Search

RespiCardia remedē System (Minnetonka, MN 2017), a transvenous phrenic nerve stimulator, is indicated to treat central sleep apnea (CSA) by stimulating the phrenic nerve to cause diaphragmatic contraction to restore normal breathing during sleep. CSA is associated with decreased patient quality of life and worsens cardiovascular outcomes. Systematic review was conducted according to the Preferred Reporting of Systematic Reviews and Meta-Analysis guidelines. PubMed/MEDLINE, Cochrane, EBSCO CINAHL, and Ovid databases were queried by 2 independent reviewers for English-language studies published between 2000 and 2021. The initial search screened for all occurrences of "remedē" then was further refined to include studies evaluating use of the RespiCardia remedē System as a treatment for CSA in multiple patients. A total of 124 articles were identified from the initial search results. A total of 37 articles were identified through screening of title and abstracts from initial results. Full-text review of all the articles was then completed. Of the 13 articles included, a total of 232 patients underwent device implantation. We sought to summarize the available evidence regarding patient selection for implantation, immediate and delayed complications, adherence to therapy, and polysomnographic evidence of efficacy. All 13 articles detailed significant decreases in central apnea index and many patients reported significant mild to marked improvement in quality of life. In conclusion, the remedē System has been demonstrated to improve sleep and respiratory parameters, with few complications. This device demonstrates safe and effective treatment of moderate to severe CSA in adult patients, including those with heart failure.

Maneuvers during drug-induced sleep endoscopy (DISE), for patients with obstructive sleep apnea (OSA), have been used as predictors for success with oral appliances. Hypoglossal nerve stimulation (HGNS) promotes opening at the velum through palatoglossus coupling. In this study, we evaluate maneuvers during DISE as predictors for HGNS treatment efficacy.We evaluated patients undergoing HGNS between November, 2014 and February, 2021. We assessed maneuvers including jaw thrust and chin lift during preoperative DISE. The impact of tongue base and palatal opening during these maneuvers were rated from 1 to 4 (1: no improvement, 2: mild improvement, 3: moderate improvement, 4: significant improvement). Patients were grouped by a score of 1-2 (weak response) or 3-4 (strong response). Apnea hypopnea index (AHI) change was calculated from the difference between preoperative and postoperative sleep study.One hundred and seventy one patients were included. With jaw thrust, there was no significant difference in AHI change between patients with weak or strong response at the palate (n = 68 vs. 94, 9.5 vs. 13.6, p = 0.21) or tongue base (n = 24 vs. 138, 16.6 vs. 11.3, p = 0.24). On chin lift, there was no significant difference at the palate (n = 82 vs. 18, 13.9 vs. 11.4, p = 0.63) or tongue base (n = 92 vs. 72, 10.8 vs. 14.6, p = 0.24). On multiple linear regression analysis, lower body mass index, higher preoperative AHI, and higher preoperative oxygen nadir were associated with a larger AHI change.Palatal coupling maneuvers during DISE are not predictive of AHI change with HGNS. Findings using these maneuvers should not preclude HGNS candidacy.Level III Laryngoscope, 2022.

Introduction: To determine if there is a recovery time difference between patients with and without obstructive sleep apnea (OSA) when using total intravenous anesthesia (TIVA) compared to volatile gas inhalational anesthesia. Patients and Methods: OSA and Non-OSA patients were identified at a tertiary institution between January 2019 and November 2020. Non-OSA patients were defined as those who have not been formerly diagnosed with OSA. A modified STOP-BANG score (MSBS) was performed to screen Non-OSA patients for OSA. Recovery was measured by Phase I recovery time, or time it took a patient to reach ≥9/10 on the Aldrete scoring system. Results: A total of 334 patients were included with 142 in the OSA cohort (59 TIVA, 83 inhalational anesthesia) and 192 in the Non-OSA cohort (119 TIVA, 73 inhalational anesthesia). In OSA patients, there was a 41.29-minute recovery time reduction when using TIVA versus sevoflurane ( P Conclusions: When utilizing TIVA over inhalational anesthesia, patients with OSA have significantly increased benefit in terms of reduced Phase I recovery times as compared to Non-OSA patients.

STUDY OBJECTIVES: The objective was to determine the prevalence and predictors of comorbid insomnia in patients presenting for sleep surgery evaluation. The insomnia severity index (ISI) was utilized to evaluate patients’ insomnia severity. METHODS: A retrospective chart review was performed in patients presenting to an otolaryngology sleep surgery clinic; patients also completed a sleep history questionnaire. Patients were divided between those with and without clinically significant insomnia defined as ISI ≥ 15. RESULTS: A total of 119 patients were included in the study: 50 (42%) with an ISI ≥ 15 and 69 (58%) with an ISI < 15. Clinically significant insomnia was associated with respiratory disturbance index (P = .028) but not apnea-hypopnea index or SaO(2) nadir (P > .05). Clinically significant insomnia was associated with frequency of wake ups (P = .008), time to fall back asleep (P = .049), history of continuous positive airway pressure device use (P = .012), Epworth Sleepiness Scale (P = .008), and Sino-nasal Outcome Test (SNOT-22) (P < .001). CONCLUSIONS: Patients reporting to a sleep surgery clinic are at an elevated risk for comorbid insomnia. The relationship between increased respiratory event–related arousals and nonsleep SNOT-22 scores to related sleep-maintenance insomnia supports the connection between insomnia, nasal obstruction, and continuous positive airway pressure intolerance. CITATION: Sagheer SH, Scott ER, Ananth A, Boon M, Huntley C. Incidence and predictors of comorbid insomnia in a sleep surgery clinic. J Clin Sleep Med. 2021;17(11):2165–2169.

OBJECTIVE To evaluate the effectiveness of neuromodulating agents for the management of atypical facial pain and primary facial neuralgias. METHODS We searched MEDLINE, Embase, CINAHL, and ClinicalTrials.gov databases for original research articles that examine the effectiveness and adverse reactions of pharmacologic therapy for the treatment of trigeminal neuralgia and atypical facial pain. Studies that included surgical interventions for atypical facial pain or facial pain secondary to other causes were excluded. Meta-analysis was conducted for reductions in symptom scores and adverse effects. RESULTS Of 3,409 articles screened, 73 full-text articles were included, consisting of 45 observational studies and 29 randomized controlled trials. Twenty-four different pharmacological agents were assessed; carbamazepine was the most frequently studied while botulinum toxin A demonstrated the highest consistency in reduction of symptom scores. Pooled estimate of three randomized controlled trials revealed that patients with trigeminal neuralgia who received botulinum toxin A had higher odds (odds ratio 7.46; 95% CI 3.53-15.78) of achieving a ≥50% reduction in visual analogue scale scores compared to controls. Pooled estimate of 15 observational studies showed that three-fourths of patients with trigeminal neuralgia who received carbamazepine experienced clinically significant pain reduction (prevalence proportion 0.75; 95% CI 0.66-0.83). CONCLUSIONS Patients receiving botulinum toxin A for trigeminal neuralgia had higher odds of achieving ≥50% reduction in pain scores. A significant proportion of patients with trigeminal neuralgia experienced positive response to carbamazepine. There was moderate evidence for amitriptyline in patients with atypical facial pain. Standardization of outcome reporting would facilitate future quantitative comparisons of therapeutic effectiveness. Laryngoscope, 131:1235-1253, 2021.

To evaluate patient-reported quality of life pertaining to gastroesophageal reflux disease symptoms in patients undergoing upper airway surgery for comorbid obstructive sleep apnea.A prospective survey-based study was conducted on patients with gastroesophageal reflux disease and comorbid obstructive sleep apnea receiving surgery from July 2020-December 2020. Patients completed the Gastroesophageal Reflux Disease-Health Related Quality of Life Questionnaire at two time-points: one week before surgery and at 6 months following surgery. Disease-related symptoms were rated from 0 (no symptoms) to 5 (incapacitating symptoms). Patient survey scores, demographics, medications, and sleep study parameters were collected for analysis. A p-value0.05 indicated statistical significance.Twenty-two patients completed the baseline preoperative and 6-month postoperative questionnaires. Median baseline vs. 6-month survey scores significantly decreased for symptoms including heartburn in general (3.0 vs. 2.0, p = 0.006), when lying down (2.5 vs. 1.5, p = 0.046), when standing (2.0 vs 1.0, p = 0.003), following meals (2.0 vs. 2.0, p = 0.042), and cumulative survey score (15.5 vs. 11.0, p = 0.029). Heartburn altering diet or sleep, odynophagia, dysphagia, and medication burden did not change following surgery (p 0.05). More patients were satisfied with their postoperative condition compared to baseline, however this did not reach statistical significance (40.9% vs. 18.2%, p = 0.18).Our results suggest that upper airway surgery to treat obstructive sleep apnea may have a positive impact on patient-reported symptoms of gastroesophageal reflux disease, and further investigation into the role of surgery in this setting for improvement of both quality of life and true clinical disease severity is merited.

Introduction Chiari malformations (CM) are congenital conditions defined by craniocervical junction anatomic anomalies with downward displacement of cerebellar structures. Sleep-disordered breathing (SDB) including obstructive sleep apnea (OSA), bradypnea, central sleep apnea (CSA), and hypoventilation are described in CM patients. Report of case(s) 31-year-old male with history of CM type 1 presented to the sleep medicine clinic for management of OSA and CSA diagnosed at age 16. PSG showed an apnea-hypopnea index (AHI) of 14.2, RDI of 29, and no central events. He was started on auto-CPAP with pressures of 5–20 with subsequent visits showing high residual AHI. A subsequent CPAP-titration study resulted in a pressure of 16cm H2O yielding AHI of 0. CPAP pressure was fixed, but a high residual AHI persisted despite excellent compliance. A split-night study resulted in a BiPAP prescription, for which a titration study noted PAP-emergent CSA. He was started on auto Bilevel with IPAP of 30, EPAP of 8 and backup rate of 12bpm. Despite treatment, elevated AHI persisted so he was switched to adaptive servo-ventilation (ASV) with nightly oxygen blended in resulting in controlled complex sleep apnea. SDB management was complicated by multiple neurosurgical decompressions. During the most recent procedure, he was found to have syringomyelia, syringobulbia, and a mass at the brainstem. Surgery, including placement of a 4th ventricle stent and mass excision, initially led to a decrease of residual AHI to a low of 0.7, before progressively increasing to 15. A repeat PSG demonstrated severe OSA (AHI of 35), without evidence of CSA or nocturnal hypoventilation. ASV was resumed and patient’s AHI progressively decreased to an average of 8, with improvement in his sleep apnea symptoms. Conclusion SDM in CM patients can be explained by condition-related anatomical changes and depression of respiratory centers due to possible extrinsic compression leading to complex sleep apnea presentations. While it is unclear why this patient’s sleep apnea improved then worsened after his latest decompression surgery, we believe that CSF recirculation and postoperative inflammation may be responsible. Close monitoring of SDB in patients with CM is important as they may require advanced therapies for proper control. Support (if any)

Objective Angioedema is a potentially life-threatening complication of angiotensin-converting enzyme inhibitor (ACEI) use, occurring in up to 0.5% of users. Although the pathophysiology of ACEI-induced angioedema is attributable to elevated serum bradykinin, standard management typically includes corticosteroids and antihistamines. We sought to summarize the evidence supporting pharmacotherapy for ACEI-induced angioedema. Data Sources PubMed, MEDLINE, and Embase portals. Methods A systematic literature review was conducted according to the PRISMA guidelines. Databases were queried by 3 independent reviewers for English-language studies published between 1980 and 2017. The initial search screened for all occurrences of "angioedema" and then was further refined to include studies of ACEI-related cases and exclude hereditary angioedema. Results Five articles representing 218 cases were identified, including 3 randomized controlled trials and 2 prospective case series with historical controls. One of 2 studies of icatibant (bradykinin B2 receptor antagonist) found more rapid symptom improvement than that with a control group of corticosteroids and antihistamines. Two studies of ecallantide (plasma kallikrein inhibitor) and 1 study of C1 inhibitor replacement found no significant benefit over control. No studies were identified that compared the efficacy of corticosteroids with antihistamines, of one dose with another, of fresh frozen plasma, or of combination therapy. Conclusion The efficacy of treatment of ACEI-induced angioedema with bradykinin antagonists, kallikrein inhibitor, and C1 inhibitor warrants further study. Although consistent benefit of these medications has not been demonstrated, their use has not caused harm. One study examining off-label use of icatibant has demonstrated efficacy over control. In addition, further study is needed to establish the efficacy and mechanism of action of standard pharmacotherapy such as corticosteroids and antihistamines in treatment of this condition.

Neonatal nasal septal deviations can occur as a result of trauma in utero or during birth. They are associated with significant clinical implications, including respiratory distress and failure to thrive. The incidence and classification of these deformities are varied in the otolaryngology literature. Some authors advocate conservative observation, but no consensus on management has been reached. We present 2 cases of neonatal septal deviation that occurred during difficult deliveries. We repaired the defects with closed reduction in the operating room within the first 2 weeks of life to good effect.

Introduction Sleep disruption is common among hospitalized patients due to psychological, physiological, and environmental reasons including illness, pain, anxiety, invasive interventions, frequent monitoring, and stimuli, especially noise and light. The AASM has published guidelines for the use of actigraphy in the outpatient setting, but there is a paucity of literature evaluating the validity of actigraphy in inpatients. We sought to summarize the evidence surrounding the use of actigraphy for inpatient sleep evaluation. Methods Systematic review was conducted according to the Preferred Reporting of Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Databases were queried by two independent reviewers for English-language studies published between 1990 and 2020. The initial search screened for all occurrences of “actigraphy AND sleep AND hospital” then was further refined to include studies of actigraphy used for monitoring in the inpatient hospital setting and exclude studies evaluating actigraphy in outpatient, rehabilitation, immediate postoperative, or intensive care unit settings. Results 1221 were screened from initial search results. 48 articles were identified through screening of abstracts. Full-text review of the articles was then completed. Of the 48 articles, a total of 12 studies examined general medical inpatients, 12 studies examined inpatients with neurologic disorders, 5 studies examined inpatients with cancer, 6 studies examined patients with mental illness, 9 studies examined elderly patients, and 4 studies examined other defined populations (pregnancy, trauma, liver transplantation, and hip arthroplasty). We summarize the qualitative findings of inpatient actigraphy as it relates to each of these populations. Commonly reported outcome measures were total sleep time (TST), number of nighttime awakenings, and concordance with polysomnography (PSG). Conclusion We summarize the existing evidence for the use of actigraphy in the inpatient setting. Actigraphy may provide a simple and effective method for screening of sleep disorders in the inpatient setting. With regard to the published literature, there is support for the use of actigraphy in the inpatient setting in certain patient populations, especially traumatic brain injury. Variation in data output of actigraphy devices and outcome measures presents a barrier to meta-anaylsis of pooled data. Standardization of outcome measures will allow for effective synthesis of future studies. Support (if any)

Introduction Sleep disruption is common among hospitalized patients due to psychological, physiological, and environmental reasons including illness, pain, anxiety, invasive interventions, frequent monitoring, and stimuli, especially noise and light. The AASM has published guidelines for the use of actigraphy in the outpatient setting, but there is a paucity of literature evaluating the validity of actigraphy in inpatients. The aim of this study is to evaluate sleep in hospitalized general medicine patients undergoing sleep medicine consultation using actigraphy and qualitative surveys. Methods A single-site prospective study in hospitalized medicine patients. Patients were observed with a Fitbit® Charge3 wrist actigraphy device overnight, then administered 7 surveys: Richards-Campbell Sleep Questionnaire (RCSQ), qualitative questionnaires assessing sleep history, sleep hygiene, barriers to sleep, STOP-BANG, Epworth Sleepiness Scale (ESS), and Patient-Health Questionniare-2 (PHQ-2). Actigraphy data including total sleep time, slow wave sleep time, and number of awakenings was compared with patient-reported data. Results In preliminary analysis, six patients met inclusion criteria and underwent sleep medicine consultation, overnight actigraphy, and completed 7 surveys. Based on subjective sleep history questionnaires, average total sleep time was 437 + 215 minutes. Actigraphy revealed average total sleep time was 228 + 80 minutes with an average of 3.6 nocturnal awakenings. Increased number of awakenings on actigraphy was not correlated with increased number of awakenings by survey. The most frequently reported barriers to sleep on patient surveys were pain and being woken up for labs or vital signs. The average STOP-BANG score was 6 out of 8 and average ESS was 14 out of 24. Conclusion Restorative sleep warrants consideration alongside complex medical care during hospitalization. Patients experience decreased total sleep time and increased number of awakenings while in the hospital compared with their subjective estimates of sleep at home. Actigraphy provides a non-invasive and reliable way to monitor some sleep parameters in the inpatient setting. An elevated STOP-BANG score could represent sleep disordered breathing and impact perceptions of sleep quality. Patient-identified barriers to sleep are targets for quality improvement. Future studies should compare inpatient actigraphy data to polysomnographic data and the effect of sleep-directed interventions on sleep quality in the hospital. Support (if any)

Introduction The remedē® system is a transvenous phrenic nerve stimulator used to treat central sleep apnea (CSA). It is a safe and effective implantable device that has demonstrated significant improvements in objective sleep and quality of life. While complex sleep apnea represents development of central sleep apnea during treatment with continuous positive airway pressure (CPAP) for obstructive sleep apnea, and has been well-described in the literature, we present a novel case of predominantly central sleep apnea treated with phrenic nerve stimulation with augmentation of obstructive events thereafter. This patient is, at the time of submission, being evaluated for hypoglossal nerve stimulation. Report of case(s) A 63-year-old male patient with a past medical history of hypertension, atrial fibrillation, asthma, chronic obstructive pulmonary disease, GERD, obesity, and prior pulmonary embolism presented to the sleep medicine clinic with complaints of difficulty initiating and maintaining sleep, snoring, multiple nocturnal awakenings, excessive daytime sleepiness, and physical fatigue. On exam, BMI was 30.4, and airway classification was Mallampati 4. Split night polysomnography revealed severe mixed sleep apnea with an overall apnea hypopnea index (AHI) of 58.6 and a central AHI of 53.8 per hour after successful PAP titration at 7 cm H2O. He underwent phrenic nerve stimulator implantation and activation without complications. Phrenic nerve stimulation titration study four months after activation showed a normal central AHI of 4.6 per hour at a therapeutic voltage of 1.6 to 1.9mA, but significantly worse OSA with obstructive AHI of 53.1. In follow up, he reported intolerance of CPAP due to significant nasal congestion as well as asthma. Presently, he is scheduled for drug induced sleep endoscopy to visualize airway obstruction and is being evaluated for Inspire hypoglossal nerve stimulation and nasal surgery. Conclusion The treatment of sleep apnea has recently evolved rapidly with the development of implantable devices to treat both central and obstructive sleep apnea. We present a novel case of the first known patient to be considered for dual nerve stimulation for sleep apnea. Further study needs to be done to determine the safety and efficacy of concurrent implantable devices for the simultaneous treatment of OSA and CSA. Support (if any)

Introduction RespiCardia™ remedē® System, a transvenous phrenic nerve stimulator, is indicated to treat central sleep apnea (CSA) in certain patients. CSA involves disruption of the normal breathing pattern during sleep; CSA is associated with decreased patient quality of life and worsens cardiovascular outcomes. Existing therapies for CSA are often complicated by poor patient adherence to therapy and occasional adverse effects. The remedē® System uses electrical stimulation of the phrenic nerve to cause diaphragmatic contraction and attempts to restore normal breathing during sleep. Methods Systematic review was conducted according to the Preferred Reporting of Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Databases were queried by two independent reviewers for English-language studies published between 2000 and 2020. The initial search screened for all occurrences of “remede” then was further refined to include studies evaluating use of the RespiCardia™ remedē® System as a treatment for CSA in multiple patients. Results Two hundred twenty-seven articles were identified from initial search results. Fourteen articles were identified through screening of title and abstracts from initial results. Seven additional articles were identified through reference review. Full-text review of all the articles was then completed. All articles were published after 2010. Of the 21 articles, a total of 1621 patients underwent device implantation. We sought to summarize the available evidence regarding patient selection for implantation, immediate and delayed complications, adherence to therapy, and polysomnographic evidence of efficacy. Conclusion The remedē® System has been demonstrated to improve sleep and respiratory parameters including AHI, CAI, arousal index, REM sleep, and ODI with few complications. This device proves to be a safe and effective treatment for moderate to severe CSA in adult patients, especially those with HF. Future studies examining long-term outcomes and delayed complications are needed. Support (if any)

To identify factors associated with efficient operating room work flow on high volume pediatric otolaryngology days and the effects on provider and perceived parent satisfaction.Retrospective review was performed of a sample of 20 days with greater than 10 cases per day performed by a pediatric otolaryngologist operating in 2 rooms. Turnover time and complications were the main outcome measures. Providers from otolaryngology and anesthesia that participated in these days were surveyed regarding efficiency, safety, and satisfaction.223 cases were performed over 20 operative days. The average turnover time was significantly longer in "major" surgeries (p=0.03), cases with multispecialty involvement (p=0.01), cases requiring intubation (p0.001), and in cases where a fellowship trained pediatric anesthesiologist (p=0.01) or CRNA was present (p0.001). When comparing "fast" (25min average turnover) operative days vs. "slow" (25min average turnover) days, presence of a non-fellowship trained anesthesiologist (p0.001), and the presence of an anesthesiology resident (p=0.03) were significantly associated with "fast" days, while the presence of a CRNA was associated with "slow" days (p0.001). A significantly greater proportion of patients required intubation on "slow" turnover days vs. "fast" days (p=0.13). Only one complication was observed (0.4%). 48 providers were surveyed with a 63% response rate. Reported satisfaction amongst providers was significantly greater on days with at least 10 cases (p=0.047) and on days with turnover times of 25min or less (p0.001). Pre-operative nursing evaluation/preparation of the patient, inter-provider communication and delays in room cleaning/setup were identified most often as causative factors responsible for delays in turnover.High-operative volume operating days are common in pediatric otolaryngology and can be safely performed in an efficient manner. Appropriate scheduling and high-level communication between providers is needed to ensure success on these days. Identified areas of potential inefficiency can be a starting point for work flow optimization practices.

Objectives/Hypothesis: The human cochlea is most commonly considered to have two and a half turns. Although the causes of cochlear hypoplasia are well described, cochlear hyperplasia is a rarer entity that is poorly understood. We describe rare anatomic cochlear malformations identified in a 4-month-old male originally referred for evaluation after a failed newborn hearing screening. The full diagnostic evaluation, imaging findings, treatment, and follow-up are described in detail. Cochleae with three turns are an uncommon malformation that is not included in current classifications schemes and may represent a distinct type of anomaly not caused by developmental arrest.

Human papillomavirus (HPV) is a preventable disease that plays a causative role in a significant proportion of malignant neoplasms of the head and neck. Inner-city populations are at risk for HPV-related oropharyngeal cancer, are least likely to receive HPV vaccination, and report a lack of information regarding HPV.To determine whether an educational platform affects knowledge, attitudes, and practices regarding HPV vaccination in an inner-city community.This prospective cohort study, conducted from March 1 to December 31, 2014, surveyed 128 participants at multiple inner-city community centers regarding their knowledge of, attitudes toward, and practices regarding HPV vaccination before and after a brief educational presentation. No eligible individuals refused to participate in the educational session. Surveys were excluded from analysis if they were incomplete.Participants completed two 20-question surveys separated by a 15-minute educational session on HPV-related disease, including a short PowerPoint presentation.Presence of statistically significant differences in survey scores before and after the educational session.Eighty-six participants met eligibility criteria (61 male [70.9%]; 68 with a high school education [79.1%]). Baseline knowledge of HPV, its causal association with cancer, and the existence of a vaccine against HPV were poor: of a total composite score of 20, the mean knowledge score before the educational session was 9.69. Participants' self-rated knowledge regarding HPV disease and vaccination improved significantly as a result of the educational session; the absolute increase in mean knowledge composite score from before the educational session to after the session was 3.52 (17.6%) (95% CI, -2.87 to 9.92; P .01). Attitudes regarding government involvement in vaccination did not change as a result of the educational session (composite attitudes score before the educational session, 16.57 of 28; score after the session, 15.22; P = .98). Participants' intent to vaccinate their children increased significantly following the educational presentation: before the presentation, 34 respondents (40%) intended to have their children vaccinated; after the presentation, 60 (70%) intended to do so (P = .002).Lack of knowledge regarding HPV vaccination and unwillingness to undergo vaccination contribute to low rates of HPV vaccination within urban populations. Community-based educational sessions successfully teach the link between HPV and various cancers, provide information regarding the risks and benefits of vaccination, and increase participants' willingness to vaccinate their children against HPV. Attitudes regarding government involvement in health programs are resistant to change.

Objectives/Hypothesis: The human cochlea is most commonly considered to have two and a half turns. Although the causes of cochlear hypoplasia are well described, cochlear hyperplasia is a rarer entity that is poorly understood. We describe rare anatomic cochlear malformations identified in a 4-month-old male originally referred for evaluation after a failed newborn hearing screening. The full diagnostic evaluation, imaging findings, treatment, and follow-up are described in detail. Cochleae with three turns are an uncommon malformation that is not included in current classifications schemes and may represent a distinct type of anomaly not caused by developmental arrest.

A biodegradable positron-emitting dendritic nanoprobe targeted at α v β 3 integrin, a biological marker known to modulate angiogenesis, was developed for the noninvasive imaging of angiogenesis. The nanoprobe has a modular multivalent core-shell architecture consisting of a biodegradable heterobifunctional dendritic core chemoselectively functionalized with heterobifunctional polyethylene oxide (PEO) chains that form a protective shell, which imparts biological stealth and dictates the pharmacokinetics. Each of the 8 branches of the dendritic core was functionalized for labeling with radiohalogens. Placement of radioactive moieties at the core was designed to prevent in vivo dehalogenation, a potential problem for radiohalogens in imaging and therapy. Targeting peptides of cyclic arginine–glycine–aspartic acid (RGD) motifs were installed at the terminal ends of the PEO chains to enhance their accessibility to α v β 3 integrin receptors. This nanoscale design enabled a 50-fold enhancement of the binding affinity to α v β 3 integrin receptors with respect to the monovalent RGD peptide alone, from 10.40 nM to 0.18 nM IC 50 . Cell-based assays of the 125 I-labeled dendritic nanoprobes using α v β 3 -positive cells showed a 6-fold increase in α v β 3 receptor-mediated endocytosis of the targeted nanoprobe compared with the nontargeted nanoprobe, whereas α v β 3 -negative cells showed no enhancement of cell uptake over time. In vivo biodistribution studies of 76 Br-labeled dendritic nanoprobes showed excellent bioavailability for the targeted and nontargeted nanoprobes. In vivo studies in a murine hindlimb ischemia model for angiogenesis revealed high specific accumulation of 76 Br-labeled dendritic nanoprobes targeted at α v β 3 integrins in angiogenic muscles, allowing highly selective imaging of this critically important process.