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Hearing Disorders: HELP
Articles by Hung Jeffrey Kim
Based on 19 articles published since 2010
(Why 19 articles?)
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Between 2010 and 2020, H. Jeff Kim wrote the following 19 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Guideline ACR Appropriateness Criteria 2018

Anonymous7200967 / Sharma, Aseem / Kirsch, Claudia F E / Aulino, Joseph M / Chakraborty, Santanu / Choudhri, Asim F / Germano, Isabelle M / Kendi, A Tuba / Kim, H Jeffrey / Lee, Ryan K / Liebeskind, David S / Luttrull, Michael D / Moritani, Toshio / Murad, Gregory J A / Shah, Lubdha M / Shih, Robert Y / Symko, Sophia C / Bykowski, Julie. ·Mallinckrodt Institute of Radiology, Saint Louis, Missouri. Electronic address: sharmaa@mir.wustl.edu. · Panel Chair, North Shore-Long Island Jewish Hospital, Hofstra Medical School, Hempstead, New York. · Vanderbilt University Medical Center, Nashville, Tennessee. · Ottawa Hospital Research Institute and the Department of Radiology, The University of Ottawa, Ottawa, Ontario, Canada. · Le Bonheur Children's Hospital, University of Tennessee Health Science Center, Memphis, Tennessee. · Mount Sinai School of Medicine, New York, New York; neurosurgical consultant. · Mayo Clinic, Rochester, Minnesota. · Georgetown University Hospital, Washington, District of Columbia; American Academy of Otolaryngology-Head and Neck Surgery. · Einstein Healthcare Network, Philadelphia, Pennsylvania. · University of California Los Angeles, Los Angeles, California; American Academy of Neurology. · The Ohio State University Wexner Medical Center, Columbus, Ohio. · University of Iowa Hospitals and Clinics, Iowa City, Iowa. · University of Florida, Gainesville, Florida; neurosurgical consultant. · University of Utah, Salt Lake City, Utah. · Walter Reed National Military Medical Center, Bethesda, Maryland. · Neuroradiology consultant, Denver, Colorado. · Specialty Chair, UC San Diego Health, San Diego, California. ·J Am Coll Radiol · Pubmed #30392601.

ABSTRACT: This article presents guidelines for imaging utilization in patients presenting with hearing loss or vertigo, symptoms that sometimes occur concurrently due to proximity of receptors and neural pathways responsible for hearing and balance. These guidelines take into account the superiority of CT in providing bony details and better soft-tissue resolution offered by MRI. It should be noted that a dedicated temporal bone CT rather than a head CT best achieves delineation of disease in many of these patients. Similarly, optimal assessment often requires a dedicated high-resolution protocol designed to assess temporal bone and internal auditory canals even though such a study will be requested and billed as a brain MRI. Angiographic techniques are helpful in some patients, especially in the setting of vertigo. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

2 Article Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2. 2018

deTorres, Alvin T / Brewer, Carmen C / Zalewski, Chris K / King, Kelly A / Walker, Robert / Scott, Gretchen C / Asthagiri, Ashok R / Chittiboina, Prashant / Kim, Hung Jeffrey. ·Department of Otolaryngology-Head and Neck Surgery, Georgetown University Hospital, Washington, DC. · Audiology Unit, Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD. · Howard University College of Medicine, Washington, DC. · Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD. · Department of Neurosurgery, University of Virginia School of Medicine, Charlottesville, VA. · Neurosurgery Unit for Pituitary and Inheritable Disorders, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, MD. · Office of Clinical Director, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD. ·Otol Neurotol · Pubmed #29342057.

ABSTRACT: OBJECTIVE: To characterize the audiometric natural progression in patient-ears with small volume (<1,000 mm), treatment-naïve cochleovestibular schwannomas (CVSs) in Neurofibromatosis Type 2 (NF2). STUDY DESIGN: Prospective, longitudinal cohort study. SETTING: Quaternary medical research institute. PATIENTS: One hundred eleven ears in 71 NF2 patients with small, treatment-naïve CVSs observed from July 2006 to July 2016. INTERVENTION: Serial audiometric testing, including pure tone audiometry, speech audiometry, and magnetic resonance imaging (MRI). OUTCOME MEASURES: Four-frequency pure tone average (4f-PTA) of 0.5, 1, 2, and 4 kHz and word recognition score (WRS) were recorded. Their changes were compared with MRI changes in CVS volume over time. Times to significant hearing loss (10 dB loss in 4f-PTA) and WRS based on 95% critical difference were measured. RESULTS: Linear regression analysis showed a significant correlation with baseline hearing level (4f-PTA) and internal auditory canal (IAC) tumor volume to annual hearing decrease rate (AHDR) (p = 0.003, p = 0.0004). Hearing level at baseline and tumor volume correlate with AHDR while tumor volume growth rate does not. Two-way analysis of variance found significant differences in AHDR, risk of significant hearing loss, and risk of critical difference in WRS based on baseline hearing level (abnormal or normal) and IAC tumor volume (greater or less than 200 mm). CONCLUSION: Subjects with normal baseline hearing and small IAC tumor component had a low AHDR and low risk of significant hearing loss and may warrant conservative management while the presence of baseline hearing loss and large IAC volume resulted in higher ADHR and greater risk for further hearing loss and may benefit from early treatment interventions.

3 Article Gradual Symmetric Progression of DFNA34 Hearing Loss Caused by an NLRP3 Mutation and Cochlear Autoinflammation. 2018

Nakanishi, Hiroshi / Kawashima, Yoshiyuki / Kurima, Kiyoto / Muskett, Julie A / Kim, H Jeffrey / Brewer, Carmen C / Griffith, Andrew J. ·Molecular Biology and Genetics Section. · Office of Clinical Director. · Audiology Unit, National Institute on Deafness and Other Communication Disorders (NIDCD), NIH, Bethesda, Maryland. ·Otol Neurotol · Pubmed #29342053.

ABSTRACT: OBJECTIVE: To characterize the audiometric phenotype of autosomal-dominant DFNA34 hearing loss (HL) caused by a missense substitution in the NLRP3 gene. NLRP3 encodes a critical component of the NLRP3 inflammasome that is activated in innate immune responses. STUDY DESIGN: This study was conducted under protocol 01-DC-0229 approved by the NIH Combined Neurosciences IRB. We performed medical and developmental history interviews and physical and audiological examinations of affected individuals with DFNA34 HL caused by the p.Arg918Gln mutation of NLRP3. We retrospectively reviewed audiological reports, when available, from other health care centers. SETTING: Federal biomedical research facility. SUBJECTS: Eleven members of a North American family segregating p.Arg918Gln. MAIN OUTCOME MEASURES: Pure-tone thresholds, rates of pure-tone threshold progression, and speech discrimination scores. RESULTS: Eight subjects had bilateral sensorineural HL with an onset in the late 2nd to 4th decade of life. Slowly progressive HL initially primarily affected high frequencies. Low and middle frequencies were affected with advancing age, resulting in moderate HL with a downsloping audiometric configuration. The average annual threshold deterioration was 0.9 to 1.5 dB/yr. Speech recognition scores ranging from 60 to 100% were consistent with cochlear, but not retrocochlear, etiology. Three subjects (16, 22, and 32 yr old) had normal hearing thresholds. CONCLUSION: DFNA34 HL has an onset during early adulthood and progresses approximately 1.2 dB/yr.

4 Article Association of Hearing Loss and Otologic Outcomes With Fibrous Dysplasia. 2018

Boyce, Alison M / Brewer, Carmen / DeKlotz, Timothy R / Zalewski, Christopher K / King, Kelly A / Collins, Michael T / Kim, H Jeffrey. ·Section on Skeletal Disorders and Mineral Homeostasis, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland. · Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland. · Department of Otolaryngology-Head & Neck Surgery, Georgetown University Hospital, Washington, DC. ·JAMA Otolaryngol Head Neck Surg · Pubmed #29192304.

ABSTRACT: Importance: Fibrous dysplasia (FD) and McCune-Albright syndrome (MAS) are rare bone and endocrine disorders in which expansile fibro-osseous lesions result in deformity, pain, and functional impairment. The effect of FD on hearing and otologic function has not been established. Objectives: To characterize audiologic and otologic manifestations in a large cohort of individuals with FD/MAS and to investigate potential mechanisms of hearing loss. Design, Setting, and Participants: In this natural history study, individuals with craniofacial FD seen at a clinical research center underwent clinical, biochemical, computed tomographic, audiologic, and otolaryngologic evaluations. Main Outcomes and Measures: Clinical and radiologic features associated with hearing loss and otologic disease were evaluated. Conductive hearing loss was hypothesized to be associated with narrowing of the external auditory canal (EAC), FD involving the epitympanum, and FD crowding the ossicular chain. Sensorineural hearing loss was hypothesized to be associated with FD affecting the internal auditory canal (IAC) and otic capsule. Results: Of the 130 study participants with craniofacial FD who were evaluated, 116 (89.2%) had FD that involved the temporal bone (median age, 19.6 years; range, 4.6-80.3 years; 64 female [55.2%]), whereas 14 (10.8%) had craniofacial FD that did not involve the temporal bone. Of the 183 ears with temporal bone FD, hearing loss was identified in 41 ears (22.4%) and was conductive in 27 (65.9%), sensorineural in 12 (29.3%), and mixed in 2 (4.9%). Hearing loss was mild and nonprogressive in most participants. Whereas EACs were narrower in ears with FD (mean difference [MD], 0.33 mm; 95% CI, 0.11-0.55 mm), this finding was associated with conductive hearing loss in only 4 participants. Fibrous dysplasia crowding of the ossicles was associated with conductive hearing loss (odds ratio [OR], 5.0; 95% CI, 2.1-11.6). The IAC length was not different between ears with and without FD (MD, -0.37; 95% CI, -0.95 to 0.211); however, canals were elongated in ears with sensorineural hearing loss (MD, -1.33; 95% CI, -2.60 to -0.07). Otic capsule involvement was noted in only 4 participants, 2 of whom had sensorineural hearing loss. Both MAS-associated growth hormone excess (OR, 3.1; 95% CI, 1.3-7.5) and neonatal hypercortisolism (OR, 11; 95% CI, 2.5-55) were associated with an increased risk of hearing loss . Conclusions and Relevance: Hearing loss in craniofacial FD is common and mild to moderate in most individuals. It typically arises from FD crowding of the ossicular chain and elongation of the IAC, whereas EAC stenosis and otic capsule invasion are less common causes. Individuals with craniofacial FD should undergo otolaryngologic evaluation and monitoring, including assessment to identify those with high-risk features.

5 Article None 2017

Nakanishi, Hiroshi / Kawashima, Yoshiyuki / Kurima, Kiyoto / Chae, Jae Jin / Ross, Astin M / Pinto-Patarroyo, Gineth / Patel, Seema K / Muskett, Julie A / Ratay, Jessica S / Chattaraj, Parna / Park, Yong Hwan / Grevich, Sriharsha / Brewer, Carmen C / Hoa, Michael / Kim, H Jeffrey / Butman, John A / Broderick, Lori / Hoffman, Hal M / Aksentijevich, Ivona / Kastner, Daniel L / Goldbach-Mansky, Raphaela / Griffith, Andrew J. ·Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892. · Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892. · Rheumatology Fellowship and Training Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD 20892. · Rady Children's Hospital and Department of Pediatrics, University of California, San Diego, La Jolla, CA 92093. · Office of the Clinical Director, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892. · Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD 20892. · Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892; kastnerd@mail.nih.gov griffita@nidcd.nih.gov. · Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892. · Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892; kastnerd@mail.nih.gov griffita@nidcd.nih.gov. ·Proc Natl Acad Sci U S A · Pubmed #28847925.

ABSTRACT: The NLRP3 inflammasome is an intracellular innate immune sensor that is expressed in immune cells, including monocytes and macrophages. Activation of the NLRP3 inflammasome leads to IL-1β secretion. Gain-of-function mutations of

6 Article Auditory and otologic profile of Alström syndrome: Comprehensive single center data on 38 patients. 2017

Lindsey, Spencer / Brewer, Carmen / Stakhovskaya, Olga / Kim, Hung Jeffrey / Zalewski, Chris / Bryant, Joy / King, Kelly A / Naggert, Jürgen K / Gahl, William A / Marshall, Jan D / Gunay-Aygun, Meral. ·Department of Otolaryngology-Head and Neck Surgery, Georgetown University Hospital, Washington, D.C. · Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland. · Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland. · The Jackson Laboratory, Bar Harbor, Maine. · NIH Undiagnosed Diseases Program, Common Fund, Office of the Director, National Institutes of Health, Bethesda, Maryland. · Office of the Clinical Director, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland. · Alström Syndrome International, Bar Harbor, Maine. · Johns Hopkins University School of Medicine, Department of Pediatrics and McKusick-Nathans Institute of Genetic Medicine, Baltimore, Maryland. ·Am J Med Genet A · Pubmed #28573831.

ABSTRACT: Alström syndrome (AS) is a rare autosomal recessive ciliopathy caused by mutations in the ALMS1 gene. Hallmark characteristics include childhood onset of severe retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, and cardiomyopathy. Here we comprehensively characterize the auditory and otologic manifestations in a prospective case series of 38 individuals, aged 1.7-37.9 years, with genetically confirmed AS. Hearing loss was preceded by retinal dystrophy in all cases, and had an average age of detection of 7.45 years (range 1.5-15). Audiometric assessments showed mean pure tone averages (0.5, 1, 2, 4 kHz) of 48.6 and 47.5 dB HL in the right and left ears, respectively. Hearing was within normal limits for only 8/74 ears (11%). For the 66 ears with hearing loss, the degree was mild (12%), moderate (54%), or severe (8%). Type of hearing loss was predominantly sensorineural (77%), while three ears had mixed loss, no ears had conductive loss, and type of hearing loss was indeterminate for the remaining 12 ears. Serial audiograms available for 33 patients showed hearing loss progression of approximately 10-15 dB/decade. Our data show that hearing loss associated with AS begins in childhood and is a predominantly symmetric, sensory hearing loss that may progress to a severe degree. Absent otoacoustic emissions, intact speech discrimination, and disproportionately normal auditory brainstem responses suggest an outer hair cell site of lesion. These findings indicate that individuals with AS would benefit from sound amplification and if necessary, cochlear implantation.

7 Article Low surgical complication rates in cochlear implantation for young children less than 1 year of age. 2017

Kalejaiye, Adedoyin / Ansari, Ghedak / Ortega, Gezzer / Davidson, Mauricia / Kim, Hung Jeffrey. ·Department of Surgery, Division of Otolaryngology-Head and Neck Surgery, Howard University College of Medicine, Washington, DC, U.S.A. · Department of Surgery, Clive O. Callender Howard-Harvard Multidisciplinary Outcomes Research Center and the Division of Otolaryngology-Head and Neck Surgery, Howard University Hospital, Washington, DC, U.S.A. · Howard University College of Medicine, Washington, DC, U.S.A. · Department of Surgery, Howard University College of Medicine, Washington, DC, U.S.A. · Department of Otolaryngology-Head and Neck Surgery, Medstar Georgetown University Hospital, Washington, DC, U.S.A. ·Laryngoscope · Pubmed #27411677.

ABSTRACT: OBJECTIVE: To identify risk factors for perioperative morbidity among a large national cohort of pediatric patients undergoing cochlear implantation. STUDY DESIGN: Retrospective study utilizing the American College of Surgeons National Surgical Quality Improvement Program Pediatric database (2012-2013). METHODS: Pediatric cochlear implantation cases were identified using current procedural terminology 69930. Patients were categorized by age, and operative characteristics along with 30-day perioperative outcomes were analyzed. RESULTS: We identified 1,351 cases of pediatric cochlear implantation. The median age was 3.6 years, and 73 patients were less than 1 year of age. Of 21 complication occurrences (1.55%), superficial incisional surgical site infection (SSI) was the most common (n = 13, 61.9%). Thirty-nine patients (2.9%) required readmission. The median operative time was 142 minutes, and the mean postoperative length of stay was 0.58 days. When comparing patients younger than 1 year old to those 1 year or older, no significant differences were noted in complication rate, postoperative length of stay, or reoperation rate. Patients less than 1 year of age were more likely to be readmitted (6.9% vs. 2.7%, P = 0.04) and had longer mean operative times (191 minutes vs. 160 minutes, P = 0.0015). Steroid use was a risk factor for unplanned reoperation, SSI, and readmission. CONCLUSION: Despite a slight increase in readmission rates and operative times among patients less than 1 year of age, cochlear implantation appears to be safe in this population, with complication rates, reoperation rates, and postoperative lengths of stay similar to children undergoing the procedure at the current U.S. Food and Drug Administration-approved age of 1 year and older. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:720-724, 2017.

8 Article Otologic manifestations of Fanconi anemia and other inherited bone marrow failure syndromes. 2016

Kalejaiye, Adedoyin / Giri, Neelam / Brewer, Carmen C / Zalewski, Christopher K / King, Kelly A / Adams, Charleen D / Rosenberg, Philip S / Kim, H Jeffrey / Alter, Blanche P. ·Howard University Hospital, Washington, District of Columbia. · National Cancer Institute, National Institutes of Health, Rockville, Maryland. girin@mail.nih.gov. · National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland. · University of Washington School of Public Health, Seattle, Washington. · National Cancer Institute, National Institutes of Health, Rockville, Maryland. · Medstar Georgetown University Hospital, Washington, District of Columbia. ·Pediatr Blood Cancer · Pubmed #27428025.

ABSTRACT: BACKGROUND: The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the association between physical findings and hearing loss. METHODS: Patients with an IBMFS underwent comprehensive clinical and laboratory evaluations and testing for syndrome-specific gene mutations. Hearing loss was measured by pure tone audiometry and otologic abnormalities by otomicroscopy. RESULTS: Patients included 33 with FA, 37 with DC, 32 with DBA, and nine with SDS. Hearing loss was most frequent in patients with FA (45%) and DBA (14%). The most common type of hearing loss in FA was conductive (65%). Absent or hypoplastic radius, noted in 21% of the patients with FA, was associated with hearing loss in all cases. Otomicroscopy was abnormal in 66% of patients with FA. Characteristic ear abnormalities included small tympanic membrane (66%), malformed malleus (57%), aberrant tympanic bony island (48%), narrow external auditory canal (EAC) (32%), and abnormal course of chorda tympani (34%). Ear malformations were almost always associated with hearing loss. Hearing loss was rare in patients with DC and SDS. CONCLUSIONS: FA is the major IBMFS with associated hearing loss, which is most commonly conductive. Radial hypoplasia or aplasia and characteristic congenital ear malformations are associated with hearing loss in patients with FA. Recognition of these syndrome-specific abnormalities should lead to earlier management of hearing loss.

9 Article Intrinsic network activity in tinnitus investigated using functional MRI. 2016

Leaver, Amber M / Turesky, Ted K / Seydell-Greenwald, Anna / Morgan, Susan / Kim, Hung J / Rauschecker, Josef P. ·Department of Neuroscience, Georgetown University Medical Center, Washington, District of Columbia. · Department of Neurology, University of California Los Angeles, Los Angeles, California. · Division of Audiology, Medstar Georgetown University Hospital, Washington, District of Columbia. · Department of Otolaryngology, Medstar Georgetown University Hospital, Washington, District of Columbia. · Institute for Advanced Study, TU Munich, Germany. ·Hum Brain Mapp · Pubmed #27091485.

ABSTRACT: Tinnitus is an increasingly common disorder in which patients experience phantom auditory sensations, usually ringing or buzzing in the ear. Tinnitus pathophysiology has been repeatedly shown to involve both auditory and non-auditory brain structures, making network-level studies of tinnitus critical. In this magnetic resonance imaging (MRI) study, two resting-state functional connectivity (RSFC) approaches were used to better understand functional network disturbances in tinnitus. First, we demonstrated tinnitus-related reductions in RSFC between specific brain regions and resting-state networks (RSNs), defined by independent components analysis (ICA) and chosen for their overlap with structures known to be affected in tinnitus. Then, we restricted ICA to data from tinnitus patients, and identified one RSN not apparent in control data. This tinnitus RSN included auditory-sensory regions like inferior colliculus and medial Heschl's gyrus, as well as classically non-auditory regions like the mediodorsal nucleus of the thalamus, striatum, lateral prefrontal, and orbitofrontal cortex. Notably, patients' reported tinnitus loudness was positively correlated with RSFC between the mediodorsal nucleus and the tinnitus RSN, indicating that this network may underlie the auditory-sensory experience of tinnitus. These data support the idea that tinnitus involves network dysfunction, and further stress the importance of communication between auditory-sensory and fronto-striatal circuits in tinnitus pathophysiology. Hum Brain Mapp 37:2717-2735, 2016. © 2016 The Authors Human Brain Mapping Published by Wiley Periodicals, Inc.

10 Article Audiovestibular Characteristics of Small Cochleovestibular Schwannomas in Neurofibromatosis Type 2. 2014

Holliday, Michael A / Kim, Hung Jeffrey / Zalewski, Christopher K / Wafa, Talah / Dewan, Ramita / King, Kelly A / Brewer, Carmen C / Butman, John A / Asthagiri, Ashok R. ·Department of Otolaryngology-Head and Neck Surgery, Georgetown University Hospital, Washington, DC, USA michael.a.holliday@gunet.georgetown.edu. · Department of Otolaryngology-Head and Neck Surgery, Georgetown University Hospital, Washington, DC, USA Office of Clinical Director, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, USA. · Audiology Unit, Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, USA. · Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA. · Radiology and Imaging Sciences, The Clinical Center at the National Institutes of Health, National Institutes of Health, Bethesda, Maryland, USA. ·Otolaryngol Head Neck Surg · Pubmed #24718755.

ABSTRACT: OBJECTIVE: Describe the relationship between cochleovestibular schwannoma (CVS) volume, audiovestibular characteristics, and magnetic resonance imaging (MRI) findings in patients with neurofibromatosis type 2 (NF2). STUDY DESIGN: Subgroup analysis of NF2 prospective natural history study from 2008 to 2011. SETTING: Quaternary medical research institute. SUBJECTS AND METHODS: NF2 patients with small treatment-naive CVSs (volume <1000 mm(3)) by ear; N = 49 ears (32 patients). Cross-sectional analysis of the following parameters was performed: tumor size, auditory brainstem response (ABR), 4-frequency pure-tone average (4f-PTA; 0.5, 1, 2, and 4KHz), cervical vestibular evoked myogenic potential (cVEMP), caloric testing, 240° velocity step test (VST), and MRI findings. RESULTS: For all physiologic measures but the 4f-PTA, larger tumors correlated with abnormal responses (P < .05). For abnormal ABR, mean tumor volume was 405 vs 151 mm(3) (P = .0007) for normal ABR. Similarly, larger tumors correlated with weak caloric responses (mean 521 vs 165 mm(3); P = .0007) and weak cVEMP (mean 357 vs 192 mm(3); P = .0262). Tumor volume was not significantly correlated with 4f-PTA. Elevated intralabyrinthine protein on MRI fluid-attenuated inversion recovery sequences was correlated with larger tumor volume (mean 333 vs 55 mm(3); P = .001) and abnormal ABR and 4f-PTA (P < .05) but did not correlate with cVEMP, VST, or caloric responses. CONCLUSION: In our cohort, ABR, caloric response, cVEMP, and elevated intralabyrinthine protein correlated with tumor volume, but 4f-PTA did not. Abnormal ABR and 4f-PTA correlated with elevated intralabyrinthine protein. These findings may provide insight on the effect of small CVS on the inner ear and cochleovestibular nerves, which may aid in their optimal management.

11 Article Clinical presentation and imaging findings in patients with pulsatile tinnitus and sigmoid sinus diverticulum/dehiscence. 2014

Grewal, Ameet K / Kim, Han Y / Comstock, Richard H / Berkowitz, Frank / Kim, Hung Jeffrey / Jay, Ann K. ·*Department of Otolaryngology, Georgetown University Hospital; †Department of Radiology, Howard University Hospital; and ‡Department of Radiology, Georgetown University Hospital, Washington, District of Columbia, U.S.A. ·Otol Neurotol · Pubmed #24005164.

ABSTRACT: OBJECTIVE: Sigmoid sinus diverticulum/dehiscence (SSDD) is an increasingly recognized venous cause for pulsatile tinnitus (PT). SSDD is amenable to surgical/endovascular intervention. We aim to understand the clinical and imaging features of patients with PT due to SSDD. STUDY DESIGN: Retrospective CT study and chart review. SETTING: Tertiary-care, academic center. PATIENTS: Cohort 1: 200 consecutive unique temporal bone CT were blindly reviewed for anatomic findings associated with PT. Cohort 2: 61 patients with PT were evaluated for otologic manifestations. INTERVENTION(S): All patients underwent a temporal bone CT for evaluation of PT. Clinical information was gathered using electronic medical records. MAIN OUTCOME MEASURE(S): Otologic symptoms and physical findings (including body mass index (BMI), mastoid/neck bruits) were analyzed. Temporal bone CT scans were evaluated for the presence of SSDD and other possible causes of PT. RESULTS: Cohort 1: 35 cases of SSDD were identified (18%); 10 (29%) true diverticula; and 25 (71%) dehiscence. Sixty-six percent were right sided. Twelve patients had PT (34%). Patients with SSDD are more likely to have PT (p = 0.003). A significant association between right SSDD and PT was found (p = 0.001). Cohort 2: 15 out of 61 patients had PT and CT-confirmed SSDD. All were female subjects; average age was 45 years (26-73 yr). Radiologic evaluation revealed 10 SSDD cases on the right (66.7%), 2 on the left (13.3%%), and 3 bilateral (20%). Sensorineural hearing loss was seen in 8 (53%), aural fullness in 12 (80%). Average BMI was 32.2 (21.0-59.82), and 4 (26%) had audible mastoid bruits. CONCLUSION: SSDD may be the most common identifiable cause for PT from venous origin and is potentially treatable. Temporal bone CT scans should be included in a complete evaluation of PT.

12 Article Mechanisms of hearing loss in neurofibromatosis type 2. 2012

Asthagiri, Ashok R / Vasquez, Raul A / Butman, John A / Wu, Tianxia / Morgan, Keaton / Brewer, Carmen C / King, Kelly / Zalewski, Chris / Kim, H Jeffrey / Lonser, Russell R. ·Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA. asthagiria@ninds.nih.gov ·PLoS One · Pubmed #23049959.

ABSTRACT: INTRODUCTION: Patients with neurofibromatosis type 2 (NF2) develop bilateral cochleovestibular schwannomas (CVSs) that cause binaural deafness in most individuals. Hearing loss occurs in an unpredictable manner and the underlying mechanisms are not known. To gain insight into the pathophysiologic basis for hearing loss in NF2, we performed a prospective cross-sectional study of untreated ears in NF2 patients. METHODS: One hundred consecutive NF2 patients in a prospective natural history study were included. Clinical and audiometric data were analyzed for treatment naïve ears. In addition to standard MR-imaging sequences, alterations in intralabyrinthine protein content were determined utilizing high resolution FLAIR, the presence of cochlear aperture obstruction was determined by examining 3D T2 sequences, and endolymphatic hydrops was identified on delayed post-contrast FLAIR sequences. RESULTS: Eighty-nine ears harboring 84 untreated CVSs in 56 consecutive NF2 patients (age 30 ± 16 years) were analyzed. Thirty-four (38%) ears had varying degrees of hearing loss. Elevated intralabyrinthine protein was identified in 70 (75%) ears by FLAIR MR-imaging and was strongly associated with the presence of hearing loss (32/34 hearing loss ears; 94%)(Fisher's exact test; P= .005). Elevated intralabyrinthine protein was associated with the presence of CVS-associated cochlear aperture obstruction (64 of 67 ears with elevated protein; 96%)(Fisher's exact test; P<0.0001) in both normal and hearing loss ears. Elevated intralabyrinthine protein was not identified in ears without CVS (5 ears). While larger tumor size was associated with hearing loss (P=0.006), 16 hearing loss ears (47%) harbored CVSs less than 0.5 cm(3), including 14 ears (88%) with block of the cochlear aperture and elevated protein. DISCUSSION: These findings are consistent with a model in which hearing loss develops as a result of cochlear aperture obstruction and accumulation of intralabyrinthine protein. MRI based identification of elevated intralabyrinthine protein may help identify the ear at-risk for developing hearing loss.

13 Article Functional MRI evidence for a role of ventral prefrontal cortex in tinnitus. 2012

Seydell-Greenwald, Anna / Leaver, Amber M / Turesky, Ted K / Morgan, Susan / Kim, Hung J / Rauschecker, Josef P. ·Department of Neuroscience, Georgetown University Medical Center, 3970 Reservoir Rd. NW, Washington, DC 20007, USA. as2266@georgetown.edu ·Brain Res · Pubmed #22982009.

ABSTRACT: It has long been known that subjective tinnitus, a constant or intermittent phantom sound perceived by 10 to 15% of the adult population, is not a purely auditory phenomenon but is also tied to limbic-related brain regions. Supporting evidence comes from data indicating that stress and emotion can modulate tinnitus, and from brain imaging studies showing functional and anatomical differences in limbic-related brain regions of tinnitus patients and controls. Recent studies from our lab revealed altered blood oxygen level-dependent (BOLD) responses to stimulation at the tinnitus frequency in the ventral striatum (specifically, the nucleus accumbens) and gray-matter reductions (i.e., anatomical changes) in ventromedial prefrontal cortex (vmPFC), of tinnitus patients compared to controls. The present study extended these findings by demonstrating functional differences in vmPFC between 20 tinnitus patients and 20 age-matched controls. Importantly, the observed BOLD response in vmPFC was positively correlated with tinnitus characteristics such as subjective loudness and the percent of time during which the tinnitus was perceived, whereas correlations with tinnitus handicap inventory scores and other variables known to be affected in tinnitus (e.g., depression, anxiety, noise sensitivity, hearing loss) were weaker or absent. This suggests that the observed group differences are indeed related to the strength of the tinnitus percept and not to an affective reaction to tinnitus. The results further corroborate vmPFC as a region of high interest for tinnitus research.This article is part of a Special Issue entitled: Tinnitus Neuroscience.

14 Article Audiovestibular dysfunction associated with adoptive cell immunotherapy for melanoma. 2012

Seaman, Bradley J / Guardiani, Elizabeth A / Brewer, Carmen C / Zalewski, Christopher K / King, Kelly A / Rudy, Susan / Van Waes, Carter / Morgan, Richard A / Dudley, Mark E / Yang, James C / Rosenberg, Steven A / Kim, H Jeffrey. ·Department of Otolaryngology-Head and Neck Surgery, Georgetown University Medical Center, Washington, DC 20007, USA. ·Otolaryngol Head Neck Surg · Pubmed #22597578.

ABSTRACT: OBJECTIVE: To understand the audiologic and vestibular toxicities associated with adoptive cell immunotherapy (ACI) targeting pigment-pathway antigens on melanoma and to investigate the use of intratympanic steroid injections in the treatment of these toxicities. STUDY DESIGN: Prospective nonrandomized study. SETTING: Tertiary clinical research center. METHODS: Thirty-two patients with progressive metastatic melanoma who failed conventional therapy underwent ACI with T cells genetically modified to target MART-1 (n = 18) or gp100 (n = 14). All patients received serial audiometric testing. Vestibular testing was performed on patients with vestibular complaints. Patients with significant deficits received intratympanic steroid injections. RESULTS: Of 32 patients, 15 had no hearing change, 9 had mild hearing loss, and 8 had moderate hearing loss following treatment. Ten patients received intratympanic steroid injections for mild (n = 2) or moderate (n = 7) hearing loss or for significant imbalance (n = 1). Of those with mild hearing loss (n = 9), all but 1 recovered to pretreatment hearing levels. Four of 8 patients with moderate hearing loss recovered to baseline hearing levels, and 4 had partial recovery. All 7 patients with posttreatment vestibular complaints had demonstrable vestibular dysfunction. Three of these patients demonstrated recovery to normal vestibular function. The number of modified T cells infused for therapy correlated with the degree of audiovestibular deficit. CONCLUSION: Adoptive cell immunotherapy targeting pigment-pathway cell proteins, a novel therapy for melanoma, can induce hearing loss and vestibular dysfunction. The presumed mechanism of autoimmune attack on normal melanocytes in the cochlear stria vascularis and in the vestibular organs demonstrates the importance of melanocytes in normal inner ear function.

15 Article Discrimination task reveals differences in neural bases of tinnitus and hearing impairment. 2011

Husain, Fatima T / Pajor, Nathan M / Smith, Jason F / Kim, H Jeff / Rudy, Susan / Zalewski, Christopher / Brewer, Carmen / Horwitz, Barry. ·Department of Speech and Hearing Science, University of Illinois at Urbana-Champaign, Champaign, Illinois, United States of America. husainf@illinois.edu ·PLoS One · Pubmed #22066003.

ABSTRACT: We investigated auditory perception and cognitive processing in individuals with chronic tinnitus or hearing loss using functional magnetic resonance imaging (fMRI). Our participants belonged to one of three groups: bilateral hearing loss and tinnitus (TIN), bilateral hearing loss without tinnitus (HL), and normal hearing without tinnitus (NH). We employed pure tones and frequency-modulated sweeps as stimuli in two tasks: passive listening and active discrimination. All subjects had normal hearing through 2 kHz and all stimuli were low-pass filtered at 2 kHz so that all participants could hear them equally well. Performance was similar among all three groups for the discrimination task. In all participants, a distributed set of brain regions including the primary and non-primary auditory cortices showed greater response for both tasks compared to rest. Comparing the groups directly, we found decreased activation in the parietal and frontal lobes in the participants with tinnitus compared to the HL group and decreased response in the frontal lobes relative to the NH group. Additionally, the HL subjects exhibited increased response in the anterior cingulate relative to the NH group. Our results suggest that a differential engagement of a putative auditory attention and short-term memory network, comprising regions in the frontal, parietal and temporal cortices and the anterior cingulate, may represent a key difference in the neural bases of chronic tinnitus accompanied by hearing loss relative to hearing loss alone.

16 Article Otologic and audiologic manifestations of Hutchinson-Gilford progeria syndrome. 2011

Guardiani, Elizabeth / Zalewski, Christopher / Brewer, Carmen / Merideth, Melissa / Introne, Wendy / Smith, Ann C M / Gordon, Leslie / Gahl, William / Kim, H Jeffrey. ·Georgetown University Hospital Department of Otolaryngology-Head and Neck Surgery, Washington, DC, USA. ·Laryngoscope · Pubmed #21898437.

ABSTRACT: OBJECTIVES/HYPOTHESIS: To define the audiologic and otologic phenotype of Hutchinson-Gilford progeria syndrome (HGPS). STUDY DESIGN: Prospective case series. METHODS: Fifteen patients with HGPS were enrolled in a prospective natural history study; 14 were evaluated in the neurotology clinic, and 11 received audiologic evaluations. The physical exam and audiologic findings of these patients were reviewed to define an otologic and audiologic phenotype for HGPS in the largest series of subjects in the literature. RESULTS: All patients were noted to have stiff auricular cartilages, small or absent lobules, and hypoplasia of the lateral soft-tissue portion of the external ear canal leading to a shortened canal. Ten of 14 patients (71%) had dry cerumen impaction, and four of 14 patients (29%) reported a history of recurrent otitis media. Nineteen of 22 ears (86.4%) demonstrated low-frequency conductive hearing loss in the 250 to 500 Hz range. Sixteen of 22 ears (73%) had type A tympanograms; three of 22 ears (14%) displayed bimodal or "W" peaked tympanograms; two of 22 ears (9%) had type B tympanograms; one of 22 ears (4%) had a type C tympanogram. Nine of 10 patients had distortion product otoacoustic emissions consistent with normal peripheral hearing sensitivity. CONCLUSIONS: HGPS is caused by a mutation in the LMNA gene resulting in the production of an abnormal nuclear protein; this in turn affects nuclear structure and function. Patients with HGPS have characteristic otologic features due to cartilaginous and subcutaneous tissue abnormalities and typically demonstrate low-frequency conductive hearing loss despite largely normal tympanometry. It is important to be aware of these conditions in managing these patients.

17 Article Cryopyrin-associated periodic syndromes: otolaryngologic and audiologic manifestations. 2011

Ahmadi, Neda / Brewer, Carmen C / Zalewski, Christopher / King, Kelly A / Butman, John A / Plass, Nicole / Henderson, Cailin / Goldbach-Mansky, Raphaela / Kim, H Jeffrey. ·Georgetown University Hospital, Washington, DC, USA. ·Otolaryngol Head Neck Surg · Pubmed #21493283.

ABSTRACT: OBJECTIVE: Cryopyrin-associated periodic syndromes (CAPS) represent a spectrum of CIAS1 gene-mediated autoinflammatory diseases characterized by recurrent systemic inflammation. The clinical spectrum of CAPS varies from mild to severe and includes the syndromes historically described as familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID). This article presents the largest cohort of patients with CAPS. The objective is to describe the pathogenesis, otolaryngologic, and audiologic manifestations of CAPS. STUDY DESIGN: Prospective (2003-2009). SETTING: National Institutes of Health. SUBJECTS AND METHODS: Fifty-seven patients with a diagnosis of CAPS were identified (31 NOMID, 11 NOMID/MWS, 9 MWS, and 6 FCAS). Comprehensive data regarding clinical manifestations, audiologic phenotype, and fluid attenuation inversion recovery MRI (FLAIR-MRI) of the brain and inner ear were obtained. RESULTS: Complete audiologic data obtained on 70% of ears revealed conductive hearing loss in 4 (11%) NOMID ears and mixed hearing loss in 5 (13%) NOMID and 2 (14%) NOMID/MWS ears. Sensorineural hearing loss (SNHL), worse in higher frequencies, was the most common type of hearing loss and was present in 23 (61%) NOMID, 10 (71%) NOMID/MWS, and 4 (33%) MWS ears. All of the patients with FCAS had normal hearing except 2, who had SNHL from 4 to 8 kHz. On FLAIR-MRI sequence, cochlear enhancement was noted in 26 of 29 (90%) NOMID, 6 of 11 (55%) NOMID/MWS, 3 of 9 (33%) MWS, and 1 of 6 (17%) FCAS patients and was significantly associated with the presence of hearing loss. Maxillary sinus hypoplasia and mucosal thickening were found in 39% and 86% of the cohort, respectively. CONCLUSION: CIAS1 pathway–mediated CAPS is associated with unregulated autoinflammation mediated by interleukin-1 in the cochlea and hearing loss. Timely diagnosis is crucial to initiate early treatment with interleukin-1 receptor antagonists.

18 Article Dysregulation of limbic and auditory networks in tinnitus. 2011

Leaver, Amber M / Renier, Laurent / Chevillet, Mark A / Morgan, Susan / Kim, Hung J / Rauschecker, Josef P. ·Georgetown University Medical Center, Washington, DC 20057, USA. ·Neuron · Pubmed #21220097.

ABSTRACT: Tinnitus is a common disorder characterized by ringing in the ear in the absence of sound. Converging evidence suggests that tinnitus pathophysiology involves damage to peripheral and/or central auditory pathways. However, whether auditory system dysfunction is sufficient to explain chronic tinnitus is unclear, especially in light of evidence implicating other networks, including the limbic system. Using functional magnetic resonance imaging and voxel-based morphometry, we assessed tinnitus-related functional and anatomical anomalies in auditory and limbic networks. Moderate hyperactivity was present in the primary and posterior auditory cortices of tinnitus patients. However, the nucleus accumbens exhibited the greatest degree of hyperactivity, specifically to sounds frequency-matched to patients' tinnitus. Complementary structural differences were identified in ventromedial prefrontal cortex, another limbic structure heavily connected to the nucleus accumbens. Furthermore, tinnitus-related anomalies were intercorrelated in the two limbic regions and between limbic and primary auditory areas, indicating the importance of auditory-limbic interactions in tinnitus.

19 Article Computed tomography and otosclerosis: a practical method to correlate the sites affected to hearing loss. 2010

Wycherly, Benjamin J / Berkowitz, Frank / Noone, Anne-Michelle / Kim, H Jeffrey. ·Department of Otolaryngology-Head and Neck Surgery, Georgetown University, Washington, DC 20007, USA. ·Ann Otol Rhinol Laryngol · Pubmed #21250549.

ABSTRACT: OBJECTIVES: We present a practical method for correlating computed tomography (CT) scans with hearing loss in otosclerosis. METHODS: We reviewed the CT scans of 18 patients (34 ears) with clinical otosclerosis who were seen between 2007 and 2008. The scans were reviewed by an otologist in a clinical office setting, followed by a blinded radiologist working at an imaging workstation. The 5 most commonly affected sites in otosclerosis were evaluated for evidence of otospongiosis and then correlated with the degree of air-bone gap and sensorineural hearing loss. RESULTS: Positive CT findings were noted in 70.5% of ears, with a 94% concordance between readings. The sites affected included the ante fenestram (21 ears), round window niche (12), cochlear promontory (4), cochlear apex (3), and posterior fenestram (2). The average air-bone gap increased with each additional site of involvement within an otic capsule (p = 0.004). The bone conduction threshold also increased, on average, with each additional affected site (p = 0.047). CONCLUSIONS: Most patients with clinical evidence of otosclerosis have evidence of otosclerosis on CT that is readily detected in the office setting. Ears with more affected sites have a significantly greater degree of air-bone gap and sensorineural hearing loss.