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Children with a history of temporary conductive hearing loss (CHL) during early development may show long-term impairments in auditory processes that persist after restoration of normal audiometric hearing thresholds. Tones in noise provide a simplified paradigm for studying hearing in noise. Prior research has shown that adults with sensorineural hearing loss may alter their listening strategy to use single-channel energy cues for tone-in-noise (TIN) detection rather than rove-resistant envelope or spectral profile cues. Our objective was to determine the effect of early CHL on TIN detection in healthy children compared to controls. Children ages 4-7 years, with and without a history of CHL due to otitis media with effusion (OME) before age 3 years, participated in a two-alternative forced choice TIN detection task. Audiometric thresholds were normal at the time of testing. Thresholds for detection of a 1000 Hz tone were measured in fixed-level noise and in roving-level noise that made single-channel energy cues unreliable. Participants included 23 controls and 23 with a history of OME-related CHL. TIN thresholds decreased with increasing age across participants. Children in both groups showed similar TIN sensitivity and little or no threshold elevation in the roving-level condition compared to fixed-level tracks, consistent with use of rove-resistant cues. In contrast to older listeners with sensorineural hearing loss, there was no detectable change in TIN sensitivity with roving level for children with a history of OME-related CHL.

Cystic fibrosis (CF) is a multisystem disease that often requires otolaryngology care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing loss and dysphonia. Given these manifestations of CF, otolaryngologists are frequently involved in the care of patients with CF; however, there is limited consensus on optimal management of sinonasal, otologic, and laryngologic symptoms.The Cystic Fibrosis Foundation convened a multidisciplinary team of otolaryngologists, pulmonologists, audiologists, pharmacists, a social worker, a nurse coordinator, a respiratory therapist, two adults with CF, and a caregiver of a child with CF to develop consensus recommendations. Workgroups developed draft recommendation statements based on a systematic literature review, and a ≥80% consensus was required for acceptance of each recommendation statement.The committee voted on 25 statements. Eleven statements were adopted recommending a treatment or intervention, while five statements were formulated recommending against a specific treatment or intervention. The committee recommended eight statements as an option for select patients in certain circumstances, and one statement did not reach consensus.These multidisciplinary consensus recommendations will help providers navigate decisions related to otolaryngology consultation, medical and surgical management of CF-CRS, hearing, and voice in individuals with CF. A collaborative and multidisciplinary approach is advocated to best care for our patients with CF. Future clinical research is needed utilizing standardized, validated outcomes with comprehensive reporting of patient outcome, effects of modulator therapies, and genetic characteristics to help continue to advance care, decrease morbidity, and improve the quality of life for individuals with CF.

To determine whether removing or retaining adenoids at the time of placement of a second set of ear tubes impacts the need for a third set of ear tubes later in childhood.Single-institution retrospective case series.Tertiary academic university hospital.We identified pediatric subjects who had undergone a second ear tube placement between 1/1/17 and 9/1/19. Subjects were stratified into two groups: 1) adenoids removed at time of second tympanostomy tube insertion (TT+A) and 2) adenoids retained at time of second tympanostomy tube insertion (TT-A). A subset of children less than age 4 was also studied independently. The primary outcome was number of patients requiring a third set of tympanostomy tubes.A total of 136 subjects met inclusion and exclusion criteria. Among children less than 4 years of age (n = 99), the incidence of requiring a third set of tubes was significantly lower in the TT+A group4 (12.8%; 6/47) compared to the TT-A group4 (44.2%; 23/52) (p = 0.0008) with an odds ratio of 0.18 (95%CI 0.067-0.51) and number needed to treat of 3.2.Performing adenoidectomy in children less than 4 years of age at the second tympanostomy procedure was associated with a reduced incidence of requiring a third set of ear tubes.

Ankyloglossia and other oral ties have been recognized for centuries, but interest in and literature on these topics has recently increased. This article presents the latest evidence on the diagnosis and management of tongue-tie and outlines some of the controversies and gaps in the existing evidence. Anterior tongue-tie is accepted in most clinical practices as a potential risk for breastfeeding difficulty, and good evidence exists that division of an anterior tongue-tie leads to improved breastfeeding outcomes. Posterior tongue ties and upper lip-ties are being studied more intensively to provide sound, evidence-based recommendations on their diagnosis and treatment.

The primary purpose of this multidisciplinary guideline is to identify quality improvement opportunities in the management of nosebleeds and to create clear and actionable recommendations to implement these opportunities in clinical practice. Specific goals of this guideline are to promote best practices, reduce unjustified variations in care of patients with nosebleeds, improve health outcomes, and minimize the potential harms of nosebleeds or interventions to treat nosebleeds. The target patient for the guideline is any individual aged ≥3 years with a nosebleed or history of nosebleed who needs medical treatment or seeks medical advice. The target audience of this guideline is clinicians who evaluate and treat patients with nosebleed. This includes primary care providers such as family medicine physicians, internists, pediatricians, physician assistants, and nurse practitioners. It also includes specialists such as emergency medicine providers, otolaryngologists, interventional radiologists/neuroradiologists and neurointerventionalists, hematologists, and cardiologists. The setting for this guideline includes any site of evaluation and treatment for a patient with nosebleed, including ambulatory medical sites, the emergency department, the inpatient hospital, and even remote outpatient encounters with phone calls and telemedicine. Outcomes to be considered for patients with nosebleed include control of acute bleeding, prevention of recurrent episodes of nasal bleeding, complications of treatment modalities, and accuracy of diagnostic measures. This guideline addresses the diagnosis, treatment, and prevention of nosebleed. It focuses on nosebleeds that commonly present to clinicians via phone calls, office visits, and emergency room encounters. This guideline discusses first-line treatments such as nasal compression, application of vasoconstrictors, nasal packing, and nasal cautery. It also addresses more complex epistaxis management, which includes the use of endoscopic arterial ligation and interventional radiology procedures. Management options for 2 special groups of patients-patients with hereditary hemorrhagic telangiectasia syndrome and patients taking medications that inhibit coagulation and/or platelet function-are included in this guideline. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group. It is not intended to be a comprehensive, general guide for managing patients with nosebleed. In this context, the purpose is to define useful actions for clinicians, generalists, and specialists from a variety of disciplines to improve quality of care. Conversely, the statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients.The guideline development group made

Background Castleman disease is a lymphoproliferative disorder that can present in multiple locations throughout the body. While considered a benign entity, Castleman disease in the pediatric head and neck can resemble other common childhood malignancies. Methods This report describes a case of Castleman disease in a 10-year-old male who presented with a progressively enlarging facial mass; work-up revealed aggressive histopathological and imaging features concerning for rhabdomyosarcoma. The patient ultimately required surgical resection for definitive diagnosis. Results The facial mass was successfully resected without complication. A diagnosis of unicentric Castleman disease was made based on final histopathological evaluation of the permanent specimen. The mass has not recurred one year postoperatively. Conclusions Castleman disease in the head and neck should be included in the differential diagnosis of a pediatric facial mass. Balancing the appropriate degree of diagnostic intervention and treatment is essential in ensuring the best possible outcome with the least morbidity.

To evaluate the frequency of potential cytochrome P450 (CYP) drug-drug interactions affecting opioid metabolism among children undergoing adenotonsillectomy.Case series with chart review.Tertiary care children's hospital.A retrospective review was conducted of 1000 patients undergoing adenotonsillectomy at Children's Hospital of Wisconsin. The discharge medication reconciliation form was reviewed. Each patient's list of medications was compared with various published sources to determine whether medications causing CYP inhibition or induction were present.There were 157 unique medications with systemic absorption given postoperatively to this patient cohort. Eight percent of patients were on at least 5 medications after surgery other than their posttonsillectomy medication. The 5 most commonly prescribed medications were albuterol, cetirizine, fluticasone nasal spray, montelukast, and polyethylene glycol. Per a list of known CYP inducers and inhibitors published by the US Food and Drug Administration, 30 (3%) patients were on a medication that inhibited CYP3A4; 1 patient was on a CYP3A4 inducer, prednisone; and 46 (5%) patients were on a medication that inhibited CYP2D6.A small fraction of patients undergoing adenotonsillectomy are on medications that potentially alter the metabolism of opioid pain medications. Given the narrow therapeutic index of opioids and increased sensitivity to opioids among patients with obstructive sleep apnea, drug-drug interactions need to be considered as they relate to whether an opioid is appropriate and at what dose.

Otitis media with effusion (OME) is considered a form of relative sensory deprivation that often occurs during a critical period of language acquisition in children. Animal studies have demonstrated that hearing loss during early development can impair behavioral sensitivity to amplitude modulation (AM), critical for speech understanding, even after restoration of normal hearing thresholds. AM detection in humans with a history of OME-associated conductive hearing loss (CHL) has not been previously investigated. Our objective was to determine whether OME-associated CHL in children ages 6 months to 3 years results in deficits in AM detection in later childhood, after restoration of normal audiometric thresholds. Children ages 4 to 7 years with and without a history of OME-associated CHL participated in an AM detection two-alternative forced-choice task at 8 and 64 Hz modulation frequencies using a noise carrier signal and an interactive touch screen interface. Thirty-four subjects were studied (17 with a history of OME-related CHL and 17 without). Modulation detection thresholds improved with age and were slightly lower (more sensitive) for the 64 Hz modulation frequency for both groups. Modulation detection thresholds of children with a history of OME-associated CHL were higher than control thresholds at 5 years, but corrected to expected levels between ages 6–7. OME-associated CHL results in impaired AM detection, even when measured years after restoration of normal audiometric thresholds. Future studies may shed light on implications for speech and language development and academic success for children affected by OME and associated conductive hearing loss.

The purpose of this study was to investigate the normal lingual frenulum anatomy in newborns and to evaluate tip-frenulum distance as an objective diagnostic tool for identifying newborns at risk for anterior and posterior tongue tie and breastfeeding difficulty.The distance from the tongue tip to the insertion of the lingual frenulum was measured in a group of 100 healthy newborns to establish normative data. The presence of a visible or palpable cord was noted. Inter-rater reliability was assessed. Breastfeeding surveys including a maternal pain scale and Infant Breastfeeding Assessment Tool (IBFAT) were administered on days of life 2 and 14 to determine whether these findings predict breastfeeding difficulty.Mean tip-frenulum length was 9.07 mm. Intraclass correlation coefficient between observers for tip-frenulum length was 0.82. A visible cord was identified in 21 subjects (21%). A palpable cord was identified in 59 subjects (59%). Unweighted κ coefficients for inter-rater reliability of visible and palpable cords were 0.91 and 0.47, respectively. Visible cord and shorter tip-frenulum distance were independently predictive of higher maternal pain scores. A positive correlation was identified between tip-frenulum length and IBFAT scores for mothers with two or more previous breastfed children.Tongue tip-frenulum length correlated with maternal nipple pain, and was useful as an objective tool for identifying newborns at risk for ankyloglossia. Maternal breastfeeding experience appears to be an important factor in the link between tongue anatomy and breastfeeding difficulty. The presence of a palpable cord was variable across examiners, and should be interpreted with caution when evaluating newborns for posterior tongue tie.

Objectives/Hypothesis The tonsils and adenoids are secondary lymphoid organs, where antigen processing and immune cell development occur to control bacterial colonization and infection in the upper respiratory tract. Both organs are abundant in follicular T helper cells (TFH), a subset of T cells specialized for promoting B-cell development. There are no prior studies on differences between the immune cells of the tonsils and adenoids and whether the cells function differently. Study Design In vitro assays to assess cell phenotype of tonsils and adenoids from young children (median age = 40 months). Methods Mononuclear cells from tonsils and adenoids were cultured with or without 1 µg/mL Staphylococcus enterotoxin B (SEB) for 4 days. Cell phenotype and function were assessed by flow cytometry and multiplex enzyme-linked immunosorbent assay. Results We found that in resting adenoids, TFH expressed higher CXCR5 and inducible costimulator but lower PD-1 than those from the tonsils, and that adenoidal B cells expressed higher CD27. Upon polyclonal stimulation with SEB, both TFH and B cells from the adenoids proliferated to a greater extent, and culture supernatants contained higher levels of interleukin 21. Conclusions We conclude that the cells of the adenoid are disposed toward the provision of more robust B-cell help than the tonsils. Level of Evidence NA Laryngoscope, 2015

Objectives/Hypothesis To review the presentation and management of malignant tumors of the mandible in children. Study Design Case series. Methods Children 0 to 21 years old presenting to a tertiary pediatric hospital with a diagnosis of a malignant tumor involving the mandible were included. Comparison groups included children from the Surveillance, Epidemiology, and End Results database with malignant mandible tumors as well as a group of children with benign mandibular lesions from the same institution. Main outcome measures for the institutional malignant group included presentation, tumor characteristics, treatment modalities, and clinical outcome. Results Sixteen patients with malignant and 183 patients with benign lesions were identified at the primary institution. The most common presentation in both groups was mandibular swelling or mass. Malignant tumors included sarcoma (n = 11), leukemia/lymphoma (n = 2), squamous cell carcinoma (n = 1), malignant spindle cell tumor (n = 1), and yolk sac tumor (n = 1). The national database identified 56 malignant cases, of which 71% were sarcomas. Thirteen patients at our institution (81%) underwent mandibulectomy and 12 were reconstructed using free tissue transfer. Eleven of the 13 (85%) were treated with adjuvant therapy. After an average follow-up of 32.8 months, seven (44%) had no evidence of disease, three (19%) were deceased, three (19%) were alive with disease, and three (19%) were lost to follow-up. Conclusions Malignant mandibular tumors in children are most often sarcomas but can include other rare lesions. Free flap reconstruction is a reasonable option for even very young children requiring extensive mandibular surgery. Level of Evidence 4. Laryngoscope, 123:2035–2042, 2013

Objective To reclassify facial nerve hemangiomas in the context of presently accepted vascular lesion nomenclature by examining histology and immunohistochemical markers. Study Design Cohort analysis of patients diagnosed with a facial nerve hemangioma between 1990 and 2008. Setting Collaborative analysis at a specialty hospital and a major academic hospital. Subjects and Methods Seven subjects were identified on composite review of office charts, a pathology database spanning both institutions, and an encrypted patient registry. Clinical data were compiled, and hematoxylin-eosin–stained specimens were reviewed. For six patients, archived pathological tissue was available for immunohistochemical evaluation of markers specific for infantile hemangioma (glucose transporter protein isoform 1 [GLUT1] and Lewis Y antigen) and for lymphatic endothelial cells (podoplanin). Results All patients clinically presented with slowly progressive facial weakness at a mean age of 45 years without prior symptomatology. Hemotoxylin-eosin–stained histopathological slides showed irregularly shaped, dilated lesional vessels with flattened endothelial cells, scant smooth muscle, and no internal elastic lamina. Both podoplanin staining for lymphatic endothelial cells and GLUT1 and LewisY antigen staining for infantile hemangioma endothelial cells were negative in lesional vessels in all specimens for which immunohistochemical analysis was performed. Conclusion Lesions of the geniculate ganglion historically referred to as “hemangiomas” do not demonstrate clinical, histopathological, or immunohistochemical features consistent with a benign vascular tumor, but instead are consistent with venous malformation. We propose that these lesions be classified as “venous vascular malformations of the facial nerve.” This nomenclature should more accurately predict clinical behavior and guide therapeutic interventions.

OBJECTIVE: To describe a case and discuss the differential diagnosis of facial nerve paresis presenting years after resection of multiple recurrent parotid pleomorphic adenoma. PATIENTS: Case report of a patient on immunosuppressive therapy with facial nerve weakness 3 years after last resection for multiple recurrent pleomorphic adenoma. INTERVENTIONS: Computed tomography and magnetic resonance imaging followed by surgical exploration, resection, and reconstruction. MAIN OUTCOME MEASURES: Histopathologic diagnosis and treatment outcome. RESULTS: Final diagnosis of recurrent pleomorphic adenoma causing compression of the facial nerve at the stylomastoid foramen. CONCLUSION: Facial nerve weakness caused by a benign salivary gland tumor is rare. Although alternate diagnoses must be considered, recurrent pleomorphic adenoma alone may impair facial function by impinging on the nerve in the stylomastoid foramen.

To review the presentation and management of malignant tumors of the mandible in children.Case series.Children 0 to 21 years old presenting to a tertiary pediatric hospital with a diagnosis of a malignant tumor involving the mandible were included. Comparison groups included children from the Surveillance, Epidemiology, and End Results database with malignant mandible tumors as well as a group of children with benign mandibular lesions from the same institution. Main outcome measures for the institutional malignant group included presentation, tumor characteristics, treatment modalities, and clinical outcome.Sixteen patients with malignant and 183 patients with benign lesions were identified at the primary institution. The most common presentation in both groups was mandibular swelling or mass. Malignant tumors included sarcoma (n = 11), leukemia/lymphoma (n = 2), squamous cell carcinoma (n = 1), malignant spindle cell tumor (n = 1), and yolk sac tumor (n = 1). The national database identified 56 malignant cases, of which 71% were sarcomas. Thirteen patients at our institution (81%) underwent mandibulectomy and 12 were reconstructed using free tissue transfer. Eleven of the 13 (85%) were treated with adjuvant therapy. After an average follow-up of 32.8 months, seven (44%) had no evidence of disease, three (19%) were deceased, three (19%) were alive with disease, and three (19%) were lost to follow-up.Malignant mandibular tumors in children are most often sarcomas but can include other rare lesions. Free flap reconstruction is a reasonable option for even very young children requiring extensive mandibular surgery.

Objective The use of image guidance for sinonasal and skull base surgery has been well-characterized in adults but there is limited information on the use of these systems in the pediatric population, despite their widespread use. The aim of this study is to evaluate the use of image guidance systems to facilitate an endoscopic minimally invasive approach to sinonasal and skull base surgery in a pediatric population. Methods A retrospective cohort study was performed at a tertiary pediatric hospital. Thirty-three children presented with complications of sinusitis, tumors, traumatic, or congenital lesions of the skull base and underwent endoscopic surgery using image guidance from March 2000 to April 2007. Patient variables including diagnosis, extent of disease, and complications were extracted from paper and computer charts. Additional surgical variables including set-up time, accuracy, surgeon satisfaction index and number of uses per case were also reviewed. Results Twenty-eight patients (85%) underwent sinonasal surgery and five (15%) underwent skull base surgery. Indications included infectious complications of acute sinusitis ( N = 15), neoplasms ( N = 12), choanal atresia ( N = 4), and cerebrospinal fluid leak ( N = 2). Thirty-one patients (94%) required only one procedure. No surgical complications were reported. Surgeon satisfaction, mean accuracy and number of uses per procedure increased over time ( p Conclusions Image guidance systems are safe and effective tools that facilitate a minimally invasive approach to sinonasal and skull base surgery in children. Consistent with adult literature, usage and surgeon comfort increased with experience. The additional anatomical information obtained by image guidance systems facilitates a minimally invasive endoscopic approach for sinonasal and skull base pathologies.

Incongruent auditory and visual stimuli can elicit audiovisual illusions such as the McGurk effect where visual /ka/ and auditory /pa/ fuse into another percept such as/ta/. In the present study, human brain activity was measured with adaptation functional magnetic resonance imaging to investigate which brain areas support such audiovisual illusions. Subjects viewed trains of four movies beginning with three congruent /pa/ stimuli to induce adaptation. The fourth stimulus could be (i) another congruent /pa/, (ii) a congruent /ka/, (iii) an incongruent stimulus that evokes the McGurk effect in susceptible individuals (lips /ka/ voice /pa/), or (iv) the converse combination that does not cause the McGurk effect (lips /pa/ voice/ ka/). This paradigm was predicted to show increased release from adaptation (i.e. stronger brain activation) when the fourth movie and the related percept was increasingly different from the three previous movies. A stimulus change in either the auditory or the visual stimulus from /pa/ to /ka/ (iii, iv) produced within-modality and cross-modal responses in primary auditory and visual areas. A greater release from adaptation was observed for incongruent non-McGurk (iv) compared to incongruent McGurk (iii) trials. A network including the primary auditory and visual cortices, nonprimary auditory cortex, and several multisensory areas (superior temporal sulcus, intraparietal sulcus, insula, and pre-central cortex) showed a correlation between perceiving the McGurk effect and the fMRI signal, suggesting that these areas support the audiovisual illusion.

OBJECTIVE. The purpose of this work was to investigate the clinical manifestations and diagnostic range of malignant entities presenting as a nasal mass in the pediatric population. PATIENTS AND METHODS. A retrospective cohort analysis was conducted at a specialty hospital and a tertiary care university hospital. Patients aged between birth and 18 years and diagnosed with a malignancy that arose within the nasal cavity between the years 1991 and 2006 were included. This institution-specific patient group was compared with a similar cohort of patients extracted from the national Surveillance Epidemiology and End Results database. The main outcome measures were the incidence, presentation, and diagnoses of nasal cancer presenting in this population. RESULTS. Sixteen patients with nasal malignancies presented institutionally in the defined pediatric age group. Patient age at the time of diagnosis ranged from 7 months to 17 years, with a slight male predominance. The main presenting symptoms were unilateral nasal congestion and ophthalmologic complaints. The median time from presentation to diagnosis was 7 weeks; patients who presented with nonspecific complaints, such as nasal obstruction, headache, and fatigue, were given a diagnosis, on average, later than those who presented with focal manifestations. Nationwide, 47 patients were identified from the Surveillance Epidemiology and End Results database. In both subject groups, the most common diagnoses were rhabdomyosarcoma (37.5% institutionally and 23% in the Surveillance Epidemiology and End Results group) and esthesioneuroblastoma (25% institutionally and 28% Surveillance Epidemiology and End Results). In the Surveillance Epidemiology and End Results cohort, the overall mean survival rate was 188 months. CONCLUSIONS. Nasal cancer in the pediatric population often presents with nonspecific signs and symptoms, and a high index of suspicion is necessary for a timely diagnosis. Soft tissue sarcomas are expectedly common. The relative high frequency of esthesioneuroblastoma is particularly noteworthy.

Objectives To report a case of paraganglioma arising from the hypoglossal nerve and review the anatomy, clinical features, and literature to date. Study design Case report and review of the literature. Methods Case records including paper and electronic chart and imaging reports were reviewed and summarized for the index case. A literature search was performed using pubmed keywords paraganglioma, hypoglossal, chemodactoma, carotid body, and glomus tumor. The available literature on the topic was reviewed and summarized. Results Paragangliomas associated with the hypoglossal nerve were reported in 4 cases over the past 47 years. Our index case presented with similar clinical features compared to those reported in the literature. Imaging with computed tomography and angiography showed a hypervascular mass at the carotid bifurcation, splaying the internal and external carotid arteries. As in previously reported cases, the source of the paraganglioma was only identified intraoperatively. The current case differs from prior reported literature in that the tumor was dissected from the associated hypoglossal nerve which was preserved. The patient clinically had no deficits in articulation or deglutination following excision of the lesion and was able to return to a normal diet within 24 hours of surgery. Conclusions Hypoglossal paraganglioma is a neck mass that may not be distinguishable from more common carotid body or vagus tumors despite the use of multiple imaging modalities. Although XIIth nerve sacrifice may be requires in some instances, nerve preserving surgery, when possible, allows for complete recovery without functional deficits.