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Int J Pediatr Otorhinolaryngol 2021 Apr 14;145:110710. Epub 2021 Apr 14.

Department of Otorhinolaryngology - Head and Neck Surgery, Meir Medical Center, Kfar Saba, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address:

Objective: To compare Ostmann's fat pad (OFP) size between healthy ears and ears with chronic otitis media with cholesteatoma (COMwC) in children, to assess its role in the disease.

Methods: MRI scans of 29 children with unilateral COMwC with conductive hearing loss were used to measure OFP, represented by the maximal diameter of the high intensity area medial to the tensor veli palatini muscle (TVP): M1. A reference diameter was defined from the medial border of OFP reaching the medial border of the medial pterygoid muscle: M2. Read More

Int J Pediatr Otorhinolaryngol 2021 Apr 14;145:110711. Epub 2021 Apr 14.

Department of Otolaryngology-Head and Neck Surgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China; Regional Key Laboratory of Early Prevention and Treatment of High-Rise Tumors, Nanning, 530021, China. Electronic address:

Objective: Waardenburg syndrome type 2 (WS2) is a rare neural-crest disorder, characterized by heterochromic irides or blue eyes and sensorineural hearing loss. The aim of this study was to analyze the clinical features and investigate the genetic cause of WS2 in a small family from Guangxi Zhuang Autonomous region.

Methods: Whole-exome sequencing and mutational analysis were used to identify disease-causing genes in this family. Read More

Dev Biol 2021 Apr 14. Epub 2021 Apr 14.

Department of Molecular Pathobiology, New York University, College of Dentistry, New York, USA. Electronic address:

Nager syndrome is a rare human developmental disorder characterized by hypoplastic neural crest-derived craniofacial bones and limb defects. Mutations in SF3B4 gene, which encodes a component of the spliceosome, are a major cause for Nager. A review of the literature indicates that 45% of confirmed cases are also affected by conductive, sensorineural or mixed hearing loss. Read More

Lancet Child Adolesc Health 2021 May;5(5):e17

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland; Division of Pediatric Hematology/Oncology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

BMJ Case Rep 2021 Apr 16;14(4). Epub 2021 Apr 16.

Infectious Diseases, Townsville Hospital and Health Service, Townsville, Queensland, Australia.

Hearing loss is an unusual presenting feature of meningoencephalitis. Two cases of HIV-negative patients who presented with hearing loss are discussed and a literature review of published cases was conducted. Possible mechanisms for hearing loss with infection are explored. Read More

J Clin Neurosci 2021 May 19;87:97-102. Epub 2021 Mar 19.

Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China; Neurosurgical Institute of Fudan University, China; Shanghai Clinical Medical Center of Neurosurgery, China. Electronic address:

Long-term unilateral hearing loss could reorganize the functional network association between the bilateral auditory cortices, while alterations of other functional networks need to be further explored. We attempted to investigate the pattern of the reorganization of functional network associations between the auditory and visual cortex caused by long-term postlingual unilateral hearing loss (UHI) and its relationship with clinical characteristics. Therefore, 48 patients with hearing loss caused by unilateral acoustic tumors and 52 matched healthy controls were enrolled, and their high-resolution structural MRI and resting-state functional MRI data were also collected to depict the brain network. Read More

J Sports Sci 2021 Apr 16:1-9. Epub 2021 Apr 16.

Vision and Hearing Sciences Research Group, School of Psychology and Sports Science, Anglia Ruskin University, Cambridge, UK.

Classification within the sport of vision impairment (VI) shooting is based upon the athlete's visual function. This study aimed to determine whether more than one class of competition is needed within VI shooting on the basis of visual field loss. Qualification scores of 23 elite athletes were obtained at World Championship events in prone and standing shooting disciplines. Read More

Eur Arch Otorhinolaryngol 2021 Apr 16. Epub 2021 Apr 16.

University Clinic of Otorhinolaryngology, Faculty of Medicine, University of Coimbra, Coimbra, Portugal.

Purpose: Our study investigates the effectiveness of aural rehabilitation to decrease depressive symptoms in older adults, and the relationship between hearing loss and depression.

Methods: A randomized controlled study was conducted at a hearing rehabilitation center with people over 65 years old. Participants were randomly allocated to the intervention group who received hearing aids, or to the control group. Read More

Front Cell Infect Microbiol 2021 30;11:640899. Epub 2021 Mar 30.

Department of Biomaterials, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

Osseointegration is a well-established concept used in applications including the percutaneous Bone-Anchored Hearing System (BAHS) and auricular rehabilitation. To date, few retrieved implants have been described. A systematic review including cases where percutaneous bone-anchored implants inserted in the temporal bone were retrieved and analyzed was performed. Read More

Case Rep Otolaryngol 2021 23;2021:5592611. Epub 2021 Mar 23.

Novena Ent-Head & Neck Surgery Specialist Centre, 04-21/22/34, Mount Elizabeth Novena Medical Centre, 38 Irrawaddy Road, Singapore.

Meniere's disease (MD) is a condition characterised by fluctuating and progressive hearing loss, aural fullness, tinnitus, and intermittent attacks of vertigo. The disabling vertigo symptoms can be controlled in most patients by lifestyle changes and medications such as diuretics. Should standard medical therapy fail, the patient may require surgery in order to control the disease, but such surgical procedures can be functionally destructive. Read More

Otol Neurotol 2021 Apr 14. Epub 2021 Apr 14.

Kolling Deafness Research Centre, Royal North Shore Hospital, University of Sydney Department of Otolaryngology-Head and Neck Surgery, Faculty of Medicine and Health Sciences, Macquarie University, Sydney, Australia.

Objective: Pneumatoceles of the temporal bone are rare entities. A symptomatic external auditory canal pneumatocele repaired endoscopically is demonstrated.

Methods: A 79-year-old man presented with fluctuating hearing loss and difficulty wearing in-ear hearing aids. Read More

Otol Neurotol 2021 Apr 14. Epub 2021 Apr 14.

Division of Otolaryngology-Head and Neck Surgery, The George Washington University, Washington DC.

Objective: To present a case of progressive sensorineural hearing loss (SNHL) caused by labyrinthine erosion secondary to expanding geniculate ganglion cerebrospinal fluid (CSF) diverticulum extending along the path of the facial nerve.

Patient: Thirteen-year-old man with no past medical history or risk factors presented with unilateral progressive SNHL and no other otologic or neurologic symptoms.

Interventions: Audiological data as well as imaging studies including a magnetic resonance imaging (MRI) of the brain and internal auditory canal and a temporal bone computed tomography (CT) are presented. Read More

Otol Neurotol 2021 Apr 14. Epub 2021 Apr 14.

Department of Otorhinolaryngology, Head and Neck Surgery, Graduate School of Biomedical and Health Sciences, Hiroshima University Department of Otorhinolaryngology, Shinshu University School of Medicine Division of Rehabilitation, Department of Clinical Practice and Support, Hiroshima University, Minami-ku, Hiroshima, Japan.

Objective: Eyes absent 4 (EYA4) is the causative gene of autosomal dominant non-syndromic hereditary hearing loss, DFNA10. We aimed to identify a copy number variation of EYA4 in a non-syndromic sensory neural hearing loss pedigree.

Family And Clinical Evaluation: A Japanese family showing late-onset and progressive hearing loss was evaluated. Read More

Ear Hear 2021 Apr 15. Epub 2021 Apr 15.

Oticon A/S, Smørum, Denmark Department of Behavioural Sciences and Learning, Linnaeus Centre HEAD, Swedish Institute for Disability Research, Linköping University, Linköping, Sweden Eriksholm Research Centre, Snekkersten, Denmark Hearing Systems, Hearing Systems Group, Department of Electrical Engineering, Technical University of Denmark, Kongens Lyngby, Denmark Department of Nordic Studies and Linguistics, University of Copenhagen, Amager, Denmark.

Objectives: Communication requires cognitive processes which are not captured by traditional speech understanding tests. Under challenging listening situations, more working memory resources are needed to process speech, leaving fewer resources available for storage. The aim of the current study was to investigate the effect of task difficulty predictability, that is, knowing versus not knowing task difficulty in advance, and the effect of noise reduction on working memory resource allocation to processing and storage of speech heard in background noise. Read More

Ear Hear 2021 Apr 15. Epub 2021 Apr 15.

Department of Speech, Language, and Hearing Sciences, University of Connecticut, Storrs, CT Connecticut Institute for Brain and Cognitive Sciences, University of Connecticut, Storrs, CT Department of Surgery, Division of Otolaryngology-Head and Neck Surgery, University of Connecticut Health, Farmington, CT.

Objectives: Serological biomarkers, common to many areas of medicine, have the potential to inform on the health of the human body and to give early warning of risk of compromised function or illness before symptoms are experienced. Serological measurement of prestin, a motor protein uniquely produced and expressed in outer hair cells, has recently been identified as a potential biomarker to inform on the health of the cochlea. Before any test can be introduced into the clinical toolkit, the reproducibility of the measurement when repeated in the same subject must be considered. Read More

Ear Hear 2021 Apr 15. Epub 2021 Apr 15.

Department of Communicative Disorders and Deaf Education, Utah State University, Logan, Utah, USA Department of Communication, University of Washington, Seattle, Washington, USA.

Objectives: The majority of children born in the United States with hearing loss (HL) are born to parents with hearing. Many of these parents ultimately choose cochlear implantation for their children. There are now decades of research showing which these children's speech, language, listening, and education seem to benefit from cochlear implantation. Read More

J Laryngol Otol 2021 Apr 16:1-6. Epub 2021 Apr 16.

Getting it Right First Time programme, NHS Improvement, NHS England, UK.

Background: Litigation in the National Health Service continues to rise with a 9.4 per cent increase in clinical negligence claims from the period 2018 and 2019 to the period 2019 and 2020. The cost of these claims now accounts for 1. Read More

J Otolaryngol Head Neck Surg 2021 Apr 15;50(1):25. Epub 2021 Apr 15.

Department of Otolaryngology-Head and Neck Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

Background: Salivary gland choristoma (SGCh) is a rare benign tumor reported in several unusual sites, such as the gastrointestinal tract, the optic nerve, and the internal auditory canal, but never reported in the inner ear.

Case Presentation: An 8-year-old girl with a history of left profound congenital hearing loss presented to us with ipsilateral progressive severe facial nerve palsy (House-Brackmann Grade VI). The left tympanic membrane was swollen with a pulsatile tumor. Read More

Am J Otolaryngol 2021 Apr 7;42(5):103034. Epub 2021 Apr 7.

West Virginia University, Department of Otolaryngology, Morgantown, WV 26505, United States of America.

Objective: (1) Describe the presenting symptoms and tumor characteristics of patients who are diagnosed with a vestibular schwannoma (VS) with normal hearing or symmetric hearing loss, and (2) report the prospective growth and treatment of each tumor.

Methods: This is a retrospective cohort study of acoustic neuroma patients who were found to have a VS and normal or symmetric hearing loss at a single tertiary care academic center from 1999 to 2012. Medical records were reviewed collecting the following information: patient demographics, symptoms, MRI characteristics, and treatment modality. Read More

Psychol Assess 2021 Apr 15. Epub 2021 Apr 15.

Department of Hearing & Speech Sciences.

Listening-related fatigue can be a significant burden for adults with hearing loss (AHL), and potentially those with other health or language-related issues (e.g., multiple sclerosis, traumatic brain injury, second language learners) who must allocate substantial cognitive resources to the process of listening. Read More

Pediatr Blood Cancer 2021 Apr 15:e29055. Epub 2021 Apr 15.

Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

The diagnosis of MYH9 disorder is guided by recognizing granulocyte Döhle body-like inclusion bodies and large/giant platelets in the peripheral blood smear. Immunofluorescence study of nonmuscle myosin heavy chain IIA is a sensitive screening method for diagnosis of MYH9 disorder. The diagnosis can then be confirmed by genetic analysis. Read More

Sci Rep 2021 Apr 14;11(1):8103. Epub 2021 Apr 14.

Department of Occupational and Environmental Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 29 Saemunan-ro, Jongno-gu, Seoul, 03181, Republic of Korea.

We examined the associations of smoking status and urinary cotinine levels, an objective measure of smoking, with the development of new-onset HL. This cohort study was performed in 293,991 Korean adults free of HL who underwent a comprehensive screening examination and were followed for up to 8.8 years. Read More

Sci Rep 2021 Apr 14;11(1):8116. Epub 2021 Apr 14.

Department of Pharmacology, Southern Illinois University School of Medicine, Springfield, IL, 62702, USA.

Regulators of G protein signaling (RGS) accelerate the GTPase activity of G proteins to enable rapid termination of the signals triggered by G protein-coupled receptors (GPCRs). Activation of several GPCRs, including cannabinoid receptor 2 (CB2R) and adenosine A receptor (AAR), protects against noise and drug-induced ototoxicity. One such drug, cisplatin, an anticancer agent used to treat various solid tumors, produces permanent hearing loss in experimental animals and in a high percentage of cancer patients who undergo treatments. Read More

J Audiol Otol 2021 Apr 16. Epub 2021 Apr 16.

Department of Otolaryngology-Head and Neck Surgery, Bozyaka Training and Research Hospital, Izmir, Turkey.

Background And Objectives: This study aimed to evaluate the audiologic results after cochlear implantation (CI) in older patients and the degree of improvement in their quality of life (QoL).

Subjects And Methods: Patients over 65 years old who underwent CI at implant center in Bozyaka Training and Research Hospital were included in this study (n=54; 34 males and 20 females). The control group was patient over 65 years old with normal hearing (n=54; 34 males and 20 females). Read More

ORL J Otorhinolaryngol Relat Spec 2021 Apr 14:1-8. Epub 2021 Apr 14.

Department of Otolaryngology, Head and Neck Surgery, College of Medicine, The Pennsylvania State University, Hershey, Pennsylvania, USA.

Introduction: The precise etiology of Ménière's disease (MD) remains unknown; however, given the association of MD with serum antibodies and human leukocyte antigen (HLA) complex, several studies have proposed a relationship between MD and thyroid disorders. Similarly, multiple hypotheses exist regarding the metabolic disturbances of fluctuating thyroid hormone as a potential contributing agent in the development of MD.

Methods: A total of 171 abstracts were identified and screened by 2 independent reviewers. Read More

Laryngoscope 2021 Apr 14. Epub 2021 Apr 14.

Michigan Ear Institute, Farmington Hills, Michigan, USA.

Objectives/hypothesis: Hearing rehabilitation after translabyrinthine resection of a vestibular schwannoma (VS) has largely been based on the transfer of acoustic stimulus to the contralateral ear, typically through a contralateral routing of signal hearing aid or bone-anchored hearing aid (BAHA). Cochlear implant, either as a subsequent surgery or simultaneously, has become a more common treatment option; however, there is still relatively limited data available on its success. The purpose of this study is to evaluate the early outcomes of simultaneous cochlear implantation in patients with sporadic VS undergoing translabyrinthine resection. Read More

iScience 2021 Apr 8;24(4):102282. Epub 2021 Mar 8.

Molecular Architecture of Synapses Group, Institute for Auditory Neuroscience, InnerEarLab and Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, 37075 Göttingen, Germany.

Endbulbs of Held are located in the anteroventral cochlear nucleus and present the first central synapses of the auditory pathway. During development, endbulbs mature functionally to enable rapid and powerful synaptic transmission with high temporal precision. This process is accompanied by morphological changes of endbulb terminals. Read More

North Clin Istanb 2021 12;8(2):167-171. Epub 2021 Mar 12.

Department of Otorhinolaryngology, University of Health Sciences, Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey.

Objective: The aim of the study was to evaluate the results of neonatal hearing screening in our hospital with the help of literature and to question and reveal the risk factors to gain healthy individuals and to raise awareness for all health workers and the public who are interested in this subject.

Methods: A total of 16,388 newborn infants were evaluated between October 2009 and January 2018. All newborns were screened with transient evoked otoacoustic emissions (TEOAEs) test. Read More

Mol Ther Methods Clin Dev 2021 Jun 10;21:209-236. Epub 2021 Mar 10.

Department of Otorhinolaryngology, Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.

There is growing attention and effort focused on treating the root cause of sensorineural hearing loss rather than managing associated secondary characteristic features. With recent substantial advances in understanding sensorineural hearing-loss mechanisms, gene delivery has emerged as a promising strategy for the biological treatment of hearing loss associated with genetic dysfunction. There are several successful and promising proof-of-principle examples of transgene deliveries in animal models; however, there remains substantial further progress to be made in these avenues before realizing their clinical application in humans. Read More