Sensorineural hearing loss

BACKGROUND: Susac syndrome (SS) classically presents with the clinical triad of retinal artery occlusion, sensorineural hearing loss, and encephalopathy and the neuroimaging triad of white matter lesions, deep gray matter lesions, and leptomeningeal disease. However, patients can present with an incomplete clinical or neuroimaging triads making diagnosis difficult in certain situations. A standard treatment paradigm also is lacking in this illness. It is important for neuro-ophthalmologists to recognize clinical and radiographic findings that are pathognomonic for this syndrome and have a basic understanding of the available treatment options...

This paper investigates the safety of a novel 'magnetic injection' method of delivering therapy to the cochlea, in a rodent model. In this method of administration, a magnetic field is employed to actively transport drug-eluting iron oxide-core nanoparticles into the cochlea, where they then release their drug payload (we delivered the steroid prednisolone). Our study design and selection of control groups was based on published regulatory guidance for safety studies that involve local drug delivery. We tested for both single and multiple delivery doses to the cochlea, and found that magnetic delivery did not harm hearing...

No abstract text is available yet for this article.

Importance: Sudden sensorineural hearing loss (SSHL) accompanied by vertigo may portend a negative prognosis in the hearing outcome. Objective: To investigate the association of vertigo with prognosis of hearing variables in SSHL. Data Sources: A literature search of eligible studies was performed in PubMed, Web of Science, and Embase from September 26, 1973, through September 26, 2017. Studies published in English were retrieved with no restrictions on the date of publication...

No abstract text is available yet for this article.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) is an emergency clinical otology condition defined as hearing loss greater than 30 dB over three consecutive frequencies within 72 h. We aimed to integrate pretreatment hearing grades with reports of treatment recovery outcomes of SSNHL using the modified Siegel's criteria. METHODS: This was a retrospective cohort study comprising 110 patients with SSNHL seen between January 2014 and January 2015. The patients were treated with combined systemic and intra-tympanic steroid therapy...

INTRODUCTION: Contralateral sensorineural hearing loss (CSNHL) after vestibular schwannoma (VS) is a severe complication, especially in those cases in which hearing preservation in the operated side was not possible. There are several theories that attempt to explain this issue, but there is no established guideline of treatment. MATERIAL AND METHODS: We report 4 patients treated in our institution who developed a severe CSNHL after surgery. RESULTS: Of the 185 cases of VS treated with surgery, 4 patients (2...

IMPORTANCE: Sudden sensorineural hearing loss (SSNHL) is an otological emergency of unknown etiology. Recent reports showed that antioxidant drugs can benefit patients with SSNHL. This study attempted to evaluate the effect of adding antioxidant vitamins as an adjuvant therapy alongside with corticosteroids. OBJECTIVE: To evaluate the effects of the 3 major antioxidant vitamins (A, C, and E) as an adjuvant therapy, administered with corticosteroids, for the treatment of SSNHL in adult patients (≥18 years)...

Background: Superficial siderosis (SS) is a rare condition in which hemosiderin, an iron storage complex, is deposited in neural tissues because of recurrent subarachnoid bleeding. Hemosiderin deposition in the vestibulocochlear nerve (CN VIII), brain, spinal cord and peripheral nerve can cause sensorineural hearing loss (SNHL) and postural imbalance, but much remains unknown about the vestibular manifestations of SS. Objectives: To report the clinical course, cochleovestibular status, and patterns of vestibulopathy during follow-up of a relatively large case series, and to discuss the possible pathophysiological mechanism of vestibular deterioration...

BACKGROUND: Genetic analysis detected excessive mono-allelic recessive GJB2 mutations in individuals with idiopathic deafness; the remaining alleles in trans/cis are underdetermined. The aim of this study was to assess the contributions of variants in GJB3 or GJB6 to non-syndromic sensorineural hearing impairment (NSHI) in Chinese patients with mono-allelic GJB2 mutations. METHODS: The entire coding sequences of GJB3/GJB6, as well as deletions in GJB6, in a cohort of NSHI patients (n = 100) carrying likely pathogenic heterozygous GJB2 mutations, were tested...

An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues...

SRY-box 10 (SOX10) mutation may lead to inner ear deformities. However, its molecular mechanisms on inner ear development are not clear. In this work, the inner ear morphology was investigated at different embryonic stages of the SOX10 mutation miniature porcine model with sensorineural hearing loss, and high-throughput RNA-seq and bioinformatics analyses were applied. Our results indicated that the SOX10 mutation in the miniature pigs led to an incomplete partition (IP) of the cochlea, a cystic apex caused by fusion from middle and apical turns, cochlear modiolar defects and a shortened cochlear duct...

Objective: To explore the clinical characteristics and treatments of patients with idiopathic sudden sensorineural hearing loss（ISSHL） with or without vertigo. Method: One hundred and twenty ISSHL cases were divided into vertigo group ( n =36) , without vertigo group ( n =84) , and with benign paroxysmal positional vertigo group ( n =15). All patients were in regular treatment. Besides, according to the types of BPPV, patients do the Epley maneuver or Barbecue roll maneuver. We summarized the result and treatment of the patients...

Objective: To study the early prelingual auditory development in the pediatric cochlear implant（CI） recipients from 1 years old to 5 years old. Method: The study was designed as retrospectively. A total of 103 children with profound prelingual sensorineural hearing loss were included in this study. All participants were divided into four groups as 1－2 yr，＞2－3 yr，＞3－4 yr and ＞4－5 yr，according to the age of cochlear implantation. The scores of IT-MAIS/MAIS（total scores，sound detection scores and sound recognition scores）before implantation（or at switch-on）and 3,6,12 months after implantation were analyzed with Mixed linear model...

Objective: To explore the epidemiological characteristics and to help accomplish accurate diagnosis and treatment strategies by analyzing the composition and clinical features of various diseases with acute constant vertigo. Method: We retrospectively analyzed medical records (including name, sex, age, diabetes, hypertension, history of vertigo, family history, etc.),otoneurological examination, vestibular function tests and radiological examination of patients with acute vestibular syndrome.We classified various diseases according to diagnostic criteria, and then analyze the clinical data...

Objective: The next-generation sequencing technology (NGS) was used to perform genetic testing on children diagnosed with sensorineural hearing loss in outpatient clinics.The information on the status and inheritance of disease causing genes in deafness was analyzed to provide a theoretical basis for genetic counseling, prenatal diagnosis, and birth defects prevention. Method: Ninety-four cases of sensorineural deafness diagnosed by medical history, audiological examination, and imaging examination were collected in our department...

This issue of Seminars in Hearing has focused on the early intervention of adult sensorineural hearing loss (SNHL). Contributions to this monograph have taken a contemporary issues approach to the prevention, diagnosis, and treatment of mild SNHL. This article looks toward the future and discusses clinical and research implications for the early intervention of adult SNHL.

Little evidence is available regarding outcomes of advanced digital technology (ADT) hearing aid wearers with mild sensorineural hearing loss (MSNHL). The purpose of this article is to report the characteristics of and outcomes for this population. A cross-sectional research design was employed with 56 participants from a private practice setting. The International Outcomes Inventory for Hearing Aids (IOI-HA), Satisfaction with Amplification in Daily Life (SADL), and the Measure of Audiologic Rehabilitation Self-Efficacy for Hearing Aids (MARS-HA) were completed, scored, and compared with normative data...

Recently, President Trump signed into law the Food and Drug Administration (FDA) Reauthorization Act of 2017, which included the Over-the-Counter Hearing Aid Act designed to provide greater public accessibility to and affordability of amplification for individuals with self-identified mild and moderate hearing loss through the provision of over-the-counter (OTC) hearing aids (HAs) with a direct-to-consumer (DTC) delivery model. American Speech-Language-Hearing Association and American Academy of Audiology Position Statements on OTC HAs state that these devices should only be used for adults with mild hearing losses...

Limited accessibility to and affordability of hearing health care (HHC) and hearing aids (HAs) are two reasons why people do not seek treatment for their hearing losses. This article is the first in a series of two and discusses affordability issues (i.e., billing models, cost-effectiveness, insurance coverage, and reimbursement) related to and provides a historical context for the Over-the-Counter Hearing Aid Act of 2017. This piece of legislation supports development of a new class of over-the-counter HAs that represents a disruptive technology that may transform the HHC industry by reducing costs specific to the device...