170 results match your criteria severe-profound hearing


Paraneoplastic cochleovestibulopathy: clinical presentations, oncological and serological associations.

J Neurol Neurosurg Psychiatry 2021 Jul 20. Epub 2021 Jul 20.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA

Objective: Cochleovestibulopathy is a distinguishable paraneoplastic phenotype. In this study, we evaluate clinical presentation, serological/cancer associations and outcomes of paraneoplastic cochleovestibulopathy.

Methods: Retrospective chart review of patients with hearing impairment and/or vestibulopathy who underwent serological evaluations for paraneoplastic antibodies between January 2007 and February 2021 was performed. Read More

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Rasch Analysis of the Listening Effort Questionnaire-Cochlear Implant.

Ear Hear 2021 May 4. Epub 2021 May 4.

Patient and Population Health and Informatics, Swansea University Medical School, Singleton Park, Swansea, United Kingdom Australian Institute of Health Innovation, Macquarie University, Sydney, NSW, Australia Faculty of Medicine and Health, School of Health Sciences, University of Sydney, Sydney, NSW, Australia HEAR Centre, Macquarie University, Sydney, NSW, Australia.

Objectives: Listening effort may be defined as the attentional and cognitive resources needed to understand an auditory message, modulated by motivation. Despite the use of hearing devices such as hearing aids or cochlear implants (CIs), the requirement for high listening effort remains a challenge for individuals with hearing loss. The Listening Effort Questionnaire-Cochlear Implant (LEQ-CI) is a hearing-specific patient-reported outcome measure (PROM), which has been designed for use in the CI candidacy and rehabilitation process to assess perceived listening effort in everyday life in adults with severe-profound hearing loss. Read More

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Screening Strategies for Deafness Genes and Functional Outcomes in Cochlear Implant Patients.

Otol Neurotol 2021 01;42(1):180-187

Department of Otolaryngology.

Objectives: To review the current state of knowledge about the influence of specific genetic mutations that cause sensorineural hearing loss (SNHL) on cochlear implant (CI) functional outcomes, and how this knowledge may be integrated into clinical practice. A multistep and sequential population-based genetic algorithm suitable for the identification of congenital SNHL mutations before CI placement is also examined.

Data Sources, Study Selection: A review was performed of the English literature from 2000 to 2019 using PubMed regarding the influence of specific mutations on CI outcomes and the use of next-generation sequencing for genetic screening of CI patients. Read More

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January 2021

Neural Mechanisms of Hearing Recovery for Cochlear-Implanted Patients: An Electroencephalogram Follow-Up Study.

Front Neurosci 2020 5;14:624484. Epub 2021 Feb 5.

School of Biomedical Engineering, Capital Medical University, Beijing, China.

Background: Patients with severe profound hearing loss could benefit from cochlear implantation (CI). However, the neural mechanism of such benefit is still unclear. Therefore, we analyzed the electroencephalogram (EEG) and behavioral indicators of auditory function remodeling in patients with CI. Read More

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February 2021

Genotype-phenotype correlation in patients with Usher syndrome and pathogenic variants in MYO7A: implications for future clinical trials.

Acta Ophthalmol 2021 Feb 11. Epub 2021 Feb 11.

Department of Genetics, University Hospital Fundacion Jimenez Diaz, IIS-FJD, UAM, Madrid, Spain.

Purpose: We aimed to establish correlations between the clinical features of a cohort of Usher syndrome (USH) patients with pathogenic variants in MYO7A, type of pathogenic variant, and location on the protein domain.

Methods: Sixty-two USH patients from 46 families with biallelic variants in MYO7A were examined for visual and audiological features. Participants were evaluated based on self-reported ophthalmological history and ophthalmological investigations (computerized visual field testing, best-corrected visual acuity, and ophthalmoscopic and electrophysiological examination). Read More

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February 2021

Speech and language outcomes in mild-moderate unilateral sensorineural hearing loss.

Int J Pediatr Otorhinolaryngol 2021 Feb 14;141:110558. Epub 2020 Dec 14.

Head and Neck Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address:

Objective: The impact of mild-moderate unilateral sensorineural hearing loss (USNHL) on speech and language delay (SLD) is not well established. Objectives included (1) determining SLD prevalence in patients with mild-moderate USNHL in comparison to prevalence in the general population and severe-profound USNHL patients and (2) examining speech, language, and auditory function testing (SLAT) results in USNHL patients.

Methods: A retrospective chart review of pediatric patients with USNHL, classified using pure tone averages (PTA) into mild-moderate (PTA 21-60) and severe-profound (PTA ≥ 61) USNHL groups was conducted. Read More

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February 2021

Vestibular Infant Screening (VIS)-Flanders: results after 1.5 years of vestibular screening in hearing-impaired children.

Sci Rep 2020 12 3;10(1):21011. Epub 2020 Dec 3.

Faculty of Medicine and Health Sciences, Department of Rehabilitation Sciences, Ghent University, 9000, Ghent, Belgium.

Due to the close anatomical relationship between the auditory and vestibular end organs, hearing-impaired children have a higher risk for vestibular dysfunction, which can affect their (motor) development. Unfortunately, vestibular dysfunction often goes unnoticed, as vestibular assessment in these children is not standard of care nowadays. To timely detect vestibular dysfunction, the Vestibular Infant Screening-Flanders (VIS-Flanders) project has implemented a basic vestibular screening test for hearing-impaired infants in Flanders (Belgium) with a participation rate of 86. Read More

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December 2020

Association between the severity of hearing loss and the risk of dementia within the 2010-2017 national insurance service survey in South Korea.

Sci Rep 2020 11 26;10(1):20679. Epub 2020 Nov 26.

Department of Otorhinolaryngology - Head and Neck Surgery, Korea University College of Medicine, Korea University Ansan Hospital, 123, Jeokgeum-ro, Danwon-gu, Ansan-si, Gyeonggi-do, 15355, Republic of Korea.

Hearing loss and dementia are highly prevalent neurologic conditions in older adults that can considerably impact the quality of life and create social and familial burdens. To investigate the impact of hearing loss on the risk of developing dementia in a nationwide long-term follow-up study using data obtained from the South Korean National Health Information Database. Retrospective medical data for patients of all ages were extracted from the database between January 2010 and December 2017. Read More

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November 2020

Bilateral cochlear implantation: an assessment of language sub-skills and phoneme recognition in school-aged children.

Eur Arch Otorhinolaryngol 2021 Jun 24;278(6):2093-2100. Epub 2020 Nov 24.

Department of Audiology, Hacettepe University, Faculty of Health Sciences, Ankara, Turkey.

Purposes: The main purpose of this study is to investigate whether there is a difference in phoneme recognition and school-age language skills in children with bilateral and unilateral cochlear implants (CI). The second aim of the study is to examine language-based skills in bilateral cochlear implanted children with the first implant, second implant and in the bilateral listening situations.

Method: 60 to 108-month-old children with similar demographic and audiological features were included. Read More

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Unilateral Cochlear Implants for Severe, Profound, or Moderate Sloping to Profound Bilateral Sensorineural Hearing Loss: A Systematic Review and Consensus Statements.

JAMA Otolaryngol Head Neck Surg 2020 10;146(10):942-953

Department of Otorhinolaryngology and Auditory and Voice Surgery, University of Tokyo Hospital, Tokyo, Japan.

Importance: Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine.

Objective: To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL.

Design, Setting, And Participants: This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Read More

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October 2020

Phoneme recognition in bimodal hearing.

Acta Otolaryngol 2020 Oct 9;140(10):854-860. Epub 2020 Jul 9.

The Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.

Background: Many unilateral cochlear implant (CI) users have residual hearing in the nonimplanted ear, allowing them to use bimodal hearing. Assessing the hearing aid (HA) contribution is important.

Objective: To examine the contribution of a contralateral HA in unilateral CI users with severe-profound hearing loss (HL) in the non-implanted ear to phonetic features perception. Read More

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October 2020

Systematic Review of Outcomes After Cochlear Implantation in Children With X-Linked Deafness-2.

Otolaryngol Head Neck Surg 2021 01 30;164(1):19-26. Epub 2020 Jun 30.

Department of Otolaryngology-Head and Neck Surgery, University of Michigan Medical School, Ann Arbor, Michigan, USA.

Objective: Outcomes following cochlear implantation in children with X-linked deafness-2 are variable, resulting in challenges in appropriate preoperative counseling. To address this uncertainty, we performed a systematic review and synthesis of the literature on audiologic and speech outcomes after cochlear implantation in these patients to inform prognostic counseling.

Data Sources: PubMed, Embase, and Cochrane Library were queried for articles published between January 2000 and July 2019. Read More

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January 2021

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas.

Ann Otol Rhinol Laryngol 2020 Dec 18;129(12):1229-1238. Epub 2020 Jun 18.

Department of Otorhinolaryngology-Head and Neck Surgery, Rush University Medical Center, Chicago, IL, USA.

Objectives: Historically, eventual loss of cochlear nerve function has limited patients with neurofibromatosis type 2 (NF2) to auditory brainstem implants (ABI), which in general are less effective than modern cochlear implants (CI). Our objective is to evaluate hearing outcomes following ipsilateral cochlear implantation in patients with NF2 and irradiated vestibular schwannomas (VS), and sporadic VS that have been irradiated or observed.

Methods: Multi-center retrospective analysis of ipsilateral cochlear implantation in the presence of observed and irradiated VS. Read More

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December 2020

Management of the Temporal Bone Fibrous Dysplasia With External Auditory Canal Stenosis and Secondary Cholesteatoma in an Asian Population: A 11-Case Series.

Ear Nose Throat J 2020 May 22:145561320927922. Epub 2020 May 22.

ENT Institute and Otorhinolaryngology Department, Affiliated Eye and ENT Hospital, Shanghai Medical College, Fudan University, Shanghai, China.

Objective: This article summarizes the experience of diagnosis and treatment of temporal bone fibrous dysplasia (FD) with external auditory canal (EAC) stenosis and secondary cholesteatoma in the Chinese population, in order to improve the quality of life of patients in the future.

Methods: Eleven patients with FD of the temporal bone who underwent surgery were retrospectively reviewed.

Results: All lesions originated from the temporal bone, and all involved of the EAC. Read More

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In silico analysis of a novel causative mutation in Cadherin23 gene identified in an Omani family with hearing loss.

J Genet Eng Biotechnol 2020 Mar 2;18(1). Epub 2020 Mar 2.

Department of Biochemistry, College of Medicine and Health Sciences, Sultan Qaboos University, P.O. Box 35, Al-Khoud, 123, Muscat, Oman.

Background: Hereditary hearing loss is a heterogeneous group of complex disorders with an overall incidence of one in every 500 newborns presented as syndromic and non-syndromic forms. Cadherin-related 23 (CDH23) is one of the listed deafness causative genes. It is found to be expressed in the stereocilia of hair cells and in the retina photoreceptor cells. Read More

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Results From a Second-Generation Vestibular Implant in Human Subjects: Diagnosis May Impact Electrical Sensitivity of Vestibular Afferents.

Otol Neurotol 2020 01;41(1):68-77

Department of Otolaryngology, Virginia Merrill Bloedel Hearing Research Center, National Primate Research Center, University of Washington, Seattle, Washington.

Objective: Auditory and vestibular outcomes after placement of a vestibular-cochlear implant in subjects with varying causes of vestibular loss.

Study Design: Prospective case study.

Setting: Tertiary referral center. Read More

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January 2020

Comparison of auditory steady-state response and click-evoked auditory brain response in infants with different types and degrees of hearing loss.

Acta Otolaryngol 2020 Feb 11;140(2):116-121. Epub 2019 Dec 11.

Department of Paediatric Ophthalmology and Otorhinolaryngology, Hunan Provincial Maternal and Child Health Care Hospital, Changsha, China.

Auditory steady-state response (ASSR) and click-evoked auditory brain response (c-ABR) have been used for hearing assessment for decades years, the correlation of the two methods and the effects of type and degree of hearing loss (HL) to the correlation in infants younger than 6 months of age are unclear. To compare the correlation of ASSR and c-ABR and then to analyse the effects of type and degree of HL on the correlation in infants younger than 6 months of age. Retrospective study comparing ASSR thresholds at various frequencies with c-ABR thresholds. Read More

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February 2020

Comprehensive hearing aid assessment in adults with bilateral severe-profound sensorineural hearing loss who present for Cochlear implant evaluation.

Am J Otolaryngol 2020 Mar - Apr;41(2):102300. Epub 2019 Sep 11.

Rocky Mountain Ear Center, 601 E. Hampden Ave #430, Englewood, CO 80113, United States of America.

Purpose: To comprehensively assess and describe functional auditory performance in a group of adults with bilateral, moderate sloping to profound sensorineural hearing loss who were dissatisfied users of well-fit bilateral hearing aids and presented for Cochlear implant evaluation. Participants were evaluated with bilateral hearing aids and after six months of bimodal (Cochlear implant and a contralateral hearing aid) hearing experience with a Cochlear implant and contralateral hearing aid.

Methods: Study participants were assessed using pure tone audiometry, aided speech understanding in quiet (CNC words) and in noise (AzBio sentences at +10 and +5 dB SNR) in the sound field with unilateral and bilateral hearing aids fit to target. Read More

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Degree of Hearing Loss Affects Bilateral Hearing Aid Benefits in Ecologically Relevant Laboratory Conditions.

J Speech Lang Hear Res 2019 10 9;62(10):3834-3850. Epub 2019 Oct 9.

GN Hearing, Chicago, IL.

Purpose Previous evidence supports benefits of bilateral hearing aids, relative to unilateral hearing aid use, in laboratory environments using audio-only (AO) stimuli and relatively simple tasks. The purpose of this study was to evaluate bilateral hearing aid benefits in ecologically relevant laboratory settings, with and without visual cues. In addition, we evaluated the relationship between bilateral benefit and clinically viable predictive variables. Read More

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October 2019

[The association between serum bilirubin levels and hearing loss in the patients with bilateral sudden deafness].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2019 Aug;33(8):700-703

Department of Otolaryngology Head and Neck Surgery,Institute of Otolaryngology,Chinese PLA General Hospital,Beijing,100853,China.

The aim of this study was to investigate the association between serum bilirubin levels and the severity of bilateral sudden sensorineural hearing loss (BSSHL). A total of 113 patients with bilateral axillary sputum were enrolled, and the relationship between serum bilirubin levels and initial hearing levels was explored using a univariate and multivariate linear regression model. Compared with the group with moderate and below hearing loss (≤70 dB HL), patients with severe profound HL(>70 dB HL) were more likely to have lower levels of total and indirect bilirubin level, magnesium and relative hearing gain, higher levels of final hearing, white blood counts, neutrophil, platelet and alkaline phosphatase. Read More

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The Effect of Cochlear Implants on Cognitive Function in Older Adults: Initial Baseline and 18-Month Follow Up Results for a Prospective International Longitudinal Study.

Front Neurosci 2019 2;13:789. Epub 2019 Aug 2.

The Royal Victorian Eye and Ear Hospital, Melbourne, VIC, Australia.

In older adults, hearing loss is independently associated with an increased rate of cognitive decline, and has been identified to be a modifiable risk factor for dementia. The mechanism underlying the cognitive decline associated with hearing loss is not understood, but it is known that the greater the hearing loss, the faster the rate of decline. It is unknown whether remediation of hearing loss with hearing devices can delay cognitive decline. Read More

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Diagnosis of congenital CMV infection via DBS samples testing and neonatal hearing screening: an observational study in Italy.

BMC Infect Dis 2019 Jul 22;19(1):652. Epub 2019 Jul 22.

Dipartimento di Scienze Biomediche per la Salute, Università degli Studi di Milano, Via Carlo Pascal, 36, 20133, Milan, Italy.

Background: Congenital Cytomegalovirus (cCMV) is the most common cause of non-genetic hearing loss in childhood. A newborn hearing screening program (NHSP) is currently running in Italy, but no universal cCMV nor statewide hearing-targeted CMV screening programs have been implemented yet. This observational monocentric study was aimed at estimating the rate of cCMV infections identified by CMV-DNA analysis on Dried Blood Spots (DBS) samples in deaf children identified via NHSP in Northern Italy in the period spanning from 2014 to 2018. Read More

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Study protocol for the validation of a new patient-reported outcome measure (PROM) of listening effort in cochlear implantation: the Listening Effort Questionnaire-Cochlear Implant (LEQ-CI).

BMJ Open 2019 07 9;9(7):e028881. Epub 2019 Jul 9.

Swansea University Medical School, Swansea University, Swansea, UK.

Introduction: Listening effort may be defined as the cognitive resources needed to understand an auditory message. A sustained requirement for listening effort is known to have a negative impact on individuals' sense of social connectedness, well-being and quality of life. A number of hearing-specific patient-reported outcome measures (PROMs) exist currently; however, none adequately assess listening effort as it is experienced in the listening situations of everyday life. Read More

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Guidelines (short version) of the French Society of Otorhinolaryngology (SFORL) on pediatric cochlear implant indications.

Eur Ann Otorhinolaryngol Head Neck Dis 2019 Oct 17;136(5):385-391. Epub 2019 Jun 17.

Service d'ORL pédiatrique, hôpital Necker - Enfants-Malades, université Paris Descartes, 75015 Paris, France.

Objectives: The authors present the guidelines of the French Society of Otorhinolaryngology - Head and Neck Surgery (Société française d'oto-rhino-laryngologie et de chirurgie de la face et du cou - SFORL) on the indications for cochlear implantation in children.

Methods: A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. Read More

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October 2019

Serum Bilirubin Level as a Potential Marker for the Hearing Outcome in Severe-Profound Bilateral Sudden Deafness.

Otol Neurotol 2019 07;40(6):728-735

Department of Otolaryngology-Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, China.

Objective: To investigate the association of serum bilirubin level with hearing outcomes in bilateral sudden sensorineural hearing loss (BSSHL) patients.

Participants: One hundred thirteen in-patient BSSHL patients were consecutively enrolled between July 2008 and December 2015 in a tertiary center.

Main Outcome Measures: Multivariable linear regression, generalized estimating equations (GEE), and stratified analyses were applied to examine the association between serum bilirubin level and hearing outcome measures such as final hearing threshold and absolute and relative hearing gains in BSSHL. Read More

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Application of Wireless Contralateral Routing of Signal Technology in Unilateral Cochlear Implant Users with Bilateral Profound Hearing Loss.

J Am Acad Audiol 2019 Jul/Aug;30(7):579-589. Epub 2018 Dec 13.

Department of Otolaryngology, University of Miami Ear Institute, University of Miami Miller School of Medicine, Miami, FL.

Purpose: The aim of the study was to determine if contralateral routing of signal (CROS) technology results in improved hearing outcomes in unilateral cochlear implant (CI) patients and provides similar gains in speech perception in noise to traditional monaural listeners (MLs).

Research Design: The study is a prospective, within-subject repeated-measures experiment.

Study Sample: Adult, English-speaking patients with bilateral severe-profound sensorineural hearing loss using an Advanced Bionics CI (n = 12) in one ear were enrolled for the study. Read More

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October 2020

Polydimethylsiloxanes biocompatibility in PC12 neuronal cell line.

Colloids Surf B Biointerfaces 2019 Jan 6;173:400-406. Epub 2018 Oct 6.

Bioacoustics Research Laboratory, Department of Neurosciences, University of Padua, 35129, Padua, Italy; Foundation Onlus "Staminali e Vita", 35138, Padua, Italy. Electronic address:

Cochlear implants, the only way to recover from severe/profound hearing loss, may cause adverse effects, among which reactions to silicone materials coating implant electrodes, leading to apoptosis and necrosis of spiral ganglion cells. Our aim was to evaluate whether three polydimethylsiloxane (PDMS) compounds (hexadimethylsiloxane, octamethyltrisiloxane, decamethylcyclopentasiloxane) used in silicone rods could exert toxic effects on an in vitro neuronal cell model (PC12). Cell viability, morphology and mRNA expression levels of apoptotic markers were evaluated on PC12 cells at different PDMS dilutions up to 6 days of exposure. Read More

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January 2019

Occurrence of major complications after cochlear implant surgery in Ireland.

Cochlear Implants Int 2018 11 29;19(6):297-306. Epub 2018 Aug 29.

a National Cochlear Implant Programme, Beaumont Hospital , Dublin , Ireland.

Objectives: Cochlear implantation (CI) is considered an effective and relatively safe procedure for patients with severe-profound hearing loss. However, severe complications are reported in several studies. The purpose of this study was to report the frequency and management of major complications following CI surgery at the National CI Programme (NCIP) in Ireland. Read More

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November 2018

Prevalence of severe-profound hearing loss in South Korea: a nationwide population-based study to analyse a 10-year trend (2006-2015).

Sci Rep 2018 07 2;8(1):9940. Epub 2018 Jul 2.

Department of Otolaryngology-Head and Neck Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

To estimate prevalence of severe-profound hearing loss (HL) in South Korea, and analyse a 10-year trend of HL according to age, sex, and region by using a nationwide population-based database. Retrospective review from Korean National Health Insurance Service from 2006 to 2015. The degree of severe-profound HL was classified into six grades, based mostly on HL worse than 60 dB HL for both ears. Read More

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