Tensor Tympani and Stapedial Tendon Reconstruction for Postoperative Hyperacusis after Tendon Lysis for Middle Ear Myoclonus.

OBJECTIVE: To present a method for repair of the stapedial and tensor tympani tendons in a patient with hyperacusis after a tendon lysis procedure. PATIENTS: A 71-year-old professional musician who presented to clinic with debilitating hyperacusis following a tensor tympani and stapedial tendon lysis procedure to treat middle ear myoclonus. INTERVENTIONS: A novel procedure for reapproximation of the tensor tympani and stapedial tendons into their native insertion points using periosteal grafts and nitinol wire. MAIN OUTCOMES MEASURES: Stapedial reflex measurements, uncomfortable loudness level, and subjective patient experience. RESULTS: Postoperatively, the patient had objective improvement in hyperacusis with return of acoustic reflexes in the affected ear and durable improvements in their frequency-specific uncomfortable loudness levels. CONCLUSIONS: This case describes the debilitating complication of hyperacusis following tendon lysis and highlights the importance of maximizing behavioral and medical measures prior to undergoing surgical intervention for middle ear myoclonus.

Coordinated Same- or Next-Day Radiosurgery and Cochlear Implantation for Vestibular Schwannoma.

OBJECTIVE: To describe the experience and results from coordinated and closely scheduled radiosurgery and cochlear implantation (CI) in a vestibular schwannoma (VS) cohort. PATIENTS: Patients with VS who underwent radiosurgery followed by CI on the same or next day. INTERVENTIONS: Interventions included sequential radiosurgery and CI. MAIN OUTCOME MEASURES: Tumor control defined by tumor growth on posttreatment surveillance and audiometric outcomes including consonant-nucleus-consonant words and AzBio sentences in quiet. RESULTS: In total, six patients were identified that met the inclusion criteria, with an age range of 38 to 69 years and tumor sizes ranging from 2.0 to 16.3 mm. All patients successfully underwent radiosurgery and CI on the same or immediately successive day. Postoperatively, all patients obtained open-set speech recognition. Consonant-nucleus-consonant word scores ranged from 40 to 88% correct, and AzBio scores ranged from 44 to 94% correct. During posttreatment magnetic resonance imaging surveillance, which ranged from 12 to 68 months, all tumors were noted to be adequately visualized, and no tumor progression was noted. CONCLUSION: Coordinated radiosurgery and CI can be safely performed in patients with VS on the same or next day, serving to decrease burden on patients and increase access to this vital rehabilitative strategy.

Anatomic Step-by-Step Dissection of Complex Skull Base Approaches for Trainees: Surgical Anatomy of the Infratemporal Fossa Approach to the Jugular Foramen.

BACKGROUND AND OBJECTIVES: The infratemporal fossa (ITF) is a complex region bounded by the temporal bone, maxilla, sphenoid, pterygoid plates, and mandibular ramus. Containing a high density of neurovascular and musculoskeletal structures, the ITF can house a number of pathologies, and access is challenging. The ITF approach and its variations can be challenging due to complex anatomy and unfamiliarity by many surgeons. The objective of this study was to present a step-by-step 3-dimensional anatomic dissection for the classic Fisch Type A and modified ITF approach from the surgeon's perspective. METHODS: Six sides of 3 formalin-fixed latex-injected specimens were dissected under microscopic magnification (JRD and AMN). Standard Fisch Type A and modified ITF approaches were performed on contralateral sides of each specimen. Representative high-quality 3-dimensional photography was performed for each key step. RESULTS: The ITF approach affords excellent access to the posterior ITF and jugular foramen. Modifications to this approach include preservation of the ear canal and limiting facial nerve transposition, thus limiting morbidity while generally still providing sufficient access to key anatomic structures. CONCLUSION: The ITF approach provides access to the lateral skull base for jugular foramen paraganglioma and other lesions. Modifications of the classic Fisch Type A technique can be used to access pathologies in this region without sacrificing conductive hearing or facial nerve function. Three dimensional operatively oriented neuroanatomy dissections provide surgeons with a valuable resource for learning this complex surgical approach.

Risk of Monopolar Electrosurgery in Cochlear Implant Recipients is Nominal: Evidence to Guide Clinical Practice.

OBJECTIVE: Comprehensively assess the prevalence of monopolar electrosurgery-related device complications among cochlear implant (CI) recipients. STUDY DESIGN: Multifaceted retrospective review and survey. SETTING: Tertiary medical center. METHODS: Multifaceted approach including: (i) review of the current literature; (ii) historical review of institutional data from an academic, tertiary CI center; (iii) review of industry data provided by 3 Food and Drug Administration-approved CI manufacturers; and (iv) survey of high-volume CI centers. RESULTS: Literature review identified 9 human studies, detailing 84 devices with 199 episodes of device-cautery exposure. From studies reporting on patients records, no implant showed evidence of damage after exposure. One cadaveric study using dental cautery reported 1 episode of device damage. Review of institutional records did not identify any CI damage in 84 instances of exposure. Data from the 3 major implant manufacturers showed a single report of damage that could be reasonably linked to monopolar electrosurgery, out of a possible 689,426 CIs. Last, a survey of 8 high-volume CI centers did not identify any adverse events associated with monopolar cautery. CONCLUSION: These data estimate the risk of adverse device-related events or tissue injury to be extraordinarily low. Short of operating in immediate proximity to the CI (ie, the ipsilateral temporoparietal scalp), these data indicate that monopolar electrosurgery can be used in the body and the head-and-neck of CI recipients with nominal risk. These findings may guide decision-making in cases that are optimally or preferably performed with monopolar electrocautery and can be used to counsel CI patients following inadvertent exposures.

Comparing Speech Recognition Outcomes Between Cochlear Implants and Auditory Brainstem Implants in Patients With NF2-Related Schwannomatosis.

OBJECTIVE: To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. INTERVENTIONS: CI or ABI ipsilateral to vestibular schwannoma. MAIN OUTCOME MEASURES: Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. RESULTS: Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12-87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0-52%) and 57% (IQR, 5-83%), respectively, for CIs, compared with 0% (IQR, 0-0%) and 0% (IQR, 0-0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. CONCLUSION: Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.

Third window lesions of the inner ear: A pictorial review.

PURPOSE: Radiographic review of pathologies that associate with third window syndrome. METHODS: Case series and literature review. RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct. CONCLUSION: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Anatomic Variants in the Anterior Inferior Cerebellar Artery Encountered During Resection of Vestibular Schwannomas.

BACKGROUND AND OBJECTIVES: Vestibular schwannomas (VS) are often phenotypically benign lesions that may be technically challenging to resect because of involvement of neurovascular structures. The anterior inferior cerebellar artery (AICA) is commonly identified near VS, with variable position in relation to the tumor; however, little published literature describes anatomic and pathologic variants of AICA observed during VS resection. METHODS: A prospectively maintained cohort of surgically managed VS with available operative reports and clinical/radiographic follow-up was queried and reviewed for noted aberrations. RESULTS: We identified 66 cases with noted AICA abnormalities among 880 reviewer cases, including 20 loops extending into the internal auditory canal (2.3%), 18 arteries embedded in dura (2.0%), 15 AICA branches directly within VS (1.7%), 8 main trunk arteries coursing between cranial nerves 7 and 8 (0.9%), 3 arteries embedded in temporal bone (0.2%), 1 aneurysm (0.1%), and 1 artery bifurcating cranial nerve 6 (0.1%). The median age of AICA-variant patients was 55 years (range 19-74), and 29 were female (45%). Compared with the other AICA variants, tumors embedded with AICA tended to be larger lesions on maximal axial diameter (2.9 vs 1.6 cm; P = .006), they more commonly underwent less than total resection (73% vs 28%; P = .0001), and they had higher rates postoperative House-Brackmann scores >2 (47% vs 20%; P = .005). Two patients had radiographic and symptomatic postoperative cerebral ischemia or hemorrhage-1 from a bone-encased AICA and 1 from a dural embedded variant. CONCLUSION: Anatomic variants of AICA occur in approximately 7% of VS operations. Most aberrations do not affect surgical or clinical outcomes, and the rate of major vascular injury was low. However, certain types variably add operative time and in the case of AICA encasement in the tumor, likely indicate a more aggressive tumor phenotype with lower rates of gross total resection and high incidences of facial nerve weakness.

Initial Experiences with the Envoy Acclaim® Fully Implanted Cochlear Implant.

Introduction: Cochlear implantation has become the standard of care for the treatment of moderate-to-profound bilateral sensorineural hearing loss. However, current technologies, all of which rely on an external sound processor, have intrinsic limitations that prevent certain activities and diagnostics, thus hampering full integration into a patient's lifestyle. The Envoy Medical (White Bear Lake, MN, USA) Acclaim® fully implanted cochlear implant is a new device currently undergoing testing that has been designed to alleviate many of the current constraints by housing all components within the patient, thus allowing for near-constant use in many environments that are not conducive to a traditional cochlear implant. Methods: As part of an Early Feasibility Study, three adult implant candidates were implanted with the Acclaim® cochlear implant. Surgical video and photography were taken, and initial observations were recorded. Implantation with the Acclaim® device is largely similar to a traditional cochlear implant, with modifications to allow room for the implanted sensor as well as the implantation of a battery in the subcutaneous tissues of the chest. Results: This study demonstrates a step-by-step overview of implanting the Acclaim® and discusses initial insight and experiences with the first three implantations with this new device. Conclusions: All three surgeries proceeded without complication, and at activation, all three patients were hearing through their devices. Surgery is more technically challenging compared to a standard cochlear implant, but the skills needed can all be mastered by a dedicated otologic surgeon.

Cochlear Implant Outcomes between Patients with Sporadic and Neurofibromatosis Type 2-Associated Vestibular Schwannoma.

OBJECTIVE: Compare cochlear implant (CI) performance between patients with ipsilateral sporadic vestibular schwannoma (VS) and NF2-related schwannomatosis (NF2). Compare CI performance according to VS management modality. STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: Forty-nine patients (52 ears) undergoing cochlear implantation in the setting of ipsilateral sporadic (n = 21) or NF2-associated VS (n = 28). INTERVENTIONS: CI ipsilateral to VS. MAIN OUTCOME MEASURES: Auditory thresholds, consonant-nucleus-consonant (CNC) word scores, and AzBio sentences in quiet scores. RESULTS: Among all patients, median post-CI pure tone average was 28 dB HL (interquartile range [IQR], 21-38), CNC word score was 39% (IQR, 6-62), and AzBio sentences in quiet score was 60% (IQR, 11-83) at a median of 12.5 months postimplantation. Despite the NF2 cohort having larger tumors, when comparing patients with sporadic versus NF2-associated VS, there were no statistically significant differences in CNC word (49% [30-70] vs. 31% [0-52]) or AzBio sentences in quiet (66% [28-80] vs. 57% [5-83]) scores. Regardless of NF2 status, all patients managed with observation, and radiosurgery achieved open-set speech. In patients who underwent microsurgery, 6 (46%) of 13 with NF2 achieved open-set speech recognition compared with 4 (67%) of 6 with sporadic disease. CONCLUSION: Select patients with VS achieve successful hearing rehabilitation with a CI. In this cohort, tumor management strategy significantly influenced CI performance, whereas differences in NF2 status exhibited less effect. Specifically, all patients managed with observation or radiosurgery achieved open-set speech perception, whereas approximately half of people with NF2-related VS and two-thirds of people with sporadic VS achieved this outcome after tumor microsurgery. When disease permits, observation and radiosurgery should be considered in patients who may later pursue a CI.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Individual Differences in Speech Recognition Changes After Cochlear Implantation.

Importance: Cochlear implantation is highly effective at improving hearing outcomes, but results have been limited to groupwise analysis. That is, limited data are available for individual patients that report comparisons of preoperative aided speech recognition and postimplantation speech recognition. Objective: To assess changes in preoperative aided vs postoperative speech recognition scores for individual patients receiving cochlear implants when considering the measurement error for each speech recognition test. Design, Setting, and Participants: This cross-sectional study used a prospectively maintained database of patients who received cochlear implants between January 1, 2012, and December 31, 2017, at a tertiary, university-based referral center. Adults with bilateral sensorineural hearing loss undergoing cochlear implantation with 6- or 12-month postoperative measures using 1 or more speech recognition tests were studied. Exposures: Cochlear implantation. Main Outcomes and Measures: Postoperative word recognition (consonant-nucleus-consonant word test), sentence recognition (AzBio sentences in quiet), and sentence recognition in noise (AzBio sentences in +10-dB signal-to-noise ratio) scores, and association of each speech recognition score change with aided preoperative score to each test's measurement error. Results: Analysis of data from a total of 470 implants from 323 patients included 253 male (53.8%) patients; the mean (SD) age was 61.2 (18.3) years. Most patients had statistically significant improvement in all speech recognition tests postoperatively beyond measurement error, including 262 (84.8%) for word recognition, 226 (87.6%) for sentence recognition, and 33 (78.6%) for sentence recognition in noise. A small number of patients had equivalent preoperative and postoperative scores, including 45 (14.5%) for word recognition, 28 (10.9%) for sentence recognition, and 9 (21.4%) for sentence recognition in noise. Four patients (1.6%) had significantly poorer scores in sentence recognition after implantation. The associations between age at implantation and change in speech recognition scores were -0.12 (95% CI, -0.23 to -0.01) for word recognition, -0.22 (95% CI, -0.34 to -0.10) for sentence recognition, and -0.10 (95% CI, -0.39 to 0.21) for sentence recognition in noise. Patients with no significant improvement were similarly distributed between all preoperative aided speech scores for word recognition (range, 0%-58%) and sentence recognition (range, 0%-56%) testing. Conclusions and Relevance: In this cross-sectional study, with respect to preoperative aided speech recognition, postoperative cochlear implant outcomes for individual patients were largely encouraging. However, improvements in scores for individual patients remained highly variable, which may not be adequately represented in groupwise analyses and reporting of mean scores. Presenting individual patient data from a large sample of individuals with cochlear implants provides a better understanding of individual differences in speech recognition outcomes and contributes to more complete interpretations of successful outcomes after cochlear implantation.

Factors implicated in response to treatment/prognosis of vestibular migraine.

PURPOSE: To identify patient factors that influence response to therapy in patients with vestibular migraines. METHODS: A retrospective cohort study was performed at a university-based tertiary medical center. PATIENTS: 47 patients evaluated for treatment of definite vestibular migraine, per the Barany Society criteria, from 2015 to 2019. INTERVENTIONS: A protocol of antidepressants, antiepileptics, beta blockers, and vestibular rehabilitation. Patients failing initial therapy received botulinum toxin per the PREEMPT protocol. Vestibular rehabilitation for motion desensitization in case of known vestibular dysfunction. OUTCOME MEASURES: Quality of life measured per the dizziness handicap inventory (DHI). Pre- and post-treatment DHI scores (total and domain scores) and change in DHI were correlated against patient-specific variables to determine factors associated with change in response to therapy. Patient factors included demographic variables, medical comorbidities, comorbid otologic or pain symptoms, treatment modality, and initial DHI scores. RESULTS: 47 patients underwent therapy for vestibular migraine. This population had a significant DHI reduction of 17.3 ± 25.2 (p < 0.001) with therapy. Univariate analysis showed that female gender, comorbid benign paroxysmal positional vertigo, and high initial DHI were significantly associated with greater reduction in DHI scores (ß = - 7.92, p = 0.033; ß = - 18.65, p = 0.028; ß = - 0.458, p = 0.016, respectively). Conversely, cervicalgia and oscillopsia were significantly associated with a lower reduction in DHI scores (ß = 5.525, p = 0.024 and ß = 21.48, p = 0.027, respectively). CONCLUSIONS: Vestibular migraine is a complex disorder with heterogeneous response to therapy. This study shows that patient-specific factors of gender, cervicalgia, oscillopsia, BPPV, and high DHI scores on presentation may influence response to common vestibular migraine therapy.

Vitamin D Deficiency, Hypocalcemia, and Hearing Loss in Children.

OBJECTIVE: Characterize relations between vitamin D deficiency (VDD), hypocalcemia, and hearing loss (HL) in children. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral hospital. PATIENTS: Children in the Audiological and Genetic Database with a diagnosis of VDD, rickets, or osteomalacia. INTERVENTION: None. MAIN OUTCOME MEASURES: Prevalence, type, severity (4-tone pure-tone average, PTA), and progression of HL. HL was defined as greater than 15 dB HL at any threshold by pure tone, greater than 20 dB HL by sound field audiometry, or greater than 25 dB in infants less than 1 year of age. RESULTS: Of 888 children with VDD, 474 (53.4%) had HL, with 17% having moderate-profound HL. Compared with an age-matched cohort of 13,320 children drawn from the same database, children with VDD were significantly more likely to have sensorineural HL (SNHL) (adjusted odds ratios [aOR] 1.26 [95% confidence interval [CI] 1.01-1.58]). Among children with VDD, children with femur fracture had a significantly higher rate of HL (81% versus 53%, p = 0.008) and children with hypocalcemia had a significantly higher rate of moderate-profound HL (36% versus 18%, p = 0.016). Additionally, hypocalcemia with and without VDD was associated with SNHL (aOR 2.30 [1.07-4.56]). CONCLUSIONS: Both vitamin D deficiency and hypocalcemia were found to be independently associated with SNHL, a type of HL that is less likely to improve over time. Recognition of VDD and hypocalcemia as independent risk factors for the development of SNHL could allow for better evaluation and treatment of this patient population. Routine audiological evaluation should be considered in this population.

Association of Patient-Related Factors With Adult Cochlear Implant Speech Recognition Outcomes: A Meta-analysis.

Importance: Multiple studies have evaluated associations between post-cochlear implant (CI) speech recognition outcomes and patient-related factors. Current literature often appears equivocal or contradictory, so little is known about the factors that contribute to successful speech recognition outcomes with CIs. Objective: To use a meta-analysis to pool data from the extant literature and provide an objective summary of existing evidence on associations of patient-related factors and CI speech recognition outcomes. Data Sources: A literature search was performed using PubMed, Scopus, and CINAHL databases in January 2019 using the following search terms: cochlear implant or cochlear implants or cochlear implantation and speech recognition or word recognition or sentence recognition. Studies of postlingually deafened adult CI recipients that reported word or sentence recognition scores were included. Study Selection: Inclusion criteria were postlingual adult CI recipients 18 years or older with word or sentence recognition scores at minimum 6-month postimplantation. Studies that included patients undergoing revision or reimplantation surgery were excluded. Data Extraction and Synthesis: Following the Preferred Reporting Items for Systemic Reviews and Meta-analyses (PRISMA) guidelines, 1809 unique articles underwent review by abstract, and 121 articles underwent full-text review, resulting in 13 articles of 1095 patients for a meta-analysis of correlations. Random-effects model was used when the heterogeneity test yielded a low P value (P < .05). Main Outcomes and Measures: The planned primary outcome was the pooled correlation values between postimplant speech recognition scores and patient-related factors. Results: Of the 1095 patients included from the 13 studies, the mean age at implantation ranged from 51.2 to 63.7 years and the mean duration of hearing loss ranged from 9.5 to 31.8 years; for the 825 patients for whom sex was reported, 421 (51.0%) were women. A weak negative correlation was observed between age at implantation and postimplant sentence recognition in quiet (r = -0.31 [95% CI, -0.41 to -0.20]). Other correlations between patient-related factors and postimplant word or sentence recognition were statistically significant, but all correlations were absent to negligible (r = 0.02-0.27). Conclusions and Relevance: Given that most associations were weak, negligible, or absent, patient-related factors often thought to affect CI speech recognition ability offer limited assistance in clinical decision-making in cochlear implantation. Additional research is needed to identify patient-related and other factors that predict CI outcomes, including speech recognition and other important variables related to success with CIs.

When to replace legacy cochlear implants for technological upgrades: Indications and outcomes.

OBJECTIVE: To determine indications, surgical efficacy, and audiologic outcomes of replacing Advanced Bionics Clarion C1.2 internal devices (Advanced Bionics, LLC, Valencia, CA) as a means of technology upgrade. STUDY DESIGN: Retrospective review, case series. METHODS: Ten patients were initially implanted as a child (mean age = 3.87 years) and underwent cochlear implant reimplantation (CIR) with current Advanced Bionics internal device as a young adult (mean duration of implant use = 15.66 years). Demographic data and pre- and post-CIR speech perception scores were collected. RESULTS: Technology upgrade was the primary (9) or secondary (1) motivation for CIR. No surgical complications were noted, and full insertion was obtained in nine cases. Intraoperative impedance levels and neural response imaging measures were within normal limits for eight patients. At most recent post-CIR follow-up evaluation, all patients (100%) performed within or better than the 95% confidence interval of their pre-CIR word and sentence recognition scores; and 55.6%, 50.0%, and 50.0% of patients performed above the 95% confidence interval of their pre-CIR scores for the CNC words, sentences in quiet, and sentences in noise, respectively. CONCLUSION: Post-CIR audiological benefit was stable or improved compared to pre-CIR results in all categories by 3 months after reactivation. Given these results, patients who are unable to use the most current external processors due to incompatibility with a legacy internal device could consider reimplanation to optimize their overall performance with a cochlear implant. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:748-753, 2019.

Gene Expression Differences in Pediatric Lymphatic Malformations: Size Really Matters.

Lymphatic malformations (LMs) are congenital vascular anomalies characterized by dilated and cystic lymphatic channels. They are subdivided into macrocystic and microcystic lesions based upon the predominant size of the cysts involved. However, significant differences in clinical characteristics, treatment outcomes, and prognosis between macrocytic and microcytic disease suggest variation in underlying biologic and genetic influences. Indirect differential expression analysis revealed that 426 genes are significantly different (p < 0.01) in a small sample of LM subtypes. Functional analyses on the differentially expressed gene sets showed that microcystic LM gene expression favors a prooncogenic profile with upregulation of MYC target genes and cell cycle proteins, whereas macrocystic expression demonstrates hypoxic events that lead to angiogenesis and cell proliferation. Therefore, microcystic and macrocystic LMs, although histologically and physiologically similar, may occur under the influence of vastly different biological pathways and mechanisms of action.

The expression of renin-angiotensin-aldosterone axis components in infantile hemangioma tissue and the impact of propranolol treatment.

BackgroundPropranolol's mechanism of action for controlling infantile hemangioma (IH) remains unclear. We hypothesize that this nonselective beta antagonist downregulates renin-angiotensin-aldosterone (RAA) axis components, preventing angiogenic substrate induction of IH.MethodsIH tissue and serum were collected from children with propranolol-treated or -untreated IH during surgery. Normal skin and serum from demographically matched children were used as controls. Real-time PCR and western blot quantified RAA components in proliferative (n=10), involuting (n=10), propranolol-treated (n=12) IH, and normal specimens (n=11). Serum was analyzed by enzyme-linked immunosorbent assay (ELISA).ResultsThere were significantly greater messenger RNA (mRNA) levels of angiotensinogen (AGT) in proliferating IH, but not in involuting or treated IH, when compared with controls (P<0.05). Angiotensin-converting enzyme (ACE) and angiotensin II receptor 1 (AGTR1) mRNA expression was higher in all IH specimens when comparedwith controls (P<0.05). ACE and AGTR1 protein expression was greater in proliferating IH tissue compared with that in controls and in involuting and treated IH tissue (P<0.05). ELISA showed no significant difference in ACE serum levels but did show a significant reduction in renin in involuting compared with proliferating IH (P<0.05).ConclusionsThe protein and mRNA expression of several RAA pathway constituents is elevated in IH tissue when compared with that in normal tissue. The action of propranolol on IH may be the result of reductions in ACE and AGTR1.

Pediatric unilateral sensorineural hearing loss: implications and management.

PURPOSE OF REVIEW: The purpose of this review is to summarize current studies detailing the impact of unilateral sensorineural hearing loss in children and the most current modalities of treatment used in its management. RECENT FINDINGS: Current studies corroborate historic views on the impact of unilateral sensorineural hearing loss on patient wellbeing and academic success and stress the use of additional surveillance and studies to diagnose those patients that may pass standard screening practices and suffer from lack of prompt and proper care. With respect to management, notable findings include the continuous development of improved conventional and contralateral routing of signal amplification devices that may act to provide alternatives to percutaneous bone-anchored hearing aid implantation. These include improvements in more conventional hearing aid technology, so as to bridge the performance gap with the classical bone-anchored hearing aid implant, and the development of partially implanted transcutaneous bone conduction hearing devices. Due to dissatisfaction with sound localization, a new and significant development is the burgeoning accumulation of research on cochlear implantation for the treatment of unilateral sensorineural hearing loss in children. SUMMARY: With advances in technology in historic modalities of treatment, and the advent of new modalities such as cochlear implantation, the clinician has a wide armamentarium by which to provide treatment to patients based on clinical circumstances and patient desires.

The Association of Valsalva Status With Smoking and Its Impact on Ossiculoplasty Outcomes and Complications.

OBJECTIVES: To assess the association of a patient's ability to perform a clinically observed Valsalva maneuver with tobacco smoking and post-ossiculoplasty outcomes. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral, university-based otology practice. PATIENTS: Adult patients (16-88 years of age) undergoing ossiculoplasty with tympanoplasty or tympanomastoidectomy using cartilage tympanic membrane grafts, retrograde mastoidectomy with canal wall reconstruction, or mastoid obliteration techniques. OUTCOME MEASURES: Patients were classified as Valsalva negative (Val) or positive (Val). Comparisons were then made between these two groups for smoking status, final audiometric outcomes, rate of cure of conductive hearing loss, anatomic outcomes, and incidence of significant complications after otologic surgery involving ossiculoplasty. RESULTS: Tobacco smoking is not significantly correlated with the inability to insufflate the middle ear with the Valsalva maneuver (p = 0.557). An insignificant difference in final pure-tone average air-bone gap (PTA-ABG) was observed between the Val and Val groups (20.2 dB HL versus 18.2 dB HL, respectively; p = 0.152). Similarly, the rate of successful PTA-ABG closure to ≤20 dB HL was not significantly different between the groups (62.5% [80 of 128] of Val patients and 56.8% [54 of 95] of Val patients [p = 0.394]). Postoperative complications were seen with a higher frequency in the Val patients (16.8% [16 of 95] versus 7.0% [9 of 128]; p = 0.022). CONCLUSIONS: A patient's inability to perform the Valsalva maneuver is not significantly associated with smoking; however, negative Valsalva status is associated with an increased incidence of significant complications after ossiculoplasty.