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Glottic and Subglottic Stenosis and Related Voice Disorders

Key Points Laryngeal stenosis can be congenital or acquired. It often involves multiple levels of the airway. The presentation may range from mild symptoms affecting the voice, respiration, and/or feeding to significant respiratory distress requiring emergent intubation or tracheotomy to secure the airway. Subglottic stenosis is the third most common congenital anomaly of the larynx, after laryngomalacia and true vocal fold immobility. Acquired laryngeal stenosis can…

Evaluation and Management of the Pediatric Airway

Key Points Initial evaluation of a child with noisy breathing involves assessment of the phase and character of the noise; patient distress in relation to states of sleep, wakefulness, and feeding; overall color and oxygen saturation; growth and weight gain; and functional anatomy via awake fiberoptic laryngoscopy. The most common cause of stridor in infants is laryngomalacia, but this cannot be assumed to be the cause…

Pediatric Head and Neck Neoplasms

Key Points Lymphomas are the most common head and neck malignancies in children, followed by retinoblastoma, rhabdomyosarcoma (RMS), neuroblastoma, and thyroid cancer. Hodgkin lymphoma is the predominant malignant lymphoma in older children. Although surgery is not the primary treatment modality for pediatric lymphoma, the otolaryngologist often plays a key role in the diagnosis by obtaining tissue. Neuroblastomas of the head and neck tend to present at…

Vascular Anomalies of the Head and Neck

Key Points Vascular anomalies are divided into vascular malformations and vascular tumors. The molecular mechanisms that give rise to vascular anomalies involve disordered angiogenesis and vasculogenesis. The most common type of vascular tumor is hemangioma of infancy. Hemangiomas of infancy (HOIs) are absent at birth, occur in infancy, proliferate until 9 to 10 months of age, and invariably involute or shrink over years. PHACE ( p…

Salivary Gland Disease in Children

Key Points Accurate diagnosis of salivary gland disorders depends on a thorough history and a good physical examination. Although most salivary gland lesions in children have an inflammatory etiology, the differential diagnosis is vast and includes acute or chronic inflammatory and/or infectious conditions, congenital lesions, vascular malformations, benign or malignant tumors, traumatic injury, and many systemic diseases. Common bacterial etiologies of sialadenitis include Staphylococcus aureus and…

Congenital and Inflammatory Neck Masses in Children

Key Points At approximately 4 to 5 weeks of gestation, the area of the embryo’s future face and neck consists of pairs of fingerlike masses of tissue called the pharyngeal arches. The outer surfaces of the arches and the clefts are lined with ectoderm; the substance of the pharyngeal arches contains mesoderm and neural crest cells. The pharyngeal pouches are outpouchings from the foregut region and…

Pediatric Infectious Disease

Key Points The diagnosis of pharyngitis requires excellent history and physical documentation and appropriate culture and laboratory testing. The majority of sore throats in children are caused by viral pharyngitis, which will not improve with antibiotics or tonsillectomy. Corynebacterium diphtheriae causes an early exudative pharyngotonsillitis with a thick pharyngeal membrane and produces a lethal exotoxin that can damage cells in distant organs. Mortality rates are high,…

Pediatric Chronic Rhinosinusitis

Key Points Chronic rhinosinusitis (CRS) in children has a negative effect on quality of life. Distinction between adenoiditis and CRS in children is difficult because of similar symptoms and often similar findings on physical examination. In children with chronic respiratory complaints, lower Lund-Mackay computed tomography (CT) scores indicate primarily adenoid disease, whereas higher scores correlate better with CRS. The inflammatory reaction in the sinus tissues of…

Evaluation and Management of Pediatric Vestibular Disorders

Key Points Vestibular and balance impairment in children is common, especially in the setting of sensorineural hearing loss. Detecting vestibular and balance deficits in children can be challenging. A screening assessment can be an effective tool for identifying children at risk of vestibular impairment. The effect of vestibular and balance impairment on development, cognition, and learning is significant. Introduction The balance organ is the most primitive…

Evaluation and Management of Congenital Aural Atresia

Key Points Microtia/congenital aural atresia (CAA) can occur in conjunction with ocular, cervical, cardiac, renal, and other congenital anomalies that must be evaluated and treated if present. The vast majority of patients with CAA have a maximal (60 dB) conductive hearing loss and normal cochlear function. Hearing evaluation by auditory brainstem response (ABR) testing with air and bone conduction thresholds early in infancy (6 months and…

Microtia Reconstruction

Key Points Normal adult ear height ranges from 5.5 to 6.5 cm; 95% of this height is achieved by 8 to 10 years of age. Microtia encompasses a spectrum of congenital anomalies of the ear. Patients with microtia should be evaluated with age-appropriate hearing assessment. Treatment options for microtia reconstruction include (1) observation, (2) adhesive- or implant-retained prosthetics, (3) single-stage reconstruction with temporoparietal fascia flap and…

Pediatric Cochlear Implantation

Key Points Cochlear implantation (CI) services should be integrated into a well-coordinated early identification and intervention program with multidisciplinary collaborators within the center and surrounding community. The core team includes a surgeon, a pediatric audiologist, and a speech and language pathologist or teacher of the deaf who are trained in the principles of listening and spoken language rehabilitation. Criteria for pediatric CI are evolving and currently…

Pediatric Otologic Surgery

Middle Ear and Mastoid Surgery Chronic Suppurative Otitis Media and Tympanic Membrane Repair A number of factors are thought to influence the success of tympanic membrane repair in chronic suppurative otitis media (CSOM), such as age, type of graft material, and surgical technique. Several published meta-analyses have tried to elucidate the importance of these factors. The optimum age at which to perform tympanoplasty is an ongoing…

Acute Otitis Media and Otitis Media With Effusion

Key Points The incidence of otitis media (OM) is highest in the first years of life and declines as children grow older and the functions of the immune system and eustachian tube mature. The cause of OM is multifactorial; risk factors are genetic, social, and environmental. The presence of middle ear effusion (MEE) is an important prerequisite for diagnosing acute OM (AOM) and OM with effusion…

Enlarged Vestibular Aqueduct

Key Points Enlargement of the vestibular aqueduct is a commonly detected inner ear anomaly. The hearing loss of enlargement of the vestibular aqueduct (EVA) characteristically shows fluctuation, progression, or both. Patients with EVA often have a low-frequency conductive hearing loss in the presence of a normal middle ear. The otolaryngologist should have a high index of suspicion to avoid unnecessary placement of tympanostomy tubes in such…

Congenital Malformations of the Inner Ear

Key Points In congenital sensorineural hearing loss, approximately 20% of inner ears demonstrate a radiographically detectable abnormality. Many patterns of deformity appear to result from disturbances in the embryogenesis of the inner ear during the first trimester. Enlargement of the vestibular aqueduct is the most common anomaly, followed in frequency by semicircular canal and cochlear malformations. Cochlear anomalies apparent on imaging include aplasia, hypoplasia, incomplete partition,…

Early Detection and Diagnosis of Infant Hearing Impairment

Key Points Protocols for newborn hearing screening include a hearing screen by 1 month of age, diagnostic hearing testing in those who failed the hearing screen by 3 months of age, and early intervention for infants diagnosed with hearing loss by 6 months of age. The most common causes of congenital hearing loss in infants without apparent risk factors are recessive genetic hearing loss and congenital…

22q11.2 Deletion Syndrome

Key Points 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome. There is a large phenotypic variance in 22q11.2DS, thus requiring a high index of diagnostic suspicion for genetic testing. Otolaryngologists play a key role in the care of 22q11.2DS patients as otitis media, hearing loss, velopharyngeal dysfunction (VPD), obstructive sleep apnea, sinusitis, dysphagia, and airway anomalies are common. Children with 22q11.2DS often require a…

Velopharyngeal Dysfunction

Key Points Velopharyngeal dysfunction (VPD) refers to any type of abnormality in form or function and is further categorized as insufficiency, incompetence, or mislearning and as phoneme- or sound-specific VPD. Phoneme- or sound-specific VPD involves abnormal production of one or more phonemes with increased nasal airflow while other phonemes are normal. Phonemes most commonly involved with phoneme-specific VPD include /s/, /sh/, /z/, and, rarely, Compensatory…

Cleft Lip and Palate

Key Points Cleft lip and palate deformities constitute the most common congenital defects of the head. The etiology of clefting is multifactorial. Its prevalence varies among ethnic groups and within families. Syndromes are common, especially among patients with cleft palate. Surgical decisions should be based on careful analysis of the defects. The goals are to achieve normal lip closure, a patent nasal airway, age-appropriate speech, eustachian…