Donald G. Keamy

The epidemiology of hearing impairment in the United States: Newborns, children, and adolescents

Objective: Hearing loss ranks high among disabilities in the United States. The epidemiologic parameters of hearing impairment in the United States have not been systematically studied and important historic data have not diffused to relevant stakeholders; even otolaryngologists are unfamiliar with epidemiologic data. We wished to compile known studies to establish an epidemiologic baseline beginning with pediatric data. Data Sources: Relevant literature was retrieved from medical databases and Centers for Disease Control and Prevention reports. Methods: Candidate articles and national data sets encompassing pediatric hearing loss were analyzed and compared. Whenever possible, group analyses were performed. Results: The average incidence of neonatal hearing loss in the United States is 1.1 per 1000 infants, with variation among states (0.22 to 3.61 per 1000). Childhood and adolescent prevalence rates demonstrate variability. The prevalence of mild hearing impairment or worse (>20 dB) is 3.1 percent based on the average of comparable audiometric screening studies; self-reporting prevalence is 1.9 percent. Hispanic Americans demonstrate a higher prevalence of hearing impairment than other children. Low-income households demonstrate a higher prevalence of hearing loss compared to households with higher income levels. Genetic causes were attributed to 23 percent across studies. Conclusions: Analysis of the data reveals gaps in our knowledge of the epidemiology of hearing loss and stresses the importance of consistent definitions of hearing impairment for systematic assessment of changes over time. Hearing loss in childhood deserves further epidemiologic investigation and elevated awareness among health care professionals and the public. Genetic etiologies are likely underestimated in this review.

A pilot study to identify pre- and peri-operative risk factors for airway complications following adenotonsillectomy for treatment of severe pediatric OSA

OBJECTIVE A pilot study to identify risk factors predicting post-operative complications in children with severe OSA undergoing adenotonsillectomy. METHODS Retrospective review in a tertiary care academic institution. Two-stage least squares regression analysis and instrumental variable analysis to allow for modeling of pre- and peri-operative risk factors as having significance in predicting post-operative morbidity. RESULTS Eighty-three children (mean age 4.88 ± 3.09 years) with apnea-hypopnea index (AHI) ≥ 10 who were observed overnight following adenotonsillectomy were evaluated for rates of major (increased level of care, CPAP/BiPAP use, pulmonary edema and reintubation) and minor (oxygen saturation <90%) airway complications as well as total observation costs. Major and minor complications occurred in 4.8% and 19.3% of children, respectively. Age <2 years (p<0.01), AHI >24 (p<0.05), intra-operative laryngospasm requiring treatment (p<0.05), oxygen saturations <90% on room air in PACU (p<0.05) and PACU stay >100 min (p<0.01) independently predicted post-operative complications. Children with any one of these factors experienced a 38% complication rate versus 4% in all others. CONCLUSIONS This pilot study identified pre- and peri-operative risk factors that collectively can be investigated as predictors of post-operative airway complications in a prospective study. By identifying preliminary results comparing the complication rates between those children with and without these risk factors, we will be able to calculate the sample size for a future prospective validation study. Such a study is necessary to understand the safety and potential significant cost savings of observing children without risk factors on the pediatric floor and not in an ICU setting. A best practice algorithm can be created for children with severe OSA only after completing this prospective study.

Successfully Treated Rhinocerebral Phycomycosis in Well Controlled Diabetes

RHINOCEREBRAL phycomycosis (mucormycosis) is a fulminant fungal infection most commonly seen in diabetes mellitus, usually with ketoacidosis,1 2 3 4 or occasionally some other debilitating conditio...

Medical and Surgical Interventions for Hearing Loss Associated with Congenital Cytomegalovirus A Systematic Review

Background. Hearing loss associated with congenital cytomegalovirus (CMV) infection occurs in 0.2 to 0.6 per 1000 neonates. Objective. The primary goal of this systemic review was to test the following null hypotheses: (1) antiviral therapy has no impact on congenital CMV-related sensorineural hearing loss and (2) surgical therapy has no impact on congenital CMV-related sensorineural hearing loss. Data Sources. Computerized searches of MEDLINE and EMBASE databases through September 2010 were performed, supplemented with manual searches and inquiries to topic experts. Review Methods. Studies were included based on review of 387 studies according to criteria developed a priori. Data extraction was performed by independent reviewers and focused on relevant audiologic measurements, study designs, and potential confounders. Results. Criterion-meeting studies (n = 19) included a total of 446 participants. The largest randomized controlled trial (RCT) suggested a significant protective effect of intravenous ganciclovir against deterioration of hearing in neonates with central nervous system manifestations of CMV infection. It also, however, suggested a 3-fold increase in neutropenia. The second RCT suggested that there may be no significant benefit of intravenous ganciclovir for normal-hearing infants with asymptomatic congenital CMV. Additional prospective and retrospective data evaluated the impact of oral therapy and cochlear implantation in affected patients. Conclusion. Although results are mixed, the highest level of evidence suggests that antiviral therapy confers a protective benefit on neonates with hearing loss and symptomatic CMV. Cochlear implantation can result in advancement of speech and language skills, but there are mixed results compared with non–CMV-infected patients.

Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea

Obstructive sleep apnea (OSA) is more common in children with Down syndrome, affecting up to 60% of patients, and may persist in up to 50% of patients after adenotonsillectomy. These children with persistent moderate to severe OSA require continuous positive airway pressure, which is often poorly tolerated, or even tracheotomy for severe cases. The hypoglossal nerve stimulator is an implantable device that produces an electrical impulse to the anterior branches of the hypoglossal nerve, resulting in tongue protrusion in response to respiratory variation. It is an effective treatment of sleep apnea in select adult patients because it allows for alleviation of tongue base collapse, improving airway obstruction. Herein we describe the first pediatric hypoglossal nerve stimulator implantation, which was performed in an adolescent with Down syndrome and refractory severe OSA (apnea hypopnea index [AHI]: 48.5 events/hour). The patient would not tolerate continuous positive airway pressure and required a long-standing tracheotomy. Hypoglossal nerve stimulator therapy was well tolerated and effective, resulting in significant improvement in the patient’s OSA (overall AHI: 3.4 events/hour; AHI: 2.5–9.7 events/hour at optimal voltage settings depending on sleep stage and body position). Five months after implantation, the patient’s tracheotomy was successfully removed and he continues to do well with nightly therapy.

Lobular capillary hemangioma of the nasal cavity in a five-year-old boy.

Afive-year-old male was referred to the pediatric otolaryngology clinic with recurrent right anterior epistaxis and unilateral nasal obstruction. There was no history of systemic disease, neurologic changes, trauma, or foreign body insertion. He had no significant medical or surgical history. Upon examination, he was noted to have an obstructing friable right intranasal mass (Fig 1). Anterior rhinoscopy was normal on the opposite side. The remainder of his head and neck examination was normal. Imaging studies revealed a lobular heterogeneous nasal mass causing mass effect on the septum and lateral nasal wall. There was no erosion or remodeling of bone, and no extension outside of the nasal cavity. No prominent feeding vessels were identified. The mass was excised endoscopically using image guidance. Intraoperatively, the mass was noted to have a single pedicle attachment to the nasal septum. Under direct visualization, the posterior septal attachment was lysed. Due to the size of the mass, it could not be extracted through the nose. It was pushed into the nasopharynx and removed transorally. The final histopathologic diagnosis was lobular capillary hemangioma. On follow-up at eight months, the patient reported resolution of his epistaxis and improved nasal breathing, and fiberoptic endoscopy revealed no evidence of recurrence. Lobular capillary hemangioma (LCH), formerly known as pyogenic granuloma, is a benign vascular lesion of the skin and mucosa. These lesions are more common in male children and in female adults during pregnancy. The head and neck are the most frequent sites of occurrence, with cutaneous or lip lesions presenting most commonly, followed by oral mucosa and nasal mucosa. LCH of the nasal mucosa can present with recurrent unilateral epistaxis and unilateral nasal obstruction or rhinorrhea. Most nasal lesions occur in Little’s area on the anterior septum, in the nasal vestibule or inferior turbinate. Repetitive trauma or manipulation has been suspected as a causative factor. LCH can evolve rapidly over the course of a few weeks but does not demonstrate the proliferative and involutional phases characteristic of classic hemangioma of infancy. Diagnosis involves a complete history and physical, with endoscopic evaluation of the nasal cavities. Imaging with CT and MRI can be useful for narrowing the differential diagnosis and defining a treatment plan. The definitive treatment is complete removal. Endoscopic excision is the preferred technique to ensure localization of the mucosal origin and to avoid facial incisions. Embolization and adjuvant therapies are unwarranted. With complete resection, recurrences are rare. This case report was deemed exempt from human studies committee review.

Predictors of complications following adenotonsillectomy in children with severe obstructive sleep apnea.

OBJECTIVE To identify pre-operative risk factors predicting complications following adenotonsillectomy in children with severe OSA. METHODS Retrospective chart review in an academic tertiary care center. Children with symptoms of OSA with overnight polysomnography (PSG) revealing apnea-hypopnea index (AHI) >10, who underwent adenotonsillectomy with overnight postoperative observation between 2008 and 2012. Univariate logistic regression was used to assess odds ratio (OR) of individual risk factors versus postoperative complications such as overnight desaturations <90%, length of stay (LOS)>24 h, supplemental oxygen requirement, and transfer to a higher level of care. RESULTS All patients (n=157) with severe OSA were observed overnight. Mean age was 5.3±3.7 years. Twenty-five (15.9%) patients had LOS>24 h. Forty-two (26.8%) had overnight desaturations <90%. AHI ≥15 and O2 saturation nadir <80% on preop polysomnography (PSG) were independent predictors of post-op O2 saturation <90% and LOS>24 h. (p<0.05). PSG minimum saturation <80% was the strongest predictor of all variables examined with an OR of 6.98 (3.15-15.48, 95% CI) for desaturation <90% and 5.19 (2.11-12.75, 95% CI) for LOS>24 h. Preop PSG O2 saturation<90% predicted overnight post op oxygen requirement with an OR of 3.38 (1.39-8.25, 95%CI). CONCLUSIONS Preoperative polysomnography yields significant independent predictors of post-op complications in children with OSA. While AHI is a significant independent predictor, minimum O2 saturation on preop PSG appeared the strongest predictor when <80%. Patients with these risk factors, especially low O2 on PSG, warrant overnight observation with continuous pulse oximetry.

Radiology Quiz Case 3

A 30-YEAR-OLD WOMAN IN HER SECOND TRImester of pregnancy (20 weeks) presented with a severe headache and pain in the right side of her face. Magnetic resonance imaging showed a soft tissue lesion in the right ethmoidal sinus, extending into the right orbit (Figure 1). The patient had a history of very poor vision in her right eye because of a cataract that had been present since childhood. Under anesthesia, she underwent a nasal examination and biopsy of the lesion, which was occupying the superior meatus and extending posteriorly toward the sphenoidal sinus. Wide surgical excision of the lesion by a combined endoscopic endonasal and craniofacial facial approach was planned. Proptosis and swelling of the patient’s right eyelid developed as she was waiting for surgery and workup, with further investigation to rule out a secondary focus in her neck and chest. After a couple of days, she suddenly became unconscious. She was not responding to any stimuli, but all her vital signs were within normal limits. An urgent computed tomographic scan of her brain did not show any signs of hemorrhage or increased intracranial pressure. She underwent intubation and was transferred to an intensive care unit. She suddenly gained consciousness the next day, without any neurologic deficits. She was treated with high-dose steroids, but within the next 48 hours she started to complain of blurred vision in her left eye. An urgent computed tomographic scan of her paranasal sinuses (Figure 2) showed a homogeneous mass involving the right ethmoidal sinuses, right orbit, and cavernous sinuses; invading the cribriform plate, left ethmoidal sinus, and left orbit; eroding the pituitary fossa; and compressing the left optic nerve at the apex of the left orbit. What is your diagnosis?

Hypoglossal Nerve Stimulation in Adolescents With Down Syndrome and Obstructive Sleep Apnea

Importance Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome (DS) and may persist in half of patients after adenotonsillectomy. Children with DS who have persistent OSA often do not tolerate treatment with positive pressure airway support devices or tracheotomy for their residual moderate to severe OSA. The hypoglossal nerve stimulator is an implantable device that delivers an electrical impulse to anterior branches of the hypoglossal nerve in response to respiratory variation, resulting in tongue base protrusion that alleviates upper airway obstruction in adults. Objective To determine whether hypoglossal nerve stimulation is safe and effective in children with DS. Design, Setting, and Participants Case series of the first 6 adolescents with DS to undergo hypoglossal nerve stimulator implantation. Participants were 6 children and adolescents (12-18 years) with DS and severe OSA (apnea hypopnea index [AHI] > 10 events/h) despite prior adenotonsillectomy. Intervention Inspire hypoglossal nerve stimulator placement. Main Outcomes and Measures Patients were monitored for adverse events. Adherence to therapy was measured by hours of use recorded by the device. Efficacy was evaluated by comparing AHI and OSA-18, a validated quality-of-life instrument, scores at baseline and follow-up. Results In 6 patients (4 male, 2 female; aged 12-18 years), hypoglossal nerve stimulator therapy was well tolerated (mean use, 5.6-10.0 h/night) and effective, resulting in significant improvement in OSA. At 6- to 12-month follow-up, patients demonstrated a 56% to 85% reduction in AHI, with an overall AHI of less than 5 events/h in 4 children and less than 10 events/h in 2 children. Children also demonstrated a clinically significant improvement (mean [SD] overall change score, 1.5 [0.6]; range, 0.9-2.3) on the OSA-18, a validated quality-of-life instrument. Conclusions and Relevance Hypoglossal nerve stimulation was well tolerated and effective in the study population, representing a potential therapeutic option for patients with DS and refractory OSA after adenotonsillectomy who are unable to tolerate positive pressure airway devices. Trial Registration clinicaltrials.gov Identifier: NCT2344108

Utilization of diagnostic testing for pediatric sensorineural hearing loss

BACKGROUND/OBJECTIVE Sensorineural hearing loss is a common diagnosis among children. The diagnostic workup varies widely among practitioners. This studys aim was to assess the utilization of diagnostic testing for SNHL and determine the yield of each test. STUDY DESIGN Retrospective chart review. SETTING Tertiary care center. SUBJECTS 827 patients with a diagnosis of SNHL from January 1, 2011 to January 1, 2015. RESULTS 746 patients met inclusion criteria. Temporal bone imaging was performed on 561 (75%) of patients with 224 (40%) having positive results that explained the etiology of the SNHL. Congenital SNHL was more likely to be associated with abnormal imaging than acquired SNHL (109/299 versus 106/316 respectively) (p = 0.001). Unilateral SNHL was more likely to be associated imaging abnormalities than bilateral SNHL (101/221 and 123/340 respectively) (p = 0.028). Genetic testing was performed on 244 (33%) patients, of which 94 (39%) had abnormalities. Positive genetics results were more common with bilateral than unilateral SNHL (82/191 and 12/53 respectively) (p = 0.007). There was no statistically significant difference in the utility of genetic testing for congenital and acquired SNHL (p = 0.0836). Cytomegalovirus (CMV) testing was available for 104 (14%) of patients with 13 (12.5%) being positive and consistent with congenital CMV. Electrocardiogram, urinalysis, and Lyme titers were less useful. CONCLUSIONS Imaging and genetic testing had the highest yield in the evaluation of children with SNHL and were the most commonly performed. CMV testing was valuable in neonates who failed newborn hearing screening.