSNHL usually involves systemic steroid therapy. Unfortunately, some patients do not respond successfully to or are poorly tolerant of systemic steroids. Transtympanic administration of steroids has been suggested as an alternative to systemic therapy. A prospective study was designed to evaluate the hearing outcomes in SSNHL patients treated with transtympanic steroids. Patients received transtympanic steroids if oral steroids had failed to work or if they were not able to tolerate oral steroids. Transtympanic steroids were administered through a ventilation tube placed with the patient under local anesthesia. Steroid administration was performed on 4 separate occasions over the course of 10 to 14 days. Hearing was assessed immediately before therapy and within 1 to 2 weeks after therapy. RESULTS: Hearing improvement was documented in 10 of 23 patients (44%) who underwent transtympanic steroid administration. This represents a 44% hearing salvage in patients for whom steroid treatment would otherwise have been considered a failure. CONCLUSION: Transtympanic steroid therapy may be an alternative treatment for patients with SSNHL for whom systemic steroid therapy had failed or who could not tolerate systemic steroid therapy.     

Idiopathic sudden sensorineural hearing loss in dialysis patients

Although sudden sensorineural hearing loss (SSNHL) affects chronic kidney disease (CKD) patients more frequently than non-CKD patients, few reports have described SSNHL in dialysis patients. We aimed to review the characteristics of SSNHL in chronic dialysis patients and evaluate treatment responses to steroid therapy. We retrospectively reviewed the records of dialysis patients diagnosed with idiopathic SSNHL at Asan Medical Center between January 2000 and December 2014. Pure tone and speech audiometry analyzes were performed before and 2?weeks and 2?months after treatment onset to evaluate outcomes. Twenty-two patients (11 men, 11 women; mean age: 49.9?±?11.7?years) were included; 16 (72%) and 6 (28%) had undergone hemodialysis and peritoneal dialysis, respectively, for a median of 49.2?±?41.4 (1–144) months. End-stage renal disease was most frequently caused by diabetic nephropathy (11 cases), chronic glomerulonephritis (1 case) and unknown factors (7 cases). Common accompanying symptoms included tinnitus (68.2%), ear fullness (45.5%) and vertigo (27.3%). The mean pure tone audiometry threshold at the initial presentation was 82.6?±?22.4?dB. At 2?months post-steroid treatment, 4 (18.2%), 4 (18.2%) and 6 (27.3%) patients exhibited a complete, partial, or slight recovery, respectively; 8 patients (36.3%) showed no improvement. Although we could not identify the specific cause of SSNHL in this population, our relatively large case series elucidates the precise clinical features of SSNHL in this population and demonstrates the outcomes of steroid treatment.     

Intratympanic steroids for treatment of sudden hearing loss after failure of intravenous therapy.

OBJECTIVE: We sought to describe our experience with intratympanic steroid treatment of sudden sensorineural hearing loss after failure of intravenous steroid treatment. STUDY DESIGN AND SETTING: We conducted a nonrandomized prospective clinical trial. Fifty patients presenting with sudden onset idiopathic hearing loss were treated intravenously over five days. After this period, patients with treatment failure (18 cases) were offered intratympanic steroid treatment. Nine patients refused, whereas the other nine patients received three weekly injections of methylprednisolone. Recovery of hearing was reported as improvement of more than 15 dB in pure tone average. RESULTS: Intratympanic steroid treatment improved hearing loss in five patients (55%). This is significant compared with those patients who refused intratympanic treatment, who showed no further improvement (P < 0.05). No serious adverse effects were observed. CONCLUSION: Intratympanic steroids significantly improve the recovery outcome of sudden hearing loss that had not recovered after intravenous steroid treatment. SIGNIFICANCE: Intratympanic steroids are an effective and safe therapy in sudden sensorineural hearing loss cases that are refractory to standard treatment.     

Vestibular schwannoma in patients with sudden sensorineural hearing loss

Sudden sensorineural hearing loss (SSNHL) has several etiologies. It may be a presenting symptom of vestibular schwannoma (VS). This study aimed to establish the incidence of VS in patients with SSNHL, and we report several unusual cases among these patients. We reviewed retrospectively the charts and magnetic resonance imaging (MRI) findings of all adult patients who presented with SSNHL between 2002 and 2008. We utilized three-dimensional fast imaging with steady-state acquisition temporal MRI as a screening method. Of the 295 patients with SSNHL, VS was found in 12 (4%). All patients had intrameatal or small to medium-sized tumors. There were three cases with SSNHL in one ear and an incidental finding of intracanalicular VS in the contralateral ear. There were four cases of VS that showed good recovery from SSNHL with corticosteroid treatment. There were two cases that mimicked labyrinthitis with hearing loss and vertigo. A greater number of cases than expected of VS were detected in patients with SSNHL, as a result of increasing widespread use of MRI. Various unusual findings in these patients were identified. MRI would seem to be mandatory in all cases of SSNHL.     

Bilateral versus unilateral sudden sensorineural hearing loss.

OBJECTIVES: To analyze the clinical characteristics and treatment results between bilateral (bi-) and unilateral (uni-) sudden sensorineural hearing loss (SSNHL). STUDY DESIGN AND SETTING: A retrospective study. METHODS: Three hundred twenty-four patients with SSNHL were classified into two groups; simultaneous bi-SSNHL (n = 16) and uni-SSNHL (n = 308). We compared clinical characteristics, medical history, hearing level, and treatment results between the 2 groups. RESULTS: The incidence of bi-SSNHL was 4.9 percent of overall patients with SSNHL. Bi-SSNHL occurs more commonly in patients of older age, with preexisting diabetes mellitus, and lipid panel abnormalities compared with uni-SSNHL. Ten patients (62.5%) in the bi-SSNHL group showed hearing recovery in 1 or both ears compared with 56.5 percent of patients with uni-SSNHL. Only 12 (37.5%) of all 32 ears recovered in bi-SSNHL, which was significantly lower than in uni-SSNHL. CONCLUSION: Bi-SSNHL has a very low incidence and lower recovery rate than uni-SSNHL. Recognition of similarities and differences between bilateral and unilateral SSNHL can help in counseling and managing the patients.     

Audiometric Patterns and Prognosis in Sudden Sensorineural Hearing Loss in Southern Taiwan

OBJECTIVES: To investigate factors affecting the prognosis of sudden sensorineural hearing loss (SSNHL). STUDY DESIGN AND SETTING: This is a retrospective study of patients with SSNHL hospitalized at an academic medical center. All patients in this study were treated with prednisolone and dextran. We compared a new 7-pattern classification for audiometric pattern and prognosis analysis with Sheehy classification. RESULTS: We analyzed 148 affected ears in 146 patients. Occurrence of SSNHL was associated with changes in season. The best prognosis was with the midtone pattern of the 7-pattern classification and with the low-tone pattern of Sheehy classification. The older patients, those with vertigo, or those treated after 6 days had a poor prognosis. There was no significant association between ESR level and prognosis. CONCLUSIONS: SSNHL patients with midtone loss have the best prognosis. This study of the audiometric patterns and prognostic factors of SSNHL allow us to better predict its outcome.     

Audiovestibular dysfunction associated with adoptive cell immunotherapy for melanoma

Objective To understand the audiologic and vestibular toxicities associated with adoptive cell immunotherapy (ACI) targeting pigment-pathway antigens on melanoma and to investigate the use of intratympanic steroid injections in the treatment of these toxicities. Study Design Prospective nonrandomized study. Setting Tertiary clinical research center. Methods Thirty-two patients with progressive metastatic melanoma who failed conventional therapy underwent ACI with T cells genetically modified to target MART-1 (n = 18) or gp100 (n = 14). All patients received serial audiometric testing. Vestibular testing was performed on patients with vestibular complaints. Patients with significant deficits received intratympanic steroid injections. Results Of 32 patients, 15 had no hearing change, 9 had mild hearing loss, and 8 had moderate hearing loss following treatment. Ten patients received intratympanic steroid injections for mild (n = 2) or moderate (n = 7) hearing loss or for significant imbalance (n = 1). Of those with mild hearing loss (n = 9), all but 1 recovered to pretreatment hearing levels. Four of 8 patients with moderate hearing loss recovered to baseline hearing levels, and 4 had partial recovery. All 7 patients with posttreatment vestibular complaints had demonstrable vestibular dysfunction. Three of these patients demonstrated recovery to normal vestibular function. The number of modified T cells infused for therapy correlated with the degree of audiovestibular deficit. Conclusion Adoptive cell immunotherapy targeting pigment-pathway cell proteins, a novel therapy for melanoma, can induce hearing loss and vestibular dysfunction. The presumed mechanism of autoimmune attack on normal melanocytes in the cochlear stria vascularis and in the vestibular organs demonstrates the importance of melanocytes in normal inner ear function.     

Oral steroid regimens for idiopathic sudden sensorineural hearing loss.

OBJECTIVE: To determine hearing recovery in patients with idiopathic sudden hearing loss treated with varying amounts of oral steroids. STUDY DESIGN AND SETTING: A retrospective chart review (n = 75) in a tertiary care clinic examined sudden hearing loss patients treated with 1 60-mg prednisone taper, 1 course of steroid less than a 60-mg taper, or any 2 courses of oral steroid. RESULTS: Overall, 35% of the patients recovered a clinically significant amount of hearing. Recovery was associated with immediate treatment (within 2 weeks from onset), better hearing at the onset of treatment, and treatment with the higher dose of prednisone in patients with just 1 additional symptom (dizziness or tinnitus). Patients tended to continue to experience some recovery in hearing up to 4 months after treatment. CONCLUSION: Immediate treatment of patients with unilateral idiopathic sudden hearing loss and additional symptoms (dizziness or tinnitus) with a 14-day course of 60 mg prednisone (with taper) is recommended. EBM rating: B-3.     

Saccular damage in patients with idiopathic sudden sensorineural hearing loss without vertigo

OBJECTIVE: Saccule could be damaged in patients with idiopathic sudden sensorineural hearing loss (ISSHL) with vertigo and with high-frequency sensorineural hearing loss. Thus, the saccule might be deteriorated subclinically in ISSHL cases without vertigo. Therefore, we investigated saccular damage in ISSHL patients without vertigo through vestibular evoked myogenic potentials (VEMP). STUDY DESIGN: A prospective study. SUBJECTS AND METHODS: Fifty-two patients with ISSHL without vertigo were enrolled in the study. We identified VEMP in patients with ISSHL and analyzed the association of VEMP with initial hearing threshold, each threshold according to frequency, the type of audiogram, and hearing recovery. RESULTS: For cases with absent VEMP, we found significant differences between patients with 90 dB or more hearing loss and those with a hearing loss less than 55 dB with frequencies over 1000 Hz. Patients with profound hearing loss presented significantly high abnormal and absent VEMP than patients with audiograms of other types. CONCLUSION: These findings suggest that the subclinical deterioration of the saccular neuroepithelium is associated with patients with ISSHL having profound hearing loss at the high frequency.     

Intratympanic dexamethasone for profound idiopathic sudden sensorineural hearing loss

OBJECTIVES: To determine hearing recovery by using intratympanic dexamethasone for profound, idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY DESIGN AND SETTING: A prospective, clinical study was performed of 25 consecutive patients seen with profound ISSNHL. Patients received 4 intratympanic treatments of dexamethasone over the course of 2 weeks. Complete hearing recovery was defined as the final pure-tone average (PTA) within 10 dB of baseline. Partial recovery was defined as a final PTA with >/=50% hearing. The hearing in the contralateral ear was used as baseline. RESULTS: The average time to treatment was 28 days. Two patients had significant hearing recovery, and 1 additional patient had partial recovery. The 2 patients with significant hearing recovery were treated within 9 days of onset of hearing loss. CONCLUSIONS: By using the treatment protocol and definition of hearing recovery of this study, intratympanic dexamethasone does not result in significant hearing improvement for patients with profound ISSNHL. There is a possible trend for improved hearing results if intratympanic treatment is performed within 11 days of onset of hearing loss.     

Intratympanic steroid perfusion for refractory sudden sensorineural hearing loss.

OBJECTIVE: Patients with sudden sensorineural hearing loss (SSHL) can benefit from systemic steroid therapy. Unfortunately, some patients are not candidates for steroid therapy due to concern over possible complications. Furthermore, not all patients will benefit from steroid administration. This study evaluates the potential benefits and safety of treating patients with SSHL refractory to oral steroids with intratympanic steroid therapy. METHODS: A retrospective case review was performed on all patients who presented with sudden sensorineural hearing loss refractory to oral steroid therapy during the past year. Seventeen patients were identified. All patients underwent intratympanic steroid administration, via MicroWick placement and/or round window catheter placement. RESULTS: Nine patients with sudden sensorineural hearing loss showed an improvement with intratympanic steroid therapy, consisting of MicroWick placement with dexamethasone drop (Decadron) administration for 1-2 weeks and/or round window catheter placement with steroid perfusion. Only one of the patients presenting with hearing loss present for greater than 8 weeks benefited from intratympanic therapy. Complications were few and included tympanic membrane perforation, chronic otitis media, dysequilibrium, and dysguesia. CONCLUSIONS: Intratympanic steroid therapy can be beneficial in treating patients with sudden sensorineural hearing loss refractory to oral steroid use. EBM rating: C.     

Effects of intratympanic injection of latanoprost in Meniere's disease: a randomized, placebo-controlled, double-blind, pilot study.

OBJECTIVE: To determine the short-term effects of latanoprost, a selective FP prostanoid receptor agonist, in Meniere's disease. STUDY DESIGN AND METHODS: Latanoprost was administered by intratympanic injection once daily for 3 days. Before the first injection (day 1) and on days 5 and 15, hearing and tinnitus were determined. The patients assessed vertigo on a visual analogue scale on days 1-15. The study was randomized, double-blind, and placebo-controlled. RESULTS: Latanoprost reduced vertigo/dysequilibrium around 30% (P < 0.05), and improved speech discrimination around 15% (P < 0.05). Tinnitus loudness deteriorated after injection of placebo (P < 0.01) but not after latanoprost. Side effects were few. CONCLUSION AND SIGNIFICANCE: Latanoprost alleviated vertigo/dysequilibrium and improved hearing. The results indicate that the drug potentially could be useful for treatment of Meniere's disease.     

Intratympanic steroid treatment in idiopathic sudden sensorineural hearing loss: A control study

BACKGROUND AND OBJECTIVE: Although systemic steroids in sudden sensorineural hearing loss (SSHL) appears to be the most effective and the most widely accepted treatment today, a significant number of patients do not respond to steroid treatment or they cannot receive steroids for medical reasons. Intratympanic (IT) administration of steroids appears to be an alternative or additional method of management without the side effects of intravenous steroids. The aim of this study is to investigate the effectiveness and safeness of IT administration of steroids in patients who had not responded to IV treatment and to compare treatment efficacy with controls. STUDY DESIGN AND SETTING: Our study consisted of 37 patients with SSHL who, at the end of 10 days of therapy with intravenous steroids as a 1st line treatment, had pure-tone 4-frequency (0.5, 1, 2, and 4 kHz) average (PTA) of worse than 30 dB or worse than 10 dB from the contralateral ear (defined as failed intravenous treatment). They were randomized into 2 groups, treatment and control. The 19 patients of the treatment group received approximately 0.5 mL sterile aqueous suspension of methylprednisolone acetate in a concentration of 80 mg/2 mL by direct injection. The procedure was carried out 4 times within a 15-day period. An audiogram was performed before each injection and approximately 1.5 months after the last session. RESULTS: All patients tolerated the procedure well. No perforation or infection was noticed in any of the patients at their last visit. With regard to the 19 patients who received intratympanic treatment, in 9 patients, the PTA threshold improved more than 10 db, in 10 patients there was no change greater than 10 db, and no patients deteriorated more than 10 db. In the control group, none of the patients showed any change greater than 10 db. The difference was statistically significant (P = 0.002). The treatment group showed an improvement in mean PTA of 14.9 dB, whereas the control group showed a deterioration of 0.8 dB, and this difference also was statistically significant (P = 0.0005). IT treatment (P = 0.0001), better post-IV PTA (P = 0.0008), and absence of vertigo (P = 0.02) were good predictors of the outcome. In contrast, sex, age, affected ear, days to admission, and pattern of the initial audiogram showed no significant influence on the outcome. CONCLUSION AND SIGNIFICANCE: IT steroid administration after failed intravenous steroids is a safe and effective treatment in sudden sensorineural hearing loss.     

鼓室内注射地塞米松治疗突发性感音神经性聋的效果

探究鼓室内注射地塞米松治疗突发性感音神经性聋的临床效果。 方法 选取2020年 6月-2023年2月湖南省人民医院收治的80例突发性感音神经性聋患者为研究对象,按照随机数字表法分为 对照组和试验组,每组40例。对照组给予前列地尔治疗,试验组在对照组基础上增加鼓室内注射地塞米松 治疗,比较两组纯音听阈值、临床症状恢复时间、血清指标水平及不良反应发生情况。 结果 试验组治疗 后0.5 kHz、1.0 kHz及2.0 kHz的纯音听阈值均优于对照组（P＜0.05）;试验组眩晕消失时长、听力恢复时 长、耳鸣消失时长和耳闷消失时长均短于对照组（P＜0.05）;试验组治疗后ET水平低于对照组,NO水平 高于对照组（P＜0.05）;试验组不良反应发生率为2.50%,低于对照组的17.50%（P＜0.05）。 结论 针对突 发性感音神经性聋患者给予鼓室内注射地塞米松治疗的效果良好,可有效改善患者的纯音听阈值,减轻其 临床症状,有利于缩短恢复时间,提高患者的生活质量,且治疗后不良反应发生几率较小,安全性较高。     

Sudden hearing loss as the sole manifestation of neurosarcoidosis

Sarcoidosis is a chronic, idiopathic granulomatous disease with frequent pulmonary, ocular, and lymphatic systemic manifestations. Central nervous system involvement is rare (1% to 5%), yet the most frequent presenting symptoms (48%) in patients with neurosarcoidosis are neurologic. Eighth cranial nerve abnormalities occur in 20% of these patients, typically in association with other cranial neuropathies or overt systemic disease. Isolated eighth nerve disease is very rare. We report two cases of neurosarcoidosis manifest as isolated, sudden onset sensorineural hearing loss, one of which had a cerebellopontine angle (CPA) granuloma that mimicked an acoustic neuroma. Both patients experienced marked steroid-induced hearing improvement that persisted after termination of therapy. Brainstem evoked response audiometry (BSER) abnormality persisted despite normalization of audiologic parameters. The implications for diagnosis and treatment of sarcoidosis-induced hearing loss are described. Neurosarcoidosis must be considered when evaluating patients with sudden hearing loss. Prompt steroid therapy offers hope for significant hearing recovery in these patients.     

Intratympanic gentamicin therapy for persistent vertigo after endolymphatic sac surgery.

OBJECTIVES: To evaluate the role of intratympanic gentamycin therapy (ITGM) in controlling vertigo in patients with refractory Meniere's who have recurrent or persistent vertigo after endolymphatic sac surgery. STUDY DESIGN AND SETTING: A retrospective review of 491 patients seen in a university balance clinic between July 1999 and July 2000. Of these, 68 patients had Meniere's disease; 4 patients had recurrent vertigo after endolymphatic sac surgery and were followed for at least 1 year. These 4 patients received a series of ITGM injections. Outcome measures included control of vertigo and audiometric results. RESULTS: Three of 4 (75%) patients had control of their vertigo with ITGM therapy. The 1 patient who failed ITGM therapy underwent a successful vestibular neurectomy with ultimate control of his vertigo. No patients had progression of their sensorineural hearing loss. CONCLUSION: ITGM therapy can be used to control vertigo in selected patients with Meniere's disease who have persistent vertigo after unsuccessful endolymphatic sac surgery. SIGNIFICANCE: ITGM therapy may reduce the need for vestibular neurectomy in patients with refractory Meniere's disease after endolymphatic sac surgery.     

Titration streptomycin therapy for bilateral Meniere's disease: a preliminary report

Intramuscular dosages of streptomycin sulfate were titrated in eight patients with bilateral Meniere's disease in an attempt to alleviate disabling vertigo and hearing loss. It appears possible to eliminate the vertiginous episodes, preserve or improve hearing, and avoid ataxia and oscillopsia in most of these individuals. At present we consider this treatment regimen our first choice of therapy in people with bilateral active Meniere's disease or in patients whose only hearing ear is actively fluctuating and in whom associated disabling vertigo is present. Great caution and supervision should be exercised in the use of streptomycin titration therapy in the individual with bilateral Meniere's disease, as further experience is required to determine the efficacy of this form of management.     

Acoustic neuroma presenting as sudden hearing loss with recovery

In our series of patients operated on for acoustic neuromas at New York University Medical Center between 1974 and 1983, 13% (17 of 133) had sudden hearing loss. Of these, approximately 23% (four of 17) had recovered auditory function before acoustic neuroma extirpation. Three patients spontaneously recovered, while one improved with steroid therapy. Contrast computerized tomography demonstrated a widened internal auditory canal and evidence of cerebellopontine angle tumor, respectively, in 88% and 59% of patients with sudden hearing loss and acoustic neuroma. Clinical characteristics suggesting acoustic neuroma as the cause of sudden hearing loss with or without auditory recovery could not be identified in our series. Our data support the rationale that patients with unilateral sudden hearing loss, even with recovery, must be evaluated for a possible cerebellopontine lesion.     

Acute hearing loss

Opinion statement Acute hearing loss (AHL) is a medical urgency. The management of patients presenting with sudden deafness involves detecting the causal mechanism and administering emergency therapeutic drugs to restore hearing by minimizing the period of cellular ischemia to the inner ear. Acute management of AHL consists of administering a 10-day course of high-dose corticosteroids (prednisone 60 to 80 mg) until a cause can be established. Magnetic resonance imaging with gadolinium is indicated, with a study dedicated to the internal auditory canals. The natural history of idiopathic AHL is characterized by spontaneous improvement in two thirds of patients. Maximum improvement occurs within 2 weeks of onset of AHL. In the vast majority of patients (>90%), the AHL is idiopathic. For an identifiable etiology, the treatment is specific and may consist of stopping ototoxic medications, repair of perilymphatic fistulas, administering antimicrobial agents for viral or bacterial infections, correction of metabolic imbalances, management of stroke, and possible surgery for cerebellopontine angle tumors. Management of idiopathic AHL is controversial. Various therapeutic agents, such as vasodilators, diuretics, anticoagulants, plasma expanders, contrast agents, and carbogen inhalation, have been tried in single therapy or as a combination therapy. The empiric use of these drugs is mainly based on improving the blood circulation and restoring the oxygen tension to the inner ear. The use of interventional procedures, such as low-density lipoprotein apheresis as well as newer drug delivery systems for corticosteroids, and immunosuppressive agents have opened new options in the treatment of AHL secondary to immunemediated diseases of the inner ear. Prognosis for AHL is best when patients are seen early, begin recovery within 2 weeks, and have a mild hearing loss (<90 dB) with upward-sloping audiograms. Greater than 90 dB of hearing loss along with flat or down sloping audiogram, advanced age, and presence of vertigo are adverse prognostic factors for recovery of hearing loss.