Objective: Ménière's disease (MD) is a clinical condition defined by spontaneous vertigo attacks (each lasting 20 minutes to 12 hours) with documented low- to midfrequency sensorineural hearing loss in the affected ear before, during, or after one of the episodes of vertigo. It also presents with fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness) in the affected ear. The underlying etiology of MD is not completely clear, yet it has been associated with inner ear fluid (endolymph) volume increases, culminating in episodic ear symptoms (vertigo, fluctuating hearing loss, tinnitus, and aural fullness). Physical examination findings are often unremarkable, and audiometric testing may or may not show low- to midfrequency sensorineural hearing loss. Conventional imaging, if performed, is also typically normal. The goals of MD treatment are to prevent or reduce vertigo severity and frequency; relieve or prevent hearing loss, tinnitus, and aural fullness; and improve quality of life. Treatment approaches to MD are many and typically include modifications of lifestyle factors (eg, diet) and medical, surgical, or a combination of therapies.
Purpose: The primary purpose of this clinical practice guideline is to improve the quality of the diagnostic workup and treatment outcomes of MD. To achieve this purpose, the goals of this guideline are to use the best available published scientific and/or clinical evidence to enhance diagnostic accuracy and appropriate therapeutic interventions (medical and surgical) while reducing unindicated diagnostic testing and/or imaging.
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http://dx.doi.org/10.1177/0194599820909438 | DOI Listing |
Adv Sci (Weinh)
December 2024
Department of Neurology, Aerospace Center Hospital, School of Life, Beijing Institute of Technology, Beijing, 100081, China.
The World Health Organization (WHO) reports that by 2050, nearly 2.5 billion people are expected to have some degree of hearing loss (HL) and at least 700 million will need hearing rehabilitation. Therefore, there is an urgent need to develop treatment strategies for HL.
View Article and Find Full Text PDFCochlear Implants Int
December 2024
Department of ENT and Head & Neck Surgery, Seth GS Medical College & K.E.M. Hospital, Mumbai, India.
Introduction: Wolfram syndrome, a rare autosomal recessive disorder, is characterised by diabetes insipidus, juvenile diabetes mellitus, optic nerve atrophy and deafness (DIDMOAD).
Case Report: We present a case of a 21-year-old male diagnosed with Wolfram syndrome who underwent cochlear implantation due to progressive hearing loss. The patient first complained of bilateral hearing loss at the age of 8 years.
Ann Otol Rhinol Laryngol
December 2024
Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN, USA.
Introduction: External ear malformations represent a spectrum of congenital anomalies that may involve the external auditory canal (EAC), tympanic membrane (TM), or associated structures. A rare anomaly, the EAC skin wedge, results from incomplete canalization during embryologic development. This report presents the clinical presentation, diagnostic evaluation, and surgical management of this condition.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China.
Mitochondrial transcription factor A (TFAM) deficiency may cause mtDNA depletion syndrome, which manifests as neonatal liver failure or primary ovarian insufficiency, hearing loss, seizures, and intellectual disability. Treatment focusing on symptomatic management, and the clinical prognosis remains poor. Here, we describe a novel case of TFAM mutation presenting with progressive neonatal cholestasis, hypoglycemia and abnormal amino acid profiling.
View Article and Find Full Text PDFLaryngoscope
December 2024
Division of Surgery, University of Western Australia, Perth, Western Australia, Australia.
Objective: To determine which ear environment risk factors impact ossiculoplasty hearing outcomes and to generate a statistically-valid grading system for ossiculoplasty outcome reporting.
Study Type: Retrospective case series.
Methods: A multi-institutional database was generated from cases performed between 2011 and 2019.
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