An Italian case series' description of thiamine responsive megaloblastic anemia syndrome: importance of early diagnosis and treatment.

BACKGROUND: Individuals with thiamine-responsive megaloblastic anemia (TRMA) mainly manifest macrocytic anemia, sensorineural deafness, ocular complications, and nonautoimmune diabetes. Macrocytic anemia and diabetes may be responsive to high-dosage thiamine treatment, in contrast to sensorineural deafness. Little is known about the efficacy of thiamine treatment on ocular manifestations. CASES PRESENTATION: Our objective is to report data from four Italian TRMA patients: in Cases 1, 2 and 3, the diagnosis of TRMA was made at 9, 14 and 27 months. In 3 out of 4 subjects, thiamine therapy allowed both normalization of hyperglycemia, with consequent insulin suspension, and macrocytic anemia. In all Cases, thiamine therapy did not resolve the clinical manifestation of deafness. In Cases 2 and 3, follow-up showed no blindness, unlike Case 4, in which treatment was started for megaloblastic anemia at age 7 but was increased to high doses only at age 25, when the genetic diagnosis of TRMA was performed. CONCLUSIONS: Early institution of high-dose thiamine supplementation seems to prevent the development of retinal changes and optic atrophy in TRMA patients. The spectrum of clinical manifestations is broad, and it is important to describe known Cases to gain a better understanding of this rare disease.

Case Report: Leptospirosis Complicated by Persistent, Bilateral Sensorineural Hearing Loss.

The clinical manifestations of leptospirosis range from mild to life-threatening and can impact on multiple organ systems. A wide array of neurological manifestations of leptospirosis have been reported, although the pathophysiology of neuroleptospirosis remains incompletely understood. We present a case of leptospirosis complicated by bilateral sensorineural deafness, with nodular meningitis demonstrated in the internal auditory meatus on magnetic resonance imaging. The patient was treated with doxycycline, ceftriaxone, systemic and topical steroids, and hyperbaric oxygen therapy, with modest, but incomplete, improvement.

Space Station-like Composite Nanoparticles for Co-Delivery of Multiple Natural Compounds from Chinese Medicine and Hydrogen in Combating Sensorineural Hearing Loss.

Ototoxic drugs such as aminoglycoside antibiotics and cisplatin (CDDP) can cause sensorineural hearing loss (SNHL), which is closely related to oxidative stress and the acidification of the inner ear microenvironment. Effective treatment of SNHL often requires multifaceted approach due to the complex pathology, and drug combination therapy is expected to be at the forefront of modern hearing loss treatment. Here, space-station-like composite nanoparticles (CCC@mPP NPs) with pH/oxidation dual responsiveness and multidrug simultaneous delivery capability were constructed and then loaded with various drugs including panax notoginseng saponins (PNS), tanshinone IIA (TSIIA), and ammonia borane (AB) to provide robust protection against SNHL. Molecular dynamics simulation revealed that carboxymethyl chitosan/calcium carbonate-chitosan (CCC) NPs and monomethoxy poly(ethylene glycol)-PLGA (mPP) NPs can rendezvous and dock primarily by hydrogen bonding, and electrostatic forces may be involved. Moreover, CCC@mPP NPs crossed the round window membrane (RWM) and entered the inner ear through endocytosis and paracellular pathway. The docking state was basically maintained during this process, which created favorable conditions for multidrug delivery. This nanosystem was highly sensitive to pH and reactive oxygen species (ROS) changes, as evidenced by the restricted release of payload at alkaline condition (pH 7.4) without ROS, while significantly promoting the release in acidic condition (pH 5.0 and 6.0) with ROS. TSIIA/PNS/AB-loaded CCC@mPP NPs almost completely preserved the hair cells and remained the hearing threshold shift within normal limits in aminoglycoside- or CDDP-treated guinea pigs. Further experiments demonstrated that the protective mechanisms of TSIIA/PNS/AB-loaded CCC@mPP NPs involved direct and indirect scavenging of excessive ROS, and reduced release of pro-inflammatory cytokines. Both in vitro and in vivo experiments showed the high biocompatibility of the composite NPs, even after long-term administration. Collectively, this work suggests that composite NPs is an ideal multi-drug-delivery vehicle and open new avenues for inner ear disease therapies.

Steroid Efficacy on Audiologic Recovery in Patients With Sudden Sensorineural Hearing Loss and Vestibular Schwannoma: A Retrospective Review.

OBJECTIVES: The objectives of this study are to (i) estimate the incidence of vestibular schwannoma (VS) among patients in an integrated healthcare system who present for evaluation of sudden sensorineural hearing loss (SSNHL) and (ii) evaluate the efficacy of empiric steroid therapy on audiologic recovery among SSNHL patients ultimately diagnosed with VS. METHODS: A retrospective chart review was performed on patients presenting with SSNHL in 2021 at a multicenter integrated healthcare system serving over 4 million members. Patient demographics, audiometric data, VS diagnosis, therapeutic steroid intervention, and data regarding treatment response were recorded. A clinically significant audiometric improvement was defined as (i) an increase of 15% in word recognition score, (ii) a decrease of 15 dB in four-frequency pure-tone average (PTA) using frequencies of 500, 1000, 2000, and 4000 Hz, or (iii) a PTA of <20 dB on follow-up audiogram. RESULTS: Six hundred fifty-eight patients were reviewed, of which 309 (56.0% male; mean, 57.5 years) met the inclusion criteria with audiometric data and magnetic resonance imaging data. Ten patients (70.0% male; mean, 51.3 years) were found to have VS. Of these, five patients received oral steroid therapy alone, and five had combination therapy (oral + intratympanic steroid injections). No patients received intratympanic steroid therapy alone. Median PTA improvement with steroid therapy was 3.1-dB hearing loss, and median word recognition score improvement was 16.5%. Six of 10 patients demonstrated clinically significant audiometric improvement with steroid therapy. CONCLUSION: This study represents the largest US-based study showcasing the prevalence of VS in patients originally presenting with SSNHL. It also reinforces previous findings that VS does not preclude trials of steroid therapy.

Sudden hearing loss and early hyperbaric oxygen therapy: A preliminary study.

Purpose: Sudden sensorineural hearing loss (SSNHL) is a time-sensitive urgent condition. The aim of this study was to evaluate the frequency of hearing improvement in patients with idiopathic SSNHL who only received hyperbaric oxygen (HBO2) therapy within three days of symptom onset, instead of conventional corticosteroid treatment. Methods: The medical charts of patients who experienced SSNHL between January 1, 2012, and December 31, 2021, were reviewed. The present study included all adult patients who were diagnosed with idiopathic SSNHL and started HBO2 therapy within 72 hours of symptom onset. These subjects did not take corticosteroids due to contraindications or because they were concerned about possible side effects. The HBO2 therapy protocol consisted of at least 10 sessions of 85 minutes each with pure oxygen inhalation at 2.5 atmospheres absolute pressure. Results: Overall, 49 subjects (26 males and 23 females) met the inclusion criteria, with a mean age of 47 (± 20.4) years. The mean initial hearing threshold was 69.8 dB (±18.0). After HBO2 therapy, complete hearing recovery was observed in 35 patients (71.4%), and the mean hearing threshold improved significantly (p≺0.001) to 31.4 dB (±24.5). In patients with complete hearing recovery, no significant differences were found between males and females (p=0.79), right and left ears (p=0.72) or initial grades of hearing loss (p=0.90). Conclusion: This study suggests that, in the absence of the confounding effect of concurrent steroid therapy, starting HBO2 therapy within three days of symptom onset could have a positive impact on patients with idiopathic SSNHL.

Combination of Hyperbaric Oxygen Therapy and Oral Steroids for the Treatment of Sudden Sensorineural Hearing Loss: Early or Late?

Background and Objectives: Several treatments are available for sudden sensorineural hearing loss (SSNHL), but no studies have compared the different treatments based on the delay from the onset of the disease. Our study aims to compare the effect of hyperbaric oxygen therapy (HBOT), oral steroids (OS) and combination of both therapies (HBOT + OS) for treating SSNHL. Materials and Methods: This randomized study analyzed 171 patients with SSNHL. Patients were evaluated by pure tone audiometry test (PTA) at baseline (T0) and 20 days after treatment (T1). Three groups were available HBOT-A-, OS-B- and HBOT + OS-C-. After baseline PTA, patients were randomly assigned to each group. Statistical analysis was performed by one-way ANOVA and Chi-square. Results: Patients in the HBOT + OS and HBOT groups improved their auditory function (p < 0.05). HBOT was the best choice for treatment when started by 7 days from SSNHL onset, while HBOT + OS in case of late treatment. Profound SNHL recovered equally by HBOT and HBOT + OS (p < 0.05). Upsloping SNHL obtained better auditory results by HBOT compared to HBOT + OS (p < 0.05). Downsloping and flat SSNHL had the most improvement with HBOT + OS compared to HBOT only (p < 0.05). Conclusions: Combination of HBOT and OS is a valid treatment for SSNHL both in case of early and late treatment. Combination of HBOT and OS was the choice with the best results in case of treatment started >14 days from symptom onset.

Efficacy and safety of standardized Ginkgo biloba L. leaves extract as an adjuvant therapy for sudden sensorineural hearing loss: A systematic review and meta-analysis.

ETHNOPHARMACOLOGICAL RELEVANCE: Ginkgo biloba L. has been used for medical purposes in China for centuries. Standardized Ginkgo biloba L. leaves extract (GBE) is a widely used botanical drug which displays a variety of pharmacological effects against sudden sensorineural hearing loss (SSNHL). AIM OF THE STUDY: To evaluate the efficacy and safety of GBE as an adjuvant therapy, administered with corticosteroids, for the initial management of patients with SSNHL. MATERIALS AND METHODS: We searched seven databases for randomized clinical trials (RCTs) comparing GBE plus corticosteroids with corticosteroids alone for SSNHL treatment. Data analysis was carried out by Review Manager 5.4 and Stata 16.0 software. The study was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. We subsequently evaluated the certainty of evidence using the GRADE (Grading of Recommendation Assessment, Development and Evaluation) approach. RESULTS: A total of 11 RCTs involving 1069 patients were included. Meta-analysis indicated that the clinical cure rate (RR = 1.33, 95% CI = 1.12 to 1.58, P = 0.001) and total effective rate (RR = 1.24, 95%CI = 1.17 to 1.31, P < 0.001) in SSNHL patients receiving GBE plus corticosteroids was superior to patients receiving corticosteroids alone. After treatment, pure tone average (PTA) improvement of observation group was better than that in the control group (WMD = 10.33, 95%CI = 6.46 to 14.21, P < 0.001). The levels of whole blood high shear viscosity (WMD = 0.93, 95%CI = 0.28 to 1.59, P = 0.005), whole blood medium shear viscosity (WMD = 0.53, 95%CI = 0.11 to 0.95, P = 0.01), whole blood low shear viscosity (WMD = 1.26, 95%CI = 0.80 to 1.72, P < 0.001), plasma viscosity (WMD = 0.19, 95%CI = 0.09 to 0.30, P < 0.001) and fibrinogen (WMD = 0.80, 95%CI = 0.25 to 1.35, P = 0.004) were lower than those in the control group. There was no significant difference in the change of hematokrit between two groups (WMD = 4.23, 95%CI = -0.54 to 8.99, P = 0.08). GBE was generally well tolerated, and there was no significant difference in the incidence of adverse reactions between two groups (RR = 1.01, 95%CI = 0.57 to 1.79, P = 0.97). CONCLUSION: The results of the current study suggested that GBE might be effective and promising as an adjuvant to corticosteroids in the initial treatment of moderate to profound SSNHL. However, the GRADE assessment indicated that the overall strength of evidence was not high. Further studies with high methodological quality and low risk of bias are needed to confirm the positive results. PROSPERO registration No. CRD 42020190113.

A case report of sudden-onset auditory neuropathy spectrum disorder associated with Brown-Vialetto-Van Laere syndrome (riboflavin transporter deficiency).

OBJECTIVE: The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes. DESIGN: We report the audiological and clinical profile of a child who presented with a complaint of sudden loss of speech understanding associated with an atypical form of ANSD. He was later diagnosed with BVVL. STUDY SAMPLE: An 11-year-old boy with ANSD associated with BVVL. RESULTS: The patient's severe neurological symptoms improved within a year of supplementation with high doses of riboflavin. His fluctuating hearing loss and 0% WDS remained unchanged. The patient was able to use hearing aids without any discomfort after treatment initiation, but he stopped using them again due to a lack of benefit in speech understanding. Although cochlear implantation was recommended, the patient and his family decided not to consider it for another year since they still had hope for complete recovery. CONCLUSIONS: Sudden-onset ANSD can be the earliest sign of undetected BVVL syndrome. Early detection of BVVL is crucial since all symptoms can be reversible with an early intervention of high doses of riboflavin supplementation.

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.

Leber congenital amaurosis as an initial manifestation in a Chinese patient with thiamine-responsive megaloblastic anemia syndrome.

Thiamine-responsive megaloblastic anemia syndrome (TRMA) is an autosomal recessive disorder, inherited by the defective SLC19A2 gene that encodes a high-affinity thiamine transporter (THTR-1). TRMA is characterized by the occurrence of classical triad manifestations including megaloblastic anemia, diabetes mellitus, and sensorineural deafness. In addition to the systemic manifestations, ophthalmic features can be present and include retinitis pigmentosa, optic atrophy, cone-rod dystrophy, maculopathy, and Leber congenital amaurosis. Here we report a 6-year-old boy presenting severe early-onset retinal dystrophy with the initial diagnosis of Leber congenital amaurosis, which followed for 12 years. Diabetes mellitus occurred 3 years after vision problem. Eosinophilic granuloma of the left scapula was confirmed at 13 years old. Whole-exome sequencing was performed to identify two novel compound heterozygous variants c.725dupC (p.Ala243Serfs*3) and c.121G>A (p.Gly41Ser) in SLC19A2 gene (NM_006996.3). Oral thiamine supplementation treatment was initiated at 13 years. This case demonstrates Leber congenital amaurosis can present as the first clinical feature before systemic manifestations. Phenotypic variety should be aware and multidisciplinary teamwork and regular follow-up are important for TRMA patient care.

Hyperbaric Oxygen Therapy Versus Intratympanic Steroid for Salvage Treatment of Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis.

OBJECTIVE: The aim of this meta-analysis is to compare the efficacy of hyperbaric oxygen therapy (HBOT) and intratympanic steroids (ITS) as salvage treatment for patients with refractory sudden sensorineural hearing loss (SSNHL). DATA SOURCES: Electronic search was performed in the PubMed, Embase, Cochrane Library, CNKI, and Wan Fang databases to June 1, 2020. METHODS: For each outcome measure, a forest plot was generated and a pooled relative risk (RR) or mean difference (MD) was calculated. Potential publication bias in the meta-analysis was assessed using funnel plot. RESULTS: The numbers of cases with hearing improvements and pure tone averages (PTA) changes after salvage treatment were entered into the R software to calculate the pooled effect of HBOT compared with ITS. When pooling the results of the studies reporting the proportion of patients with hearing improvement, a fixed-effects model was used. We calculated the RR and found no significant difference when HBOT compared with ITS (RR = 1.09, 95% confidence interval [CI]: 0.83-1.42, p = 0.55). With respect to the PTA changes, a fixed-effects model was used. The improvement in the PTA (in dB) was calculated in MD and no significant difference was found between the two groups (MD = 0.55, 95% CI: -1.76-2.86, p = 0.64). CONCLUSION: Both HBOT and ITS offer some benefits for refractory SSNHL patients, and there were no significant differences in hearing outcomes between the two modalities. Future RCTs that include large samples are needed to demonstrate superiority of one of the treatments.

Combined Acupuncture-Hyperbaric Oxygen-Steroids Therapy for Idiopathic Sudden Sensorineural Hearing Loss: A Retrospective Observational Study.

Objectives: Acupuncture, widely used in Chinese society, has been studied as an adjunct treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). The purpose of this study is to investigate the effectiveness of combined acupuncture and hyperbaric oxygen therapy (HBOT) with conventional steroid therapy for ISSNHL. Methods: This retrospective chart review enrolled 154 patients who met the ISSNHL criteria and were categorized into three groups according to the different treatment regimens. Among these patients, 43 underwent steroid therapy only (S) group, 74 received steroid and HBOT (S-H) group, and the remaining 37 were treated with combined acupuncture-HBOT in addition to steroid therapy (S-H-A) group. The outcome was determined by comparing the differences in pure-tone thresholds and absolute hearing gains after treatment calculated at each audiometric octave frequency or grouped frequencies of audiograms. Hearing recoveries classified into three grades: complete, partial, and poor were also analyzed and compared among different treatment groups. Results: All subjects presented with initial severe hearing loss with averaged hearing thresholds >70 dB. The S-H-A group exhibited good hearing improvement outcomes at each audiometric octave frequency and grouped frequencies of audiograms, with greater hearing gain and had more favorable outcomes in hearing recovery grades compared with the S group and the S-H group. Conclusions: The results obtained in this study revealed a preliminary finding of ISSNHL patients benefiting from combined acupuncture, HBOT, and conventional steroid therapy. Acupuncture is a safe and nonpharmacologic treatment option and can be considered as an initial treatment strategy in such a clinical scenario.

Riboflavin in Neurological Diseases: A Narrative Review.

Riboflavin is classified as one of the water-soluble B vitamins. It is part of the functional group of flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD) cofactors and is required for numerous flavoprotein-catalysed reactions. Riboflavin has important antioxidant properties, essential for correct cell functioning. It is required for the conversion of oxidised glutathione to the reduced form and for the mitochondrial respiratory chain as complexes I and II contain flavoprotein reductases and electron transferring flavoproteins. Riboflavin deficiency has been demonstrated to impair the oxidative state of the body, especially in relation to lipid peroxidation status, in both animal and human studies. In the nervous system, riboflavin is essential for the synthesis of myelin and its deficiency can determine the disruption of myelin lamellae. The inherited condition of restricted riboflavin absorption and utilisation, reported in about 10-15% of world population, warrants further investigation in relation to its association with the main neurodegenerative diseases. Several successful trials testing riboflavin for migraine prevention were performed, and this drug is currently classified as a Level B medication for migraine according to the American Academy of Neurology evidence-based rating, with evidence supporting its efficacy. Brown-Vialetto-Van Laere syndrome and Fazio-Londe diseases are now renamed as "riboflavin transporter deficiency" because these are autosomal recessive diseases caused by mutations of SLC52A2 and SLC52A3 genes that encode riboflavin transporters. High doses of riboflavin represent the mainstay of the therapy of these diseases and high doses of riboflavin should be rapidly started as soon as the diagnosis is suspected and continued lifelong. Remarkably, some mitochondrial diseases respond to supplementation with riboflavin. These include multiple acyl-CoA-dehydrogenase deficiency (which is caused by ETFDH gene mutations in the majority of the cases, or mutations in the ETFA and ETFB genes in a minority), mutations of ACAD9 gene, mutations of AIFM1 gene, mutations of the NDUFV1 and NDUFV2 genes. Therapeutic riboflavin administration has been tried in other neurological diseases, including stroke, multiple sclerosis, Friedreich's ataxia and Parkinson's disease. Unfortunately, the design of these clinical trials was not uniform, not allowing to accurately assess the real effects of this molecule on the disease course. In this review we analyse the properties of riboflavin and its possible effects on the pathogenesis of different neurological diseases, and we will review the current indications of this vitamin as a therapeutic intervention in neurology.

Long-term efficacy of triple semicircular canal plugging in the treatment of patients with ipsilateral delayed endolymphatic hydrops.

This study aims to explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable ipsilateral delayed endolymphatic hydrops (DEH), so as to provide an alternative therapy for this disease. Forty-eight patients diagnosed with ipsilateral DEH referred to vertigo clinic of our hospital between Dec. 2010 and Dec. 2017, were included in this study for retrospective analysis. All patients were followed up for 2 years. Vertigo control and auditory functions were measured and analyzed. Pure tone audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) were performed in two-year follow-up. Forty-five patients who accepted intratympanic gentamicin (26.7 mg/mL) twice given one week apart were selected as a control group. The total control rate of vertigo in TSCP group was 97.9% (47/48) in the two-year follow-up, with complete control rate of 83.3% (40/48) and substantial control rate of 14.6% (7/48). The rate of hearing loss was 22.9% (11/48). The total control rate of vertigo in intratympanic gentamicin group was 80.0% (36/45), with complete control rate of 57.8% (26/45) and substantial control rate of 22.2% (10/45), and the rate of hearing loss was 20.0% (9/45). The vertigo control rate of TSCP was significantly higher than that of intratympanic gentamicin (χ2 = 6.01, p < 0.05). There was no significant difference of hearing loss rate between two groups. (χ2 = 0.12, p > 0.05). TSCP, which can reduce vertiginous symptoms in patients with intractable ipsilateral DEH, represents an effective therapy for this disorder.

Effectiveness of Ginkgo biloba diterpene lactone in the treatment of sudden sensorineural hearing loss.

PURPOSE: To evaluate the effectiveness of systemic Ginkgo biloba diterpene lactone therapy for sudden sensorineural hearing loss. METHODS: This retrospective review investigated 56 patients with unilateral sudden sensorineural hearing loss. Among them, 26 patients received conventional therapy (group C, intravenous methylprednisolone), and 30 received conventional therapy supplemented with Ginkgo biloba diterpene lactone (group G). Pure tone audiometry was measured before treatment and 1 month after treatment. The average pure tone audiometry gain, pure tone audiometry gain at each frequency, pure tone audiometry gain according to initial hearing loss, and rate of effectiveness were defined as functionally relevant recovery of hearing and compared between the two groups. RESULTS: The average pure tone audiometry gain was significantly greater in group G (20.6 ± 15.1 dB) than in group C (11.9 ± 13.3 dB) (p = 0.025), with similar trends at 250, 1 k, and 8 k Hz. In the subgroup of patients with profound hearing loss (initial pure tone audiometry >70 dB), hearing gain was significantly higher in group G (26.7 ± 14.4 dB) than in C (5.5 ± 9.0 dB) (p = 0.034). In the mild-moderate hearing loss subgroup (initial pure tone audiometry ≤70 dB), the pure tone audiometry gain did not differ significantly (group G: 18.4 ± 14.3 dB; group C: 13.0 ± 13.4 dB) (p = 0.209). The overall rate of effectiveness was 73.3% and 57.7% in groups G and C, respectively; however, the difference was statistically insignificant (p = 0.218). CONCLUSIONS: Compared with conventional therapy alone, supplementary systemic administration of Ginkgo biloba diterpene lactone to treat sudden sensorineural hearing loss could improve hearing recovery, especially, in patients with profound hearing loss.

Prediction of hearing outcomes by auditory steady-state response in patients with sudden sensorineural hearing loss.

BACKGROUND: There has been no report on the predictive value of auditory steady-state response (ASSR) in the hearing prognosis of sudden sensorineural hearing loss (SSNHL). AIMS/OBJECTIVES: To investigate whether ASSR can be a prognostic indicator of hearing outcome in patients with SSNHL after systemic steroid treatment. MATERIAL AND METHODS: Fifty-three patients with unilateral mild to severe SSNHL (≤90 dB HL at 0.5k, 1k, 2k, and 4 kHz, 4FA) were included. All patients received systemic high dose steroid therapy within one month after onset. The difference between the threshold levels measured by ASSR and PTA on the same day [ASSR - PTA] was calculated. The hearing recovery (HR) was defined as a < 30 dB HL of final degree of hearing loss and a > 15 dB HL of hearing gain. RESULTS: The HR (+) group showed significantly worse ASSR predicted threshold than pure-tone threshold in univariate (t(51) = 2.412, p = .020) and multivariate analysis (OR 0.910, p = .012). The [ASSR - PTA] threshold showed significantly moderate correlation with hearing gain (r = -0.303, p = .028). CONCLUSIONS: Worse ASSR predicted threshold than pure-tone threshold predicted poor hearing outcome after systemic steroid treatment in mild to severe unilateral SSNHL.

Relapse of rare diseases during COVID-19 pandemic: bicytopenia in an adult patient with thiamine-responsive megaloblastic anaemia.

Thiamine-responsive megaloblastic anaemia (TRMA) is a syndrome associated with megaloblastic anaemia, diabetes mellitus and sensorineural deafness, due to mutations in the SLC19A2gene, which codes for a thiamine carrier protein. Oral thiamine supplementation is the main treatment. We report the case of a 19-year-old man known for TRMA, who presented in the emergency department with bicytopenia (haemoglobin 5,4 g/dL, thrombocytes 38×109/L) revealed by dyspnea and chest pain. Investigations excluded bleeding, hemolysis, coagulopathy and iron deficiencies. A recent infection and an acute coronary syndrome have also been eliminated. We later found out that thiamine treatment had been discontinued three months before, due to general confinement in Tunisia during the COVID-19 pandemic. Parenteral administration of 100 mg of thiamine daily resulted in the recovery of haematopoiesis within three weeks.

Nimodipine and Steroid Combination Therapy for Idiopathic Sudden Sensorineural Hearing Loss.

OBJECTIVE: To evaluate the treatment outcomes of nimodipine and steroid combination therapy for idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Seventy-eight patients who were diagnosed with ISSNHL were divided into two group based on the treatment strategies used: steroid+nimodipine (SN, n = 36) and steroid only (SO, n = 42) groups. Based on the level of hearing loss before treatment, subgroup analysis (<90 dB HL, SN-S versus SO-S groups; ≥90 dB HL, SN-P versus SO-P groups) was performed. INTERVENTIONS: Nimodipine+dexamethasone versus dexamethasone alone. MAIN OUTCOME MEASURES: Hearing thresholds and complete/partial recovery rate after treatment. RESULTS: Hearing thresholds after treatment were not significantly different between the SN and SO groups (46.8 ±â€Š29.4 versus 54.8 ±â€Š27.6 dB HL, p = 0.218). However, the complete recovery rate was significantly higher in the SN group than in the SO group (41.7% versus 16.8%, p = 0.014). In subgroup analysis, the complete recovery rate was significantly higher in the SN-S group than in the SO-S group (60.9% versus 19.2%, p = 0.003), whereas the difference between the SN-P and SO-P groups was not significant (7.7% versus 12.5%, p = 0.672). The cumulative incidence of complete recovery was significantly higher in SN-S group than in the SO-S group (p = 0.005); the mean recovery time was 4.4 weeks (95% confidence interval [CI], 2.8-6.1) in the SN-S group and 8.8 weeks (95% CI, 7.0-10.5) in the SO-S group. CONCLUSIONS: The results of this study suggest that nimodipine and steroid combination therapy for ISSNHL results in a higher complete recovery rate than steroid alone in patients with moderate to severe hearing loss.

Frequency-specific efficacy of intratympanic steroid on idiopathic sudden sensorineural hearing loss.

Background: Hearing recovery would be different in each sound frequency in patients with idiopathic sudden sensorineural hearing loss (ISSNHL).Aims/objectives: To analyze frequency-specific efficacy of intratympanic steroid on ISSNHL.Materials and methods: Of a total of 381 patients with ISSNHL (hearing threshold ≥40 dB; ≤30 days until treatment), 174 patients (174 ears) received systemic steroid plus hyperbaric oxygen therapy (HBO group), and 207 patients (208 ears) received systemic plus intratympanic steroid (IT group). Hearing thresholds at 125-8000 Hz were measured at every octave before and after treatment.Results: % of patients with hearing gains ≥10 dB in the IT group was significantly higher for 500 Hz and the average of 5 mid-frequencies, tended to be higher for 1000 Hz, but was significantly lower for 8000 Hz, compared to the HBO group. Multiple regression analysis showed that hearing recovery was negatively correlated with patients' age for 125/2000/4000/8000 Hz and with days from onset to treatment for all frequencies, and also revealed better hearing recovery at 500/1000 Hz in the IT group than in the HBO group.Conclusions: Intratympanic steroid is more effective than hyperbaric oxygen to yield better hearing outcomes at mid-frequencies and would be advantageous to restore sound/speech perception.Significance: Superiority of intratympanic steroid over hyperbaric oxygen for treating ISSNHL was verified.