Faculty Bibliography

OBJECTIVE:To present a case of an iatrogenic inner ear third window after vestibular schwannoma microsurgery. PATIENTS:A 42-year-old male presented 9-months after left-sided retrosigmoid approach for an intracanalicular vestibular schwannoma with hearing-preservation attempt performed elsewhere. Immediately postoperatively, he developed the following disabling and persistent symptoms on the ipsilateral side: autophony, pulsatile tinnitus, high-pitched ringing tinnitus, and hearing his footsteps. He denied vertigo. Otoscopy was normal. Tuning fork (512-Hz) lateralized to the left and Rinne was negative on the left. Audiogram demonstrated a severe mixed hearing loss and 10% aided word-recognition score. High-resolution CT demonstrated violation of the common crus and dehiscence of bone along the medial vestibule suggestive of an iatrogenic inner ear third window. INTERVENTIONS:Labyrinthectomy and concurrent cochlear implantation. MAIN OUTCOME MEASURES:Resolution of third window symptoms, open-set speech recognition, tinnitus suppression. RESULTS:Patient reported immediate resolution of third window symptoms after labyrinthectomy and cochlear implantation. He demonstrates open-set word recognition of 64% at 1-year postoperatively and tinnitus suppression with his cochlear implant on. CONCLUSIONS:Iatrogenic third window symptoms can occur after hearing-preservation vestibular schwannoma microsurgery. Patients with sufficient hearing preservation who are disabled by third window symptoms yet lack sound clarity and useful hearing may be considered for labyrinthectomy and concurrent cochlear implantation. This intervention effectively extinguishes third window symptoms by destroying residual auditory function and simultaneously provides an opportunity to restore useful hearing and suppress tinnitus, thereby enhancing overall quality of life.

INTRODUCTION/BACKGROUND:Neonates with severe Pierre Robin sequence (PRS) can be treated by mandibular distraction osteogenesis (MDO), tongue-lip adhesion, or tracheostomy; however, there is an active debate regarding the indications of MDO in this patient population. Published algorithms identify tracheomalacia, bronchomalacia, laryngomalacia, hypotonic syndromes, and central sleep apnea as contraindications for MDO and indications for tracheostomy, but these comorbidities may exist along a spectrum of severity. The authors propose that appropriately selected neonates with PRS who concurrently express 1 or more of these traditional contraindications may be successfully treated with MDO. METHODS:The authors performed a 5-year retrospective chart review of all neonates who underwent MDO for treatment of severe PRS. All patients expressed a comorbidity previously identified as an indication for tracheostomy. Pre- and postoperative characteristics were recorded. Apnea/hypopnea index (AHI) before and after MDO were compared using 2-tailed repeated measures t-test. RESULTS:The authors identified 12 patients with severe PRS and conditions associated with contraindications to MDO: 9 (75.0%) patients had laryngomalacia, 6 (50.0%) patients had tracheomalacia, 2 (16.6%) patients had bronchomalacia, 1 (8.3%) patient had central sleep apnea, and 3 (25.0%) patients had hypotonia. Five (41.7%) patients underwent concurrent gastrostomy tube placement due to feeding insufficiency. Average birthweight was 3.0 kg. Average pre-op AHI was 34.8. Average post-op AHI was 7.3. All patients successfully underwent MDO with avoidance of tracheostomy. CONCLUSIONS:By employing an interdisciplinary evaluation of patient candidacy, MDO can safely and effectively treat upper airway obstruction and avoid tracheostomy in higher-risk neonatal patients with traditional indications for tracheostomy.

OBJECTIVE:Concurrent bilateral congenital aural atresia (CAA) and profound sensorineural hearing loss are rare. While not a contraindication, temporal bone and cochleovestibular abnormalities are an important consideration for cochlear implantation (CI) candidacy. Intraoperative image-guided surgical navigation may play a role during CI surgery in patients with complex anatomy, such as CAA. PATIENT/METHODS:One patient with bilateral CAA, cochlear dysplasia, speech delay, and profound sensorineural hearing loss underwent candidacy evaluation for cochlear implantation. INTERVENTIONS/METHODS:Cochlear implantation using intraoperative image-guided navigation. MAIN OUTCOME MEASURES/METHODS:(1) Registration accuracy, (2) surgical outcomes, (3) audiometry. RESULTS:A four-year-old girl with complete bilateral CAA and profound sensorineural hearing loss successfully underwent a right transmastoid approach for CI using intraoperative image-guided navigation with sticker fiducials. Bony landmarks included the mastoid tip, tympanomastoid suture line, helical root, zygomatic root, and lateral brow. A registration accuracy of 0.9 mm was achieved. There were no intraoperative or immediate postoperative complications. Postoperatively, Neural Response Imaging was confirmed on 9 electrodes and behavioral testing demonstrated Ling-6 access at 30 dB. On most recent follow-up, she has demonstrated gains in language development, vocalizations, and uses total communication in a hearing-impaired educational environment. CONCLUSIONS:Children with CAA and profound sensorineural hearing loss may be candidates for cochlear implantation, with successful outcomes in the setting of complex anatomy. Surgical navigation may play a role corroborating intraoperative landmarks.

OBJECTIVES/HYPOTHESIS/OBJECTIVE:We sought to identify changes that occur in spirometric values between surgical interventions in patients with recurrent laryngotracheal stenosis and assess the utility of tracking those changes in predicting the need to return to surgery. METHODS:), peak expiratory flow (PEF), and peak inspiratory flow (PIF), and body mass index (BMI) were tabulated. Calculations were then performed to determine deviations in spirometric measurements from maximums. Comparing the patients who required intervention to those who did not, we used a regression analysis to generate a decision tree based on factors with the strongest predictive power. We then calculated receiver operating characteristic (ROC) curves for all calculated variables. RESULTS:from each patient's maximums had strong predictive power in determining return to surgery. PIF was the only fixed measurement found to have a statistically significant role in predicting return to surgery. BMI did not play a role. CONCLUSION/CONCLUSIONS:For each patient, the deviation from their overall spirometric maximums had the statistically strongest predictive power in determining need to return to surgery. This suggests the importance of the trends in spirometric measures for each individual, and implies these trends have greater import than fixed measures alone. LEVEL OF EVIDENCE/METHODS:IV. Laryngoscope, 2020.

BACKGROUND:Insufficient exposure may require termination of procedure in transoral robotic surgery (TORS). The aim of study was to develop a "Pharyngoscore" to quantify the risk of difficult oropharyngeal exposure (DOE) before TORS. METHODS:Three-hundred six patients undergoing any surgical procedure at one Academic Hospital were prospectively enrolled. Oropharynx was exposed with Feyh-Kastenbauer retractor. Exposure was evaluated by direct and endoscopic visualization of the four oropharyngeal subsites. Preoperative clinical/anthropometric parameters were studied in good oropharyngeal exposure and DOE groups. Logistic regression was performed to explore association between clinical/anthropometric parameters and DOE. Statistically significant parameters at multivariate analysis were incorporated into a nomogram. RESULTS:Sixty-five (21.2%) subjects were characterized by DOE. Variables associated with DOE at univariate analysis were male (p = 0.031), modified Mallampati Class (MMC) ≥ III (p < 0.001), smaller interincisor gap (p < 0.001), and larger neck circumference (p = 0.006). MMC, interincisor gap, and neck circumference were significant at multivariate analysis and were presented with a nomogram for creating the Pharyngoscore. CONCLUSIONS:The Pharyngoscore is a promising tool for calculating DOE probability before TORS.

OBJECTIVE:Pituitary carcinoma is a rare tumor, defined as a tumor of adenohypophyseal cells with systemic or craniospinal metastasis. We present a case of a growth hormone (GH)-secreting pituitary carcinoma with a review of literature to better characterize this disease. DESIGN:Case report and literature review of 25 cases of GH-secreting pituitary carcinomas RESULTS: The age of diagnosis of GH-secreting carcinomas ranged 24-69 years old with a mean age of 44.4 with 52% of cases present in females. Mean latency period between diagnosis of acromegaly and transition to pituitary carcinoma was 11.4 years with mean survival being 3.4 years. CONCLUSION:Growth hormone (GH)-secreting pituitary carcinomas are rare and hard to distinguish from aggressive pituitary adenomas. From review of literature, treatment options include debulking surgery, radiotherapy, or chemotherapy with dismal outcomes. There are no diagnostic markers or features which can predict metastatic progression of these tumors. Future studies with genomic landscapes and relevant tumor markers are needed to identify pituitary tumors most likely to metastasize.

OBJECTIVES/HYPOTHESIS/OBJECTIVE:To document the history of hearing seeing in children and adults. STUDY DESIGN/METHODS:A literature search in all languages was carried out with the terms of hearing screening from the following sources: Pub Med, Science Direct, World Catalog, Index Medicus, Google scholar, Google Books, National Library of Medicine, Welcome historical library and The Library of Congress. METHODS:The primary sources consisting of books, scientific reports, public documents, governmental reports, and other written material were analyzed to document the history of hearing screening. RESULTS:The concept of screening for medical conditions that, when found, could influence some form of the outcome of the malady came about during the end of 19th century. The first applications of screening were to circumscribe populations, schoolchildren, military personnel, and railroad employees. During the first half of the 20th century, screening programs were extended to similar populations and were able to be expanded on the basis of the improved technology of hearing testing. The concept of universal screening was first applied to the inborn errors of metabolism of newborn infants and particularly the assessment of phenylketonuria in 1963 by Guthrie and Susi. A limited use of this technique has been the detection of genes resulting in hearing loss. The use of a form of hearing testing either observational or physiological as a screen for all newborns was first articulated by Larry Fisch in 1957 and by the end of the 20th century newborn infant screening for hearing loss became the standard almost every nation worldwide. CONCLUSIONS:Hearing screening for newborn infants is utilized worldwide, schoolchildren less so and for adults many industrial workers and military service undergo hearing screening, but this is not a general practice for screening the elderly. LEVEL OF EVIDENCE/METHODS:NA Laryngoscope, 2021.

OBJECTIVES/HYPOTHESIS/OBJECTIVE:Vocal fold fibrosis remains a significant clinical challenge. Estrogens, steroid hormones predominantly responsible for secondary sexual characteristics in women, have been shown to alter wound healing and limit fibrosis, but the effects on vocal fold fibrosis are unknown. We sought to elucidate the expression of estrogen receptors and the effects of estrogens on TGF-β1 signaling in rat vocal fold fibroblasts (VFFs). STUDY DESIGN/METHODS:In vitro. METHODS: M) were employed as antagonists of ERα or GPR30, respectively. qPCR was employed to determine estrogen receptor-mediated effects of E2 on genes related to fibrosis. RESULTS: M) and TGF-β1 significantly increased Smad7 (P = .03) and decreased Col1a1 (P = .04) compared to TGF-β1 alone; this response was negated by the combination of ICI and G36 (P = .009). CONCLUSIONS:E2 regulated TGF-β1/Smad signaling via estrogen receptors in VFFs. These findings provide insight into potential mechanisms of estrogens on vocal fold injury with the goal of enhanced therapeutics for vocal fold fibrosis. LEVEL OF EVIDENCE/METHODS:NA. Laryngoscope, 2020.

Initial diagnosis of peripheral vestibulopathy requires a detailed history, physical examination, and, in some cases, audiovestibular testing, radiographic imaging, or serology. Differentiation of a peripheral vestibulopathy as progressive or degenerative is often nuanced and influenced by a characterization of a patient's symptoms or natural history over time. A diverse group of vestibular pathology may fit into this category, including Ménière's disease, autoimmune conditions, congenital pathologies, ototoxic medications, radiation therapy, and perilymphatic fistula. Differentiation among these entities may be guided by initial or subsequent symptomatology, with various combinations of audiovestibular testing, serology, and imaging. Treatment options are disparate and disease-specific, ranging from observation to medical management or surgical intervention, underscoring the need for astute investigation and diagnosis.

Binaural unmasking, a key feature of normal binaural hearing, can refer to the improved intelligibility of masked speech by adding masking that facilitates perceived separation of target and masker. A question relevant for cochlear implant users with single-sided deafness (SSD-CI) is whether binaural unmasking can still be achieved if the additional masking is spectrally degraded and shifted. CIs restore some aspects of binaural hearing to these listeners, although binaural unmasking remains limited. Notably, these listeners may experience a mismatch between the frequency information perceived through the CI and that perceived by their normal hearing ear. Employing acoustic simulations of SSD-CI with normal hearing listeners, the present study confirms a previous simulation study that binaural unmasking is severely limited when interaural frequency mismatch between the input frequency range and simulated place of stimulation exceeds 1-2 mm. The present study also shows that binaural unmasking is largely retained when the input frequency range is adjusted to match simulated place of stimulation, even at the expense of removing low-frequency information. This result bears implications for the mechanisms driving the type of binaural unmasking of the present study and for mapping the frequency range of the CI speech processor in SSD-CI users.