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Hearing Disorders: HELP
Articles by Alan G. Cheng
Based on 5 articles published since 2009
(Why 5 articles?)

Between 2009 and 2019, Alan Cheng wrote the following 5 articles about Hearing Disorders.
+ Citations + Abstracts
1 Guideline International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient. 2016

Liming, Bryan J / Carter, John / Cheng, Alan / Choo, Daniel / Curotta, John / Carvalho, Daniela / Germiller, John A / Hone, Stephen / Kenna, Margaret A / Loundon, Natalie / Preciado, Diego / Schilder, Anne / Reilly, Brian J / Roman, Stephane / Strychowsky, Julie / Triglia, Jean-Michel / Young, Nancy / Smith, Richard J H. ·Department of Otolaryngology -Head and Neck Surgery, University of Iowa Health Care, Iowa City, IA, USA. Electronic address: Bryan-liming@uiowa.edu. · Department of Otolaryngology- Head and Neck Surgery, Ochsner Medical Center, New Orleans, LA, USA. · Sydney Children's Hospital Network, Sydney, Australia. · Cincinnati Children's Hospital, Cincinnati, OH, USA. · Rady Children's Hospital, San Diego, CA, USA. · Children's Hospital of Philadelphia, Philadelphia PA, USA. · Our Lady's Children's Hospital, Crumlin, Dublin, Ireland. · Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston MA, USA. · Pediatric ENT Department, Hopital Necker-Enfants Malades, AP-HP Universite Paris Descartes, Paris, France. · Department of Otolaryngology, Children's National Hospital, Washington DC, USA. · Evident, UCL Ear Institute, Royal National Throat, Nose and Ear Hospital, London UK. · Department of Pediatric Otolaryngology, La Timone Children's Hospital, Aix-Marseille Universite', Marseille, France. · Paediatric Otolaryngology-Head and Neck Surgery-Children's Hospital at London Health Sciences Centre, London, Ontario, Canada. · Division of Otolaryngology-Head and Neck Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago IL, USA. · Department of Otolaryngology -Head and Neck Surgery, University of Iowa Health Care, Iowa City, IA, USA. ·Int J Pediatr Otorhinolaryngol · Pubmed #27729144.

ABSTRACT: OBJECTIVE: To provide recommendations for the workup of hearing loss in the pediatric patient. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group. RESULTS: Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. CONCLUSION: The workup of children with hearing loss can be guided by the recommendations provided herein.

2 Article Uncoordinated maturation of developing and regenerating postnatal mammalian vestibular hair cells. 2019

Wang, Tian / Niwa, Mamiko / Sayyid, Zahra N / Hosseini, Davood K / Pham, Nicole / Jones, Sherri M / Ricci, Anthony J / Cheng, Alan G. ·Department of Otolaryngology-Head and Neck Surgery, Stanford University School of Medicine, Stanford, California, United States of America. · Department of Otolaryngology-Head and Neck Surgery, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China. · Department of Special Education and Communication Disorders, College of Education and Human Sciences, University of Nebraska, Lincoln, Nebraska, United States of America. · Department of Molecular and Cellular Physiology, Stanford University School of Medicine, Stanford, California, United States of America. ·PLoS Biol · Pubmed #31260439.

ABSTRACT: Sensory hair cells are mechanoreceptors required for hearing and balance functions. From embryonic development, hair cells acquire apical stereociliary bundles for mechanosensation, basolateral ion channels that shape receptor potential, and synaptic contacts for conveying information centrally. These key maturation steps are sequential and presumed coupled; however, whether hair cells emerging postnatally mature similarly is unknown. Here, we show that in vivo postnatally generated and regenerated hair cells in the utricle, a vestibular organ detecting linear acceleration, acquired some mature somatic features but hair bundles appeared nonfunctional and short. The utricle consists of two hair cell subtypes with distinct morphological, electrophysiological and synaptic features. In both the undamaged and damaged utricle, fate-mapping and electrophysiology experiments showed that Plp1+ supporting cells took on type II hair cell properties based on molecular markers, basolateral conductances and synaptic properties yet stereociliary bundles were absent, or small and nonfunctional. By contrast, Lgr5+ supporting cells regenerated hair cells with type I and II properties, representing a distinct hair cell precursor subtype. Lastly, direct physiological measurements showed that utricular function abolished by damage was partially regained during regeneration. Together, our data reveal a previously unrecognized aberrant maturation program for hair cells generated and regenerated postnatally and may have broad implications for inner ear regenerative therapies.

3 Article Sox2 haploinsufficiency primes regeneration and Wnt responsiveness in the mouse cochlea. 2018

Atkinson, Patrick J / Dong, Yaodong / Gu, Shuping / Liu, Wenwen / Najarro, Elvis Huarcaya / Udagawa, Tomokatsu / Cheng, Alan G. ·Department of Otolaryngology - Head and Neck Surgery, Stanford University School of Medicine, Stanford, California, USA. · Department of Otology, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China. ·J Clin Invest · Pubmed #29553487.

ABSTRACT: During development, Sox2 is indispensable for cell division and differentiation, yet its roles in regenerating tissues are less clear. Here, we used combinations of transgenic mouse models to reveal that Sox2 haploinsufficiency (Sox2haplo) increases rather than impairs cochlear regeneration in vivo. Sox2haplo cochleae had delayed terminal mitosis and ectopic sensory cells, yet normal auditory function. Sox2haplo amplified and expanded domains of damage-induced Atoh1+ transitional cell formation in neonatal cochlea. Wnt activation via β-catenin stabilization (β-cateninGOF) alone failed to induce proliferation or transitional cell formation. By contrast, β-cateninGOF caused proliferation when either Sox2haplo or damage was present, and transitional cell formation when both were present in neonatal, but not mature, cochlea. Mechanistically, Sox2haplo or damaged neonatal cochleae showed lower levels of Sox2 and Hes5, but not of Wnt target genes. Together, our study unveils an interplay between Sox2 and damage in directing tissue regeneration and Wnt responsiveness and thus provides a foundation for potential combinatorial therapies aimed at stimulating mammalian cochlear regeneration to reverse hearing loss in humans.

4 Article Designer aminoglycosides prevent cochlear hair cell loss and hearing loss. 2015

Huth, Markus E / Han, Kyu-Hee / Sotoudeh, Kayvon / Hsieh, Yi-Ju / Effertz, Thomas / Vu, Andrew A / Verhoeven, Sarah / Hsieh, Michael H / Greenhouse, Robert / Cheng, Alan G / Ricci, Anthony J. · ·J Clin Invest · Pubmed #25555219.

ABSTRACT: Bacterial infections represent a rapidly growing challenge to human health. Aminoglycosides are widely used broad-spectrum antibiotics, but they inflict permanent hearing loss in up to ~50% of patients by causing selective sensory hair cell loss. Here, we hypothesized that reducing aminoglycoside entry into hair cells via mechanotransducer channels would reduce ototoxicity, and therefore we synthesized 9 aminoglycosides with modifications based on biophysical properties of the hair cell mechanotransducer channel and interactions between aminoglycosides and the bacterial ribosome. Compared with the parent aminoglycoside sisomicin, all 9 derivatives displayed no or reduced ototoxicity, with the lead compound N1MS 17 times less ototoxic and with reduced penetration of hair cell mechanotransducer channels in rat cochlear cultures. Both N1MS and sisomicin suppressed growth of E. coli and K. pneumoniae, with N1MS exhibiting superior activity against extended spectrum β lactamase producers, despite diminished activity against P. aeruginosa and S. aureus. Moreover, systemic sisomicin treatment of mice resulted in 75% to 85% hair cell loss and profound hearing loss, whereas N1MS treatment preserved both hair cells and hearing. Finally, in mice with E. coli-infected bladders, systemic N1MS treatment eliminated bacteria from urinary tract tissues and serially collected urine samples, without compromising auditory and kidney functions. Together, our findings establish N1MS as a nonototoxic aminoglycoside and support targeted modification as a promising approach to generating nonototoxic antibiotics.

5 Article Sensorineural hearing loss in patients with cystic fibrosis. 2009

Cheng, Alan G / Johnston, Patrick R / Luz, Jennifer / Uluer, Ahmet / Fligor, Brian / Licameli, Greg R / Kenna, Margaret A / Jones, Dwight T. ·Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, and Department of Otology and Laryngology, Harvard Medical School, Boston, MA, USA. aglcheng@stanford.edu ·Otolaryngol Head Neck Surg · Pubmed #19559964.

ABSTRACT: OBJECTIVE: To determine the prevalence of sensorineural hearing loss (SNHL) in cystic fibrosis (CF) patients and its relationship to antibiotic use. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: We reviewed the medical records of CF patients seen in our children's hospital between March 1994 and December 2007. Data collected included patient demographics, audiograms, tympanograms, genotype, and use of potentially ototoxic antibiotics. RESULTS: Seven of 50 (14%) patients had SNHL. Three percent of patients who received 10 courses (P<0.01). No patients who received five or fewer courses of nasal irrigation with aminoglycosides had SNHL versus 23 percent of those who received more than five courses (P<0.05). Nine percent of patients who received five or fewer courses of macrolides had SNHL versus 60 percent of those who received more than five courses (P=0.079). CONCLUSION: CF patients receiving aminoglycosides are at high risk for developing SNHL.