The impact of eustachian tube function on intra-tympanic steroid administration.

OBJECTIVES: This study investigated the impact of eustachian tube (ET) function (ETF) on therapeutic success on candidates for intra-tympanic administration of steroids (ITAoS), due to idiopathic sudden sensorineural hearing loss (ISSNHL). METHODS: Medical chart review in two university-affiliated medical centers was performed. Included were consecutive adult patients diagnosed with unilateral ISSNHL between 2012 and 2019 who were treated with ITAoS due to incomplete or no recovery following systemic steroidal therapy. ETF was assessed by means tympanometry, before the initiation of ITAoS. The cohort was divided into an ET dysfunction group (ETD(+)) and a functioning ET group (control: ETD(-)). The audiologic response to treatment was recorded at the last follow-up. RESULTS: A total of 64 suitable patients [median (interquartile, IQR) age 49 (38-63) years] were enrolled. The ETD(+) group included 20 patients and the remaining 44 patients served as controls. Demographic and clinical parameters were not significantly different between the two groups at presentation. Hearing thresholds were improved significantly better, at frequencies 250, 500, 1, 2, 4, and 8 kHz (p = 0.001-0.040) in the ETD(+) group. CONCLUSION: ETD(+) is associated with better efficacy of ITAoS.

Intratympanic steroid injection versus hyperbaric oxygen therapy in refractory sudden sensorineural hearing loss: a meta-analysis.

OBJECTIVE: The present meta-analysis aims to compare the efficacy of intratympanic steroid (ITS) injection and hyperbaric oxygen (HBO) therapy as salvage treatments for refractory sudden sensorineural hearing loss (SSNHL). DATA SOURCES: Comprehensive searches were performed in PubMed, EMBASE and the Cochrane Library from the date of the database inception to June 2020. All studies reporting the use of salvage ITS and HBO treatments in refractory SSNHL patients were included. Subsequently, the full texts of the eligible studies were evaluated. METHODS: The quality and bias of the studies were assessed using the Newcastle-Ottawa Scale and Cochrane's risk of bias tools for nonrandomized and randomized studies, respectively. The data were analyzed using Comprehensive Meta-Analysis software (Version 3; Biostat, Englewood, NJ). RESULTS: Three hundred and fourteen subjects in 3 observational studies and 1 randomized controlled trial met our inclusion criteria. The pooled results demonstrated that there were no significant differences in the mean posttreatment hearing gain between the ITS and HBO groups. The changes in word discrimination and hearing gain at 250, 500, 1000, 2000, 4000 and 8000 Hz were also comparable between the two salvage treatment groups. CONCLUSIONS: The pooled results demonstrated that there were no significant differences in hearing improvements between salvage ITS injection and salvage HBO therapy after failed primary systemic steroid treatment in patients with SSNHL. However, spontaneous recovery could bias the treatment outcomes, and these results should be interpreted with caution. Clinicians may choose these salvage treatments according to personal experience and treatment availability. In cases in which specialized HBO facilities are difficult to access, salvage ITS injection can be provided with comparable responses.

Differences in responsiveness of intratympanic steroid injection for intractable vertigo in Meniere's disease.

OBJECTIVES: The efficacy of intratympanic steroid (ITS) injection for intractable Meniere's disease has been reported; however, its differences in responsiveness are not fully understood. This study investigated the clinical characteristics of patients who responded to ITS injection treatment. METHODS: This retrospective study included 32 patients with Meniere's disease who were unable to control frequent vertigo attacks despite conservative treatment for at least 3 months. They received an intratympanic injection of dexamethasone (3.3 mg/mL) in the affected side at least three times. We measured hearing threshold, subjective symptom scores, cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP), and performed glycerol and bithermal caloric tests. RESULTS: Satisfactory control of vertigo for 1 year after the first round of injection was found in 18 patients (56.3%; the response group). However, the injections failed to control vertigo in the other 14 patients (43.8%; the non-response group), and they were then treated with middle ear micropressure therapy. The response group showed improvement in low-frequency hearing, whereas hearing acuity did not change in the non-response group. Significantly reduced amplitude of cVEMP on the affected side was found in 62.5% of patients in the response group; however, no patients in the non-response group showed reduced amplitude of cVEMP. CONCLUSIONS: ITS injection significantly improved the subjective symptoms for intractable Meniere's disease; however, the long-term effects were heterogeneous. Our results suggest that reduced amplitude in cVEMP is associated with the effectiveness of ITS injection treatment.

Cluster Analysis to Identify Clinical Subtypes of Ménière's Disease.

OBJECTIVE: To identify distinct clinical subtypes of Ménière's disease by analyzing data acquired from a UK registry of patients who have been diagnosed with Ménière's disease. STUDY DESIGN: Observational study. METHODS: Patients with Ménière's disease were identified at secondary/tertiary care clinics. Cluster analysis was performed by grouping participants sharing similar characteristics and risk factors into groups based on a defined measure of similarity. RESULTS: A total of 411 participants were recruited into this study. Two main clusters were identified: participants diagnosed with ear infections (OR = 0.30, p < 0.014, 95% CI: 0.11-0.78) were more likely to be allocated in Cluster 1 (C1). Participants reporting tinnitus in both ears (OR = 11.89, p < 0.001, 95% CI: 4.08-34.64), low pitched tinnitus (OR = 21.09, p < 0.001, 95% CI: 7.47-59.54), and those reporting stress as a trigger for vertigo attacks (OR = 14.94, p < 0.001, 95% CI: 4.54-49.10) were significantly more likely to be in Cluster 2 (C2). Also, participants diagnosed with Benign Paroxysmal Positional Vertigo (OR = 13.14, <0.001, 95% CI: 4.35-39.74), autoimmune disease (OR = 5.97, p < 0.007, 95% CI: 1.62-22.03), depression (OR = 4.72, p < 0.056, 95% CI: 0.96-23.24), migraines (OR = 3.13, p < 0.008, 95% CI: 1.34-7.26), drug allergy (OR = 3.25, p < 0.029, 95% CI: 1.13-9.34), and hay fever (OR = 3.12, p < 0.009, 95% CI: 1.33-7.34) were significantly more likely to be clustered in C2. CONCLUSIONS: This study supports the hypothesis that Ménière's disease is a heterogeneous condition with subgroups that may be identifiable by clinical features. Two main clusters were identified with differing putative etiological factors. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3286-3292, 2024.

Trends in Procedural Management of Meniere's Disease: Analysis of a National Insurance Claims Database.

Objective: This study used a national insurance claims database to analyze trends in procedural management of Meniere's disease. Study Design: Retrospective cohort analysis. Setting: Database study using United States inpatient and outpatient insurance claims submitted from January 2003 to December 2021. Subjects and Methods: The Merative MarketScan Commercial and Medicare Claims Databases were queried for adults (≥18 years) with a diagnosis of Meniere's Disease according to International Classification of Diseases codes. Patients receiving procedures per Current Procedural Terminology codes for endolymphatic sac surgery, vestibular nerve section, labyrinthectomy, and intratympanic dexamethasone or gentamicin were identified. Temporal trends were analyzed by calculating annual percent change (APC) in the proportion of patients receiving procedures using Joinpoint regression. Results: A total of 16,523 unique patients with MD receiving procedural management were identified. From 2003 to 2021, the proportion of patients managed with intratympanic dexamethasone increased (APC 1.76 [95% CI 1.53-1.98], P < .001). The proportion of patients receiving intratympanic gentamicin increased from 2003 to 2015 (APC 4.43 [95% CI 1.29-7.66], P = .008) but decreased from 2015 to 2021 (APC -10.87 [95% CI -18.31 to -2.76], P = .013). The proportion of patients receiving endolymphatic sac surgery (APC: -10.20 [95% CI -11.19 to -9.20], P < .001) and labyrinthectomy (APC: -6.29 [95% CI -8.12 to -4.42], P < .001) decreased from 2003 to 2021. Conclusion: From 2003 to 2021, there has been an increase in the use of intratympanic dexamethasone and a decrease in the use of intratympanic gentamicin, endolymphatic sac surgery, and labyrinthectomy for procedural management of Meniere's Disease.

Idiopathic sudden sensorineural hearing loss: effectiveness of salvage treatment with intratympanic dexamethasone or hyperbaric oxygen therapy in addition to systemic steroids.

Background: The development of standardized treatments for idiopathic sudden sensorineural hearing loss (ISSNHL) is hampered by uncertainty over the etiology of this disorder. Systemic steroids are historically the primary therapy, with variable hearing outcomes. Over the last two decades, intratympanic steroids (ITS) and hyperbaric oxygen therapy (HBOT) have been proposed as salvage treatments in case of failure of systemic steroids. The present study aims to evaluate the effectiveness of these salvage treatments in addition to systemic steroids. Methods: We performed a retrospective study on 75 consecutive patients with a diagnosis of ISSNHL who were admitted to the Department of Otorhinolaryngology of our hospital between December 2018 and December 2022. All patients received primary treatment with systemic steroids. In case of slight or no hearing recovery within the 5th day from the beginning of the therapy (T1), a salvage treatment with ITS or HBOT was proposed. Patients were divided into three groups according to the therapy received: systemic steroids (group A), systemic steroids + HBOT (group B), and systemic steroids + ITS (group C). Pure-tone average at 500, 1000, 2000, and 3000 Hz and the mean gain were evaluated at T1 and 3 months after the beginning of the salvage treatment (T2). The hearing recovery was assessed according to the Siegel's criteria. Results: Sixty-two patients (31 men and 31 women, mean age 56 years) with failure of the primary treatment were definitively enrolled in the study: 34 (54.8%) in group A, 16 (25.8%) in group B, and 12 (19.4%) in group C. The ratio of patients responding to therapy was higher in group A (29.4%) than in groups B (18.75%) and C (16.7%). We did not find any statistically significant difference between groups in terms of mean hearing gain at T2 (17.4 ± 15.4 dB in group A vs. 18.6 ± 21.1 dB in group B and 15.7 ± 14.2 dB in group C, p = 0.9). Conclusion: In our experience, ITS or HBOT associated with systemic steroids, as salvage treatment, did not show significant improvement in hearing outcomes. The evolution of ISSNHL, regardless of the treatment, remains unpredictable.

Sudden Sensorineural Hearing Loss Post Coronavirus Disease: A Systematic Review of Case Reports.

The coronavirus disease of 2019 is a global pandemic disease severely affecting the upper respiratory tract that can be fatal in some instances. The virus most commonly affects the respiratory system. However, in certain cases it affects the other systems, including cardiovascular, renal, gastrointestinal, neurological, and auditory. Concerning the hearing and balance system, the microcirculation supply to the inner ear is hampered thus causing audiovestibular symptoms. Several case studies have reported sudden sensorineural hearing loss post-coronavirus disease and its detrimental impact on overall hearing. As both sudden sensorineural hearing loss and coronavirus disease deals with an emergency situation, there is a need to document case studies on how these individuals have been assessed and treated. The article has systematically reviewed these case reports involving a search strategy in databases like PubMed, PubMed Central, science direct, J-GATE, Google Scholar, and a manual Google Search.

Optimal timing of salvage intratympanic steroids in idiopathic sudden sensorineural hearing loss.

Background: Salvage intratympanic steroids (ITS) works in patients with idiopathic sudden sensorineural hearing loss (ISSNHL) after failure of initial therapy, but optimal timing of administration is unknown. Methods: Two hundred and seventy patients with ISSNHL were included. Among them, 180 were treated with ITS and standard medical treatment (SMT) and the other 90 received SMT along. The hearing threshold before and after salvage treatment were compared. The relationship between the salvage starting time and hearing recovery was analyzed. Results: The hearing of ITS group improved more than that of the SMT group in all frequency bands. The effect of both strategies decreases with the delay of the starting time. ITS can improve hearing even if being administrated 5 weeks after onset while SMT failed after 3 weeks. Conclusion: Patients with profound ISSNHL can obtain extra hearing recovery from salvage ITS. The earlier salvage starts, the greater the patient benefits.

Intratympanic Steroid Injection Complicated by Iatrogenic Perilymphatic Fistula: A Cautionary Tale.

Intratympanic (IT) steroid therapy is a mainstay treatment for sudden sensorineural hearing loss (SSNHL) for both initial therapy and salvage therapy. We report a rare case of iatrogenic perilymphatic fistula that resulted from trauma during an IT steroid injection for SSNHL. We discuss the diagnosis and treatment in the current case and compare it with previous reports from the literature. Laryngoscope, 131:2088-2090, 2021.

Residual Perforation Risk Assessment of Intratympanic Steroids via Tympanostomy Tube Versus Transtympanic Injections.

OBJECTIVES/HYPOTHESIS: To investigate the risk of residual tympanic membrane (TM) perforation after intratympanic (IT) steroidal treatment administered via transtympanic injection compared with trans-tympanostomy tube (TyT). STUDY DESIGN: Case series, systematic review and meta analysis. METHODS: Data were retrieved from the medical files of an original cohort of all consecutive patients with sudden sensorineural hearing loss necessitating IT steroidal treatment in a tertiary medical center between January 1, 2016 and November 20, 2020. A systematic literature search of "MEDLINE" via "PubMed," "Embase," and "Web of Science" on comparable published cases was performed and meta-analysis was established. RESULTS: Eighteen studies describing 818 ears were included in the quantitative meta-analysis in addition to a local cohort of 140 ears. The proportion of residual TM perforation was 1.11% and 1.14% (95% confidence interval: 0.01%-3.27% and 0.028%-2.38%) in the TyT and trans-tympanic groups, respectively, suggesting no significant difference in residual TM perforation risk between these techniques. CONCLUSION: IT steroid therapy via trans-TyT is not associated with more residual perforations than IT steroid therapy via transtympanic injections. LEVEL OF EVIDENCE: NA Laryngoscope, 131:E2583-E2591, 2021.

Sudden sensorineural hearing loss during pregnancy: etiology, treatment, and outcome.

OBJECTIVE: To analyze the etiologies, treatments, and outcomes of sensorineural hearing loss (SSNHL) during pregnancy. STUDY DESIGN: Retrospective chart review of 25 pregnant patients treated for SSNHL between January 2012 and September 2019. Forty-nine age matched non-pregnant women with severe and profound hearing loss diagnosed with SSNHL during the same period served as controls. Data were recorded on age, symptoms, onset of hearing loss, audiometric results, treatments, and outcomes. RESULTS: The mean age was 29.6 years (range 23-38 years). Intratympanic steroids (ITS) were administered in 15 (60.0%) pregnant women with SSNHL. Three women were treated with postauricular steroids only, while another woman was treated with intravenous ginkgo leaf extract and dipyridamole. The remaining six women received no medications. More than half (8/15, 53.3%) of pregnant women with SSNHL receiving ITS experienced hearing improvement. Pregnant women with profound hearing loss who received no medication had no hearing improvement. Most pregnant women with SSNHL (12/15, 80.0%) had higher fibrinogen levels than controls (mean values 3.77±0.71 g/L and 2.54±0.48 g/L, respectively). CONCLUSION: Fibrinogen could be a risk factor for SSNHL during pregnancy. ITS may benefit pregnant women with severe and profound SSNHL.

Pediatric sudden sensorineural hearing loss: Experience in a pediatric ENT emergency care center.

OBJECTIVES: Sudden sensorineural hearing loss (SSNHL) is relatively rare and its physiopathology remains unclear, particularly in children. Our goal was to evaluate clinical characteristics, etiologies, management, treatment outcomes and prognostic factors in the pediatric population. METHODS: We performed a retrospective chart review of all children registered for SSNHL between August 2004 and September 2017 in a tertiary care pediatric hospital. We analysed data regarding clinical symptoms, audiological characteristics, diagnostic investigations and treatment outcomes. RESULTS: Thirty-five patients were included. Mean age was 12 years (range 4-18 years). Male:female ratio was 15:20. Hearing loss was left-sided for 18 patients, right-sided for 12 patients and bilateral for 5 patients. Degree of hearing loss varied from mild to profound across frequencies in the 40 ears studied. Thirty-four patients had associated otologic symptoms: the most frequent was tinnitus (28 ears), followed by vertigo (23 ears), otalgia (5 ears) and sensation of blocked ear (5 ears). Twenty-nine patients received systemic steroids and 3 intra-tympanic steroids. In the treated group, 69% had improvement on the audiograms (14% total, 55% partial). Vestibular tests were performed in 16 patients and were abnormal in 10 patients. Radiological examination included computed tomography scan (n = 16) and/or magnetic resonance imaging (n = 33). They revealed 2 bilateral enlarged vestibular aqueducts, 1 labyrinthitis, 1 intra-cochlear haemorrhage. CONCLUSION: SSNHL can affect speech and language development in children. There are differences among the pediatric population, including inner ear malformation and immune disease. Specific work up is proposed. Appropriate diagnosis and therapeutic management are discussed.

Intratympanically Delivered Steroids Impact Thousands More Inner Ear Genes Than Systemic Delivery.

OBJECTIVES: Glucocorticoids are given for sensorineural hearing loss, but little is known of their molecular impact on the inner ear. Furthermore, in spite of claims of improved hearing recovery with intratympanic delivery of steroids, no studies have actually documented the inner ear molecular functions that are enhanced with this delivery method. METHODS: To assess steroid-driven processes in the inner ear, gene chip analyses were conducted on mice treated systemically with the glucocorticoids prednisolone or dexamethasone or the mineralocorticoid aldosterone. Other mice were given the same steroids intratympanically. Inner ears were harvested at 6 hours and processed on the Affymetrix 430 2.0 Gene Chip for expression of its 34 000 genes. Results were statistically analyzed for up or down expression of each gene against control (untreated) mice. RESULTS: Analyses showed approximately 17 500 genes are normally expressed in the inner ear and steroids alter expression of 55% to 82% of these. Dexamethasone changed expression of 9424 (53.9%) inner ear genes following systemic injection but 14 899 ear genes (85%) if given intratympanically. A similar pattern was seen with prednisolone, as 7560 genes were impacted by oral delivery and 11 164 genes (63.8%) when given intratympanically. The mineralocorticoid aldosterone changed expression of only 268 inner ear genes if given orally, but this increased to 10 124 genes (57.9%) if injected intratympanically. Furthermore, the glucocorticoids given actually impacted more inner ear genes via the mineralocorticoid receptor than the glucocorticoid receptor. CONCLUSIONS: Thousands of inner ear genes were affected by steroids, and this number increased significantly if steroids were delivered intratympanically. Also, the impact of glucocorticoids on inner ear mineralocorticoid functions is more substantial than previously known. Thus, the application of therapeutic steroids for hearing loss needs to be reassessed in light of their more comprehensive impact on inner ear genes. Furthermore, simply ascribing the efficacy of steroids to immunosuppression no longer appears to be warranted.

Sudden Sensorineural Hearing Loss Following Wasp Sting and Successful Treatment With Intratympanic Steroids.

OBJECTIVE: A sting by a Vespula vulgaris (wasp) should be considered as a cause of sudden sensorineural hearing loss. Although the mechanism of this cause is not well understood, management approach is similar to idiopathic sudden sensorineural hearing loss. METHODS: We describe a novel case of sudden sensorineural hearing loss encountered at a community otolaryngology clinic. It developed in a 26-year-old man after a sting to the ear canal by a V vulgaris (wasp) species. RESULTS: The patient failed to respond to oral steroids, but had complete recovery to normal hearing levels with intratympanic steroids. CONCLUSIONS: Sudden sensorineural hearing loss can be caused by the sting of a V vulgaris species and may be resolved with the use intratympanic steroids.

Sudden sensorineural hearing loss during pregnancy: clinical characteristics, management and outcome.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) may occur during pregnancy with a rare prevalence, and little is known about it. AIMS: To retrospectively analyze cases of SSNHL during pregnancy and investigate their clinical characteristics, management and outcome. MATERIAL AND METHODS: Records of 30 SSNHL patients during pregnancy were reviewed, including age, localization, duration from onset to treatment, gestation period, accompanying symptoms, initial hearing threshold, final hearing threshold, audiogram, treatment and outcome. RESULTS: Twenty-four patients (80.0%) suffered SSNHL in the second trimester or the last trimester with a high rate of tinnitus (70.0%). The initial hearing threshold was 63.4 ± 25.1 dB, and most audiograms were flat and profound. The overall recovery rate was 60.0%, including complete recovery (33.3%) and partial recovery (26.7%). Further, 16 patients received adjuvant intratympanic steroid showed a better audiologic outcome (improvement 27.1 ± 16.4 vs. 15.7 ± 12.0 dB, p = .042) than those who had not. CONCLUSIONS AND SIGNIFICANCE: SSNHL during pregnancy often occurred in the second trimester or the last trimester with a severe hearing loss, the most audiogram configurations are flat and profound. Dextran-40 is a safe and beneficial therapy for SSNHL patients during pregnancy and adjuvant intratympanic steroid increase the probability of hearing recovery.

Clinical Practice Guideline: Sudden Hearing Loss (Update).

OBJECTIVE: Sudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently but not universally accompanied by tinnitus and/or vertigo. Sudden sensorineural hearing loss affects 5 to 27 per 100,000 people annually, with about 66,000 new cases per year in the United States. This guideline update provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with sudden hearing loss. It focuses on sudden sensorineural hearing loss in adult patients aged ≥18 years and primarily on those with idiopathic sudden sensorineural hearing loss. Prompt recognition and management of sudden sensorineural hearing loss may improve hearing recovery and patient quality of life. The guideline update is intended for all clinicians who diagnose or manage adult patients who present with sudden hearing loss. PURPOSE: The purpose of this guideline update is to provide clinicians with evidence-based recommendations in evaluating patients with sudden hearing loss and sudden sensorineural hearing loss, with particular emphasis on managing idiopathic sudden sensorineural hearing loss. The guideline update group recognized that patients enter the health care system with sudden hearing loss as a nonspecific primary complaint. Therefore, the initial recommendations of this guideline update address distinguishing sensorineural hearing loss from conductive hearing loss at the time of presentation with hearing loss. They also clarify the need to identify rare, nonidiopathic sudden sensorineural hearing loss to help separate those patients from those with idiopathic sudden sensorineural hearing loss, who are the target population for the therapeutic interventions that make up the bulk of the guideline update. By focusing on opportunities for quality improvement, this guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. METHODS: Consistent with the American Academy of Otolaryngology-Head and Neck Surgery Foundation's "Clinical Practice Guideline Development Manual, Third Edition" (Rosenfeld et al. Otolaryngol Head Neck Surg. 2013;148[1]:S1-S55), the guideline update group was convened with representation from the disciplines of otolaryngology-head and neck surgery, otology, neurotology, family medicine, audiology, emergency medicine, neurology, radiology, advanced practice nursing, and consumer advocacy. A systematic review of the literature was performed, and the prior clinical practice guideline on sudden hearing loss was reviewed in detail. Key Action Statements (KASs) were updated with new literature, and evidence profiles were brought up to the current standard. Research needs identified in the original clinical practice guideline and data addressing them were reviewed. Current research needs were identified and delineated. RESULTS: The guideline update group made strong recommendations for the following: (KAS 1) Clinicians should distinguish sensorineural hearing loss from conductive hearing loss when a patient first presents with sudden hearing loss. (KAS 7) Clinicians should educate patients with sudden sensorineural hearing loss about the natural history of the condition, the benefits and risks of medical interventions, and the limitations of existing evidence regarding efficacy. (KAS 13) Clinicians should counsel patients with sudden sensorineural hearing loss who have residual hearing loss and/or tinnitus about the possible benefits of audiologic rehabilitation and other supportive measures. These strong recommendations were modified from the initial clinical practice guideline for clarity and timing of intervention. The guideline update group made strong recommendations against the following: (KAS 3) Clinicians should not order routine computed tomography of the head in the initial evaluation of a patient with presumptive sudden sensorineural hearing loss. (KAS 5) Clinicians should not obtain routine laboratory tests in patients with sudden sensorineural hearing loss. (KAS 11) Clinicians should not routinely prescribe antivirals, thrombolytics, vasodilators, or vasoactive substances to patients with sudden sensorineural hearing loss. The guideline update group made recommendations for the following: (KAS 2) Clinicians should assess patients with presumptive sudden sensorineural hearing loss through history and physical examination for bilateral sudden hearing loss, recurrent episodes of sudden hearing loss, and/or focal neurologic findings. (KAS 4) In patients with sudden hearing loss, clinicians should obtain, or refer to a clinician who can obtain, audiometry as soon as possible (within 14 days of symptom onset) to confirm the diagnosis of sudden sensorineural hearing loss. (KAS 6) Clinicians should evaluate patients with sudden sensorineural hearing loss for retrocochlear pathology by obtaining magnetic resonance imaging or auditory brainstem response. (KAS 10) Clinicians should offer, or refer to a clinician who can offer, intratympanic steroid therapy when patients have incomplete recovery from sudden sensorineural hearing loss 2 to 6 weeks after onset of symptoms. (KAS 12) Clinicians should obtain follow-up audiometric evaluation for patients with sudden sensorineural hearing loss at the conclusion of treatment and within 6 months of completion of treatment. These recommendations were clarified in terms of timing of intervention and audiometry and method of retrocochlear workup. The guideline update group offered the following KASs as options: (KAS 8) Clinicians may offer corticosteroids as initial therapy to patients with sudden sensorineural hearing loss within 2 weeks of symptom onset. (KAS 9a) Clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy within 2 weeks of onset of sudden sensorineural hearing loss. (KAS 9b) Clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy as salvage therapy within 1 month of onset of sudden sensorineural hearing loss. DIFFERENCES FROM PRIOR GUIDELINE: Incorporation of new evidence profiles to include quality improvement opportunities, confidence in the evidence, and differences of opinion Included 10 clinical practice guidelines, 29 new systematic reviews, and 36 new randomized controlled trials Highlights the urgency of evaluation and initiation of treatment, if treatment is offered, by emphasizing the time from symptom occurrence Clarification of terminology by changing potentially unclear statements; use of the term sudden sensorineural hearing loss to mean idiopathic sudden sensorineural hearing loss to emphasize that >90% of sudden sensorineural hearing loss is idiopathic sudden sensorineural hearing loss and to avoid confusion in nomenclature for the reader Changes to the KASs from the original guideline: KAS 1-When a patient first presents with sudden hearing loss, conductive hearing loss should be distinguished from sensorineural. KAS 2-The utility of history and physical examination when assessing for modifying factors is emphasized. KAS 3-The word "routine" is added to clarify that this statement addresses nontargeted head computerized tomography scan that is often ordered in the emergency room setting for patients presenting with sudden hearing loss. It does not refer to targeted scans, such as temporal bone computerized tomography scan, to assess for temporal bone pathology. KAS 4-The importance of audiometric confirmation of hearing status as soon as possible and within 14 days of symptom onset is emphasized. KAS 5-New studies were added to confirm the lack of benefit of nontargeted laboratory testing in sudden sensorineural hearing loss. KAS 6-Audiometric follow-up is excluded as a reasonable workup for retrocochlear pathology. Magnetic resonance imaging, computerized tomography scan if magnetic resonance imaging cannot be done, and, secondarily, auditory brainstem response evaluation are the modalities recommended. A time frame for such testing is not specified, nor is it specified which clinician should be ordering this workup; however, it is implied that it would be the general or subspecialty otolaryngologist. KAS 7-The importance of shared decision making is highlighted, and salient points are emphasized. KAS 8-The option for corticosteroid intervention within 2 weeks of symptom onset is emphasized. KAS 9-Changed to KAS 9A and 9B. Hyperbaric oxygen therapy remains an option but only when combined with steroid therapy for either initial treatment (9A) or salvage therapy (9B). The timing of initial therapy is within 2 weeks of onset, and that of salvage therapy is within 1 month of onset of sudden sensorineural hearing loss. KAS 10-Intratympanic steroid therapy for salvage is recommended within 2 to 6 weeks following onset of sudden sensorineural hearing loss. The time to treatment is defined and emphasized. KAS 11-Antioxidants were removed from the list of interventions that the clinical practice guideline recommends against using. KAS 12-Follow-up audiometry at conclusion of treatment and also within 6 months posttreatment is added. KAS 13-This statement on audiologic rehabilitation includes patients who have residual hearing loss and/or tinnitus who may benefit from treatment. Addition of an algorithm outlining KASs Enhanced emphasis on patient education and shared decision making with tools provided to assist in same.

Clinical Practice Guideline: Sudden Hearing Loss (Update) Executive Summary.

OBJECTIVE: Sudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently, but not universally, accompanied by tinnitus and/or vertigo. Sudden sensorineural hearing loss affects 5 to 27 per 100,000 people annually, with about 66,000 new cases per year in the United States. This guideline update provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with sudden hearing loss. It focuses on sudden sensorineural hearing loss in adult patients aged 18 and over and primarily on those with idiopathic sudden sensorineural hearing loss. Prompt recognition and management of sudden sensorineural hearing loss may improve hearing recovery and patient quality of life. The guideline update is intended for all clinicians who diagnose or manage adult patients who present with sudden hearing loss. PURPOSE: The purpose of this guideline update is to provide clinicians with evidence-based recommendations in evaluating patients with sudden hearing loss and sudden sensorineural hearing loss, with particular emphasis on managing idiopathic sudden sensorineural hearing loss. The guideline update group recognized that patients enter the health care system with sudden hearing loss as a nonspecific primary complaint. Therefore, the initial recommendations of this guideline update address distinguishing sensorineural hearing loss from conductive hearing loss at the time of presentation with hearing loss. They also clarify the need to identify rare, nonidiopathic sudden sensorineural hearing loss to help separate those patients from those with idiopathic sudden sensorineural hearing loss, who are the target population for the therapeutic interventions that make up the bulk of the guideline update. By focusing on opportunities for quality improvement, this guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. METHODS: Consistent with the American Academy of Otolaryngology-Head and Neck Surgery Foundation's Clinical Practice Guideline Development Manual, Third Edition, the guideline update group was convened with representation from the disciplines of otolaryngology-head and neck surgery, otology, neurotology, family medicine, audiology, emergency medicine, neurology, radiology, advanced practice nursing, and consumer advocacy. A systematic review of the literature was performed, and the prior clinical practice guideline on sudden hearing loss was reviewed in detail. Key action statements (KASs) were updated with new literature, and evidence profiles were brought up to the current standard. Research needs identified in the original clinical practice guideline and data addressing them were reviewed. Current research needs were identified and delineated. RESULTS: The guideline update group made strong recommendations for the following: clinicians should distinguish sensorineural hearing loss from conductive hearing loss when a patient first presents with sudden hearing loss (KAS 1); clinicians should educate patients with sudden sensorineural hearing loss about the natural history of the condition, the benefits and risks of medical interventions, and the limitations of existing evidence regarding efficacy (KAS 7); and clinicians should counsel patients with sudden sensorineural hearing loss who have residual hearing loss and/or tinnitus about the possible benefits of audiological rehabilitation and other supportive measures (KAS 13). These strong recommendations were modified from the initial clinical practice guideline for clarity and timing of intervention. The guideline update group made strong recommendation against the following: clinicians should not order routine computed tomography of the head in the initial evaluation of a patient with presumptive sudden sensorineural hearing loss (KAS 3); clinicians should not obtain routine laboratory tests in patients with sudden sensorineural hearing loss (KAS 5); and clinicians should not routinely prescribe antivirals, thrombolytics, vasodilators, or vasoactive substances to patients with sudden sensorineural hearing loss (KAS 11). The guideline update group made recommendations for the following: clinicians should assess patients with presumptive sudden sensorineural hearing loss through history and physical examination for bilateral sudden hearing loss, recurrent episodes of sudden hearing loss, and/or focal neurologic findings (KAS 2); in patients with sudden hearing loss, clinicians should obtain, or refer to a clinician who can obtain, audiometry as soon as possible (within 14 days of symptom onset) to confirm the diagnosis of sudden sensorineural hearing loss (KAS 4); clinicians should evaluate patients with sudden sensorineural hearing loss for retrocochlear pathology by obtaining a magnetic resonance imaging or auditory brainstem response (KAS 6); clinicians should offer, or refer to a clinician who can offer, intratympanic steroid therapy when patients have incomplete recovery from sudden sensorineural hearing loss 2 to 6 weeks after onset of symptoms (KAS 10); and clinicians should obtain follow-up audiometric evaluation for patients with sudden sensorineural hearing loss at the conclusion of treatment and within 6 months of completion of treatment (KAS 12). These recommendations were clarified in terms of timing of intervention and audiometry, as well as method of retrocochlear workup. The guideline update group offered the following KASs as options: clinicians may offer corticosteroids as initial therapy to patients with sudden sensorineural hearing loss within 2 weeks of symptom onset (KAS 8); clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy within 2 weeks of onset of sudden sensorineural hearing loss (KAS 9a); and clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy as salvage therapy within 1 month of onset of sudden sensorineural hearing loss (KAS 9b). DIFFERENCES FROM PRIOR GUIDELINE: Incorporation of new evidence profiles to include quality improvement opportunities, confidence in the evidence, and differences of opinion Included 10 clinical practice guidelines, 29 new systematic reviews, and 36 new randomized controlled trials Highlights the urgency of evaluation and initiation of treatment, if treatment is offered, by emphasizing the time from symptom occurrence Clarification of terminology by changing potentially unclear statements; use of the term sudden sensorineural hearing loss to mean idiopathic sudden sensorineural hearing loss to emphasize that over 90% of sudden sensorineural hearing loss is idiopathic sudden sensorineural hearing loss and to avoid confusion in nomenclature for the reader Changes to the key action statements (KASs) from the original guideline: KAS 1: When a patient first presents with sudden hearing loss, conductive hearing loss should be distinguished from sensorineural. KAS 2: The utility of history and physical examination when assessing for modifying factors is emphasized. KAS 3: The word routine is added to clarify that this statement addresses a nontargeted head computed tomography scan that is often ordered in the emergency room setting for patients presenting with sudden hearing loss. It does not refer to targeted scans such as a temporal bone computed tomography scan to assess for temporal bone pathology. KAS 4: The importance of audiometric confirmation of hearing status as soon as possible and within 14 days of symptom onset is emphasized. KAS 5: New studies were added to confirm the lack of benefit of nontargeted laboratory testing in sudden sensorineural hearing loss. KAS 6: Audiometric follow-up is excluded as a reasonable workup for retrocochlear pathology. Magnetic resonance imaging, computed tomography scan if magnetic resonance imaging cannot be done, or, secondarily, auditory brainstem response evaluation are the modalities recommended. A time frame for such testing is not specified, nor is it specified which clinician should be ordering this workup; however, it is implied that it would be the general or subspecialty otolaryngologist. KAS 7: The importance of shared decision making is highlighted, and salient points are emphasized. KAS 8: The option for corticosteroid intervention within 2 weeks of symptom onset is emphasized. KAS 9: Changed to KAS 9a and 9b; hyperbaric oxygen therapy remains an option but only when combined with steroid therapy for either initial treatment (9a) or for salvage therapy (9b). The timing is within 2 weeks of onset for initial therapy and within 1 month of onset of sudden sensorineural hearing loss for salvage therapy. KAS 10: Intratympanic steroid therapy for salvage is recommended within 2 to 6 weeks following onset of sudden sensorineural hearing loss. The time to treatment is defined and emphasized. KAS 11: Antioxidants were removed from the list of interventions that the clinical practice guideline recommends against using. KAS 12: Follow-up audiometry at conclusion of treatment and also within 6 months posttreatment is added. KAS 13: This statement on audiologic rehabilitation includes patients who have residual hearing loss and/or tinnitus who may benefit from treatment. Addition of an algorithm outlining KASs Enhanced emphasis on patient education and shared decision making with tools provided to assist in the same.

Idiopathic Sudden Sensorineural Hearing Loss: Average Time Elapsed Before Presentation to the Otolaryngologist and Effectiveness of Oral and/or Intratympanic Steroids in Late Presentations.

Objectives To determine how long after symptom onset that the average patient with an idiopathic sudden sensorineural hearing loss (ISSNHL) presents to the otolaryngology clinic.  In late presentations, to determine the time to presentation cutoff after which intervention may not be effective.  To evaluate the effectiveness of oral steroids versus a combination of oral and intratympanic steroid therapy in late presentations of ISSNHL.  Methods and procedures Sixty-four patients met inclusion criteria after chart review of 2,037 patients seen at Metro Health Hospital from 2006 to 2016 for sensorineural hearing loss. All sixty-four patients were used to calculate the average time to presentation, but only 40 were included to evaluate treatment efficacy because 24 were lost to follow-up or declined treatment. Audiograms were analyzed for baseline status and response to treatment. Therapy was either oral steroids or intratympanic (IT) steroids. Thirty-nine of the 40 treated patients received oral steroid therapy. Eighteen of these 39 patients received both oral and IT steroids. One patient received IT steroids only.  Results For all 64 patients in the study, the average time to presentation was 55 days, ranging from one day to 240 days. Data for 32 of the 40 treated patients were analyzed. These patients were further divided into smaller groups: Group 1 (N = 11) - treatment within seven days of symptom onset, Group 2 (N = 17) - time to treatment greater than seven days but less than 90 days of symptom onset, and Group 3 (N = 4) - greater than 90 days of symptom onset. In Group 2, there was a significant improvement in pure tone average (P-value: 0.005). Forty-seven percent of patients in this group had objective treatment response utilizing Wilson's criteria. Two patients had a complete recovery and six had a partial recovery. Hearing gains ranged from 10 dB (decibels) to 23 dB. Sixty-three percent of patients with objective improvement also had subjective improvement. In Group 3, none of the patients met Wilson's criteria for recovery. There was no statistically significant difference in response between patients treated with oral steroids only versus a combination of oral and IT steroids. Conclusion Patients with ISSNHL present to an otolaryngologist on average 55 days after symptom onset. There is statistically and clinically significant response to treatment in late presenters. Improvement can be seen up to three months from symptom onset. Oral steroid therapy is effective. IT steroid therapy may have an added benefit.

Potential Benefits of Combination Therapy as Primary Treatment for Sudden Sensorineural Hearing Loss.

OBJECTIVE: We analyzed the effectiveness of combination therapy (CT) for idiopathic sudden sensorineural hearing loss (ISSNHL) and the utility of intratympanic dexamethasone injection (ITDI) reapplication as salvage treatment for ISSNHL refractory to CT. STUDY DESIGN: Case series with chart review. SETTING: Academic university hospital. SUBJECTS AND METHODS: We reviewed 229 patients with ISSNHL and divided these patients into 2 groups according to treatment: systemic steroid therapy (SST) and CT groups. The SST group received prednisolone therapy. The CT group also received ITDI daily. Patients who demonstrated no recovery (<10 dB) after initial treatment were defined as refractory and received salvage ITDI therapy: ITDI reapplication in the CT group and ITDI application in the SST group. RESULTS: Hearing recovery rates were 77.8% (77/99) in the CT group and 60.8% (79/130) in the SST group. The difference was statistically significant (P = .011). Initial pure-tone audiometry and vertigo were affective factors on hearing recovery rates in the CT group. After salvage therapy, hearing improvement of 10 dB or greater was noted in 6 of the 22 (27.3%) patients in the CT group and 16 of the 51 (31.4%) patients in the SST group. The difference in efficacy of salvage therapy between the CT and SST groups was simply not significant (P = .612). CONCLUSIONS: Combination therapy was more effective for ISSNHL in achieving hearing gain than SST alone. Furthermore, ITDI reapplication for ISSNHL refractory to CT was as effective as salvage ITDI for ISSNHL refractory to SST.

Menière's disease.

Menière's disease (MD) is a chronic multifactorial disorder of the inner ear characterized by episodic vestibular symptoms associated with sensorineural hearing loss, tinnitus, and aural pressure. Epidemiologic and genomic evidence supports a genetic susceptibility with multiple biochemical pathways involved, including the endocrine system, innate immune response, and autonomic nervous system. Allergens, infectious agents, vascular events, or genetic factors could modify inner-ear homeostasis and trigger MD. The diagnosis of MD is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing loss, tinnitus, and/or fullness) in the affected ear. Headache is also found during the attacks and bilateral involvement is found in 25-40% of cases. Audiologic and vestibular assessment is recommended to monitor the clinical course. The treatment of MD is symptomatic to obtain relief of vestibular episodes and preventive to limit hearing loss progression. Treatment options include sodium restriction, betahistine, intratympanic gentamicin, or steroids and eventually surgery, such as cochlear implantation.