Publications by authors named "Jeffrey T Vrabec"

47 Publications

Otosclerosis in an Urban Population.

Otol Neurotol 2021 01;42(1):24-29

Houston Methodist ENT Specialists, Houston, Texas.

Objective: To investigate the evolving prevalence of otosclerosis in a large urban population.

Methods: A retrospective review of patients in a large, urban, public health system was conducted from January 2010 to August 2019 to identify subjects with otosclerosis. Diagnostic testing included audiometry and computed tomography scans. Sex, age at diagnosis, treatment received, race, ethnicity, and country of birth were analyzed for each subject and compared with all eligible patients in the reference population.

Results: A total of 134 patients from a reference population of 672,839 were diagnosed with otosclerosis and analyzed. The otosclerosis patients were predominantly Hispanic (73%), of which the majority were foreign born (87%). The average age at onset was 46 years and 59% were women. The overall prevalence of otosclerosis was 20 of 100,000 patients. The crude prevalence of otosclerosis by ethnicity was 43 of 100,000 for Hispanics, 12.6 of 100,000 for Caucasians, and 3 of 100,000 for African Americans. Within the Hispanic population, the prevalence of otosclerosis was 60 of 100,000 for foreign-born individuals and 16 of 100,000 for those born in the USA (odds ratio [OR] = 3.69, [95% confidence interval [CI], 2.02-6.76], p < 0.0001). Prevalence was not significantly different among Caucasians and US-born Hispanics.

Conclusion: Otosclerosis in the studied population was most common among Hispanic patients, though it was strongly influenced by country of birth. The "imported" otosclerosis cases are best explained by environmental influence rather than ethnic susceptibility. This discrepancy is likely due to variance in measles immunization rates among North and Central American countries before 1990.
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http://dx.doi.org/10.1097/MAO.0000000000002870DOI Listing
January 2021

The Rise and Fall of Otosclerosis: A Population-based Study of Disease Incidence Spanning 70 Years.

Otol Neurotol 2020 10;41(9):e1082-e1090

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota.

Objective: Although it is commonly held that otosclerosis has become increasingly uncommon over recent decades, no population-based data exist to characterize this trend. Moreover, because most large epidemiologic databases within the United States primarily include cancer data, even the modern incidence of otosclerosis is unknown. The chief objective of the current work was to characterize the trend in the incidence of otosclerosis over 70 years using the unique resources of the Rochester Epidemiology Project.

Study Design: Population-based study.

Patients: Residents of Olmsted County, Minnesota diagnosed with otosclerosis.

Main Outcome Measure: Disease incidence from 1950 to 2017.

Results: From 1950 to 2017, 614 incident cases of otosclerosis were identified. The incidence rose from 8.9 per 100,000 person-years in the 1950s to a peak of 18.5 from 1970 to 1974. From this peak, the incidence significantly declined to 6.2 per 100,000 person-years by the early-1990s and reached a nadir of 3.2 from 2015 to 2017 (p<0.001). From 1970 to 2017, age at diagnosis (p = 0.23) and the proportion of bilateral cases (p = 0.16) did not significantly change; pure-tone average at diagnosis did not clinically appreciably change over the study period (median difference <5 dB across decades, p = 0.034).

Conclusions: The incidence of otosclerosis drastically declined since the early-1970s. Historically considered one of the most common causes of acquired hearing loss, the low modern incidence of otosclerosis renders it legally a "rare disease" within the United States. These trends require consideration when determining trainee case requirements and developing practice guidelines.
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http://dx.doi.org/10.1097/MAO.0000000000002763DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8055300PMC
October 2020

The Potential for Preserved Cochleovestibular Function in the Setting of Prolonged Pneumolabyrinth.

Otol Neurotol 2020 08;41(7):929-933

Houston Methodist ENT and Facial Plastic Surgery Associates, Houston, Texas.

Objective: To discuss the natural history and management of pneumolabyrinth, with particular attention to illustrative cases where hearing improved after a fluid-filled vestibule was restored.

Patients: Adults at two academic medical centers with documented pneumolabyrinth on computed tomography (CT) imaging.

Intervention: Middle ear surgery or observation.

Main Outcome Measures: Cochlear and vestibular symptoms are reported. Inner ear function was documented with audiometry and vestibular testing. Resolution of pneumolabyrinth was documented on imaging for a subset of patients.

Results: All patients presented with severe hearing loss following temporal bone fracture (three cases), penetrating trauma (one case), or stapedectomy (three cases). The radiographic finding was typically seen when imaging was obtained in the first few days after the injury. In two cases, vestibular symptoms resolved and partial recovery of hearing was seen despite a relatively long time interval between the inciting event and repair.

Conclusion: Management of pneumolabyrinth is individualized based on the mechanism of injury, severity of symptoms, and timing of the imaging study. In most cases, the barrier between the middle ear and inner ear is expected to heal with rapid resolution of pneumolabyrinth. Late repair is demonstrated to result in favorable recovery in selected cases. The presence of air in the vestibule cannot be assumed to be indicative of irreversible inner ear damage.
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http://dx.doi.org/10.1097/MAO.0000000000002675DOI Listing
August 2020

A Systematic Review of Cochlear Implant Outcomes in Prelingually-deafened, Late-implanted Patients.

Otol Neurotol 2020 04;41(4):444-451

Bobby R. Alford Department of Otolaryngology - Head and Neck Surgery, Baylor College of Medicine.

Objective: To analyze outcomes of cochlear implantation (CI) in prelingually-deafened, late-implanted patients.

Data Sources: A search of MEDLINE and EMBASE was performed in February 2018 using SCOPUS for the intersection of "cochlear implant," "prelingual," "deaf," and "delay."

Review Methods: Two independent reviewers screened all abstracts and titles for relevance, with conflicts resolved by either the primary or senior author. All articles passing this screen were subjected to a full-text review, during which the primary and senior author each examined manuscripts for inclusion and exclusion criteria. The Cochrane Risk of Bias Assessment Tool was used to assess potential sources of systematic error, and postoperative clinical outcomes were collected at the latest clinical follow-up.

Results: Twenty-eight articles were yielded in the final systematic review, accounting for 542 patients. For open-set sentence scores, 10 studies representing 240 patients showed an overall estimated improvement of 44.6% (95% CI: 38.0-51.2%). In terms of quality of life, studies generally showed improvement when looking at specific emotional, social, or hearing-specific domains, but not in global measures. Nonuser rates ranged from 0 to 9.5%.

Conclusion: Despite performance that is generally poorer than what is generally seen in "traditional" candidates, prelingually-deafened, late-implanted (PL-LI) CI users can experience benefit in terms of both QOL and audiometric scores. The wide range of performance that is reported in the literature highlights the importance of careful patient counseling.
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http://dx.doi.org/10.1097/MAO.0000000000002555DOI Listing
April 2020

The Role of Obesity in Iatrogenic Encephaloceles of the Temporal Bone.

Otol Neurotol 2020 03;41(3):e342-e348

Houston Methodist ENT Specialists, Houston Methodist Hospital, Houston, Texas.

Objective: Acquired encephaloceles of the temporal bone may be traumatic, spontaneous, or from chronic ear disease or previous surgery. Iatrogenic encephaloceles arise in the setting of previous mastoidectomy and traditionally involve both bony dehiscence and dural injury. We aim to classify and analyze the pathogenesis, clinical presentation, and treatment options for patients with iatrogenic tegmen defects and encephaloceles of the temporal bone.

Study Design: Retrospective chart review.

Setting: Single tertiary academic center.

Patients: Subjects with iatrogenic tegmen defects and encephaloceles of the temporal bone were included.

Intervention: Patient demographics, history, symptoms, radiographic data, intraoperative findings, management, follow-up, and outcomes were recorded.

Outcome Measures: Primary outcome measures included patient characteristics, time from primary otologic surgery to surgical repair, location of the defect, and management strategy including surgical approach, methods, and follow-up.

Results: Iatrogenic tegmen injuries or encephaloceles were identified in 18 patients and divided into intentional or unintentional. The latter group presented immediately, early, or late, as determined by intraoperative identification or from delayed symptoms. Eleven patients presented late with previously unrecognized encephaloceles. Compared with patients presenting with incidentally noted tegmen dehiscence during revision mastoidectomy, a significant proportion of patients with late encephaloceles had BMI ≥30 kg/m (p = 0.03).

Conclusion: The majority of iatrogenic encephaloceles are unintentional, unrecognized, and may present many years after primary surgery. Similar to spontaneous encephaloceles of the temporal bone, obesity is associated with iatrogenic encephaloceles, although the rate of progression and timing is unclear. We recommend primary repair of any unintentional tegmen defect encountered during mastoid surgery, especially in obese patients.
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http://dx.doi.org/10.1097/MAO.0000000000002527DOI Listing
March 2020

Microvascular free tissue transfer and cochlear implants: A case series and literature review.

Laryngoscope 2020 06 25;130(6):1552-1557. Epub 2019 Oct 25.

Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas, U.S.A.

Objective: The use of microvascular free tissue transfer (MVFTT) for defects of the scalp and lateral temporal bone in patients with cochlear implants (CI) is uncommon. Herein, we report our experience with the utility, indications, and outcomes for MVFTT in patients with cochlear implants.

Methods: A retrospective review of patients at our institution from September 2016 to December 2017 identified subjects with coexistent cochlear implant and ipsilateral MVFTT of the lateral temporal bone or scalp. Information including demographics, indication for MVFTT, timing of CI and MVFTT, donor site, and previous radiation to the head and neck was collected. To assess the current literature on MVFTT in CI patients, a MEDLINE search was performed using key search terms.

Results: Two patients with cochlear implants and MVFTT of the ipsilateral temporal bone or scalp were identified. One patient underwent MVFTT for advanced device extrusion with stable audiometric parameters rather than locoregional reconstruction or device explantation. The second patient had primary cochlear implantation at oncologic lateral temporal bone resection (LTBR) and MVFTT for locally advanced squamous cell carcinoma and concurrent profound sensorineural hearing loss (SNHL). A literature review identifies MVFTT as an option for advanced device extrusion, treatment of osteoradionecrosis, and reconstruction after primary oncologic surgery.

Conclusion: MVFTT is an important reconstructive tool for patients with functional, exposed cochlear implants. Cochlear implantation for severe to profound SNHL should be considered at the time of primary oncologic surgery and MVFTT of the lateral temporal bone or scalp.

Level Of Evidence: IV Laryngoscope, 130:1552-1557, 2020.
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http://dx.doi.org/10.1002/lary.28300DOI Listing
June 2020

A Start Codon Variant in Underlies Symphalangism and Ossicular Chain Malformations Affecting Both the Incus and the Stapes.

Case Rep Genet 2019 22;2019:2836263. Epub 2019 Jul 22.

Center for Statistical Genetics, Department of Neurology, Gertrude H. Sergievsky Center, Columbia University Medical Center, New York, NY, USA.

We performed exome sequencing to evaluate the underlying molecular cause of a patient with bilateral conductive hearing loss due to multiple ossicular abnormalities as well as symphalangism of the fifth digits. This leads to the identification of a novel heterozygous start codon variant in the gene (c.2T>C:p.Met1?) that hinders normal translation of the noggin protein. Variants in lead to a spectrum of otologic, digit, and joint abnormalities, a combination suggested to be referred to as -related-symphalangism spectrum disorder (-SSD). Conductive hearing loss from such variants may stem from stapes footplate ankylosis, fixation of the malleoincudal joint, or fixation of the incus short process. In this case, the constellation of both stapes and incus fixation, an exceptionally tall stapes suprastructure, thickened long process of the incus, and enlarged incus body was encountered, leading to distinct challenges during otologic surgery to improve hearing thresholds. This case highlights multiple abnormalities to the ossicular chain in a patient with a start codon variant in . We provide detailed imaging data on these malformations as well as surgical considerations and outcomes.
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http://dx.doi.org/10.1155/2019/2836263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6679842PMC
July 2019

Isolated Acute Vestibular Loss After Embolization of Arteriovenous Malformation for Pulsatile Tinnitus.

Otol Neurotol 2019 08;40(7):e753-e754

Department of Otolaryngology, Houston Methodist Hospital, Houston, Texas.

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http://dx.doi.org/10.1097/MAO.0000000000002300DOI Listing
August 2019

Facial Nerve Trauma: A Call for Minimum Reporting Guidelines.

Otol Neurotol 2018 04;39(4):494-498

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas Department of Otorhinolaryngology, Mayo Clinic School of Medicine, Rochester, Minnesota Department of Neurosurgery, Mayo Clinic School of Medicine, Rochester, Minnesota Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas Department of Neurosurgery, Baylor College of Medicine, Houston, Texas Division of Otolaryngology, Department of Surgery, Texas Children's Hospital, Houston, Texas.

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http://dx.doi.org/10.1097/MAO.0000000000001754DOI Listing
April 2018

Imaging of labyrinthine fistula after repair with bone pate.

Authors:
Jeffrey T Vrabec

Laryngoscope 2018 Jul 2;128(7):1643-1648. Epub 2017 Jun 2.

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, and Houston Methodist Hospital, Houston, Texas, U.S.A.

Objectives/hypothesis: To demonstrate imaging of labyrinthine fistula due to cholesteatoma and illustrate outcome following repair with bone pate.

Study Design: Retrospective review.

Methods: Patients with labyrinthine fistula due to cholesteatoma were identified, and pre- and postoperative imaging was assessed. Method of repair was recorded. The fistula site was examined for patency and bone density after repair.

Results: Twenty-nine fistulae were seen in a cohort of 375 cholesteatoma cases (8%). Preoperative computed tomography (CT) imaging for fistula detection showed sensitivity of 96% and specificity of 90%. The lateral semicircular canal was the most common site of dehiscence occurring in all cases. Facial nerve dehiscence is observed in 91% of fistula cases and stapes erosion in 69%. Severe hearing loss was present preoperatively in four (14%) cases. Bone pate was used to repair the fistula with CT-documented restoration of the otic capsule in 11/13 cases with postoperative imaging. When bone pate was not used, the otic capsule defect persisted.

Conclusions: Spontaneous repair of the otic capsule following treatment of cholesteatoma is expected to be an uncommon occurrence. Repair of the fistula with bone can result in long-lasting restoration of the otic capsule.

Level Of Evidence: 4. Laryngoscope, 128:1643-1648, 2018.
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http://dx.doi.org/10.1002/lary.26709DOI Listing
July 2018

Diagnostic Criteria for Detection of Vestibular Schwannomas in the VA Population.

Otol Neurotol 2016 12;37(10):1510-1515

*Bobby R. Alford Department of Otolaryngology, Baylor College of Medicine, Houston, Texas†Department of Otolaryngology-Head and Neck Surgery‡Department of Epidemiology, Gillings School of Global Public Health, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

Objective: To investigate the prevalence of vestibular schwannoma (VS) and asymmetric sensorineural hearing loss in the Veterans Administration hospital population and analyze a more efficient method of diagnosing VS in a population with significant noise exposure.

Study Design: Retrospective review of South Central (VISN 16) Veterans Administration hospitals.

Methods: Record query for ICD-9 codes for asymmetric sensorineural hearing loss or VS between 1999 and 2012. Patient demographics, signs and symptoms at presentation, audiogram and imaging data, and management data were collected and analyzed. Audiograms from tumor patients were compared with controls matched for age, sex, combat experience, and medical comorbidity (2:1 control to case ratio).

Results: The prevalence of VS was 1 per 1,145 patients in this population, with average age at diagnosis of 62. Patients with VS presented more commonly with unilateral tinnitus, rollover, and absent acoustic reflexes when compared with matched controls, but positive predictive value was low. Published criteria for defining hearing asymmetry showed variable sensitivity (51-89%) and low specificity (0-42%) for the detection of VS in this population. Criteria meeting the definitions of significant asymmetry with specificity for VS of 80% or greater were as follows: >15 dB threshold difference at 3 kHz and unilateral tinnitus, ≥45 dB threshold difference at 3 kHz regardless of tinnitus, or when the word recognition score difference was ≥80%. With serial audiograms 2.5 years apart or greater, a ≥10 dB threshold increase at any frequency between 0.5 and 4 kHz had a 100% sensitivity for tumor and a ≥10 dB increase at 3 kHz had a specificity of 84%. The majority of patients were observed, whereas only 30% had surgery. Patients who were observed were older than those treated with surgery or radiation (p <0.001).

Conclusion: Typical audiometric screening criteria should be modified in the veteran population to improve cost efficiency of diagnosis. Observation is the primary management strategy in the veteran population because of age.
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http://dx.doi.org/10.1097/MAO.0000000000001251DOI Listing
December 2016

Iatrogenic cholesteatoma arising from the vascular strip.

Laryngoscope 2017 03 16;127(3):698-701. Epub 2016 Jun 16.

Department of Otorhinolaryngology, Mayo Clinic School of Medicine, Rochester, Minnesota, U.S.A.

Objectives/hypothesis: To highlight the phenomenon of cholesteatoma arising from the vascular strip following tympanoplasty and tympanomastoidectomy.

Study Design: Multi-institutional retrospective chart review.

Methods: Consecutive adult and pediatric patients evaluated between 2000 and 2015 with acquired cholesteatoma arising from the skin of a prior vascular strip were identified. Patients with evidence of residual or recurrent cholesteatoma elsewhere in the middle ear, mastoid, or ear canal were excluded.

Results: Seventeen cases (71% female, 53% right-sided) were identified, and the mean age at presentation was 39.2 years. Patients presented on average 12.9 years following prior otologic surgery, which was most commonly tympanoplasty without mastoidectomy (59%). However, younger patients presented with symptoms sooner following prior surgery (r = 0.61, r = 0.37, P = 0.0095). The most common presenting symptom was otorrhea (76%). All patients were found to have mastoid cholesteatoma with bony erosion, and three patients exhibited additional tegmen or posterior fossa bone defects. The middle ear was not involved with cholesteatoma in any case. A canal wall down procedure was performed due to extensive bony canal erosion in 29% of cases.

Conclusion: The vascular strip is an uncommon source of iatrogenic cholesteatoma that can present years following an otherwise uncomplicated otologic surgery. The findings presented herein highlight the importance of careful vascular strip orientation at the conclusion of otologic surgery.

Level Of Evidence: 4. Laryngoscope, 127:698-701, 2017.
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http://dx.doi.org/10.1002/lary.26093DOI Listing
March 2017

Is chronic suppurative otitis media a neglected tropical disease?

PLoS Negl Trop Dis 2015 Mar 26;9(3):e0003485. Epub 2015 Mar 26.

Baylor College of Medicine, Department of Otolaryngology-Head and Neck Surgery, Houston, Texas, United States of America.

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http://dx.doi.org/10.1371/journal.pntd.0003485DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4374690PMC
March 2015

Early practice: neurotology.

Otolaryngol Clin North Am 2015 Apr 31;48(2):257-62. Epub 2015 Jan 31.

Department of Otolaryngology - Head and Neck Surgery, Baylor College of Medicine, Houston, TX, USA.

Besides technical and surgical proficiency, some of the most important skills for a young Neurotologist to refine include communication and critical thinking abilities. This Early Practice article provides perspectives on common challenges and career development from a current Neurotology fellow and his mentor.
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http://dx.doi.org/10.1016/j.otc.2014.12.001DOI Listing
April 2015

Workforce analysis of neurotologists in the United States.

Authors:
Jeffrey T Vrabec

Otol Neurotol 2013 Jun;34(4):755-61

Bobby R Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA.

Objective: To analyze current ratio of neurotologists to the population and examine regional variation.

Study Design: Population survey examining hospital referral regions (HRRs) as defined in the Dartmouth Atlas of Health Care Project and location of members of the American Neurotology Society.

Main Outcome Measure: Ratio of neurotologists to the population within designated HRRs.

Results: The national estimate of practicing neurotologists per population in 2006 was 1:922664 persons. The ratio is not uniform across HRRs, indicating that referral patterns for neurotologic care are wider than the care for indicators used in designating HRRs. Distribution of specialists is closely correlated with population. Estimated procedures per population are stable or declining for lateral cranial base surgery, implying current or impending oversupply of neurotologists.

Conclusion: The number of specialists in Neurotology likely exceeds projected needs. The current supply of trainees predicts this trend will continue for decades. Development and maintenance of lateral cranial base surgical skills will be difficult for some practitioners.
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http://dx.doi.org/10.1097/MAO.0b013e318280492bDOI Listing
June 2013

Audiology in the sudden hearing loss clinical trial.

Otol Neurotol 2012 Aug;33(6):907-11

Department of Audiology, Massachusetts Eye and Ear Infirmary, USA.

Objective: To report the pretreatment and posttreatment population characteristics and the overall stability of the audiologic outcomes found during the Sudden Hearing Loss Clinical Trial (ClinicalTrials.gov: Identifier NCT00097448).

Study Design: Multicenter, prospective randomized noninferiority trial of oral versus intratympanic (IT) steroid treatment of sudden sensorineural hearing loss (SSNHL).

Setting: Fifteen academically based otology practices.

Patients: Two hundred fifty patients with unilateral SSNHL presenting within 14 days of onset with 50 dBHL or greater pure tone average hearing threshold in the affected ear.

Intervention: Either 60 mg/d oral prednisone for 14 days with a 5-day taper (121 patients) or 4 IT doses for 14 days of 40 mg/ml methylprednisolone (129 patients).

Main Outcome Measure: Primary end point was change in hearing [dB PTA] at 2 months after treatment. Noninferiority was defined as less than 10 dB difference in hearing outcome between treatments. In this article, pretreatment and posttreatment hearing findings will be reported in detail.

Results: A general (and stable) effect of treatment and a specific effect of greater improvement at low frequencies were found in both treatment groups.

Conclusion: Hearing improvements are stable, and a significantly greater improvement occurs with lower frequency after either oral or IT steroid treatment of SSNHL.
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http://dx.doi.org/10.1097/MAO.0b013e31825d9a44DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3400088PMC
August 2012

Chondroblastoma-like chondroma of soft tissue: report of the first case in the base of skull.

Ann Diagn Pathol 2013 Jun 27;17(3):298-301. Epub 2012 Jun 27.

Department of Pathology, Northwestern University, Chicago, IL, USA.

Chondroblastoma-like chondroma (CLC) of soft tissue is a rare benign neoplasm that usually involves the soft tissues of the hand. This report describes the first case of CLC of soft tissue arising in the base of the skull. A 33-year-old man was seen with a slow growing mass in the right parotid region of his face. The noncontrast computed tomographic scans showed an 8.5-cm mass with calcifications involving the right masticator space and extending through the bone into the middle cranial fossa. The radiologic differential diagnosis included osteosarcoma, leiomyosarcoma, chondrosarcoma, and giant cell tumor. During surgery, the large lateral skull base tumor appeared to involve the middle and infratemporal fossae and eroded the surrounding bone. Although the tumor was removed piecemeal, total excision was performed. On microscopic examination, the tumor displayed lobules of mature hyaline cartilage with numerous chondroblasts, coarse calcifications including chicken wire calcifications, and scattered osteoclasts. No atypia, mitoses, necrosis, or osteoid formation was seen. The tumor was diagnosed as chondroma with chondroblastoma features of the soft tissue. His postoperative clinical course was uneventful; however, after 7 months, he had a local recurrence identified on follow-up magnetic resonance imaging. He underwent repeat surgical excision of the tumor, which showed similar histology as the previous excision. This large skull based tumor eroding the bone, which clinically and radiologically mimicked a malignant process, was an unusual presentation of a benign cartilaginous neoplasm. Pathologists should be aware that CLC may occur in the base of the skull and this lesion should be differentiated from the other benign or malignant tumors arising in this area. These lesions have a potential for local recurrence; hence, a close follow-up is recommended.
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http://dx.doi.org/10.1016/j.anndiagpath.2012.05.005DOI Listing
June 2013

Oral vs intratympanic corticosteroid therapy for idiopathic sudden sensorineural hearing loss: a randomized trial.

JAMA 2011 May;305(20):2071-9

Department of Otology and Laryngology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114, USA.

Context: Idiopathic sudden sensorineural hearing loss has been treated with oral corticosteroids for more than 30 years. Recently, many patients' symptoms have been managed with intratympanic steroid therapy. No satisfactory comparative effectiveness study to support this practice exists.

Objective: To compare the effectiveness of oral vs intratympanic steroid to treat sudden sensorineural hearing loss.

Design, Setting, And Patients: Prospective, randomized, noninferiority trial involving 250 patients with unilateral sensorineural hearing loss presenting within 14 days of onset of 50 dB or higher of pure tone average (PTA) hearing threshold. The study was conducted from December 2004 through October 2009 at 16 academic community-based otology practices. Participants were followed up for 6 months.

Intervention: One hundred twenty-one patients received either 60 mg/d of oral prednisone for 14 days with a 5-day taper and 129 patients received 4 doses over 14 days of 40 mg/mL of methylprednisolone injected into the middle ear.

Main Outcome Measures: Primary end point was change in hearing at 2 months after treatment. Noninferiority was defined as less than a 10-dB difference in hearing outcome between treatments.

Results: In the oral prednisone group, PTA improved by 30.7 dB compared with a 28.7-dB improvement in the intratympanic treatment group. Mean pure tone average at 2 months was 56.0 for the oral steroid treatment group and 57.6 dB for the intratympanic treatment group. Recovery of hearing on oral treatment at 2 months by intention-to-treat analysis was 2.0 dB greater than intratympanic treatment (95.21% upper confidence interval, 6.6 dB). Per-protocol analysis confirmed the intention-to-treat result. Thus, the hypothesis of inferiority of intratympanic methylprednisolone to oral prednisone for primary treatment of sudden sensorineural hearing loss was rejected.

Conclusion: Among patients with idiopathic sudden sensorineural hearing loss, hearing level 2 months after treatment showed that intratympanic treatment was not inferior to oral prednisone treatment.

Trial Registration: clinicaltrials.gov Identifier: NCT00097448.
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http://dx.doi.org/10.1001/jama.2011.679DOI Listing
May 2011

Middle ear volume as an adjunct measure in congenital aural atresia.

Int J Pediatr Otorhinolaryngol 2011 Jul 12;75(7):910-4. Epub 2011 May 12.

Bobby R. Alford Department of Otolaryngology - Head and Neck Surgery, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.

Objective: To examine middle ear volume in patients with aural atresia and investigate the role of middle ear volume as an adjunct measure in determining surgical candidacy.

Methods: We performed a retrospective review of children with aural atresia in a tertiary academic pediatric otolaryngology practice. High resolution multiplanar CT scans of the temporal bones were analyzed for middle ear volume and staged according to existing clinical grading scales. Atretic ears were compared to the nonatretic ears of the same patient as well as to ears of a control population.

Results: The average age of patients at the time CT was performed was 4.7 years (range <0.1-13.8 years). The average middle ear volume of the atretic ears was 0.34 cc compared to an average of 0.51 cc for the nonatretic ears. The mean ratio of the atretic to nonatretic volume was 0.67. In patients who underwent serial scans, no statistically significant difference in rates of growth existed between atretic and nonatretic ears. Finally, measures of middle ear volume correlated well with clinical grading scales.

Conclusions: Both middle ear volume and the ratio of the atretic volume to nonatretic volume serve as useful adjunct measurements in determining surgical candidacy. The practitioner may be better able to assess surgical candidacy by supplementing classic atresia classification systems with middle ear volume measurements.
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http://dx.doi.org/10.1016/j.ijporl.2011.04.004DOI Listing
July 2011

Inner ear anomalies in congenital aural atresia.

Otol Neurotol 2010 Dec;31(9):1421-6

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA.

Objectives: To define the prevalence of inner ear anomalies in aural atresia patients and to recognize patterns of developmental anomalies in aural atresia patients.

Study Design: Retrospective review.

Setting: Academic medical center.

Intervention: Physical exam, audiometry, and temporal bone CT in selected patients.

Patients: Pediatric patients with aural atresia.

Main Outcome Measure: Prevalence of inner ear anomalies and coexisting facial paralysis or sensorineural hearing loss.

Results: In this series of 118 patients with aural atresia, associated facial palsy was seen in 13%, whereas inner ear anomalies were present in 22%, including all patients with facial palsy. Interestingly, the inner ear anomalies often did not display a significant sensorineural hearing loss. Bilateral inner ear anomalies were frequently encountered despite unilateral atresia. Most anomalies involved the semicircular canals including several uncommon variants of posterior semicircular canal anatomy.

Conclusion: Inner ear anomalies are common in the presence of aural atresia, especially when there is concurrent congenital facial palsy. The presence of inner ear anomalies should be recognized as a common feature of craniofacial microsomia.
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December 2010

Genetic investigations of Meniere's disease.

Authors:
Jeffrey T Vrabec

Otolaryngol Clin North Am 2010 Oct;43(5):1121-32

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery Baylor College of Medicine, 6550 Fannin Street, SM1727, Houston, TX 77030, USA.

Meniere's disease remains a disorder of unknown origin despite the collective efforts to determine the pathogenesis, although experts have long recognized that disease development likely has some heritable component. Although genetic studies of Meniere's disease have been inconclusive, increasing knowledge of human genetic structure and mutation and investigative techniques have potential to further understanding of this disorder.
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http://dx.doi.org/10.1016/j.otc.2010.05.010DOI Listing
October 2010

Characteristics of malfunctioning channels in pediatric cochlear implants.

Laryngoscope 2010 Feb;120(2):399-404

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Hearing Center at Texas Children's Hospital, Houston, Texas 77030, USA.

Objectives/hypothesis: To examine the characteristics of pediatric cochlear implant channel malfunction preceding device failure.

Study Design: : Retrospective review.

Methods: All pediatric patients who underwent cochlear implantation at a tertiary academic medical center were reviewed regarding device type, reason for replacement, time to replacement, and timing and pattern of channel faults in failed versus nonfailed devices.

Results: Between 1993 and 2008, 264 pediatric cochlear implantations were performed. With an average 894-day follow-up, the replacement rate was 9.5% (25/264). Reasons for replacement were device failure (6.4%), medical/surgical failure (2.3%), and obsolescence (0.8%). Replacement rates were comparable among Advanced Bionics (13.3%), Cochlear Corporation (6.3%), and MED-EL (10.3%) devices. Fifty-two cochlear implants developed at least one channel fault, and 13 eventually progressed to failure requiring replacement. MED-EL devices comprised 12 of these 13 failures. At the 12-month follow-up interval, one, three, and five channel faults predicted 40%, 75%, and 100% probabilities of eventual electrode failure, respectively. Channels destined to fail demonstrated small, yet statistically significant, impedance elevations 12 months before failure and large elevations 3 months before failure.

Conclusions: Replacement of cochlear implants in pediatric patients is common and is due to device malfunction about one half of the time. Earlier initial channel fault, earlier subsequent channel faults, adjacent channel faults, and a greater total number of channel faults were associated with the need for replacement surgery. Elevations in a channel's impedance should raise the concern for an impending failure. These predictors can help the cochlear implant team when considering surgery to replace the device.
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http://dx.doi.org/10.1002/lary.20668DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3623674PMC
February 2010

Auditory performance after cochlear implantation in late septuagenarians and octogenarians.

Otol Neurotol 2009 Oct;30(7):916-20

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA.

Objective: Previous studies have demonstrated consistent benefit in older adults undergoing cochlear implantation as compared with younger control groups, with age category thresholds between 60 and 70 years. The objective of this study is to report auditory performance in implant recipients older than 75 years, a cohort for which few data have been reported.

Study Design: Retrospective chart review.

Setting: Academic cochlear implant program in a tertiary-care hospital.

Patients: : Twenty-eight cochlear implant recipients were subdivided into implant users older than 80 years (Group 1) and recipients currently older than 75 years (Group 2).

Intervention: Cochlear implantation.

Main Outcome Measures: Open-set speech perception scores.

Methods: : Postoperative open-set speech perception scores were compared with preoperative scores in the best-aided condition. Criteria were developed to define situations where the implant was considered to be nonbeneficial or less beneficial than amplification, and those data were then subjected to Kaplan-Meier analysis.

Results: Group 1 included 13 patients with mean age of 80.7 years at the time of implantation. Group 2 included 15 patients with a mean age of 71.6 years. Scores were significantly better postoperatively at 6 months (p < 0.01) for Group 2 and at 12 months (p < 0.01) for both Groups 1 and 2. Kaplan-Meier curves were constructed for both groups.

Conclusion: Cochlear implantation in patients older than 75 years is beneficial, and Kaplan-Meier analysis demonstrates that the clinical benefit is durable over time. Patients older than 80 years obtain similar benefit, although auditory performance was less robust.
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http://dx.doi.org/10.1097/MAO.0b013e3181b4e594DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3607509PMC
October 2009

Advanced pediatric mastoiditis with and without intracranial complications.

Laryngoscope 2009 Aug;119(8):1610-5

Bobby R. Alford Department of Otolaryngology, Texas Children's Hospital, Houston, USA.

Objectives/hypothesis: Recently, several groups have noticed an increase in cases of advanced pediatric mastoiditis and intracranial complications. The objective of this study was to review the bacteriology of advanced mastoiditis in pediatric patients, with the hypothesis that a difference in bacteriology might explain the development of an intracranial complication.

Study Design: Retrospective chart review.

Methods: All pediatric patients with coalescent mastoiditis requiring surgery treated at a tertiary care children's hospital between 2002 and 2007 were reviewed. Every patient included was treated either with mastoidectomy alone (for coalescent mastoiditis without an intracranial complication) or with transtemporal craniotomy (for coalescent mastoiditis with an intracranial complication). All patients had surgical specimens sent for pathology, Gram stain, and aerobic and anaerobic cultures.

Results: One hundred eight pediatric patients with coalescent mastoiditis were identified: 58 (53%) presented with coalescent mastoiditis alone, 17 (16%) presented with coalescent mastoiditis and an intracranial complication, and 33 (31%) were excluded because they were treated with myringotomy and tubes alone, had incomplete data, or had an unclear diagnosis. Streptococcus pneumoniae was the most commonly cultured organism in patients with and without intracranial complications. Anaerobic isolates were present in 29.4% of patients with intracranial complications and 5.7% of patients without intracranial complications (P = .015).

Conclusions: Nearly a quarter of pediatric patients with coalescent mastoiditis presented with a simultaneous intracranial complication. There was an increased incidence of anaerobic organisms in patients with intracranial complications compared to those without, indicating the importance of culture and antibiotic coverage appropriate for anaerobes. This series demonstrates the role of aggressive surgical management and close collaboration with the infectious disease service for long-term intravenous antibiotic therapy in treating pediatric patients with advanced mastoiditis.
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http://dx.doi.org/10.1002/lary.20259DOI Listing
August 2009

Facial Nerve Grading System 2.0.

Otolaryngol Head Neck Surg 2009 Apr;140(4):445-50

Bobby R Alford Department of Otolaryngology, Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA.

Objective: To present an updated version of the original Facial Nerve Grading Scale (FNGS), commonly referred to as the House-Brackmann scale.

Study Design: Controlled trial of grading systems using a series of 21 videos of individuals with varying degrees of facial paralysis.

Results: The intraobserver and interobserver agreement was high among the original and revised scales. Nominal improvement is seen in percentage of exact agreement of grade and reduction of instances of examiners differing by more then one grade when using FNGS 2.0. FNGS 2.0 also offers improved agreement in differentiating between grades 3 and 4.

Conclusion: FNGS 2.0 incorporates regional scoring of facial movement, providing additional information while maintaining agreement comparable to the original scale. Ambiguities regarding use of the grading scale are addressed.
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http://dx.doi.org/10.1016/j.otohns.2008.12.031DOI Listing
April 2009

Intra-operative monitoring of cochlear function during cochlear implantation.

Cochlear Implants Int 2009 Mar;10(1):1-18

The Hearing Center at Texas Children's Hospital, Houston, Texas, USA.

The objective of this study was to determine if intra-operative auditory monitoring is feasible during cochlear implantation and whether this can be used as feedback to the surgeon to improve the preservation of residual hearing. This prospective non-randomised study was set in a paediatric tertiary referral hospital. Thirty eight consecutive paediatric patients undergoing cochlear implantation who had measurable auditory thresholds pre-operatively were divided into two cohorts. The unmonitored cohort included the first 22 patients and the monitored cohort included the last 16 patients. The main outcome measure(s) were pre-operative, intra-operative and more than one month post-operative average auditory thresholds at 500, 1000 and 2000 Hz measured using auditory steady-state response audiometry. The average pre-operative thresholds were 103.5 dB HL and 99.7 dB HL in the unmonitored and monitored cohorts, respectively. These were not statistically different (p > 0.3). In the monitored cohort, we measured auditory thresholds to assess cochlear function at multiple time points during the operation. Compared to baseline, thresholds were increased 0.7 dB after drilling the mastoidectomy and well, 0.2 dB after opening the cochlea and 4.6 dB after inserting the electrode array. One month post-operatively, the average thresholds were 114.0 dB HL in the unmonitored cohort but only 98.8 dB HL in the monitored cohort (p < 0.001). Both the use of intra-operative auditory monitoring and higher pre-operative thresholds were associated with improved preservation of residual hearing (p
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http://dx.doi.org/10.1002/cii.372DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597106PMC
March 2009

Sequence variants in host cell factor C1 are associated with Ménière's disease.

Otol Neurotol 2008 Jun;29(4):561-6

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas, U.S.A.

Hypothesis: There is a genetic basis for the development of Ménière's (MD) disease.

Background: The cause of MD is unknown, although many potential theories have been proposed. A genetic basis for the disease is suggested by greater prevalence in Caucasians and familial cases that display an autosomal dominant pattern of inheritance.

Methods/design: Case-control association study of selected candidate genes among patients with MD and selected control individuals.

Results: Several single-nucleotide polymorphisms (SNPs) within the host cell factor C1 (HCFCI) gene displayed a significant increase in prevalence of the major allele in subjects with MD disease. The most individually significant SNP is rs2266886. The minor allele at this site displays an odds ratio of 0.26 (95% confidence intervals, 0.010-0.65; p = 0.003) for disease development.

Conclusion: The minor allele at each SNP site was significantly more common in controls, suggesting that individuals bearing these alleles are at reduced risk of developing MD. The functional consequences of the SNPs in host cell factor C1 are unknown. A viable hypothesis for disease development is presented based on the known interaction between HCFC1 and the herpes simplex viral protein VP16.
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http://dx.doi.org/10.1097/MAO.0b013e318168d23bDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2757044PMC
June 2008

Bell's palsy and pregnancy.

Otolaryngol Head Neck Surg 2007 Dec;137(6):858-61

Baylor College of Medicine, Department of Otolaryngology, Houston, TX 77030, USA.

Objective: To describe the epidemiology, pathophysiology, presentation, treatment, and prognosis of Bell's palsy (BP) in pregnancy.

Results: The incidence of BP in pregnant women is not significantly greater than expected compared to all women of childbearing age. There is a high incidence of cases in the third trimester and corresponding low incidence during early pregnancy.

Conclusion: There is no conclusive evidence that the etiology of BP in pregnancy is different than in nonpregnant patients. Altered susceptibility to herpes simplex viral reactivation during pregnancy is the most likely explanation for concentration of cases in the third trimester. Outcome may be poorer in pregnant patients, though historically, treatment is often withheld from these patients.

Significance: Management of BP in pregnancy can mirror that of nonpregnant individuals with the exception of first-trimester cases.
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http://dx.doi.org/10.1016/j.otohns.2007.09.009DOI Listing
December 2007

Survey of Ménière's disease in a subspecialty referral practice.

Otolaryngol Head Neck Surg 2007 Aug;137(2):213-7

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA.

Objectives: To define the prevalence of definite Ménière's disease (MD) among patients presenting with characteristic symptoms and examine the utility of published diagnostic guidelines.

Study Design And Setting: Retrospective review in an academic referral practice.

Results: The prevalence of definite MD in these 295 individuals was 64%. The next-largest group (23%) consisted of patients with only cochlear symptoms. Those initially classified as probable are usually reclassified as definite with extended follow-up. Of those with definite MD, the mean duration of disease at last follow-up was 7.6 years, 56% were female, 19% had bilateral disease, and 34% required surgical management for vertigo.

Conclusions: The 1995 AAO-HNS guidelines are useful for classification of MD according to certainty of diagnosis and severity of disease, though some modifications could be considered.

Significance: Application of consistent diagnostic criteria is essential for epidemiological, genetic, or outcomes studies of Ménière's disease.
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http://dx.doi.org/10.1016/j.otohns.2007.02.033DOI Listing
August 2007

Hydrocodone use and sensorineural hearing loss.

Pain Physician 2007 May;10(3):467-72

Baylor College of Medicine, Houston, TX, USA.

Background: The hydrocodone/acetaminophen combination is one of the most commonly used analgesic preparations. Isolated incidences of suspected association between hydrocodone abuse and rapidly progressive hearing loss have been reported. In this study, we describe the clinical characteristics of 5 patients presenting with progressive hearing loss and a history of hydrocodone use.

Methods: Patients presenting with rapidly progressive bilateral hearing loss who had a documented history of hydrocodone use were selected for the study. The presentation, audiologic findings, associated comorbidities, and treatment outcomes were reviewed.

Results: All patients displayed rapidly progressive sensorineural hearing loss without vestibular symptoms. Hearing loss was asymmetric in 3 patients at initial presentation, but progressed to profound loss, usually within months. Steroid treatment has no effect on the progression of the hearing loss. The admitted quantity of hydrocodone consumed ranged from 10 to 300 mg per day. Hepatitis C was the most common comorbidity, present in 60% of the patients. All patients underwent cochlear implantation with satisfactory results.

Conclusions: The chronic use of hydrocodone can be associated with progressive sensorineural hearing loss. Successful auditory rehabilitation can be achieved with cochlear implantation. Genetic polymorphisms of drug metabolizing enzymes as well as associated comorbidities such as hepatitis C infection may be significant in the development of hydrocodone ototoxicity, though additional investigations are necessary.
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May 2007
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