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CASP9 genotype confers gentamicin susceptibility in intratympanic treatment of intractable vertigo caused by Ménière’s disease

https://www.tandfonline.com/doi/abs/10.1080/00016489.2019.1575525?journalCode=ioto20

https://www.ncbi.nlm.nih.gov/pubmed/30907242?dopt=Abstract

CASP9 genotype confers gentamicin susceptibility in intratympanic treatment of intractable vertigo caused by Ménière’s disease.

CASP9 genotype confers gentamicin susceptibility in intratympanic treatment of intractable vertigo caused by Ménière’s disease.

Acta Otolaryngol. 2019 Mar 25;:1-4

Authors: Huang CJ, Wan TK, Fang TY, Wang PC

Abstract

BACKGROUND: Ménière’s disease (MD) is a disorder of the inner ear, causing episodes of vertigo. Although surgery is reserved for intractable MD, intratympanic gentamicin (ITG) injection has become an alternative for controlling vertigo.

AIMS/OBJECTIVES: To investigate the genetic basis of ITG efficacy.

MATERIAL AND METHODS: We hypothesized that single nucleotide polymorphisms (SNPs) affect outcomes in patients with MD who receive ITG injections. Whole-exome sequencing was used to determine variations in coding regions.

RESULTS: Multivariate analysis revealed two SNPs, rs1052571 in caspase 9 (CASP9; p = .017) and rs3745274 in cytochrome P450 2B6 (p = .053), which were associated with susceptibility to ITG injections. Only the C-allele in the rs1052571 SNP was significantly associated with susceptibility (p = .027; odds ratio: 5.95; 95% confidence interval: 1.26-28.57, by Fisher’s exact test).

CONCLUSIONS AND SIGNIFICANCE: Our results elucidated the role of the rs1052571 SNP and provide a genetic perspective on gentamicin efficacy (susceptibility) in treating intractable MD.

PMID: 30907242 [PubMed – as supplied by publisher]

PubMed:30907242

Cochlear Implantation During Vestibular Schwannoma Removal or During Labyrinthectomy Surgery for Treatment of Meniere’s Disease

https://clinicaltrials.gov/ct2/show/NCT03795675?type=Intr&cond=Meniere+Disease&lupd_s=02%2F28%2F2019&lupd_d=14

Cochlear Implantation During Vestibular Schwannoma Removal or During Labyrinthectomy Surgery for Treatment of Meniere’s Disease

Conditions :   Vestibular Schwannoma;   Meniere Disease

Intervention :   Device: Cochlear Implant

Sponsors :   Ohio State University;   Advanced Bionics

Recruiting

CI Following VS Removal or Labyrinthectomy

NCT03795675

Tue, 08 Jan 2019 12:00:00 EST

Menière’s Disease and Disorders of the Carbohydrate Metabolism Involving the Inner Ear

https://www.thieme-connect.de/products/ejournals/abstract/10.1055/s-0038-1673698
https://www.ncbi.nlm.nih.gov/pubmed/30956708?dopt=Abstract

Menière’s Disease and Disorders of the Carbohydrate Metabolism Involving the Inner Ear.

Related Articles

Menière’s Disease and Disorders of the Carbohydrate Metabolism Involving the Inner Ear.

Int Arch Otorhinolaryngol. 2019 Apr;23(2):218-220

Authors: Albernaz PLM

Abstract

Introduction  Menière’s disease was described in 1861, but there are still uncertainties regarding its pathophysiology and treatment. Endolymphatic hydrops is recognized as a fundamental pathological characteristic of the disease, as a result of an inadequate absorption of the endolymph. A milder type of endolymphatic hydrops results from an altered chemical composition of the endolymph, due to disorders of the carbohydrate metabolism. Objective  To describe the association of both types of hydrops in patients with Menière disease. Methods  This was a retrospective study of 98 patients with Menière’s disease, 62 of whom also presented disorders of the carbohydrate metabolism, and 5 patients with delayed endolymphatic hydrops, 2 of whom also presented disorders of the carbohydrate metabolism. Results  The follow-up of these patients showed that the correction of the metabolic disorders may help in the clinical treatment of Menière’s disease and of delayed endolymphatic hydrops, but this does not happen in the more severe types of the diseases. Conclusion  Patients with Menière’s disease may present simultaneous disorders of the carbohydrate metabolism, affecting the inner ear. The correction of these disorders helps the clinical treatment but does not preclude the progression of the more severe cases of Menière disease.

PMID: 30956708 [PubMed]

PubMed:30956708

Treatment of Ménière’s disease focusing on vascular risk factors may allow a better control of symptoms and result in a decreased need for ablative procedures

https://www.sciencedirect.com/science/article/pii/S1672293018301028?via%3Dihub

https://www.ncbi.nlm.nih.gov/pubmed/31223301?dopt=Abstract

The cardiovascular aspects of a Ménière’s disease population – A pilot study.

The cardiovascular aspects of a Ménière’s disease population – A pilot study.

J Otol. 2019 Jun;14(2):51-56

Authors: Rego ÂR, Dias D, Pinto A, E Castro SS, Feliciano T, E Sousa CA

Abstract

Introduction: MD is considered a rare disease. An adequate model that explains MD’s pathophysiology is not well established. Recently, the vascular theory of the disease has been revived.

Objectives: To characterize a MD population according to its cardiovascular risk and correlate it to the MD clinical course.

Methods: In this retrospective chart study the data of 31 MD patients observed between January 2017 and April 2018 in a tertiary university hospital were reviewed. Patients included in the study were diagnosed according to the Bárány Society criteria. Lost follow-ups, patients with autoimmune disease, atopy or allergy, major psychiatric disease and migraine were excluded. Age, gender, cardiovascular risk factors, audiometric and vestibular parameters, occurrence of MD attacks in the previous 6 months, vestibular medication in course and time course of MD were recorded and compared between groups (with and without cardiovascular risk factors).

Results: 31 patients (61.3% females) mean aged 60.3 years (±9.7) were studied. 74% of the population had at least one risk factor and 51.6% of patients had attacks in the last 6 months. There was a statistically significant difference in the occurrence of MD attacks in the last 6 months (p = 0.014) between MD patients with and without risk factors. Mean PTA thresholds were higher and speech discrimination was lower in individuals with more cardiovascular risk factors.

Conclusions: Treatment of MD focusing on vascular risk factors may allow a better control of symptoms and result in a decreased need for ablative procedures in this disorder.

PMID: 31223301 [PubMed]

PubMed:31223301

Meniere’s Disease: A Review of Pathologic Mechanism, Clinical Diagnosis, and New Treatments

https://www.karger.com/Article/Abstract/490274

https://www.ncbi.nlm.nih.gov/pubmed/30947172?dopt=Abstract

Meniere’s Disease.

Meniere’s Disease.

Adv Otorhinolaryngol. 2019;82:77-86

Authors: Gibson WPR

Abstract

This article reviews 3 aspects of Meniere’s disease (MD), which have been recently revisited: namely, the pathologic mechanism causing the attacks of vertigo, the clinical diagnosis, and the medical and surgical treatments. The characteristic attacks of vertigo are unlikely to be due to membrane ruptures, so a hypothesis that the vertigo is caused by a volume of endolymph shifting suddenly from the cochlea into the pars superior is suggested. The definite diagnosis according to the American Academy of Otolaryngology HNS 1995 criteria [13] failed to exclude vestibular migraine sufficiently and a revision in 2015 [14] has partly addressed this problem but another method which stresses the interaction of the cochlear and vestibular symptoms is described. The treatment of MD has been updated, providing evidence for each popular therapy. Newer treatments using intratympanic medications including steroid solutions and gentamicin are discussed. Finally, the role of cochlear implants is mentioned.

PMID: 30947172 [PubMed – in process]

PubMed:30947172

Novel therapy which uses gelatin sponge particles to help deliver high-dose steroids to the inner ear, relieves vertigo, tinnitus, and hearing loss in refractory Ménière’s disease

https://www.sciencedirect.com/science/article/pii/S1672293018300916

https://www.ncbi.nlm.nih.gov/pubmed/31223300?dopt=Abstract

Autoinflammatory characteristics and short-term effects of delivering high-dose steroids to the surface of the intact endolymphatic sac and incus in refractory Ménière’s disease.

J Otol. 2019 Jun;14(2):40-50

Authors: Zou J

Abstract

Objective: To investigate immune-related genetic background in intractable Meniere’s disease (MD) and the immediate results of a novel therapy by delivering steroids to the surface of the intact endolymphatic sac (ES) and incus in a sustainable manner.

Case report and methods: Candidate genes involved in immune regulation were sequenced using a next-generation sequencing method in a patient with intractable MD. Mutations were confirmed using the Sanger sequencing method. The ES was exposed, and gelatin sponge particles were immersed in high-dose methylprednisolone solution and placed onto the surface of ES. “L”-shaped gelatin sponge strips were immersed in dexamethasone solution and served as a guiding device for the steroids by touching the incus and gelatin sponge particles on the surface of the ES. Gelatin sponge particles immersed in dexamethasone solution were placed around the gelatin sponge strips and sealed using fibrin glue.

Results: Autoinflammation in the refractory MD case was indicated by genotype, including novel heterozygous mutations of PRF1, UNC13D, SLC29A3, ITCH, and JAK3, as well as phenotype. The vertigo was fully relieved immediately after operation. Tinnitus and aural fullness were resolved 3 weeks after operation, whereas hearing improved in 2 mon postoperation. No recurrence was noted during the 5-monfollow-up, and the final MRI supported the novel therapeutic hypothesis.

Conclusion: Autoinflammation was involved in a refractory MD. This novel therapy, which involves the delivery of steroids to the surface of the intact ES and incus, is effective in relieving vertigo and tinnitus and improves hearing function of refractory MD.

PMID: 31223300 [PubMed]

PubMed:31223300

Effects of dietary restriction of salt, caffeine and alcohol intake in patients with Ménière’s disease or syndrome

https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD012173.pub2/full

https://www.ncbi.nlm.nih.gov/pubmed/30596397?dopt=Abstract

Restriction of salt, caffeine and alcohol intake for the treatment of Ménière’s disease or syndrome.

Cochrane Database Syst Rev

Restriction of salt, caffeine and alcohol intake for the treatment of Ménière’s disease or syndrome.

Cochrane Database Syst Rev. 2018 Dec 31;12:CD012173

Authors: Hussain K, Murdin L, Schilder AG

Abstract

BACKGROUND: Ménière’s disease or syndrome is a chronic inner ear disorder that results in sporadic attacks of vertigo, sensorineural hearing loss, aural fullness and tinnitus.There is no definitive treatment for Ménière’s disease and treatment options range from dietary modification through medication to surgery.Modification of diet, including restriction of salt, caffeine and alcohol intake, is a management option that is widely recommended to patients with Ménière’s as a first-line treatment. There has not previously been a systematic review of this intervention.

OBJECTIVES: To assess the effects of dietary restriction of salt, caffeine and alcohol intake in patients with Ménière’s disease or syndrome.

SEARCH METHODS: The Cochrane ENT Information Specialist searched the Cochrane ENT Trials Register; Central Register of Controlled Trials (CENTRAL); PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 28 March 2018.

SELECTION CRITERIA: Randomised controlled trials of dietary modification, specifically salt, caffeine and alcohol restriction or substitution (or both), compared to no modification of these agents or a placebo intervention, in adult patients with Ménière’s disease or syndrome.

DATA COLLECTION AND ANALYSIS: We used the standard methodological procedures expected by Cochrane. Our primary outcomes were control of vertigo or decrease in vertigo attacks, and adverse effects. Secondary outcomes included hearing (change in hearing loss or its progression), tinnitus (severity), perception of aural fullness, well-being and quality of life (overall changes), and other adverse effects. We planned to use GRADE to assess the quality of the evidence for each outcome.

MAIN RESULTS: We did not identify any studies that met the inclusion criteria for the review.

AUTHORS’ CONCLUSIONS: There is no evidence from randomised controlled trials to support or refute the restriction of salt, caffeine or alcohol intake in patients with Ménière’s disease or syndrome. High-quality research in this field is warranted. The best evidence may come from a randomised controlled trial comparing dietary interventions (e.g. low salt versus general healthy diet advice), using rigorous methodology for patient selection, randomisation and blinding, and strictly adhering to the Bárány Society diagnostic criteria. However, this research question might be more pragmatically addressed by using information from carefully constructed patient registries that include information on dietary intake of substances of interest such as salt, caffeine and alcohol. It will be important to address the question of any possible harms or unwanted effects of dietary modification.

PMID: 30596397 [PubMed – as supplied by publisher]

PubMed:30596397

The pathophysiological mechanism of Ménière’s disease: Has the key been found?

https://www.ncbi.nlm.nih.gov/pubmed/30289103?dopt=Abstract

Ménière’s disease: ‘a riddle wrapped in a mystery inside an enigma’. Has the key been found?

Related Articles

Ménière’s disease: ‘a riddle wrapped in a mystery inside an enigma’. Has the key been found?

J Laryngol Otol. 2018 Sep;132(9):763

Authors: Fishman J, Fisher E, Hussain M

PMID: 30289103 [PubMed – indexed for MEDLINE]

PubMed:30289103

Long Term Outcomes of Intratympanic Dexamethasone in Intractable Unilateral Meniere’s Disease

https://link.springer.com/article/10.1007%2Fs12070-018-1431-3

https://www.ncbi.nlm.nih.gov/pubmed/31750179?dopt=Abstract

Long Term Outcomes of Intratympanic Dexamethasone in Intractable Unilateral Meniere’s Disease.

Long Term Outcomes of Intratympanic Dexamethasone in Intractable Unilateral Meniere’s Disease.

Indian J Otolaryngol Head Neck Surg. 2019 Nov;71(Suppl 2):1369-1373

Authors: Pradhan P, Lal P, Sen K

Abstract

To evaluate the long term effect of Intratympanic dexamethasone in intractable Meniere’s disease. 30 patients with refractory Meniere’s disease which did not respond to the standard medical management, were treated with Intratympanic dexamethasone injections. Post treatment hearing outcome and dizziness scores were compared with the pretreatment values respectively. The mean dizziness handicap inventory (DHI) score was reduced from 91.58 (range 80-100) to be 31.00 (p = 0.00) at 3 months of treatment. With the successive follow-up periods, the mean DHI scores were reduced to 51.50, 46.6, and 50.90 at the end of, 6, 12, and 24 months (p = 0.04, 0.35, and 0.49 respectively). Again at the end of 24 months, 23.80% of patients were free of vertigo (p = 0.01). No patient had improvement in the hearing (> 10 dB) in any of the follow-up periods and 6.6% demonstrated deterioration in hearing. There were no major intraoperative or postoperative complications detected. Intratympanic injection of steroid is a safe and effective method for treating intractable Meniere’s disease. Although short term improvement in the vertigo is well documented, still in 23% of the patients were found to be free of vertigo at even the end of 24 months. There was no significant improvement in hearing noticed, either in short term or in long term.

PMID: 31750179 [PubMed]

PubMed:31750179

Meniere’s Disease – Reddit

https://www.reddit.com/r/Menieres/

A place for people with Meniere’s to talk, complain, and share … – Reddit

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