Volunteer-based universal newborn hearing screening program.

OBJECTIVE: To evaluate efficacy and costs of a volunteer-based universal newborn hearing screening program. METHODS: The Lucile Packard Children's Hospital at Stanford newborn hearing screening program database was reviewed. Results and costs of the hearing screens were analyzed. RESULTS: Hearing screens were performed on 5771 newborns treated in the well-baby nursery and nine infants from this population were identified with hearing loss, seven of whom had no risks factors for hearing loss. Using volunteers to perform the first-line screen with the automated auditory brainstem response (AABR) technology, 91% of infants registered for screening were evaluated prior to discharge. An additional 4% of infants were screened as outpatients. If an infant failed the AABR on two occasions, he or she was rescreened with the AABR or transient evoked otoacoustic emissions by a licensed audiologist, often while the infant was still in the hospital. Using this algorithm, 5% of infants tested in the well-baby nursery needed additional follow-up as an outpatient. Cost analysis of this volunteer-based program reveals a per/baby screening cost of $27.41. CONCLUSIONS: A volunteer-based hearing screening program is a viable option for hearing screening in well-baby nurseries but does not result in significant cost savings during the first 2 years of the program.

Fungal malignant otitis externa due to Scedosporium apiospermum.

Malignant otitis externa (MOE) is an infection of the external auditory canal that invades the skull base. Aspergillus species fungi were the pathological organism in 21 of 23 reported cases of fungal MOE. We report on a 21-year-old man with end-stage acquired immunodeficiency syndrome (AIDS) and fungal MOE caused by Scedosporium apiospermum. Fungal MOE is most common in patients with end-stage AIDS and hematologic malignancies. Granulation tissue is not a common finding in these patients, and the infectious process often starts in the mastoid air cells or middle ear space, as opposed to the external auditory canal. Surgical debridement and amphotericin B are the mainstays of therapy; resolution of the infection depends greatly on the severity of the underlying disease.

Ankyloglossia: controversies in management.

PURPOSE: To determine current beliefs regarding ankyloglossia and its treatment. DESIGN: Anonymous written survey. PARTICIPANTS: Otolaryngologists (OTO, n=423), pediatricians (PD, n=425), speech pathologists (SP, n=400), and lactation consultants (LC, n=350) were randomly selected from professional membership lists, with a response rate of 209 (49%), 235 (55%), 150 (37.5%), and 203 (58%), respectively. CLINICAL FINDINGS: Sixty-nine percent of LCs, but a minority of physician respondents, believe tongue-tie is frequently associated with feeding problems. Sixty percent of OTOs, 50% of SPs, but only 23% of PDs believe tongue-tie is at least sometimes associated with speech difficulties. Sixty-seven percent of OTOs versus 21% of PDs believe tongue-tie is at least sometimes associated with social/mechanical issues. Surgery is recommended at least sometimes for feeding, speech, and social/mechanical issues by 53, 74, and 69% of OTOs, respectively, but by only 21%, 29%, and 19% of PDs. CONCLUSION: The significance of ankyloglossia in children remains controversial, both within, and between, specialty groups.

Impact of tympanostomy tubes on child quality of life.

BACKGROUND: The objective benefits of tympanostomy tubes for otitis media are well established, but the subjective impact of surgery on child quality of life (QOL) has not been systematically studied. OBJECTIVES: To determine the subjective impact of tympanostomy tubes on child QOL, and to compare the variability in QOL before surgery with that observed after surgery. DESIGN: Prospective, observational, before-and-after trial. SETTING: Fourteen referral-based pediatric otolaryngology practices in the United States. PATIENTS: Consecutive (64%) and convenience (36%) sample of 248 children (median age, 1.4 years) with otitis media scheduled for bilateral tympanostomy tube placement as an isolated surgical procedure. INTERVENTION: Tympanostomy tubes were inserted as part of routine clinical care. Validated measures of QOL (OM-6 survey), satisfaction with health care decision (Satisfaction With Decision Scale), and satisfaction with office visit; surveys were completed at baseline (visit 1), at surgery (visit 2), and after surgery (visit 3). MAIN OUTCOME MEASURES: Short-term changes in QOL before surgery (visit 1 to visit 2) and after surgery (visit 2 to visit 3). RESULTS: Changes in QOL before surgery were mostly trivial, and were smaller than changes observed after surgery (P<.001). Large, moderate, and small improvements in QOL occurred after surgery in 56%, 15%, and 8% of children, respectively. Physical symptoms, caregiver concerns, emotional distress, and hearing loss were most improved, but significant changes were also seen for activity limitations and speech impairment. Trivial changes occurred in 17% of children, and 4% had poorer QOL. Predictors of poorer QOL were otorrhea 3 or more days (10% of variance) and decreased satisfaction with surgical decision (3% of variance). Hearing status, child age, type of otitis media (recurrent vs chronic), and office visit satisfaction were unrelated to outcome. CONCLUSIONS: Tympanostomy tubes produce large short-term improvements in QOL for most children. The best outcomes occur when postoperative otorrhea is absent or minimal, and when parents are satisfied with their initial decision to have surgery. Further research is needed to document the long-term impact of tubes on child QOL.

Pediatric sleep-related breathing disorders.

Sleep-related breathing disorders (SRBD) can occur at any age. Obstructive sleep apnea, upper airway resistance syndrome and obstructive hypopnea syndrome all lie on the pathological continuum of SRBD. These disorders can have a great impact on a child's quality of life and can progress to significant complications. The symptoms, signs, work-up, and treatment of SRBD in children are discussed.

Isolated large vestibular aqueduct syndrome in a family.

This paper presents the second case in the literature of large vestibular aqueduct syndrome without associated cochlear anomalies in 2 members of the same family. The syndrome is frequently associated with sensorineural hearing loss presenting in childhood. The onset is commonly sudden, following an event causing increased intracranial pressure. On the basis of an emerging pattern of inheritance, we recommend screening siblings of an affected child. We also discuss the importance of characterizing the extent of disease of the inner ear.

Ankyloglossia: incidence and associated feeding difficulties.

OBJECTIVES: To determine the incidence of ankyloglossia (tongue-tie) in the well-baby population, and to determine whether patients with ankyloglossia experience breastfeeding difficulties. DESIGN: Prospective controlled study. SETTING: Tertiary care children's hospital. PATIENTS: A total of 1041 neonates in the well-baby nursery were screened for ankyloglossia. Those positively identified were invited to participate in the study. Mothers of newborns with ankyloglossia and mothers of a matched control group of unaffected newborns were contacted by telephone on a monthly basis for 6 months after their children were discharged from the hospital to determine the presence of breastfeeding difficulties. MAIN OUTCOME MEASURES: Incidence of ankyloglossia, percentage of infants successfully breastfed, and incidence of breastfeeding difficulties. RESULTS: Fifty newborns were identified with ankyloglossia, for an incidence of 4.8% The male-female ratio was 2.6:1.0. Of the 36 mothers of affected infants who were followed up and who intended to breastfeed, 30 (83%) successfully breastfed their infants for at least 2 months, compared with 33 (92%) of the 36 mothers of infants in the matched control group (P = .29). Breastfeeding difficulties were experienced by 9 (25%) of the mothers of infants with ankyloglossia compared with 1 (3%) of the control mothers (P<.01). CONCLUSION: Ankyloglossia, which is a relatively common finding in the newborn population, adversely affects breastfeeding in selected infants.

Retropharyngeal abscess management in children: current practices.

Retropharyngeal abscesses (RAs) in children are uncommon in the modern antibiotic era. As a result, there are few large series outlining the management of these infections in contemporary literature. The goal of this study is to determine the current standard of care for RA. The membership of the American Society of Pediatric Otolaryngology was surveyed, and the response rate was 77.5%. Seventy-two percent of practitioners reported that CT is their preferred diagnostic method. Nearly two thirds recommended a trial of intravenous antibiotics at least occasionally for suspected RA before operative drainage was considered; 51% of respondents indicated that 20% to 40% of RA may resolve with antibiotics alone. Intraoral incision and drainage is the surgical technique preferred by 83% of respondents. Tracheotomy and short-term intubation (24 to 72 hours) are rarely required. This study defines current management practices for RA in children among pediatric otolaryngologists. Results are compared with those in the existing literature.

Safety of pediatric short-stay tonsillectomy.

OBJECTIVE: To determine the safety of a relatively brief (<3-hour) period of postoperative observation prior to discharge in children undergoing outpatient tonsillectomy. DESIGN: Retrospective chart review. SETTING: Tertiary care children's hospital and public teaching hospital. PATIENTS: The records of all patients (12 years of age who underwent tonsillectomy or adenotonsillectomy from November 1995 through July 1997 were reviewed. A total of 143 patients scheduled for ambulatory treatment were identified; 9 were excluded owing to insufficient follow-up. The remaining 134 patients made up the study group. MAIN OUTCOME MEASURES: (1) Duration of observation prior to discharge; (2) complication rates. RESULTS: The mean age of the study population was 6.1+/-2.6 (mean+/-SD) years. Obstructive sleep apnea was an indication for surgery in 86.5%. Eleven (8.2%) of 134 planned outpatients were electively admitted from the recovery room for inpatient observation, most often because of respiratory compromise. Patients admitted from the recovery room were significantly younger (mean age, 4.0 years) than those who were discharged as planned (6.3 years, P<.001). One hundred twenty-three patients were discharged from the recovery room as anticipated, following a mean+/-SD duration of postoperative observation of 144+/-48 minutes. Overall, 5 (4.1%) of these 123 outpatients suffered complications after discharge. Two patients (1.6%) experienced primary bleeding, both at 8 hours after surgery. Four patients (3.2%) were readmitted. The complication rate did not vary significantly with the duration of postoperative observation (P= .71). CONCLUSION: A short postoperative observation period is safe, with a low rate of complications, in appropriately selected children scheduled for ambulatory tonsillectomy.

Safety and biological efficacy of an adeno-associated virus vector-cystic fibrosis transmembrane regulator (AAV-CFTR) in the cystic fibrosis maxillary sinus.

OBJECTIVE: The host immune response and low vector efficiency have been key impediments to effective cystic fibrosis transmembrane regulator (CFTR) gene transfer for cystic fibrosis (CF). An adeno-associated virus vector (AAV-CFTR) was used in a phase I dose-escalation study to transfer CFTR cDNA into respiratory epithelial cells of the maxillary sinus of 10 CF patients. STUDY DESIGN: A prospective, randomized, unblinded, dose-escalation, within-subjects, phase I clinical trial of AAV-CFTR was conducted. PATIENTS: Ten patients with previous bilateral maxillary antrostomies were treated. MAIN OUTCOME MEASURES: Safety, gene transfer as measured by semiquantitative polymerase chain reaction (PCR), and sinus transepithelial potential difference (TEPD) were measured. RESULTS: The highest level of gene transfer was observed in the range of 0.1-1 AAV-CFTR vector copy per cell in biopsy specimens obtained 2 weeks after treatment. When tested, persistence was observed in one patient for 41 days and in another for 10 weeks. Dose-dependent changes in TEPD responses to pharmacologic intervention were observed following treatments. Little or no inflammatory or immune responses were observed. CONCLUSION: AAV-CFTR administration to the maxillary sinus results in successful, dose-dependent gene transfer to the maxillary sinus and alterations in sinus TEPD suggestive of a functional effect, with little or no cytopathic or host immune response. Further study is warranted for AAV vectors as they may prove useful for CFTR gene transfer and other in vivo gene transfer therapies. A prospective, randomized, double-blind, placebo-controlled, within-subjects, phase II clinical trial of the effect AAV-CFTR on clinical recurrence of sinusitis will determine the clinical efficacy of AAV gene therapy for CF.

Maxillary sinusitis as a surrogate model for CF gene therapy clinical trials in patients with antrostomies.

BACKGROUND: Assessing the biological activity and clinical efficacy of gene therapy is critically important in cystic fibrosis (CF). It is widely accepted that clinical testing using surrogate markers including pulmonary function will be useful in assessing clinical efficacy. One problem with pulmonary surrogate markers of CF disease is the large number of patients and length of time required to demonstrate clinical efficacy. An alternative to pulmonary testing of new CF treatments is use of the maxillary sinuses as a surrogate model of CF lung disease. Using CF sinusitis as a surrogate model for testing clinical efficacy of new treatments is attractive because CF upper respiratory disease is similar to the lower respiratory disease with respect to electrophysiology and microbiology. METHODS: Sinusitis recurrence in untreated sinuses was analyzed during a prospective, randomized, unblinded, dose-escalation, within-subjects, phase I clinical trial of the adeno-associated virus mediated cystic fibrosis transmembrane conductance regulator (AAV-CFTR) gene transfer. RESULTS: Clinical symptoms combined with sinus endoscopy proved useful in the diagnosis of unilateral and bilateral sinusitis recurrence. Sinusitis recurred at a rate of 45% during one month of follow-up. IL-8 concentration rose in sinus fluids from affected sinuses. Bacterial cultures and increased sinus leukocytes corroborated recurrent sinusitis. Sinus CT scans were also useful in diagnosing recurrent sinusitis in this surrogate model of CF infectious exacerbations. CONCLUSIONS: CF sinusitis as a surrogate for lung disease is particularly well-suited for phase II clinical trials of gene transfer agents, with the potential for measuring clinical efficacy in relatively small numbers of patients over relatively short periods of time.

Pitfalls in the diagnosis of aerodigestive tract foreign bodies.

In the young child, particularly those between the ages of 1 and 3 years, aerodigestive tract foreign bodies continue to be a significant problem. Early diagnosis and treatment can decrease morbidity and length of hospital stay in these children. Three cases of delayed diagnosis in children with bronchial (2) and esophageal (1) foreign bodies are presented. In dealing with young children it is important to maintain a high clinical suspicion of a foreign body and to perform a detailed history and physical examination. Options in radiographic analysis of the patient with a suspected foreign body, probable locations of the foreign body, and the likelihood of definitive radiographic findings are discussed. Importantly, a negative radiographic analysis does not rule out the presence of an aerodigestive tract foreign body. Two flow charts for the evaluation of patients who have possibly ingested or inhaled a foreign body are presented to aid the primary care physician in diagnosis.

Oral fluid intake following tonsillectomy.

Children undergoing tonsillectomy surgery traditionally have been required to drink a specified amount of fluid before being discharged home. With increasing economic pressures, same-day discharge tonsillectomy has become common, and several studies have shown it to be safe for the appropriately selected child. To examine the role of required oral fluid intake following tonsillectomy, a non-randomized cohort study was performed with 200 consecutive tonsillectomy patients scheduled for same-day discharge. The first 100 patients (group 1) were required to drink 20 cc/kg prior to discharge, and the next 100 patients (group 2) were not required to drink. The mean oral fluid intake prior to discharge decreased significantly between the two groups from 524 cc in group 1 to 130 cc in group 2 (P = 0.0001). The mean length of stay also decreased significantly from 13.8 h for group 1 to 10.0 h for group 2 (P = 0.0001). Sixty percent of children in each group vomited at least once following surgery. Fewer children in group 2 had protracted vomiting (8 versus 15) although this was not statistically significant (P = 0.12). No children were readmitted to the hospital for dehydration following discharge. Overall, 71% of children in group 1 scheduled to go home the same day were discharged on time, compared to 94% of children in group 2. In conclusion, it is safe and economically efficient to discharge tonsillectomy patients home prior to the resumption of normal oral fluid intake.

Nasal growth after external septoplasty in children.

OBJECTIVE: To assess the impact of external septoplasty surgery on nasal growth in children. DESIGN: Twelve anthropometric measurements (9 linear and 3 angular) were obtained in patients who previously underwent external septoplasty surgery for severe nasal obstruction caused by septal deviation anterior to the nasal spine. Surgery consisted of excision, refashioning, and reinsertion of the quadrilateral cartilage. From these 12 measurements, 5 proportional indexes were calculated, and then all measurements and proportions were compared with previously published norms. Follow-up measurements were taken at least 2 years after surgery (average, 3.4 years). SETTING: The Hospital for Sick Children, Toronto, Ontario, a tertiary care children's hospital. PARTICIPANTS: Twenty-eight patients who underwent external septoplasty surgery between the ages of 6 and 15 years. RESULTS: The principal measurements of the face and nose were within 1 SD of the normative mean for the majority of those in the study group. This was true for nasal height, nasal tip protrusion, nasal width, columella width, columella length, upper face height, face height, face width, inclination of the upper face, inclination of the nasal dorsum, and inclination of the columella. Values for 4 of the 5 proportional indexes were also overwhelmingly in the normal range. Twenty-nine percent of nasal dorsum measurements and 57% of nasal dorsum indexes were more than 2 SDs from the mean, indicating a predominance of short nasal dorsums. CONCLUSIONS: External septoplasty does not affect most aspects of nasal and facial growth, but it may negatively influence growth of the nasal dorsum. Prospective studies are needed to clarify this issue.

Otoplasty. Clinical protocol and long-term results.

OBJECTIVE: To evaluate the long-term results after otoplasty on prominent ears. DESIGN: Between 1988 and 1993, ear protrusion was measured preoperatively and postoperatively in pediatric patients undergoing otoplasty by means of a standard protocol based on the Frankfort horizontal line. Patients were asked to return for follow-up measurements a minimum of 1 year after surgery. At the time of follow-up, a patient satisfaction survey was completed by the patients and their families. SETTING: The Hospital for Sick Children, Toronto, Ontario, a tertiary care children's hospital. PARTICIPANTS: Thirty-one of 51 patients returned for follow-up an average of 3.7 years after surgery. RESULTS: One third of ears returned to their original position, one third of ears stayed in a position equal to the immediate postoperative position, and one third of ears had final positions between the preoperative and post-operative positions. At the superior rim, an average of 58% of the operative medialization was lost. Good to excellent ear-to-ear symmetry was obtained in 78% of patients who returned for follow-up. Retrospective chart review showed a revision surgery rate of 3%; stitch granulomas were removed in 9% of patients. The patient satisfaction survey found that 85% of patients were happy or very happy with their ears. CONCLUSIONS: With time, a substantial loss of correction can be expected in most (but not all) patients who undergo otoplasty, particularly at the upper pole. Overall, patients and their families are happy with the results of otoplasty surgery.

Effect of intermittent acid and pepsin exposure on burned esophageal mucosa.

PURPOSE: To determine if gastroesophageal reflux affects the healing of esophageal mucosa damaged by alkaline substances. MATERIALS AND METHODS: In a rabbit model, the effects of intermittent acid and pepsin exposure (thereby mimicking gastroesophageal reflux) on post-caustic-burn esophageal mucosa were examined. Exposures were group I (n = 9), sodium hydroxide exposure alone; group II (n = 9), daily acid and pepsin exposure; and group III (n = 8), sodium hydroxide, then daily acid and pepsin exposure. After 3 weeks, the animals were killed and their esophagi examined. RESULTS: Grossly, specimens in group II appeared normal; specimens in groups I and III were abnormal, displaying some scar tissue, but were not significantly different. Histologically, the differences between groups I and III approached significance. Increased numbers of intraepithelial eosinophils were seen in those specimens exposed to reflux. CONCLUSIONS: Gastroesophageal reflux may retard the healing of injured esophageal mucosa and should be studied further in an effort to improve the care of patients who sustain alkaline esophageal burns.