Pretreatment Hearing Levels Research Articles

Effect of long-term betahistine treatment on the clinical outcomes of patients with cochlear Meniere’s disease

Background Many studies have discussed the betahistine treatment for Meniere’s disease (MD). However, regarding cochlear MD, there is no consensus on the long-term betahistine treatment. Aims/objectives This study aims to investigate the relationship between the betahistine treatment duration in patients with cochlear MD and their clinical outcomes. Material and Methods We enrolled 78 patients with 96 ears who were diagnosed with cochlear MD and received the treatment for more than 6 months. Outcomes included the hearing status, frequency of acute hearing loss attack, and whether the disease progressed to MD. Clinical characteristics including age, sex, side of affected ear, treatment duration of betahistine and trichlormethiazide, and pre-treatment hearing level was recorded from medical charts. Results Comparing the clinical characteristics by outcomes, the average betahistine treatment duration was the independent factor for hearing status of four-tone average (p = 0.01) and low-tone average (p = 0.03). Patients with average betahistine treatment duration of at least 277 days per year had higher odds ratio for improvement of the hearing status of four-tone and low-tone average. Conclusions For patients with cochlear MD, regular and long-term betahistine treatment can benefit their hearing outcome in the low- and medium-frequency.

Prevalence of perilymphatic fistula in patients with sudden-onset sensorineural hearing loss as diagnosed by Cochlin-tomoprotein (CTP) biomarker detection: its association with age, hearing severity, and treatment outcomes

PurposeTo determine the prevalence of perilymphatic fistula (PLF) in sudden-onset sensorineural hearing loss (SSNHL) patients by employing the Cochlin-tomoprotein (CTP) detection test, a specific diagnostic marker for perilymph. We also analyzed the clinical characteristics associated with hearing outcomes in this cohort.MethodsA total of 74 eligible patients were prospectively enrolled. Following myringotomy, middle ear lavage (MEL) samples underwent the CTP test to identify perilymph leakage. Intratympanic dexamethasone (IT-DEX) therapy was administered, and hearing outcomes were assessed. Control groups comprised patients with chronic otitis media (n = 40) and non-inflammatory middle ears (n = 51) with concurrent MEL sample collection.ResultsCTP was positive in 16 (22%) patients. No control samples showed positive results. Multiple regression analysis indicated that age and pre-treatment hearing levels significantly contributed to the CTP value. We found a positive correlation between CTP values, age, and pre-treatment pure-tone averages. Notably, CTP values in SSNHL cases aged 60 and above were significantly higher than in those below 60 years. Patients with positive CTP had significantly worse recovery rates after IT-DEX treatment.ConclusionThis study is the first prospective investigation demonstrating a positive relationship between CTP values, age, and hearing severity in SSNHL, indicating that PLF might be the essential cause of SSNHL, particularly in the elderly. Our findings suggest that IT-DEX may be less effective for PLF-associated SSNHL. Future research could reveal that PLF repair surgery is a viable treatment strategy for SSNHL. This study was registered under the UMIN Clinical Trials Registry (UMIN000010837) on 30/May/2013.

Evaluation of Role of Prognostic Factors on Hearing Outcome in Unilateral Idiopathic Sudden Onset Sensorineural Hearing Loss

Introduction: Owing to its uncertain and multiple possible etiologies, use of multiple empirical treatment regimens and possibility of spontaneous recovery, it becomes important to look for the possible prognostic factors affecting the outcome of sudden sensorineural hearing loss (SSNHL). Material and methods: This prospective observational study was conducted with the primary aim of analyzing the role of various possible prognostic factors on the outcome of idiopathic SSNHL including demographic (Age, gender), audio-vestibular (Pre-treatment hearing loss in the affected ear, hearing in the contralateral ear, time elapse between onset of hearing loss and start of treatment, type of hearing loss in term of frequencies affected), presence of tinnitus / vertigo and Comorbidities (Diabetes, Hypothyroidism & Deranged lipid profile/ dyslipidemia). Result: The absence of vertigo and tinnitus led to better hearing outcomes while raised HbA1c levels, deranged lipids and thyroid profiles adversely affected the outcome. Conclusion: Vertigo, tinnitus, raised HbA1c levels, deranged lipids and thyroid profiles are possible negative prognostic factors. Better pretreatment hearing levels in the affected and unaffected ears prompted improved recovery.

The prognostic value of peripheral blood parameters on all-frequency sudden sensorineural hearing loss

ObjectiveTo determine whether peripheral blood parameters have any predictive value for all-frequency Sudden Sensorineural Hearing Loss (SSNHL). MethodsWe chose 78 individuals with all-frequency SSNHL who had been admitted to our department. They were divided into two groups: the effective group and the ineffective group. In patients with all-frequency SSNHL, the prognostic variables, including peripheral blood tests and clinical traits, were examined by a logistic regression analysis. In addition, the predictive value was carried out. ResultsThe effective rate of all-frequency SSNHL was 61.5%. Pre-treatment hearing level and the proportion of patients with diabetes were both significantly lower in the effective group than in the ineffective group (p = 0.024 and 0.000, respectively). The levels of fibrinogen and C-reactive protein were also significantly different between the two groups (p = 0.001 and 0.025, respectively). Pre-treatment hearing level and fibrinogen level both significantly impacted the prognosis of all-frequency SSNHL (p = 0.032 and 0.002, respectively), according to a logistic regression analysis. Furthermore, the prognosis was significantly predicted by both fibrinogen level and pre-treatment hearing level (p = 0.001 and 0.0002, respectively). The receiver operating characteristic curve showed that the fibrinogen level had a sensitivity of 85.4% and a specificity of 60.0% for predicting the prognosis of all-frequency SSNHL. ConclusionFor the prognosis of all-frequency SSNHL, the fibrinogen level can be regarded as a useful predictor. The level of C-reactive protein, however, does not have a significant prognostic effect on predicting all-frequency SSNHL. Therefore, more attention should be devoted to the level of fibrinogen in the acute period of all-frequency SSNHL. Level of Evidence4.

Comparison of Auditory Outcomes between Inpatient- and Outpatient-Based Treatment in Sudden Sensorineural Hearing Loss.

Objective: The primary treatment for sudden hearing loss is high-dose steroid therapy. In some countries, hospitalization has been taken for granted. Although most countries appear to treat sudden hearing loss on an outpatient basis, some other countries have considered hospitalization as necessary. Only a few studies have been conducted on the effect of hospitalization on hearing outcomes. Therefore, we compared the hearing outcome of inpatient- and outpatient-based treatments to determine whether hospitalization affects the recovery of sudden hearing loss. Methods: We conducted a retrospective case review of patients diagnosed with sudden sensorineural hearing loss (SSNHL). In total, 439 patients with SSNHL were enrolled and categorized as either inpatients (group I) or outpatients (group O). Pure-tone audiometry was initially performed before the treatment and 3 months post-treatment. “Recovery” was defined as a hearing gain of 15 dB HL and a final hearing of better than 25 dB. “No recovery” was defined as an improvement of hearing gain of <15 dB 3 months after treatment. To exclude the effect of the level of pretreatment hearing loss, we divided the patients into three subgroups based on their hearing level: <40 dB, 40–70 dB, and >70 dB. To assess the effect of the treatment modality, the patients were divided into three treatment subgroups: systemic steroids (SS), intratympanic steroids (ITS), and a combination of both (SS and ITS). Results: The pretreatment hearing level was significantly higher in group I (61.5 ± 25.4 dB) than in group O (50.3 ± 23.0 dB; p < 0.05). The hearing gain was significantly higher in group I (33.3 ± 24.4 dB) than in group O (24.0 ± 21.8 dB; p < 0.05). The “Recovery” ratio was significantly higher in group I (70.2%) than in group O (63.1%) (p < 0.05). A repeated measures ANOVA was performed to assess the statistical differences between hospitalization, treatment modalities, and pretreatment subgroups. The inpatient group showed a significant hearing improvement in all SSNHL patients (p < 0.05). There was a significant hearing improvement in the inpatient group with pretreatment hearing <40 and 40–70 dB (p < 0.05). There was no significant difference between the inpatient and outpatient groups in pretreatment hearing >70 dB (p > 0.05). Conclusions: This retrospective study showed that inpatient treatment for sudden hearing loss is more beneficial for hearing improvement than outpatient treatment. The positive effect of inpatient treatment appears to be significant in patients with a pretreatment hearing level of 70 dB or less.

ANİ İDİYOPATİK SENSÖRİNÖRAL İŞİTME KAYBINDA PROGNOSTİK FAKTÖRLERİN ANALİZİ

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 (&amp;gt; 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 &amp;lt; 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.

Clinical features and hearing prognosis of idiopathic sudden sensorineural hearing loss in patients undergoing hemodialysis: A retrospective study.

BackgroundPatients undergoing hemodialysis (HD) tend to experience hearing loss, including idiopathic sudden sensorineural hearing loss (ISSHL). However, little is known about the relationship between HD and ISSHL.ObjectiveTo investigate the effects of HD on the hearing level and the treatment prognosis of ISSHL.MethodsWe reviewed the medical records of 23 patients with ISSHL receiving HD treatment (HD group) and 101 patients with ISSHL not receiving HD treatment (non‐HD group), and assessed clinical features, results of audiometric tests and blood examination results.ResultsStatistically significant differences were not observed in pretreatment hearing level and hearing recovery of the ear affected with ISSHL between the two groups (P > .05). Conversely, hearing thresholds in the unaffected ear were statistically different (P < .0001), and the hearing thresholds of the HD groups were significantly increased compared with those of the non‐HD groups, especially at high frequency. In addition, patients with renal dysfunction not receiving HD treatment showed similar hearing thresholds in the unaffected ear when compared with patients receiving HD treatment.ConclusionHD itself did not influence the treatment prognosis of ISSHL. Renal dysfunction itself, and not HD treatment, worsened the hearing level. As similar treatment results are expected, standard treatment should be administered to patients undergoing HD.Level of Evidence3b.

Heparin therapy as adjuvant treatment for profound idiopathic sudden sensorineural hearing loss.

This study aimed to provide evidence of whether unfractionated heparin used as adjuvant therapy in conjunction with systemic corticosteroid therapy improves hearing recovery in patients with profound idiopathic sudden sensorineural hearing loss (ISSNHL), and to compare the effect of this treatment with those of additional intratympanic corticosteroid therapy. Retrospective chart review. Eighty-seven patients with profound ISSNHL (≥90 dB) and who had been admitted at a tertiary referral center between 2010 and 2018 were retrospectively reviewed, 67 patients for additional intratympanic corticosteroid injection (ITSI) (ITSI group) and 21 for adjuvant heparin therapy (heparin group). Hearing recovery was evaluated by grade assessment according to the American Academy of Otolaryngology-Head and Neck Surgery criteria. Of the patients in the heparin group, 42.8% recovered serviceable hearing, which was significantly higher than the recovery rates (19.7%) of those in the ITSI group. Particularly, in patients with pretreatment hearing level of 90 to 100 dB, adjuvant heparin therapy enhanced therapeutic effects with a significant hearing recovery rate of 80%. However, in patients with initial hearing level >100 dB, the rates of significant hearing recovery in the two groups were roughly equal and remained unsatisfactory (8.1% in the ITSI group and 9.1% in the heparin group). The results of this study suggest that the treatment of profound ISSNHL with adjuvant heparin therapy, in combination with systemic steroid therapy, results in higher hearing recovery rates when compared to combined local and systemic corticosteroid therapy, without serious complications. 3b Laryngoscope, 130:1310-1315, 2020.

Progression of hearing loss after LINAC-based stereotactic radiotherapy for vestibular schwannoma is associated with cochlear dose, not with pre-treatment hearing level

BackgroundAlthough stereotactic radiotherapy (SRT) for vestibular schwannoma has demonstrated excellent local control rates, hearing deterioration is often reported after treatment. We therefore wished to assess the change in hearing loss after SRT and to determine which patient, tumor and treatment-related factors influence deterioration.MethodsWe retrospectively analyzed progression of hearing loss in patients with vestibular schwannoma who had received stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) as a primary treatment between 2000 and 2014. SRS had been delivered as a single fraction of 12 Gy, and patients treated with FSRT had received 30 fractions of 1.8 Gy. To compare the effects of SRS and FSRT, we converted cochlear doses into EQD2. Primary outcomes were loss of functional hearing, Gardner Robertson (GR) classes I and II, and loss of baseline hearing class. These events were used in Kaplan Meier plots and Cox regression. We also calculated the rate of change in Pure Tone Average (PTA) in dB per month elapsed after radiation—a measure we use in linear regression—to assess the associations between the rate of change in PTA and age, pre-treatment hearing level, tumor size, dose scheme, cochlear dose, and time elapsed after treatment (time-to-first-audiogram).ResultsThe median follow-up was 36 months for 67 SRS patients and 63 months for 27 FSRT patients. Multivariate Cox regression and in linear regression both showed that the cochlear V90 was significantly associated with the progression of hearing loss. But although pre-treatment PTA correlated with rate of change in Cox regression, it did not correlate in linear regression. The time-to-first-audiogram was also significantly associated, indicating time dependency of the rate of change. None of the analysis showed a significant difference between dose schemes.ConclusionsWe found no significant difference between SRS and FSRT. As the deterioration in hearing after radiotherapy for vestibular schwannoma was associated with the cochlea V90, restricting the V90 may reduce progression of hearing loss. The association between loss of functional hearing and baseline PTA seems to be biased by the use of a categorized variable for hearing loss.

Classification of sudden deafness prognosis based on deep learning method

Objective:This study aimed to develop predictive models for sudden sensorineural hearing loss through deep belief network (DBN) and explore whether the model performances differ when adopting different outcome criteria. Method: 228 potential predictors involving the clinical characteristics, audio logical data, and serological parameters out of 1 220 hospitalized SSHL patients who were admitted from June 2008 to December 2015 were analyzed retrospectively. The hearing data of sudden deafness were classified into two or four categories based on Chinese criteria and Siegel criteria, which were used to develop the DBN models. The area under the receiver operating characteristic curve (ROC-AUC) and accuracy were used to compare the predictive performance of different models. Result: The DBN model developed for predicting the dichotomized outcomes had better performance than that of the fourcategory outcomes. When the iteration number reached 500 times, DBN model constructed for prediction of dichotomized outcomes based on Siegel's criteria had demonstrated the best performance with an accuracy of 76.25% and an AUC of 0.81. According to indices from first layer weights, DBN gave a rank of top 10 sensitive features for hearing outcome prediction focusing on indicators regarding coagulation, demographics and pre-treatment hearing levels independent of the outcome assessment criteria. Conclusion: DBN provides a robust outcome prediction ability in SSHL datasets with rich and complex variables, especially when utilized to predict dichotomized outcomes based on the Siegel criteria. In addition, this advanced deep learning technique can automatically extract valuable predictors, which is consistent with those that had been verified in previous studies by traditional statistical methods. This study provides further evidence for extending the use of DBN algorithm to the field of developing prediction or classification models for other otological diseases in the future..

Radiotherapy in patients with vestibular schwannoma and neurofibromatosis type 2: clinical results and review of the literature.

To evaluate the long-term outcome of patients with vestibular schwannoma (VS) and neurofibromatosis type 2 (NF2) treated with fractionated stereotactic radiotherapy (FSRT) or stereotactic radiosurgery (SRS). Sixteen VS in 14 patients with NF2 were treated with FSRT (n = 14) and SRS (n = 2). Patients with tumor progression and/or progression of clinical symptoms were selected for treatment. For patients treated with FSRT a median total dose of 57.6 Gy was prescribed with a median fractionation of 5 × 1.8 Gy per week. For patients who underwent SRS a median single dose of 17 Gy was prescribed to the 80% isodose. FSRT and SRS were well tolerated. Local control rate was 94% for a median follow-up time of 131 months; 2- and 5-year progression-free survival were 100%. The probability of maintaining the pretreatment hearing level was 44%. Useful hearing preservation was 33%. Cranial nerve toxicity was moderate. Trigeminal nerve function worsened in 2 patients (12%) and facial nerve function in 3 patients (19%). One patient developed a new tinnitus. FSRT and SRS are both safe and effective noninvasive and minimally invasive treatment options for patients with VS in the setting of NF2. The long-term local control rates are excellent. Functional hearing preservation is worse in patients with VS and NF2 than in patients with sporadic VS.

Differences between acoustic trauma and other types of acute noise-induced hearing loss in terms of treatment and hearing prognosis

Objectives: To evaluate the differences between acoustic trauma (AT) and other types of acute noise-induced hearing loss (ANIHL), we performed a literature search and case reviews.Methods: The literature search based on online databases was completed in September 2016. Articles on ANIHL and steroid treatment for human subjects were reviewed. The source sounds and treatment sequelae of our accumulated cases were also reviewed. Hearing loss caused by gun-shots and explosions was categorized into the AT group, while hearing loss caused by concerts and other noises was categorized into the ANIHL group.Results: Systemic steroid treatment did not appear to be effective, at least in the AT group, based on both the literature and our case reviews. However, effective recovery after treatment including steroids was observed in the ANIHL group. The difference in hearing recovery between the AT and ANIHL groups was statistically significant (p = .030), although differences in age, days from the onset to treatment and pretreatment hearing levels were not significant.Conclusions: Hearing recovery from AT is very poor, whereas, ANIHL is recoverable to some extent. Therefore, it is essential to differentiate between these two groups for accurate prediction of the hearing prognosis and evaluation of treatment effects.

Prognostic impact of salvage treatment on hearing recovery in patients with sudden sensorineural hearing loss refractory to systemic corticosteroids: A retrospective observational study.

To determine the prognostic factors for hearing recovery in patients with sudden sensorineural hearing loss (SSHL) refractory to systemic corticosteroids following salvage treatment. This is a retrospective observational study at nine tertiary referral hospitals. A total of 120 patients with sudden deafness refractory to systemic corticosteroids were enrolled. The patients were randomly assigned to receive topical application of recombinant human IGF-1 or intratympanic injection of dexamethasone as salvage treatment. Multiple regression analysis was performed to identify determinants of hearing recovery using pure tone audiometry results at 8 weeks after treatment. Clinical predictors that were evaluated included age, sex, pretreatment hearing level, presence of vertiginous symptoms, days to study entry from symptom onset and salvage treatment assignment (IGF-1 vs. dexamethasone). The linear regression model identified age (P=0.001), pretreatment hearing level (P<0.001), days to study entry from symptom onset (P=0.011) and treatment assignment (P=0.033) at 8 weeks after treatment as significant variables influencing the recovery of pure tone audiometry average thresholds. Younger age (<60 years), early initiation of salvage treatment and treatment with topical IGF-1 therapy had significant effects on hearing recovery. The results indicate that early initiation and choice of treatment modalities for salvage treatment may be important for the prognosis of patients with refractory SSHL. The positive effect of topical IGF-1 therapy on hearing recovery indicates its utility as salvage treatment.

Dose Effect of Intratympanic Dexamethasone for Idiopathic Sudden Sensorineural Hearing Loss: 24 mg/mL Is Superior to 10 mg/mL.

To compare outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) treated with intratympanic (IT) dexamethasone (DEX) at either 10 mg/mL or 24 mg/mL. Retrospective case series. Tertiary referral center. Thirty-seven adults with ISSNHL. In addition to concurrent prednisone taper, patients received a series of IT DEX injections for 2 weeks with either 10 mg/mL or 24 mg/mL. Greater than 30-dB improvement in pure-tone average (PTA). Baseline characteristics were similar between groups. Mean follow-up was 10 weeks. Ten (53%) of 19 patients treated with 24 mg/mL had greater than 30-dB improvement in PTA compared with 3 (17%) of 18 treated with 10 mg/mL (p = 0.0382, Fisher's exact test). There was a trend toward improved word recognition score outcome with 24 mg/mL. The interval between onset and initiation of IT DEX significantly affected outcome, with earlier treatment resulting in greater improvement in PTA and word recognition score. Multivariate logistic regression confirmed that IT DEX dose and interval to starting treatment were both independent predictors of PTA outcome. Change in PTA was not significantly affected by age, sex, pretreatment hearing levels, or concurrent treatment with hyperbaric oxygen. To our knowledge, this is the first demonstration of superiority of IT DEX at 24 mg/mL for the treatment of ISSNHL, with significantly better recovery of PTA. Our data suggest that treatment should be initiated as soon as possible. A prospective randomized trial to confirm the optimal dose is warranted.

Radiotherapy in Patients with Vestibular Schwannoma and Neurofibromatosis Type 2: Clinical Results and Review of the Literature

To evaluate the long-term outcome of patients with vestibular schwannoma (VS) and neurofibromatosis type 2 (NF2) treated with fractionated stereotactic radiotherapy (FSRT) or stereotactic radiosurgery (SRS). Sixteen VS in 14 patients with NF2 were treated with FSRT (n = 14) and SRS (n = 2). Patients with tumor progression and/or progression of clinical symptoms were selected for treatment. For patients treated with FSRT a median total dose of 57.6 Gy was prescribed with a median fractionation of 5 × 1.8 Gy per week. For patients who underwent SRS a median single dose of 17 Gy was prescribed to the 80% isodose. FSRT and SRS were well tolerated. Local control rate was 94% for a median follow-up time of 131 months; 2- and 5-year progression-free survival were 100%. The probability of maintaining the pretreatment hearing level was 44%. Useful hearing preservation was 33%. Cranial nerve toxicity was moderate. Trigeminal nerve function worsened in 2 patients (12%) and facial nerve function in 3 patients (19%). One patient developed a new tinnitus. FSRT and SRS are both safe and effective noninvasive and minimally invasive treatment options for patients with VS in the setting of NF2. The long-term local control rates are excellent. Functional hearing preservation is worse in patients with VS and NF2 than in patients with sporadic VS.

Hearing Preservation and Vestibular Schwannoma

This study aimed to evaluate the relationship between cochlear signal on fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) sequences and hearing in patients undergoing hearing preservation surgery for vestibular schwannoma (VS) and to demonstrate a new classification system to be used in imaging evaluation of patients with VS. A search of archived surgical cases at a single institution between January 1, 2006, and January 1, 2012, revealed 51 patients who underwent hearing preservation surgery for VS. Tumor size, patient age and sex, and preoperative and postoperative pure-tone average (PTA) and speech discrimination score (SDS) were recorded. Cochleae on the affected side were examined on preoperative FLAIR sequences and classified as limited hyperintensity (LH) or extensive hyperintensity (EH). Mean patient age was 51 years, and mean tumor size was 1.3 cm. Preoperative FLAIR sequences were classified into LH (n = 36) and EH (n = 15) categories. Preoperative PTA and SDS were 29.5 dB (SD, 16.7), 90% (SD, 14) and 40.6 dB (SD, 13.8), 80% (SD, 25) for LH and EH, respectively. On univariate analysis, preoperative PTA was superior in the LH group (p = 0.04). There was a trend toward superior preoperative SDS and postoperative PTA in the LH group, but these differences were not statistically significant (p = 0.08 and p = 0.06, respectively). The current study is the first to demonstrate a distinct association between cochlear FLAIR signal and pretreatment hearing levels in patients with VS. A new classification system for evaluating cochlear FLAIR signal is proposed. Improvement in FLAIR sequences will allow further investigation of this association.

Management of Primary and Recurrent Endolymphatic Sac Tumors

To report the clinical presentation, management strategies, and outcomes of 14 endolymphatic sac tumors (EST). Retrospective case series. Collective experience accrued from 2 tertiary referral centers. All patients with ESTs. Microsurgery, stereotactic radiosurgery (SRS). Treatment-related morbidity, recurrence. Fourteen ESTs (13 patients) met inclusion criteria. Eight tumors were sporadic, and 6 were associated with von Hippel-Lindau disease (VHL). Twelve lesions were managed primarily, whereas 2 were referred for treatment of sizable recurrences after subtotal resection. Including all patients, the median age at diagnosis was 36 years, and the median duration of posttreatment follow-up was 78 months. Among primary tumors, the median delay between symptom onset and diagnosis was 36 months, and the median tumor diameter at presentation was 23 mm with most lesions demonstrating intracranial involvement. Subjects with VHL frequently presented with smaller, less extensive tumors and were more commonly female compared with patients with sporadic disease. Of the 9 ESTs presenting with normal facial nerve function, 8 maintained good (HB 1-2) posttreatment capacity. Two of 5 ears with useful preoperative hearing maintained pretreatment hearing levels after surgery. One of 12 tumors managed primarily recurred after microsurgical resection. Primary SRS was used in 1 medically infirm patient providing durable tumor control (94 mo) at last follow-up. Among recurrent tumors, 2 were managed successfully with reexcision, whereas 1 underwent salvage surgery followed by 2 sequential treatments of SRS for 2 separate intracranial recurrences; no in-field recurrence has been detected to date. ESTs are rare primary neoplasms of the temporal bone that may occur sporadically or in association with VHL. Outcomes after treatment of small less-extensive tumors are favorable compared with patients with advanced primary or recurrent disease. Furthermore, subtotal resection carries a high risk of bulky or multifocal recurrence. As such, early gross total resection remains the management strategy of choice for primary and recurrent ESTs; however, SRS should be considered in poor surgical candidates or in cases of focal intracranial recurrence when the morbidity of salvage surgery is high.

Treatment of idiopathic sudden sensorineural hearing loss: systemic versus intratympanic methylprednisolone acetate

EnAbstract 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.

Audiovestibular Dysfunction Associated with Adoptive Cell Immunotherapy for Melanoma

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. Prospective nonrandomized study. Tertiary clinical research center. 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. 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. 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.

Stereotactic radiation therapy for large vestibular schwannomas

Background and purpose To evaluate the morbidity and tumor-control rate in the treatment of large vestibular schwannomas (VS) after stereotactic radiation therapy in our institution. Material and methods Twenty-five consecutive patients (17 men, 8 women) with large VS (diameter 3.0 cm or larger), treated with stereotactic radiotherapy (SRT) or stereotactic radiosurgery (SRS) between 1992 and 2007, were retrospectively studied after a mean follow-up period of three years with respect to tumor-control rate and complications. Results Actuarial 5-year maintenance of pre-treatment hearing level probability of 30% was achieved. Five of 17 patients suffered permanent new facial nerve dysfunction. The actuarial 5-year facial nerve preservation probability was 80%. Permanent new trigeminal nerve neuropathy occurred in two of 15 patients, resulting in an actuarial 5-year trigeminal nerve preservation probability of 85%. Tumor progression occurred in four of 25 (16%) patients. The overall 5-year tumor control probability was 82%. Conclusion Increased morbidity rates were found in patients with large VS treated with SRT or SRS compared to the published series on regular sized VS and other smaller retrospective studies on large VS.