Cochlear Implantation in Charcot-Marie-Tooth Patients: Speech Perception and Quality of Life.

OBJECTIVES: There is a limited understanding of the impact of cochlear implantation (CI) in patients with Charcot-Marie-Tooth disease (CMT), given the scarcity of reported cases. We aim to evaluate the audiological outcomes and quality of life (QoL) after CI in CMT. METHODS: Multi-institutional, university-affiliated, tertiary-referral centers, retrospective chart review.Our cohort includes 5 patients with CMT. Patients' charts were reviewed for demographic characteristics, operation notes, and pre- and post-implantation audiology evaluation. Patients completed the Cochlear Implant Quality of Life-10 (CIQOL-10) Global questionnaire. RESULTS: Pre-implantation, the mean pure tone average was 84.1 ± 7.2 dB, and the mean word recognition score was 2.4% in the implanted ear. AzBio sentence test was performed in quiet, revealing a mean of 4 ± 1.4% in the implanted ear. Post-implantation, PTA results were all within the mild hearing loss range (mean 33.0 ± 5.9 dB). Post-CI, AZ-Bio test results were 5%, 65%, and 74% (for 3 patients), and HINT scores were 55% and 58% (for 2 patients). The mean score of the CIQOL-10 questionnaire was 42.7 ± 10.47 (range 1-100). Patients were most satisfied with their ability to listen to the television or radio, have conversations in a quiet environment, and feel comfortable being themselves. CONCLUSION: To the best of our knowledge, this is the most extensive series of CI in CMT-associated sensorineural hearing loss and auditory neuropathy. Our cohort suggests that CI is a safe and reliable method for hearing rehabilitation that can achieve good speech performance and improve QoL in CMT patients.

Cochlear Implantation after Stereotactic Radiosurgery for Vestibular Schwannoma: Initial Hearing Improvement and Longevity of Hearing Restoration.

OBJECTIVE: The following research question was asked: In patients with vestibular schwannoma (VS) that underwent stereotactic radiosurgery (SRS) and cochlear implantation, were improvements in hearing function observed, and what was the cochlear implant (CI) failure rate of in these patients? DATA SOURCES: PubMed/Medline, CINAHL (EBSCOhost), and Web of Science articles without restrictions on publication dates were searched. STUDY SELECTION: Inclusion criteria required that the article was a report, a series, or a retrospective review with individual case data available. Non-English articles were excluded. Inclusion criteria required that patients were with VS and underwent subsequent SRS and cochlear implantation. Patients receiving microsurgery or stereotactic radiotherapy on the ipsilateral ear were excluded from this study. DATA EXTRACTION: Included studies were evaluated using full-text evaluation, and data on study characteristics (author names, gender), clinical data (syndromic information, SRS modality), hearing outcomes, and device failure were extracted. DATA SYNTHESIS: Means and averages were obtained for all continuous variables. Percentages were ascertained for all categorical variables. CONCLUSIONS: The majority of patients undergoing CI placement in VS treated with SRS achieved open-set speech perception (79.2%) or environmental sound awareness (6.8%). Twelve implants (20.3%) failed. Three patterns were associated with failure: 1) immediate-onset failure, 2) initial benefit with delayed failure, 3) poor local control with device explantation.

Overemphasis of USMLE and Its Potential Impact on Diversity in Otolaryngology.

OBJECTIVE: Applicant demographics during the 2019-2020 residency cycle were evaluated to determine if strict utilization of United States Medical Licensing Examination (USMLE) scores in applicant selection could lead to a restriction in diversity. STUDY DESIGN: Cross-sectional study. SETTING: Otolaryngology residency applicants to a single institution. METHODS: A total of 381 applicants were analyzed by age, gender, applicant type, race/ethnicity, USMLE scores, permanent zip code, and graduating medical school. RESULTS: Among applicants, 37% were women; 9% were ≥30 years of age; 12% were underrepresented minorities (URMs); 71% to 81% had above-average socioeconomic surrogate markers; 22% were from a top 25 US News & World Report-ranked institution; and 81% were from an institution with an otolaryngology residency program. There was no increase in applicants who identified as URM from the 2015-2020 cycles. Multivariable regression analysis showed that applicants who were international medical graduates, URMs, and ≥30 years of age had lower Step 1 and Step 2 scores (P < .05). Applicants who identified as women had a lower Step 1 score, and those from top 25 National Institutes of Health-funded institutions had a higher Step 1 score; however, there was no difference when Step 2 scores were compared (P > .05). CONCLUSION: Our data suggest that in the pre-USMLE Step 1 pass/fail setting, strict adherence to USMLE scores may lead to disproportionally low recruitment of applicants who are women, ≥30 years of age, URMs, and from institutions without an otolaryngology residency program. We must implement measures against overemphasizing the absolute values of USMLE scores for a true holistic review of applicants, specifically to prevent an overemphasis on the USMLE Step 2 score.

Otolaryngology Residency Interviews in a Socially Distanced World: Strategies to Recruit and Assess Applicants.

Due to concerns surrounding travel during the COVID-19 pandemic, the 2020-2021 otolaryngology residency application cycle will be conducted virtually for the first time. Residency programs should consider the logistics of video interviews, drawing on experiences of other programs that have successfully performed virtual interviews in the past. The lack of in-person interviews will create challenges in assessing applicants, and we recommend that programs develop structured and targeted questions and even consider having candidates answer standardized questions prior to the virtual interview day. From an applicant perspective, gauging the intangibles of individual residency programs, such as resident camaraderie, program culture, and program location, will be difficult. To address this, programs should consider hosting informal virtual gatherings, create videos that highlight the resident experience, and ensure that program websites are up-to-date. Ultimately, adaptability, resilience, and innovation will allow residency programs to achieve a successful 2021 otolaryngology match.

Predicting complications of pediatric temporal bone fractures.

OBJECTIVE: Our aim is to characterize complications of pediatric temporal bone fractures and identify predictive risk factors associated with fracture complications. METHODS: A retrospective review was conducted of all temporal bone fractures diagnosed in children (age less than or equal to 18 years) from a single academic institution between 2003 and 2017. Demographics, mechanism of injury, fracture characteristics, computed tomography evaluation and follow-up duration were recorded on each patient. Outcomes measured include facial nerve injury (FNI), cerebrospinal fluid (CSF) leak, sensorineural hearing loss (SNHL), and conductive hearing loss (CHL). RESULTS: One-hundred-seventeen patients with 129 temporal bone fractures were included in the study. Most fractures were otic capsule sparing (OCS) (96%, n = 124) and longitudinal (71%, n = 91). Otic capsule violating (OCV) fractures were associated with higher CSF leak rates (20% versus 2%, p = 0.14) and FNI rates (60% versus 5%, p = 0.002) compared to OCS fractures. Audiograms were available in 37 patients (34%). Patients with Glasgow coma scale (GCS) consistent with a mild traumatic brain injury (TBI) (GCS > 13) had significantly fewer complications (FNI and CSF leaks) compared to the group with moderate and severe TBI (GCS < 13), 5% versus 23% (p = 0.03). CONCLUSIONS: Higher complication rates are seen with OCV fractures and transverse fractures. Moderate and severe TBI as measured by GCS is predictive of FNI and CSF complications in pediatric temporal bone fractures.

Asymmetric sensorineural hearing loss and vestibular schwannoma: when to image?

PURPOSE OF REVIEW: We review the literature on the use of audiometric protocols in the guidance of when to obtain MRI for detection of vestibular schwannoma. This discussion will focus on the sensitivity, specificity, and cost-effective analysis of audiometric criteria of asymmetric sensorineural hearing loss (ASNHL) when used to decide when MRI scans should be performed. RECENT FINDINGS: The sensitivity for detecting vestibular schwannomas when invoking published audiometric protocols for triggering MRI acquisition in ASNHL ranged from 50 to 100%. Specificity of these protocols ranged from 23 to 83%. Such audiometric protocols are efficient, achieving sensitivity and specificity at these rates while reducing the screening rate to 18 to 35%. The reduced procurement of MRI while using such audiometric protocols is associated with annual cost savings of between 23 and 82%. While no definitive recommendations can be made from this review, some audiometric protocols offer a better balance of sensitivity and specificity than others. SUMMARY: Audiometric protocols for triggering MRI acquisition in ASNHL for evaluation of vestibular schwannoma can be both sensitive and specific. These are competitive measures, and so no protocol is both 100% sensitive and specific. Such protocols become less effective in populations with increased incidence of noise-induced hearing loss. Invocation of such audiometric protocols can considerably reduce the annual cost of MRI evaluation for vestibular schwannomas.

State of the art regeneration of the tympanic membrane.

PURPOSE OF REVIEW: One of the most common diseases of the tympanic membrane is a perforation, and tympanoplasty is one of the more common procedures in otolaryngology. Tympanic membrane regeneration and bioengineering aim to improve the success rate of the procedure, increase the availability of different scaffolds and provide innovative tools that will simplify the surgical technique and make it accessible for surgeons with varying expertise level. This review aims to raise awareness of current tissue engineering developments in tympanic membrane regeneration and how they may augment current clinical practices. We focus here on achievements in tympanic membrane cell cultures and on innovations in development of new scaffolds and growth factors that enhance regeneration of patient's native tympanic membranes. RECENT FINDINGS: In recent years, great achievements were reached in the field of tympanic membrane regeneration in the three hallmarks of bioengineering: cells, scaffolds and bioactive molecules. New techniques for modeling normal tympanic membrane proliferation were developed, as well as for isolation and expansion of normal tympanic membrane keratinocytes from miniature samples of scarred tissue. Ongoing clinical trials aim to seal the perforation by applying different scaffolds infiltrated by growth factors on the tympanic membrane. SUMMARY: Research efforts in tympanic membrane regeneration continue to seek the ideal single tissue-engineered substitute. Recent advances in tympanic membrane bioengineering include new types of scaffolds that may augment and provide a safe and effective alternative to the current gold-standard autograft. New bioactive molecules may simplify the surgical procedure and reduce surgical time by augmenting the native tympanic membrane regeneration. Several groups of bioengineering scientists and neurotologists are continuing to move forward and develop new strategies, seeking to create a fully functional tissue-engineered tympanic membrane.

Applicant-to-Residency Program Communication: Does It Matter?

OBJECTIVES: To elucidate the frequency and types of pre- and post-interview communication that applicants engage with programs, to garner the perceptions of both applicants and program directors (PDs), and determine if communication influences outcomes. SUBJECTS AND METHODS: Electronic surveys were distributed to otolaryngology residency applicants, and to PDs of ACGME-accredited otolaryngology programs after the 2018 to 2019 application cycle. RESULTS: 93 of 324 applicants (28.7%) and 33 of 106 PDs (31.3%) responded. In the pre-interview period, 58.1% of applicants sent emails of interest, and 41.9% had a mentor initiate communication. In the post-interview period, the majority of applicants (82.8%) sent notes of intent to their number one choice, and 32.3% had a faculty mentor communicate this on their behalf. The majority of PDs (84.8%) were undecided or did not believe that emails of interest influence decisions to offer an interview, whereas 81.8% believed that communication initiated by an applicant's mentor has an impact on interview offers. No PD agreed that declarations of intent from applicants have an impact on their rank lists, while only 33.3% of PDs believed that a mentor communicating this for an applicant has some impact. Our statistical findings are in agreement with these perceptions as neither applicant-initiated pre-interview (P = .54), mentor-initiated pre-interview (P = .62), applicant-initiated post-interview (P = .11) nor mentor-initiated post-interview (P = .78) communications influenced the number of interviews received or ultimate match outcome. CONCLUSION: Pre- and post-interview communication practices vary widely among otolaryngology applicants. Applicant-initiated communication has no impact on outcomes, while mentor-initiated communication is perceived to have more benefit, despite not impacting interview or match outcomes in this study.

Internal Acoustic Canal Stenosis Due to Hyperostosis.

Background Exostoses and osteomas are benign, insidious lesions of the bone involving the internal acoustic canal (IAC). We present two cases of IAC exostoses managed with surgical decompression and review the clinical outcomes of previously reported cases in the literature. Methods A comprehensive search was conducted using PubMed Central, Web of Science Core Collection, and Google Scholar databases to identify previous reports of IAC exostoses and osteomas. A total of 26 reported cases were identified, and patient presenting symptoms, management strategies, and response to surgery was obtained when available. Results Of the 13 patients who underwent surgical decompression, 8 patients had resolution of vertigo symptoms, 10 patients had improvement of tinnitus symptoms, and all patients maintained some level of serviceable hearing. Conclusion IAC exostoses and osteomas are rare lesions that lead to insidious onset of debilitating symptoms from vestibulocochlear nerve dysfunction. Although the role of surgical decompression remains unclear, it appears that patients presenting with vertigo have more favorable response to surgical decompression as compared with those presenting with tinnitus and sensorineural hearing loss.

Otolaryngology Residency Application during the SARS-CoV-2 (COVID-19) Pandemic.

The escalation of the COVID-19 pandemic has affected health care at every level, including medical education. As some fourth-year medical students graduate early to join the front lines, we must now turn our attention to those trainees in their penultimate year. In this commentary, we address the unique dilemmas facing otolaryngology residency candidates for the 2020-2021 cycle, with a focus on those applicants with no institutional otolaryngology department.

State-of-the-art methods in clinical intracochlear drug delivery.

PURPOSE OF REVIEW: Increasing awareness and prevalence of disorders in hearing and balance have placed emphasis on treatment strategies. With the rapid evolution in molecular, gene, and nanotechnology, alternate delivery methods have advanced intracochlear drug delivery. This review aims to raise awareness of recent developments in technologies to augment current clinical practices. RECENT FINDINGS: Intracochlear drug delivery research has expanded with the familiarity and accessibility to cochlear implantation. Various therapeutics are closely studied for both safety and efficacy as well as biologic effect. Agents including neurotrophins, antiapoptotics, cell therapy, gene therapy, and anti-inflammatory drugs are on the forefront of preclinical research. Cochlear implant electrode modification and drug administration at the time of implantation is a major focus of research. Improvements in study design have focused on overcoming barriers including elucidating the role of the blood-perilymph barrier. SUMMARY: Inner ear drug delivery methods include systemic, intratympanic, and intracochlear administration. Therapeutic technologies aim to overcome delivery barriers and to improve overall biologic effect while minimizing toxicity. Precision of drug application through intratympanic and intracochlear administration with minimal trauma is the future of inner ear drug development.

Treatment of Temporal Bone Fractures.

Traumatic injury to the temporal bone can lead to significant morbidity or mortality and knowledge of the pertinent anatomy, pathophysiology of injury, and appropriate management strategies is critical for successful recovery and rehabilitation of such injured patients. Most temporal bone fractures are caused by motor vehicle accidents. Temporal bone fractures are best classified as either otic capsule sparing or otic capsule disrupting-type fractures, as such classification correlates well with risk of concomitant functional complications. The most common complications of temporal bone fractures are facial nerve injury, cerebrospinal fluid (CSF) leak, and hearing loss. Assessment of facial nerve function as soon as possible following injury greatly facilitates clinical decision making. Use of prophylactic antibiotics in the setting of CSF leak is controversial; however, following critical analysis and interpretation of the existing classic and contemporary literature, we believe its use is absolutely warranted.

A Pilot Study on the Efficacy of Continuous Positive Airway Pressure on the Manifestations of Ménière's Disease in Patients with Concomitant Obstructive Sleep Apnea Syndrome.

OBJECTIVES: To evaluate the effect of continuous positive airway pressure (CPAP) therapy on Ménière's disease patients with concomitant obstructive sleep apnea syndrome (OSAS), since recent reports suggest OSAS may cause dysfunction of the vestibular system. STUDY DESIGN: Prospective study using CPAP administered to patients diagnosed with "Definite Ménière's disease" according to the guidelines of the American Academy of Otolaryngology--Head and Neck Surgery and combined with OSAS. SETTING: University hospital. METHODS: Twenty consecutive patients, 14 male and 6 female with active, unilateral, cochleovestibular Ménière's disease refractory to medical management who also had concurrent OSAS as defined by International Classification of Sleep Disorders, Second Edition were selected to undergo solitary CPAP therapy. Audiometric testing, caloric testing, and DHI survey were conducted before and after CPAP therapy and compared to assess effectiveness of CPAP therapy as utilized for treatment of Ménière's disease. RESULTS: Although caloric testing did not show significant difference, audiometric testing and results of dizziness handicap inventory were significantly improved (p < 0.05) after CPAP therapy only, without standard treatment for Ménière's disease. CONCLUSION: Recent reports have suggested that OSAS may cause dysfunction of the vestibular system. We investigated whether standard therapy for OSAS would be of benefit in the management of vertigo and hearing loss in Ménière's disease patients. Our study cohort demonstrated significant improvement in both DHI and audiometric testing following solitary CPAP therapy for OSAS. Solitary CPAP therapy may become a new effective treatment strategy for Ménière's disease patients with OSAS, not just only for control of dizziness and vertigo but also for potential benefit of hearing.

Biomimetic stochastic topography and electric fields synergistically enhance directional migration of corneal epithelial cells in a MMP-3-dependent manner.

Directed migration of corneal epithelial cells (CECs) is critical for maintenance of corneal homeostasis as well as wound healing. Soluble cytoactive factors and the intrinsic chemical attributes of the underlying extracellular matrix (ECM) participate in stimulating and directing migration. The central importance of the intrinsic biophysical attributes of the microenvironment of the cell in modulating an array of fundamental epithelial behaviors including migration has been widely documented. Among the best measures of these attributes are the intrinsic topography and stiffness of the ECM and electric fields (EFs). How cells integrate these multiple simultaneous inputs is not well understood. Here, we present a method that combines the use of (i) topographically patterned substrates (mean pore diameter 800nm) possessing features that approximate those found in the native corneal basement membrane; and (ii) EFs (0-150mVmm(-1)) mimicking those at corneal epithelial wounds that the cells experience in vivo. We found that topographic cues and EFs synergistically regulated directional migration of human CECs and that this was associated with upregulation of matrix metalloproteinase-3 (MMP3). MMP3 expression and activity were significantly elevated with 150mVmm(-1) applied-EF while MMP2/9 remained unaltered. MMP3 expression was elevated in cells cultured on patterned surfaces against planar surfaces. The highest single-cell migration rate was observed with 150mVmm(-1) applied EF on patterned and planar surfaces. When cultured as a confluent sheet, EFs induced collective cell migration on stochastically patterned surfaces compared with dissociated single-cell migration on planar surfaces. These results suggest significant interaction of biophysical cues in regulating cell behaviors and will help define design parameters for corneal prosthetics and help to better understand corneal wound healing.

Noise-induced hearing loss: a recreational noise perspective.

PURPOSE OF REVIEW: This review will discuss the real-world risk factors involved in noise-induced hearing loss as a result of common and popular recreational activities prone to mid and high levels of noise exposure. Although there are currently no interventional measures available to reverse or mitigate preexisting hearing loss from noise, we discuss the vital importance of hearing loss prevention from noise exposure avoidance and reduction. RECENT FINDINGS: Despite a seeming understanding of the effects of noise exposure from various recreational activities and devices, a large percentage of the general public who is at risk of such noise-induced hearing loss still chooses to refrain from using hearing protection instruments. SUMMARY: While occupational exposures pose the greatest traditional risk to hearing conservation in selected workers, recreational risk factors for noise-induced hearing loss may be more insidious in overall effect given the indifferent attitude of much of the general public and particularly our youths toward hearing protection during recreational activities. Active counseling regarding the consequences of excessive noise exposure and the potential benefits to hearing from usage of hearing protection instruments is critical to providing best possible care in the hearing health professions.

Labyrinthine artery aneurysm as an internal auditory canal mass.

We present the first case report of a labyrinthine artery aneurysm masquerading as an internal auditory canal tumor. A 72-year-old woman presented with sudden onset right facial paralysis, facial pain, hearing loss, and vertigo. She demonstrated dense right-sided facial paralysis involving all branches of the facial nerve, left beating horizontal nystagmus, and anacusis of the right ear. Magnetic resonance imaging with contrast demonstrated a 6 × 7 mm peripherally enhancing lesion with lack of central uptake filling the right internal auditory canal. The patient elected to proceed with translabyrinthine approach to the internal auditory canal for definitive resection of the mass as well as to decompress the neural structures of the internal auditory canal in an attempt to recover neural function, particularly of the facial nerve. Intraoperatively, the internal auditory canal mass was resected with minimal difficulty, with intraoperative dissection notable for brisk bleeding at the medial base of the tumor just as the tumor was dissected off its medial fibrous attachments. Final pathology of the resected mass revealed a blood vessel with mucinous degeneration of the medial layer of the vessel wall, with immunohistochemical staining confirming the presence and structure of aneurysmal blood vessel.

Otogenic lateral sinus thrombosis: case series and controversies.

OBJECTIVES: The aim of this study is to (1) report the clinical presentation, treatment, and sequelae in a series of pediatric patients with otogenic lateral sinus thrombosis and (2) to review the most controversial aspects of management of this rare intracranial complication of otitis media. METHODS: Retrospective chart review of inpatients treated for central venous thrombosis at a tertiary care facility between 1996 and 2012. RESULTS: Five pediatric patients (four male, one female) were identified with otogenic lateral sinus thrombosis. Age at presentation ranged from 13 months to 15 years. All underwent a surgical procedure, ranging from a simple myringotomy with tympanostomy tube placement to tympanomastoidectomy and internal jugular vein ligation or craniotomy. Three were anticoagulated with unfractionated heparin with subsequent transition to low molecular weight heparin of variable duration. One patient developed a non-life-threatening intracranial hemorrhage while on long-term anticoagulation. Follow-up imaging, when available, did not directly correlate complete thrombus resolution with use of anticoagulation or with persistent symptoms. CONCLUSIONS: Otogenic lateral sinus thrombosis is a rare intracranial complication of otitis media with significantly reduced morbidity and mortality in the modern era of antibiotic treatment, surgical intervention, and anticoagulation therapy. Due to the rarity of this condition today, the recommended extent of surgical intervention and need for routine anticoagulation are unclear, and requires further data to determine definitively.