scholarly journals Endolymphatic Hydrops in Fluctuating Hearing Loss and Recurrent Vertigo

2021 ◽  
Vol 8 ◽  
Author(s):  
Pablo Domínguez ◽  
Raquel Manrique-Huarte ◽  
Víctor Suárez-Vega ◽  
Nieves López-Laguna ◽  
Carlos Guajardo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Odds Ratio ◽  
Endolymphatic Hydrops ◽  
Sensorineural Hearing ◽  
Vestibular Tests ◽  
Risk Of Progression ◽  
Magnetic Resonance Imaging Mri ◽  
Adjusted Model

Background: Endolymphatic hydrops (EH) is the histopathological hallmark of Ménière's disease (MD) and has been found by in vivo magnetic resonance imaging (MRI) in patients with several inner ear syndromes without definite MD criteria. The incidence and relevance of this finding is under debate.Purpose: The purpose of the study is to evaluate the prevalence and characteristics of EH and audiovestibular test results in groups of patients with fluctuating audiovestibular symptoms not fulfilling the actual criteria for definite MD and compare them with a similar group of patients with definite MD and a group of patients with recent idiopathic sudden neurosensory hearing loss (ISSNHL).Material and Methods: 170 patients were included, 83 with definite MD, 38 with fluctuating sensorineural hearing loss, 34 with recurrent vertigo, and 15 with ISSNHL. The clinical variables, audiovestibular tests, and EH were evaluated and compared. Logistic proportional hazard models were used to obtain the odds ratio for hydrops development, including a multivariable adjusted model for potential confounders.Results: No statistical differences between groups were found regarding disease duration, episodes, Tumarkin spells, migraine, vascular risk factors, or vestibular tests; only hearing loss showed differences. Regarding EH, we found significant differences between groups, with odds ratio (OR) for EH presence in definite MD group vs. all other patients of 11.43 (4.5–29.02; p < 0.001). If the ISSNHL group was used as reference, OR was 55.2 (11.9–253.9; p < 0.001) for the definite MD group, 9.9 (2.1–38.9; p = 0.003) for the recurrent vertigo group, and 5.1 (1.2–21.7; p = 0.03) for the group with fluctuating sensorineural hearing loss.Conclusion: The percentage of patients with EH varies between groups. It is minimal in the ISSNHL group and increases in groups with increasing fluctuating audiovestibular symptoms, with a rate of severe EH similar to the known rate of progression to definite MD in those groups, suggesting that presence of EH by MRI could be related to the risk of progression to definite MD. Thus, EH imaging in these patients is recommended.

Imaging of endolymphatic hydrops: A comprehensive update in primary and secondary hydropic ear disease

2021 ◽  
pp. 1-8
Author(s):  
Michael Eliezer ◽  
Arnaud Attyé ◽  
Michel Toupet ◽  
Charlotte Hautefort
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Sensorineural Hearing ◽  
Ménière’S Disease ◽  
Clinical Settings ◽  
Technical Developments

BACKGROUND: Since the first description by Hallpike and Cairns, the excess of endolymphatic fluid, also known as endolymphatic hydrops (EH), has been established as being the main biomarker in patients with Menière’s disease. Recently, the concept of primary (PHED) and secondary hydropic ear disease (SHED) has been introduced. PHED corresponded to Menière’s disease while SHED was defined as the presence of EH in patients with pre-existing inner ear disease. OBJECTIVE: In this article, we would like to summarize the methodology of hydrops exploration using MRI and the previously published radiological findings in patients with PHED and SHED. RESULTS: Before the emergence of delayed inner ear MRI, the presence of EH was assumed based on clinical symptoms. However, because of the recent technical developments, inner ear MRI became an important tool in clinical settings for identifying EH in vivo, in patients with PHED and SHED. The presence of EH on MRI is related with the degree of sensorineural hearing loss whether in patients with PHED or SHED. By contrast, in PHED or SHED patients without sensorineural hearing loss, MRI showed no sign of EH. CONCLUSIONS: Thanks to the recent technical developments, inner ear MRI became an important tool in clinical settings for identifying EH in vivo, in patients with PHED and SHED.

scholarly journals Prevention of acquired sensorineural hearing loss in mice by in vivo Htra2 gene editing

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Xi Gu ◽  
Daqi Wang ◽  
Zhijiao Xu ◽  
Jinghan Wang ◽  
Luo Guo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Protective Effect ◽  
Hair Cell ◽  
Sensorineural Hearing Loss ◽  
Auditory Brainstem Response ◽  
Gene Editing ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Brainstem Response

Abstract Background Aging, noise, infection, and ototoxic drugs are the major causes of human acquired sensorineural hearing loss, but treatment options are limited. CRISPR/Cas9 technology has tremendous potential to become a new therapeutic modality for acquired non-inherited sensorineural hearing loss. Here, we develop CRISPR/Cas9 strategies to prevent aminoglycoside-induced deafness, a common type of acquired non-inherited sensorineural hearing loss, via disrupting the Htra2 gene in the inner ear which is involved in apoptosis but has not been investigated in cochlear hair cell protection. Results The results indicate that adeno-associated virus (AAV)-mediated delivery of CRISPR/SpCas9 system ameliorates neomycin-induced apoptosis, promotes hair cell survival, and significantly improves hearing function in neomycin-treated mice. The protective effect of the AAV–CRISPR/Cas9 system in vivo is sustained up to 8 weeks after neomycin exposure. For more efficient delivery of the whole CRISPR/Cas9 system, we also explore the AAV–CRISPR/SaCas9 system to prevent neomycin-induced deafness. The in vivo editing efficiency of the SaCas9 system is 1.73% on average. We observed significant improvement in auditory brainstem response thresholds in the injected ears compared with the non-injected ears. At 4 weeks after neomycin exposure, the protective effect of the AAV–CRISPR/SaCas9 system is still obvious, with the improvement in auditory brainstem response threshold up to 50 dB at 8 kHz. Conclusions These findings demonstrate the safe and effective prevention of aminoglycoside-induced deafness via Htra2 gene editing and support further development of the CRISPR/Cas9 technology in the treatment of non-inherited hearing loss as well as other non-inherited diseases.

scholarly journals Superficial Siderosis and Sudden Sensorineural Hearing Loss: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-2
Author(s):  
Kirsti S. V. Lee ◽  
Niranjan Sritharan ◽  
Allan Forrest
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Sensorineural Hearing Loss ◽  
Unusual Case ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Superficial Siderosis ◽  
Imaging Features ◽  
Delayed Complication ◽  
Magnetic Resonance Imaging Mri

This case report highlights an unusual case of sudden sensorineural hearing loss related to superficial siderosis (SS). Our patient had a craniotomy for medulloblastoma 23 years earlier, and this may represent a delayed complication related to this procedure. Magnetic resonance imaging (MRI) remains the key diagnostic investigation to illustrate the imaging features of superficial siderosis and exclude other pathologies. Increased awareness of progressive and sudden hearing complications caused by SS is important in the otolaryngologic community to expedite management and better counsel patients during the consent process.

Sensorineural hearing loss with macrolide antibiotics exposure: a meta-analysis of the association

2020 ◽  
Author(s):  
Yazed Saleh Alsowaida ◽  
Abdulaziz Saleh Almulhim ◽  
Mok Oh ◽  
Brian Erstad ◽  
Ivo Abraham
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Odds Ratio ◽  
Meta Analysis ◽  
Sensorineural Hearing ◽  
Cochrane Library ◽  
Macrolide Antibiotics ◽  
Random Effects Model ◽  
Inclusion Criteria ◽  
Meta Analyses

Abstract Objective Macrolide antibiotics are among the most commonly used antibiotics; the association of macrolide antibiotics exposure with sensorineural hearing loss (SNHL) has been hypothesized. A systematic search was conducted in PubMed, EMBASE and Cochrane Library from inception to 15 July 2019 to identify studies used macrolide antibiotics for any indication. The results were reported as odds ratio (OR) with 95% confidence interval (CI) using random-effects model to derive the association of macrolide antibiotics exposure with SNHL. The objective of this meta-analysis was to estimate the association of macrolide antibiotics exposure and SNHL from up-to-date evidence. Key findings Nine studies met the inclusion criteria. There was no statistically significant association between macrolide antibiotics exposure and SNHL; the OR was 1.20 (95% CI: 0.96 to 1.49). No significant association was found with any of the subgroup meta-analyses. Summary Whilst the frequency of SNHL was higher with macrolide antibiotics exposure compared with controls, overall, no association was found between macrolide antibiotics and SNHL.

Hypertrophic Pachymeningitis of the Internal Auditory Canal: A Rare Case of Unilateral Sudden Sensorineural Hearing Loss

2018 ◽  
Vol 127 (9) ◽  
pp. 649-652 ◽  
Author(s):  
Thomas Muelleman ◽  
Hannah Kavookjian ◽  
James Lin ◽  
Hinrich Staecker
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Internal Auditory Canal ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Hypertrophic Pachymeningitis ◽  
Eighth Cranial Nerve ◽  
Mri Scans ◽  
Magnetic Resonance Imaging Mri

Objectives: To describe and increase awareness of a rare cause of unilateral sudden sensorineural hearing loss. Methods: Case report and literature review. Results: We present a 66-year-old female who suffered left-sided sudden sensorineural hearing loss and dizziness. Diagnostic magnetic resonance imaging (MRI) did not reveal masses or lesions along the eighth cranial nerve or in the inner ear. Upon eventual referral to neurotology clinic, hypertrophic pachymeningitis of her left internal auditory canal and adjacent middle and posterior fossa dura were identified. The ensuing laboratory workup for autoimmune and infectious etiology revealed mild elevation of ACE 93 (9-67) but otherwise normal results. Conclusions: Idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion. Neoplastic, infectious, and autoimmune causes must be ruled out. The prevailing treatment for this condition is high-dose corticosteroids. This entity should be considered when evaluating MRI scans obtained in the setting of sudden sensorineural hearing loss.

Endolymphatic Hydrops Revealed by Magnetic Resonance Imaging in Patients With Acute Low-Tone Sensorineural Hearing Loss

2013 ◽  
Vol 34 (7) ◽  
pp. 1241-1246 ◽  
Author(s):  
Mariko Shimono ◽  
Masaaki Teranishi ◽  
Tadao Yoshida ◽  
Masahiro Kato ◽  
Rui Sano ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Low Tone

Significance of Endolymphatic Hydrops in Ears With Unilateral Sensorineural Hearing Loss

2017 ◽  
Vol 38 (8) ◽  
pp. 1076-1080 ◽  
Author(s):  
Yuriko Okazaki ◽  
Tadao Yoshida ◽  
Satofumi Sugimoto ◽  
Masaaki Teranishi ◽  
Ken Kato ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Sensorineural Hearing

Delayed Endolymphatic Hydrops: A Case Study

2008 ◽  
Vol 19 (03) ◽  
pp. 204-209 ◽  
Author(s):  
Rachel Lazaro ◽  
Larry Lundy ◽  
David Zapala
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Sensorineural Hearing ◽  
Ménière’S Disease ◽  
Episodic Vertigo ◽  
Delayed Endolymphatic Hydrops ◽  
Patient’S History ◽  
Estudio De Caso

Delayed endolymphatic hydrops (DEH) is an unusual variation of Ménière's disease characterized by episodic vertigo that develops some time after the onset of a profound, typically unilateral sensorineural hearing loss. This case study describes a 48-year-old male who presented with complaints of episodic vertigo and disequilibrium 15 years following the onset of unilateral sensorineural hearing loss. The patient's history, audiologic findings, and vestibular evaluation led to the diagnosis of DEH. The case highlights the diagnostic and treatment challenges associated with this condition and focuses attention on principles that guide the audiologist in collecting evidence that aids in solving these challenges. El hidrops endolinfático retardado (DEH) es una variante inusual de la Enfermedad de Ménière, caracterizada por vértigo episódico que se desarrolla en el tiempo luego del inicio de una hipoacusia sensorineural unilateral típica. Este estudio de caso describe una varón de 48 años que presentó quejas de vértigo episódico y desequilibrio, 15 años después del inicio de una hipoacusia sensorineural unilateral. La historia del paciente, los hallazgos audiológicos y la evaluación vestibular llevaron al diagnóstico de DEH. El caso destaca los retos diagnósticos y terapéuticos asociados con esta condición y concentra su atención en los principios que guían al audiólogo en la recolección de evidencia que ayude a resolver estos retos.

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