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1.
Cleft Palate Craniofac J ; 59(5): 652-658, 2022 05.
Article in English | MEDLINE | ID: mdl-34000844

ABSTRACT

OBJECTIVES: The objectives of this study were to: (1) determine the prevalence of otitis media with effusion in patients with nonsyndromic craniosynostosis; (2) determine the prevalence of hearing loss in patients with nonsyndromic craniosynostosis; and (3) identify potential patterns and outcomes in patients with nonsyndromic craniosynostosis. METHODS: A retrospective chart review was conducted at 2 academic institutions, St Christopher's Hospital for Children and SUNY Upstate Medical University, from January 2015 through August 2018, to identify patients having nonsyndromic craniosynostosis with a concurrent diagnosis of otitis media and/or hearing loss. The demographic data and categorical variables were analyzed using descriptive statistics and chi-square testing, respectively. RESULTS: In the entire cohort of patients (N = 113, age range 0-123 months), 36% had otitis media with effusion on either history, physical examination, tympanometry, and/or imaging. Half (50%) of patients with coronal synostosis had otitis media with effusion compared to sagittal (40.7%), metopic (26.3%), multiple (25%), and lambdoid (0%). However, these differences were not statistically significant (P = .190). Most patients had normal hearing (91%), while a minority had either conductive (7%) or sensorineural (2%) hearing loss. CONCLUSION: The presence of otitis media in our cohort of patients with nonsyndromic craniosynostosis appears to be at the upper limit of normal when compared to historical rates in normocephalic children. Synostosis subtype did not appear to predict the presence of otitis media. Only 9% of patients with nonsyndromic craniosynostosis were found to have a hearing loss.


Subject(s)
Craniosynostoses , Hearing Loss , Otitis Media with Effusion , Otitis Media , Acoustic Impedance Tests , Child , Child, Preschool , Craniosynostoses/complications , Craniosynostoses/epidemiology , Hearing Loss/epidemiology , Hearing Loss/etiology , Humans , Infant , Infant, Newborn , Otitis Media/complications , Otitis Media/epidemiology , Otitis Media with Effusion/diagnosis , Otitis Media with Effusion/epidemiology , Retrospective Studies
2.
Laryngoscope ; 132(3): 648-654, 2022 03.
Article in English | MEDLINE | ID: mdl-34599608

ABSTRACT

OBJECTIVES: To evaluate histologic changes in middle ear and eustachian tube (ET) mucosa of mice after exposure to tobacco or electronic cigarette (e-cigarette) smoke. To determine whether there were any mitigating effects of middle ear application of anti-IL-13 or the epidermal growth factor receptor antagonist AG1478 on noted changes within ET mucosa. STUDY DESIGN: Controlled animal study. METHODS: Fifty BALB/cJ mice were randomly assigned to one of five groups: A control group with no smoke exposure, two groups exposed to tobacco smoke, and two groups exposed to e-cigarette vapor. Within the exposed groups after 4 weeks of exposure, one ear was infiltrated with a saline hydrogel and the other ear with hydrogel of either Anti-IL-13 or AG1478. After four more weeks of exposure, the animals were euthanized and the ETs were evaluated for mucosal changes. RESULTS: Compared to control animals with no smoke exposure, there were significant decreases in the numbers of goblet cells within the ET mucosa of mice exposed to tobacco smoke and e-cigarette vapor. No significant differences in cilia, mucin, or squamous metaplasia were noted. Neither anti-IL-13 nor AG178 significantly altered goblet cell count in the ET mucosa of mice exposed to tobacco smoke; however, both agents significantly increased goblet cells within the ET mucosa of mice exposed to e-cigarette vapor. CONCLUSION: Short-term tobacco smoke and e-cigarette vapor significantly decrease goblet cell count in mouse ET mucosa. Middle ear application of both anti-IL-13 and AG1478 resulted in an increase in goblet cell count among mice exposed to e-cigarette vapor, but not to tobacco smoke. LEVEL OF EVIDENCE: NA Laryngoscope, 132:648-654, 2022.


Subject(s)
E-Cigarette Vapor/adverse effects , Eustachian Tube/drug effects , Mucous Membrane/drug effects , Tobacco Smoke Pollution/adverse effects , Animals , Electronic Nicotine Delivery Systems , Female , Goblet Cells/drug effects , Male , Mice , Mice, Inbred BALB C
3.
Laryngoscope Investig Otolaryngol ; 6(1): 145-149, 2021 Feb.
Article in English | MEDLINE | ID: mdl-33614943

ABSTRACT

OBJECTIVES: Climate variables are implied in the pathogenesis of certain otologic diseases, including benign paroxysmal positional vertigo (BPPV). Using internet search data obtained through Google Trends (GT), we explored the relationship between climate patterns and symptom search frequencies for BPPV. We hypothesized that increased latitude, as a proxy for decreased sunlight exposure, would lead to increase in BPPV symptom searches. METHODS: GT searches for symptoms related to BPPV were obtained for five U.S. cities of different latitudes via the Google Trends online interface. Comparisons were made using SPSS via ANOVA analysis. Figures were made using Microsoft Excel. RESULTS: Searches for BPPV-related symptoms increased with increasing latitude. BPPV-related symptoms did show seasonal variations, but not in predictable manners. CONCLUSIONS: GT may be a viable research tool when comparing geographical differences in searches, but may be less sensitive in detecting time dependent changes. We offer suggestions as to how big data tools may be altered for research purposes. LEVEL OF EVIDENCE: NA.

4.
Ear Nose Throat J ; 100(3_suppl): 286S-291S, 2021 Jun.
Article in English | MEDLINE | ID: mdl-32703012

ABSTRACT

OBJECTIVES: Present the case of a 67-year-old male with stage IV malignant melanoma who presented with uveitis and sensorineural hearing loss (SNHL) while on nivolumab and review the literature for likely etiologies. METHODS: A retrospective case review was conducted. The current literature was accessed to inquire about possible pathologic mechanisms and treatment options. RESULTS: A 67-year-old male with stage IV malignant melanoma was treated with nivolumab. During therapy, the patient presented with bilateral uveitis, vertigo, and bilateral moderate sloping to moderate-severe SNHL. After 4 cycles of nivolumab, restaging scans showed no evidence of disease. Nivolumab was discontinued. The patient was placed on a 3-week course of systemic high dose steroids and topical steroid eye drops. Both his uveitis and SNHL resolved after treatment. Nivolumab enhances the antitumor activity of T cells by inhibiting the programed death-1 receptor. While nivolumab has shown great promise in the treatment of many types of cancers, it has also been associated with many autoimmune side effects. We propose the etiology of this 67-year-old male's SNHL and uveitis are the result of an autoimmune process secondary to an augmented T cell response induced by nivolumab. CONCLUSION: While immunotherapeutic agents such as nivolumab have shown great promise in the treatment of cancer, one should maintain an awareness and caution of autoimmune side effects such as uveitis and SNHL.


Subject(s)
Antineoplastic Agents, Immunological/adverse effects , Hearing Loss, Bilateral/chemically induced , Hearing Loss, Sensorineural/chemically induced , Melanoma/drug therapy , Nivolumab/adverse effects , Ototoxicity/etiology , Aged , Humans , Male , Melanoma/pathology , Neoplasm Staging
5.
ACS Biomater Sci Eng ; 6(1): 727-738, 2020 01 13.
Article in English | MEDLINE | ID: mdl-33463199

ABSTRACT

Bacteria are well-known to form biofilms on biomaterials and implanted medical devices and cause serious infections that are incurable by conventional antibiotics. Consequently, such infections can lead to explantation and, in severe cases, amputation or even death. To address this unmet challenge, we developed a new method for noninvasive treatment of device-associated biofilm infections. We demonstrate that antibiotic tolerant biofilm cells of Pseudomonas aeruginosa and Staphylococcus aureus can be effectively killed by electromagnetically induced direct current generated wirelessly using a remote power source, which was further enhanced through synergy with conventional antibiotics. Electrochemical analyses attributed the cidal effects to DC-generated reactive oxygen species. The treatment conditions were found safe to the epithelial and fibroblast cell lines. On the basis of these findings, a prototype device was engineered and demonstrated for effective killing of biofilm cells using both ex vivo and in vivo models. With the capability to kill bacteria without using a directly connected power source, this platform technology has possible applications in noninvasive treatment of biofilm infections associated with cochlear, orthopedic, and other implanted medical devices.


Subject(s)
Biofilms , Electric Stimulation Therapy , Anti-Bacterial Agents/pharmacology , Pseudomonas aeruginosa , Staphylococcus aureus
7.
J Otol ; 13(2): 54-58, 2018 Jun.
Article in English | MEDLINE | ID: mdl-30559765

ABSTRACT

HYPOTHESIS: To determine the pharmacokinetics of sodium thiosulfate in the inner ear perilymph following middle ear application in Guinea pigs. BACKGROUND: Cisplatin chemotherapy is often associated with a dose-dependent high frequency sensorineural hearing loss. Sodium thiosulfate has been shown to reduce cisplatin-induced ototoxicity when given intravenously, but this may limit the tumoricidal effects of the chemotherapy. Recent animal studies looking at middle ear application of sodium thiosulfate have shown prevention of outer hair cell and hearing loss, but the perilymph pharmacokinetics have not yet been established. METHODS: Twenty Guinea pig ears were split into two groups and administered sodium thiosulfate to the middle ear at either a concentration of 250 mg/mL or 50 mg/mL for 30 min. Perilymph samples were then obtained serially through the round window over 6 h. Sodium thiosulfate concentrations were obtained using high-pressure liquid chromatography. RESULTS: The 250 mg/mL group had a maximum perilymph concentration of 7.27 mg/mL (±0.83) that decreased to 0.94 mg/mL (±0.03) over 6 h. The 50 mg/mL group had an initial concentration of 1.63 mg/mL (±0.17) and was undetectable after 1 h. The half-life of sodium thiosulfate within perilymph was 0.74 h. CONCLUSIONS: and Relevance: The results of this study show that sodium thiosulfate is capable of diffusing through round window and into the inner ear perilymph. Peak levels decline over several hours after exposure. This has a potential application as a localized therapy in the prevention of cisplatin induced ototoxicity.

8.
Int J Pediatr Otorhinolaryngol ; 115: 165-170, 2018 Dec.
Article in English | MEDLINE | ID: mdl-30368379

ABSTRACT

OBJECTIVE: Sociodemographic disparities of cochlear implantation in children have been reported. This study sought to determine if disparities in children receiving cochlear implants have narrowed, widened or remained constant. METHODS: Children 18 years or younger who underwent cochlear implantation from 1997 to 2012 were selected using the Kids' Inpatient Database. Demographic data included primary insurance payer, income quartile and race. The Cochran-Armitage test was used to determine if trends were significant. Prevalence rates of cochlear implantation by race were generated. A Poisson regression model was used to evaluate the rates of cochlear implantation within each racial group. RESULTS: The proportion of children receiving cochlear implants with private insurance decreased from 79.3% to 42.6% (p < .0001), whereas children with Medicaid increased from 17.4% to 35.2% (p < .0001). Proportion of implanted children from the lowest two income quartiles increased from 15.5% to 24.4% (p < .0001) and 10.3%-21.8% (p < .0035), respectively. Rates of implantation among children from income quartile four decreased from 50.9% to 35.3% (p < .0001). White children were implanted twice as often as Black or Hispanic children (p = .007 and p = .0012 respectively). Asian children were implanted more than twice as often as Black or Hispanic Children (p = .0154 and p = .0098 respectively). CONCLUSIONS: Income and insurance disparities have narrowed within the inpatient pediatric cochlear implantation cohort. Racial disparities still exist. White and Asian children are implanted at higher rates than Black or Hispanic children.


Subject(s)
Cochlear Implantation/trends , Cochlear Implants/trends , Healthcare Disparities/trends , Adolescent , Child , Child, Preschool , Databases, Factual , Female , Humans , Income , Infant , Insurance, Health/statistics & numerical data , Male , Poverty/statistics & numerical data , Prevalence , Racial Groups , Socioeconomic Factors , United States
9.
Ear Nose Throat J ; 97(7): 208-212, 2018 Jul.
Article in English | MEDLINE | ID: mdl-30036434

ABSTRACT

The study objective was to analyze Medicare payment data to otologists compared to otolaryngologists, using the publicly released Centers for Medicare and Medicaid Services dataset. Charges, payments, and common Current Procedural Terminology codes were obtained. Otology providers were selected from the roster of the American Otological Society. Descriptive statistics and unequal variance two-tailed t tests were used for comparisons between otologists (n = 147) and otolaryngologists (n = 8,318). The mean overall submitted charge was $204,851 per otology provider and was $211,209 per other otolaryngology providers (non-otologists) (p = 0.92). The mean payment to otologists was $56,191 (range: $297 to $555,274, standard deviation [SD] ±$68,540) and significantly lower (p = 0.005) than $77,275 to otolaryngologists (range: $94 to $2,123,900, SD ±$86,423). The mean submitted charge-to-payment ratio (fee multiplier) per otology provider was 3.87 (range 1.50 to 9.10, SD ±1.70), which was significantly higher (p < 0.0001) than the ratio for otolaryngologists (mean 2.91; range: 1.25 to 17.51, SD ±1.22). Office visit evaluation and management (E&M) codes made up the majority in terms of use and payments. Interestingly, allergy-based services comprised a substantial amount of repeat use among a small subset of otologists. Audiology services were billed by a similar percentage of otologists and other otolaryngologists (52%), but otologists received a significantly higher overall payment for these services.


Subject(s)
Insurance, Health, Reimbursement/statistics & numerical data , Medicare/economics , Otolaryngologists/economics , Otolaryngology/economics , Practice Patterns, Physicians'/economics , Centers for Medicare and Medicaid Services, U.S./statistics & numerical data , Humans , Medicare/statistics & numerical data , United States
10.
Front Cell Neurosci ; 11: 303, 2017.
Article in English | MEDLINE | ID: mdl-29033791

ABSTRACT

Cisplatin has been regarded as an effective and versatile chemotherapeutic agent for nearly 40 years. Though the associated dose-dependent ototoxicity is known, the cellular mechanisms by which cochleovestibular hair cell death occur are not well understood. We have previously shown that aminoglycoside ototoxicity is mediated in part by cytosolic protein synthesis inhibition. Despite a lack of molecular similarity, aminoglycosides were shown to elicit similar stress pathways to cisplatin. We therefore reasoned that there may be some role of protein synthesis inhibition in cisplatin ototoxicity. Employing a modification of the bioorthogonal noncanonical amino acid tagging (BONCAT) method, we evaluated the effects of cisplatin on cellular protein synthesis. We show that cisplatin inhibits cellular protein synthesis in organ of Corti explant cultures. Similar to what was found after gentamicin exposure, cisplatin activates both the c-Jun N-terminal kinase (JNK) and mammalian target of rapamycin (mTOR) pathways. In contrast to aminoglycosides, cisplatin also inhibits protein synthesis in all cochlear cell types. We further demonstrate that the multikinase inhibitor sorafenib completely prevents JNK activation, while providing only moderate hair cell protection. Simultaneous stimulation of cellular protein synthesis by insulin, however, significantly improved hair cell survival in culture. The presented data provides evidence for a potential role of protein synthesis inhibition in cisplatin-mediated ototoxicity.

11.
Otolaryngol Head Neck Surg ; 157(4): 676-682, 2017 10.
Article in English | MEDLINE | ID: mdl-28653563

ABSTRACT

Objective Describe longitudinal audiometric and otologic outcomes in patients with cleft palates. Study Design Case series with chart review. Setting Single academic medical center. Methods Charts of 564 patients with a diagnosis of cleft palate (59% syndromic etiology, 41% nonsyndromic) from 1998 to 2014 were reviewed. Patients without at least 1 audiometric follow-up were excluded from analysis. Patient demographics, surgeries, audiometric tests, and otologic data were recorded for 352 patients. Results Forty-five percent had isolated cleft palates, 34% had unilateral cleft lip and palate, and 21% had bilateral cleft lip and palate. Patients were followed for a mean of 50.3 months with a mean of 3.2 separate audiograms performed. Patients received a mean of 2.93 pressure equalization tubes. Increased number of pressure equalization tubes was not associated with incidence of cholesteatoma, which was identified in only 4 patients. Nine patients underwent eventual tympanoplasty with an 89% closure rate. Analysis of mean air-bone gap by cleft type did not reveal significant differences ( P = .08), but conductive losses and abnormal tympanometry persisted into teenage years. Conclusions Patients with cleft palates have eustachian tube dysfunction, which, in our cohort, resulted in persistent conductive hearing loss, highlighting the importance of long-term follow-up. Cholesteatoma incidence was low and not associated with number of tubes, which at our institution were placed prophylactically. Tympanoplasty was successful in those with persistent perforations.


Subject(s)
Audiometry, Pure-Tone/methods , Cleft Palate/complications , Eustachian Tube/physiopathology , Hearing Loss, Conductive/physiopathology , Hearing/physiology , Adolescent , Child , Child, Preschool , Cross-Sectional Studies , Female , Follow-Up Studies , Hearing Loss, Conductive/etiology , Hearing Loss, Conductive/surgery , Humans , Male , Prognosis , Retrospective Studies , Time Factors , Tympanoplasty
12.
Otolaryngol Head Neck Surg ; 156(6): 1119-1123, 2017 06.
Article in English | MEDLINE | ID: mdl-28419807

ABSTRACT

Objective To delineate research resources available to otolaryngology residents and their impact on scholarly productivity. Study Design Survey of current otolaryngology program directors. Setting Otolaryngology residency programs. Subjects and Methods An anonymous web-based survey was sent to 98 allopathic otolaryngology training program directors. Fisher exact tests and nonparametric correlations were used to determine statistically significant differences among various strata of programs. Results Thirty-nine percent (n = 38) of queried programs responded. Fourteen (37%) programs had 11 to 15 full-time, academic faculty associated with the residency program. Twenty (53%) programs have a dedicated research coordinator. Basic science lab space and financial resources for statistical work were present at 22 programs (58%). Funding is uniformly provided for presentation of research at conferences; a minority of programs (13%) only funded podium presentations. Twenty-four (63%) have resident research requirements beyond the Accreditation Council for Graduate Medical Education (ACGME) mandate of preparing a "manuscript suitable for publication" prior to graduation. Twenty-five (67%) programs have residents with 2 to 3 active research projects at any given time. None of the investigated resources were significantly associated with increased scholarly output. There was no uniformity to research curricula. Conclusions Otolaryngology residency programs value research, evidenced by financial support provided and requirements beyond the ACGME minimum. Additional resources were not statistically related to an increase in resident research productivity, although they may contribute positively to the overall research experience during training. Potential future areas to examine include research curricula best practices, how to develop meaningful mentorship and resource allocation that inspires continued research interest, and intellectual stimulation.


Subject(s)
Biomedical Research/organization & administration , Education, Medical, Graduate/organization & administration , Internship and Residency , Otolaryngology/education , Accreditation , Curriculum , Efficiency , Humans , Research Support as Topic , Surveys and Questionnaires , United States
13.
Otolaryngol Head Neck Surg ; 156(6): 1104-1107, 2017 06.
Article in English | MEDLINE | ID: mdl-28349746

ABSTRACT

Objective To determine the availability and purpose of away rotations during otolaryngology residency. Study Design Cross-sectional survey. Setting Otolaryngology residency programs. Subjects and Methods An anonymous web-based survey was sent to 98 allopathic otolaryngology training program directors, of which 38 programs responded. Fisher exact tests and nonparametric correlations were used to determine statistically significant differences among various strata of programs. A P value of <.05 was considered statistically significant. Results Thirty-nine percent (n = 38) of queried programs responded. Mandatory away rotations and elective away rotations were both present in 6 of 38 programs (16%). Neither number of faculty ( P = .119) nor residents ( P = .88) was predictive of away rotation. Away rotations were typically >151 miles from the home institution and typically used to address deficiencies in clinical exposure (67%) or case volume (50%). Participants of mandatory away rotations were universally provided housing, with other consideration such as stipend (33%), relocation allowance (33%), or food allowance (16%) sometimes offered. In contrast to mandatory rotations, half of elective rotations were to obtain a unique international mission trip experience. Nearly one-third of surveyed program directors (29%) would consider adding an away rotation to their curriculum in the next 3 years. Conclusions Mandatory and elective away rotations play a role in a small, but not insignificant, number of training programs. The rationale for these rotations is variable. Given that nearly one-third of program directors would consider adding an away rotation in the near future, further research into components of a meaningful away rotation and how to optimize the away rotation experience is warranted.


Subject(s)
Airway Management , Education, Medical, Graduate , Internship and Residency , Otolaryngology/education , Otorhinolaryngologic Diseases/surgery , Clinical Competence , Curriculum , Humans , United States
14.
Laryngoscope ; 127(12): 2850-2853, 2017 12.
Article in English | MEDLINE | ID: mdl-28349568

ABSTRACT

OBJECTIVE: We looked to determine the rates of audiovestibular symptoms following sports-related concussions among collegiate athletes. Further, we assessed the correlation between these symptoms and the time to return to participation in athletic activity. STUDY DESIGN: Retrospective analysis of the National Collegiate Athletic Association Injury Surveillance System (NCAA-ISS). METHODS: The NCAA-ISS was queried from 2009 through 2014 for seven men's sports and eight women's sports across divisions 1, 2, and 3. Injuries resulting in concussions were analyzed for audiovestibular symptoms, duration of symptoms, and return to participation times. RESULTS: From 2009 to 2014, there were 1,647 recorded sports-related concussions, with athletes reporting dizziness (68.2%), imbalance (35.8%), disorientation (31.4%), noise sensitivity (29.9%), and tinnitus (8.5%). Concussion symptoms resolved within 1 day (17.1%), within 2 to 7 days (50.0%), within 8 to 30 days (25.9%), or persisted over 1 month (7.0%). Return to participation occurred within 1 week (38.3%), within 1 month (53.0%), or over 1 month (8.7%). Using Mann-Whitney U testing, overall symptom duration and return to competition time were significantly increased when any of these symptoms were present (P < 0.05). Duration of concussion symptom correlated with dizziness (P = 0.043) and noise sensitivity (P = 0.000), whereas return to participation times correlated with imbalance (P = 0.011) and noise sensitivity (P = 0.000). Dizziness and imbalance (odds ratio: 4.15, confidence interval: 3.20-5.38, P < 0.001) were the two symptoms with the strongest association. CONCLUSION: Audiovestibular symptoms are common complaints among collegiate athletes sustaining concussions. Dizziness and noise sensitivity correlated with the duration of concussive symptoms, whereas imbalance and noise sensitivity was correlated with prolonged return to competition time. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2850-2853, 2017.


Subject(s)
Athletic Injuries/complications , Brain Concussion/complications , Hearing Disorders/etiology , Vestibular Diseases/etiology , Female , Hearing Disorders/epidemiology , Humans , Male , Organizations, Nonprofit , Retrospective Studies , Sports , Time Factors , United States , Vestibular Diseases/epidemiology
15.
Otolaryngol Head Neck Surg ; 157(1): 113-116, 2017 07.
Article in English | MEDLINE | ID: mdl-28195746

ABSTRACT

Objectives (1) Measure temperature variations achieved by common otomicroscopes. (2) Raise awareness about possible thermal injury during otologic procedures with the advent of newer, high-powered otomicroscopes. (3) Describe optical technology that aims to reduce the potential for thermal injury. Methods A variety of otomicroscopes, with different light sources (ranging from 100W halogen to 300W xenon), were studied. Temperatures were recorded from human auricular skin with a noncontact infrared thermometer at various microscope light intensities and with use of irrigation. Multiple recordings were done at 5-minute intervals, and a working distance of 225 mm was maintained. Results Maximum skin temperatures were found to plateau relatively quickly, with higher-wattage xenon light sources reaching greater temperatures. One-way analysis of variance revealed significant differences in temperatures with decreased light intensities. High-wattage xenon light sources reached significantly higher temperatures when compared with halogen models. Discussion There is substantial variation in maximal skin temperatures reached by otomicroscopes. Temperatures can be decreased to safe levels by reducing light intensity and with use of irrigation. The maximum temperature obtained in our study was 41.4°C. Second-degree skin burns have been described with prolonged exposures to temperatures >44°C. Implications for Practice Given the described potential for burns, surgeons performing procedures on the ear and temporal bone should take precautions to diminish temperature in the operative field.


Subject(s)
Burns/prevention & control , Ear, External/injuries , Hot Temperature , Otoscopes/adverse effects , Humans , Otologic Surgical Procedures/adverse effects , Otologic Surgical Procedures/instrumentation , Patient Safety , Skin/radiation effects , Thermometers
16.
Otol Neurotol ; 35(8): 1474-9, 2014 Sep.
Article in English | MEDLINE | ID: mdl-25080039

ABSTRACT

OBJECTIVES: To estimate the prevalence of external auditory canal (EAC) stenosis in patients with congenital aural atresia (CAA) and the prevalence of acquired ear canal cholesteatoma in patients with EAC stenosis and to identify risk factors that may predict the presence of ear canal cholesteatoma in those patients. STUDY DESIGN: Retrospective chart review. SETTING: University tertiary referral center. MATERIALS AND METHODS: Patients with EAC stenosis with and without acquired ear canal cholesteatoma were identified from the medical records of 673 patients (770 ears) with CAA. Demographic data, symptoms at presentation, and audiometric data were compared between those with and those without cholesteatoma to identify the risk factors for the presence of ear canal cholesteatoma. RESULTS: Of the 770 ears evaluated, 101 (13.1%) were found to have at least 1 stenotic ear canal. Of this group of 101 ears with canal stenosis, 18 of 94 ears (7 ears were missing data; 19.1%) had a concurrent cholesteatoma, with 1 patient having bilateral cholesteatomas. Demographic, clinical, and audiometric parameters showed that only female sex was associated with a higher rate of ear canal cholesteatoma. Within the cholesteatoma group, right ears in female patients and left ears in male patients predominated. CONCLUSION: Approximately 1 in 5 patients with congenital aural stenosis were found to have ear canal cholesteatoma. Female sex is a risk factor; basic audiometric parameters provide no diagnostic utility in distinguishing ears with cholesteatoma from those without cholesteatoma.


Subject(s)
Cholesteatoma, Middle Ear/epidemiology , Ear Canal/abnormalities , Ear Diseases/congenital , Adult , Audiometry, Pure-Tone , Constriction, Pathologic/congenital , Female , Humans , Male , Middle Aged , Prevalence , Retrospective Studies , Risk Factors
17.
Otol Neurotol ; 34(7): 1226-9, 2013 Sep.
Article in English | MEDLINE | ID: mdl-23921932

ABSTRACT

OBJECTIVE: To review the literature regarding cortical hearing loss and document a case of cortical hearing loss including its presentation, diagnosis, and evolution over 32 months of follow-up. PATIENT: A 56-year-old woman with profound bilateral sensorineural hearing loss secondary to sequential hemorrhagic, temporal lobe infarctions separated in time by 8 months. INTERVENTION: Diagnostic. RESULTS: Sequential infarctions affecting the patient's auditory radiations and primary auditory cortices bilaterally combined to cause cortical hearing loss. At presentation, audiogram revealed a bilateral profound sensorineural hearing loss with no reliable responses to pure-tone or speech audiometry. She has subsequently recovered the ability to distinguish environmental sounds. At her 32-month follow-up, she had a pure-tone average (PTA) of 62 dB on the right and 70 dB on the left but continued to display a poor word recognition score (0%). A literature review was performed from the year 1891 until the present. CONCLUSION: Cortical deafness is an exceedingly rare entity. Presentation and recovery of hearing are dependent on the extent of the initial lesions. The majority of patients can expect improvements in pure-tone auditory thresholds over time; however patients should be counseled that recovery of the ability to understand speech is unlikely.


Subject(s)
Hearing Loss, Central/pathology , Audiometry, Pure-Tone , Audiometry, Speech , Brain/pathology , Cochlear Implants , Disease Progression , Female , Follow-Up Studies , Hearing Loss, Central/diagnosis , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/etiology , Hearing Loss, Sensorineural/pathology , Humans , Intracranial Hemorrhages/complications , Intracranial Hemorrhages/pathology , Magnetic Resonance Imaging , Middle Aged , Otoacoustic Emissions, Spontaneous , Recovery of Function , Speech Perception
18.
J Neurosci ; 33(7): 3079-93, 2013 Feb 13.
Article in English | MEDLINE | ID: mdl-23407963

ABSTRACT

Ototoxicity is a main dose-limiting factor in the clinical application of aminoglycoside antibiotics. Despite longstanding research efforts, our understanding of the mechanisms underlying aminoglycoside ototoxicity remains limited. Here we report the discovery of a novel stress pathway that contributes to aminoglycoside-induced hair cell degeneration. Modifying the previously developed bioorthogonal noncanonical amino acid tagging method, we used click chemistry to study the role of protein synthesis activity in aminoglycoside-induced hair cell stress. We demonstrate that aminoglycosides inhibit protein synthesis in hair cells and activate a signaling pathway similar to ribotoxic stress response, contributing to hair cell degeneration. The ability of a particular aminoglycoside to inhibit protein synthesis and to activate the c-Jun N-terminal kinase (JNK) pathway correlated well with its ototoxic potential. Finally, we report that a Food and Drug Administration-approved drug known to inhibit ribotoxic stress response also prevents JNK activation and improves hair cell survival, opening up novel strategies to prevent and treat aminoglycoside ototoxicity.


Subject(s)
Aminoglycosides/toxicity , Anti-Bacterial Agents/toxicity , Cytosol/metabolism , Ear Diseases/chemically induced , Protein Synthesis Inhibitors/toxicity , Alanine/analogs & derivatives , Alkynes , Aminoglycosides/metabolism , Animals , Anti-Bacterial Agents/metabolism , Apoptosis/drug effects , Blotting, Western , Cell Count , Chick Embryo , Enzyme Activation/drug effects , Evoked Potentials, Auditory, Brain Stem/drug effects , Glycine/analogs & derivatives , Hair Cells, Auditory/drug effects , Hair Cells, Auditory/pathology , Immunohistochemistry , JNK Mitogen-Activated Protein Kinases/metabolism , Mice , Mice, Inbred CBA , Niacinamide/analogs & derivatives , Niacinamide/pharmacology , Organ Culture Techniques , Phenylurea Compounds/pharmacology , Protein Kinase Inhibitors/pharmacology , Protein Synthesis Inhibitors/metabolism , RNA, Ribosomal/metabolism , Sorafenib
19.
Otol Neurotol ; 33(6): 1002-6, 2012 Aug.
Article in English | MEDLINE | ID: mdl-22772017

ABSTRACT

OBJECTIVE: The purpose of this study is to explore the correlation between preoperative hearing and early postoperative hearing results in patients undergoing primary aural atresia repair. STUDY DESIGN: Retrospective review of 125 patients. SETTING: Academic tertiary referral center. PATIENTS: One hundred twenty-five patients (5-67 yr old) undergoing 133 primary aural atresia surgeries were included. MAIN OUTCOME MEASURE(S): Spearman correlation coefficients were calculated between preoperative and postoperative (mean, 7.5 wk; range, 3-40 wk after surgery) hearing outcome measures including 3-tone pure-tone average (PTA), speech reception threshold (SRT), speech discrimination scores (SDS), air-bone gap (ABG), change in ABG (ΔABG), and between preoperative SRT and Jahrsdoerfer score. RESULTS: Preoperative PTA, SRT, SDS, and ABG correlated strongly with their respective postoperative values (correlation coefficients rho of 0.356 [p < 0.01], 0.199 [p < 0.05], 0.480 [p < 0.01], and 0.223 [p < 0.05], respectively). Preoperative PTA (0.407; p < 0.01), SRT (0.348; p < 0.01), SDS (-0.247; p < 0.01), and ABG (0.514; p < 0.01) also were correlated with ΔABG. When postoperative results were dichotomized to either normal (SRT, <30dB HL) or abnormal (SRT, ≥30dB HL), preoperative SRT was found to be a positive predictor of normal postoperative hearing (p = 0.05). Probability of normal postoperative hearing was 66% when preoperative SRT was 50 dB HL or lower and 40% when greater than 60 dB HL. Preoperative hearing (SRT) also trended toward a correlation with Jahrsdoerfer score (-0.168 [p = 0.058]). CONCLUSION: Among patients undergoing primary atresia repair, better preoperative hearing strongly predicts better postoperative hearing and correlates with ear anatomy. Preoperative hearing status should be factored when counseling atresia patients on hearing rehabilitation options.


Subject(s)
Hearing Loss, Conductive/congenital , Hearing Loss, Conductive/surgery , Hearing/physiology , Otologic Surgical Procedures , Adolescent , Adult , Aged , Audiometry, Pure-Tone , Auditory Threshold/physiology , Bone Conduction/physiology , Child , Child, Preschool , Constriction, Pathologic , Ear Ossicles/surgery , Female , Hearing Loss, Conductive/pathology , Humans , Male , Middle Aged , Predictive Value of Tests , Retrospective Studies , Speech Perception/physiology , Temporal Bone/diagnostic imaging , Tomography, X-Ray Computed , Treatment Outcome , Young Adult
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