Genetics of Postlingual Sensorineural Hearing Loss

The Laryngoscope · 2020 · 24 citations

• Medicine
• Genetics
• Audiology

Literature and clinical practice around adult-onset hearing loss (HL) has traditionally focused on environmental risk factors, including noise exposure, ototoxic drug exposure, and cardiovascular disease. The most common diagnosis in adult-onset HL is presbycusis. However, the age of onset of presbycusis varies, and patients often describe family history of HL as well as individual variation in progression and severity. In recent years, there has been accumulating evidence of gene-environment interactions underlying adult cases of HL. Susceptibility loci for age-related HL have been identified, and genes related to postlingual nonsyndromic HL continue to be discovered through individual reports and genome-wide association studies. This review will outline main concepts in genetics as related to HL, identify implicated genes, and discuss clinical implications. Laryngoscope, 131:401-409, 2021.

Failure and Revision Surgery After Cochlear Implantation in the Adult Population: A 10-year Single-institution Retrospective and Systematic Review of the Literature

Otology & Neurotology · 2020 · 28 citations

• Medicine
• Surgery
• General surgery

OBJECTIVE: To characterize failure rate and etiology after cochlear implantation; to identify predictors and describe outcomes after implant failure. STUDY DESIGN: Retrospective chart review and systematic review of the literature using PubMed and Embase. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Four hundred ninety-eight devices in 439 distinct adult patients. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Implant failure rate and etiology. RESULTS: A total of 32 devices (5.9%) failed in 31 patients encompassing the following failure types in accordance with the European Consensus Statement of Cochlear Implants: 17 device failures (53.1%), 11 failures due to performance decrement/adverse reactions (34.4%), and 4 medical reasons (12.9%). There was no significant difference in age, sex, or manufacturer between patients with and without failures. Twenty-five percent of patients with failure leading to explantation had childhood onset of deafness compared to 12.1% of patients with adult-onset hearing loss (OR = 2.42; p = 0.04). Performance decrement/adverse reaction patients had an older average age at implantation compared to device failure patients (mean 68.5 yr 95% CI: 59.9-77.1 vs mean 47.6 yr, CI: 39.9-55.3, p < 0.01). There was no significant difference in time to failure, sex, or device manufacturer between the different types of failures. Twenty-nine patients who experienced CI failure underwent a revision surgery, while the remaining two opted for explantation without reimplantation. One patient who underwent revision surgery subsequently presented with a second failure and underwent a second revision, which was successful.In our systematic review, 815 citations were reviewed, and 9 studies were selected for inclusion. Overall failure rate across all studies was 5.5%. Device failure was the leading cause of failure in the majority (6/9) of studies, accounting for 40.8% of all failures. Medical reasons were the second leading cause at 33.6%, followed by performance decrement/adverse reaction (20.9%) and other (4.8%). CONCLUSIONS: Cochlear implant failure is a rare phenomenon. Childhood-onset of hearing loss appears to be associated with an increased risk of overall failure. Older patients are at increased risk for performance decrement/adverse reaction. Revision surgery success rates remain very high and patients with failure of any cause should be offered explantation with concurrent reimplantation.

Clinical Practice Guideline: Ménière’s Disease

Otolaryngology · 2020 · 300 citations

• Medicine
• Audiology
• Intensive care medicine

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.

Clinical Practice Guideline: Ménière’s Disease Executive Summary

Otolaryngology · 2020 · 84 citations

• Medicine
• Family medicine
• Business

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 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. 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 approaches 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.