Carol J. MacArthur

Neonatal Hearing Screening with Otoscopy, Auditory Brain Stem Response, and Otoacoustic Emissions

A study was performed to investigate the relationship between external and middle ear factors and hearing screening results by auditory brain stem response (ABR) and transient evoked otoacoustic emissions (EOAEs). The ears of 200 well newborns aged 5 hours to 48 hours underwent screening by ABR and EOAEs, followed by otoscopic examination. The pass rates for ABR and EOAE screening were 88.5% and 79%, respectively. On otoscopic examination, 13% (53 of 400) ears had occluding vernix obscuring the view of the tympanic membrane. Cleaning of vernix was attempted in ears that failed ABR or EOAE screening. Seventeen ears that failed ABR were cleaned, and 12 (71%) of them passed repeat ABR. Thirty-three ears that failed EOAE screening were cleaned, and 22 (67%) of them passed repeat emissions testing. Cleaning vernix increased the pass rates for ABR and EOAE screening to 91.5% and 84%, respectively. Decreased tympanic membrane mobility was found in 9% of ears that could be evaluated otoscopically. Increased failure rates for both ABR and EOAE screening were found in infant ears with decreased tympanic membrane mobility, but significance testing could not be performed because of inadequate sample size. Prevalence of occluding external canal vernix and middle ear effusion as a function of increasing infant age were studied. Implications for newborn hearing screening are discussed.

Pediatric Head and Neck Rhabdomyosarcoma

Rhabdomyosarcoma (RMS), the most common malignant soft-tissue tumor in the pediatric population, occurs most often in the head and neck region. Major advancements in the management of these aggressive tumors have been achieved in the last 20 years on three fronts: chemotherapy, radiation therapy, and surgery. Surgery, with the advent of skull-base, craniofacial, and free-tissue-transfer reconstructive techniques, has once again become part of the primary management of these tumors. Prior to 1960, RMS was uniformly fatal; now, with the introduction of multimodality treatment regimens, two thirds of patients can expect long-term survival.

Murine middle ear inflammation and ion homeostasis gene expression

Hypothesis: Ion homeostasis genes are responsible for the movement of ions and water in the epithelium of the middle ear. Background: It is not well known to what extent disruption of ion homeostasis is a factor in the accumulation of middle ear fluid during otitis media. Methods: Balb/c mice were transtympanically injected with heat-killed Hemophilus influenza bacteria. Untreated and saline-injected mice were used as controls. Mice were euthanized at 6, 24, and 72 hours and 1 week after injection, the bullae harvested, and total ribonucleic acid isolated from the middle ear tissues. Ion homeostasis genes were analyzed with real-time quantitative reverse transcription-polymerase chain reaction from the following gene families: Na+,K+-ATPase, claudins, K+ transport channels, epithelial Na+ channels, gap junctions, and aquaporins. Inflammatory genes also were analyzed to document inflammation. Results: All inflammatory genes analyzed were significantly upregulated, more at 6 hours than at 24 hours, with the exception of vascular endothelial growth factor and Mapk8. Most middle ear ion homeostasis genes experienced downregulation because of inflammation. This was most prominent in the aquaporin and Na+,K+-ATPase genes. Significant upregulation was seen in several genes in response to inflammation and saline independently. Conclusion: The innate immune response to bacteria in the middle ear induces expression of several inflammatory genes. Coinciding with this inflammation is the downregulation of numerous ion homeostasis genes that are involved in ion and water transport and maintenance of tight junctions. This may explain the fluid accumulation within the middle ear seen with both acute and chronic otitis media.

The Effect of the Palatoplasty Method on the Frequency of Ear Tube Placement

OBJECTIVEnTo determine whether the type of palate repair affects the frequency of subsequent ventilation tube placement.nnnDESIGNnCombined retrospective and prospective cohort with more than 2 years clinical follow-up after palatoplasty.nnnSETTINGnTertiary care childrens hospital and clinic.nnnPATIENTSnA total of 170 patients with cleft palate (with or without cleft lip) underwent palatoplasty between 1995 and 2003. Sixty-nine patients with less than 2 years of follow-up visits and 1 patient who did not require ear tubes were excluded from this analysis.nnnINTERVENTIONSnEither traditional 2-flap palatoplasty (group A) or double-opposing Z-plasty (group B) was performed. The type of palatoplasty performed was based on the reconstructive surgeons clinical decision. Ventilation tubes were placed for otitis media, conductive hearing loss, or eustachian tube dysfunction. Patients received routine follow-up care every 6 months or whenever acute problems arose. Data were analyzed with independent t tests, chi(2) tests, and Fisher exact tests.nnnMAIN OUTCOME MEASURESnNumber of ear tubes placed after palatoplasty in each group.nnnRESULTSnGroup A had a mean (SE) of 2.9 (0.2) sets of tubes placed, while group B had a mean (SE) of 1.8 (0.2) sets of tubes. Group A had significantly more sets of ventilation tubes placed (P < .001) than group B. Subgroup analysis based on type of cleft was performed.nnnCONCLUSIONnChildren with cleft palate who underwent double-opposing Z-plasty had fewer sets of ear tubes placed postoperatively than patients who had traditional repair.

Steroid Control of Acute Middle Ear Inflammation in a Mouse Model

OBJECTIVEnTo investigate steroids for their potential for therapeutic approaches to control otitis media. Glucocorticoids and mineralocorticoids have differential effects on inflammation and fluid absorption, but little is known of their control of middle and inner ear manifestations of acute otitis media.nnnDESIGNnBoth glucocorticoid (prednisolone and dexamethasone) and mineralocorticoid (aldosterone and fludrocortisone) steroids were investigated for their ability to reduce inflammatory symptoms in a mouse otitis media model.nnnSETTINGnAcademic medical center.nnnSUBJECTSnAcute inflammation was induced by transtympanic injection of heat killed Streptococcus pneumoniae to 100 BALB/c mice.nnnINTERVENTIONSnTwenty mice in each experimental group (prednisolone, dexamethasone, aldosterone, and fludrocortisone) were given a steroid in their drinking water the day before inoculation, and these treatments were continued until the mice were killed for histologic examination. Twenty control mice were treated with water only.nnnMAIN OUTCOME MEASURESnHistologic measure of inflammation: middle ear fluid, inflammatory cell number, and tympanic membrane thickness.nnnRESULTSnHistologic middle ear morphometrics showed significant steroid effects at both 3 and 5 days in reduction of fluid area, cell number, and tympanic membrane thickness.nnnCONCLUSIONSnGlucocorticoids were most effective in controlling inflammation. Interestingly, the mineralocorticoids were also effective in reducing the inflammatory response at 5 days, suggesting that their fluid transport function helped clear disease. Thus, steroid control of middle ear disease may be useful in alleviating symptoms faster and reducing the risk to the inner ear.

Efficacy of Tonsillectomy for Pediatric Patients With Dysphagia and Tonsillar Hypertrophy

OBJECTIVEnTo determine the effectiveness of tonsillectomy for the treatment of dysphagia related to tonsillar hypertrophy.nnnDESIGNnProspective cohort study.nnnSETTINGnTertiary care pediatric otolaryngology practice.nnnPARTICIPANTSnEighty-five children aged 2 to 14 years referred for tonsillectomy owing to dysphagia related to tonsillar hypertrophy (dysphagia cohort) or for other indications (control cohort).nnnINTERVENTIONSnSwallowing Quality of Life (SWAL-QOL) dysphagia questionnaires were administered at the initial clinic visit, on the day of surgery, and at 1 month and 6 months after surgery. Patients were weighed on the day of surgery and at 1 month after surgery.nnnMAIN OUTCOME MEASURESnThe primary outcome measure was the SWAL-QOL score. Secondary outcome measures were the type of diet consistency patients tolerated at home and the weight percentile for age.nnnRESULTSnOf 85 patients enrolled, 57 went on to have surgery, completed at least 1 postoperative questionnaire, and were included in the data analysis. At 1 month after tonsillectomy, the dysphagia cohort (nxa0=xa018) demonstrated improved SWAL-QOL scores (mean [SD], 58.4 [4.8] before surgery vs 82.4 [5.3] after surgery; Pxa0<xa0.001), more patients tolerating a regular diet (12 of 37 patients [33.3%] before surgery vs 22 of 36 [60.0%] after surgery, Pxa0=xa0.01), and increased weight percentile for age (mean [SD], 36.5 [10.7] before surgery vs 50.0 [10.6] after surgery; Pxa0=xa0.01). Similarly, at 1 month after tonsillectomy, the control cohort (nxa0=xa039) demonstrated improved SWAL-QOL scores (mean [SD], 80.8 [2.6] before surgery vs 91.7 [1.8] after surgery; Pxa0<xa0.001), more patients tolerating a regular diet (30 of 37 patients [81.1%] before surgery vs 34 of 36 patients [94.4%] after surgery, Pxa0=xa0.04), and increased weight percentile for age (mean [SD], 62.8 [5.4] before surgery vs 70.4 [5.1] after surgery; Pxa0=xa0.003).nnnCONCLUSIONSnDysphagia related to tonsillar hypertrophy is a significant problem not only among children with dysphagia with a primary complaint but also among a large subset of patients referred for tonsillectomy for other indications. Following tonsillectomy, both groups experience significant improvement in swallowing-related quality of life, ability to tolerate a regular diet, and weight percentile for age. Tonsillectomy is an effective treatment for the management of dysphagia related to tonsillar hypertrophy in children.

Paranasal phycomycosis in the immunocompetent host

Rhinocerebralphycomycosis is a rapidly destructive and often fatal infection of the immunocompromised individual. Although classically known to occur in diabetic patients with ketoacidosis, it has been observed in the clinical settings of renal failure, hematologic disorders, malnutrition, cancer, and iatrogenic immunosuppression. We present a previously unreported and unusual case of a healthy 4-year-old child in whom paranasal phycomycosis and subsequent midface bony destruction developed while he was being treated with steroids and broad spectrum antibiotics for an otologic condition. This case illustrates the potential for this lifethreatening infection in the previously healthy host. It underscores the need to consider phycomycosis as a nosocomial infectious agent in the actuely ill patient, because early recognition and aggressive treatment of this disease is integral to a favorable outcome.

Otolaryngologic manifestations of Multiple Pterygium Syndrome

Escobar Syndrome, or Multiple Pterygium Syndrome (MPS), is a rare syndrome with multiple congenital anomalies involving the head and neck area and limbs. Affected individuals have multiple pterygia, camptodactyly and/or syndactyly as the main features of this syndrome. Patients with MPS have a characteristic facies, including ptosis, antimongoloid slant of the palpebral fissures, hypertelorism, micrognathia, neck pterygia, and a sad flat, emotionless look to the face. We present a case of MPS recently treated at our institution for the purpose of further describing the clinical features of this syndrome, emphasizing the otolaryngologic manifestations. Increased awareness of MPS will facilitate appropriate management of this syndrome.

Sinus complications in mucopolysaccharidosis I H/S (Hurler-Scheie syndrome)

Hurler-Scheie syndrome is a genetic compound of two mucopolysaccharidoses, the Hurler and Scheie syndromes. The genetic error of metabolism caused by this syndrome produces intermediary systemic effects in the affected individuals. Lacking the enzyme alpha-L-iduronidase, glycosaminoglycans are deposited in the tissues, causing multiple systemic effects and creating many problems for the otolaryngologist. Although early bone marrow transplantation is currently being performed to prevent or reverse many of the systemic manifestations of this disorder, there remains a large population of children past the age at which bone marrow transplantation can be effective. Sinus complications have not previously been reported in the mucopolysaccharidoses and may be more frequently recognized as these patients longevity increases. We present a patient with Hurler-Scheie syndrome who developed severe nasal polyposis requiring sinus endoscopic removal.

Head-mounted display for use in functional endoscopic sinus surgery

Since the introduction of functional endoscopic sinus surgery (FESS), the procedure has undergone rapid change with evolution keeping pace with technological advances. The advent of low cost charge coupled device 9CCD) cameras revolutionized the practice and instruction of FESS. Video-based FESS has allowed for documentation of the surgical procedure as well as interactive instruction during surgery. Presently, the technical requirements of video-based FESS include the addition of one or more television monitors positioned strategically in the operating room. Thought video monitors have greatly enhanced surgical endoscopy by re- involving nurses and assistants in the actual mechanics of surgery, video monitors require the operating surgeon to be focused on the screen instead of the patient. In this study, we describe the use of a new low-cost liquid crystal display (LCD) based device that functions as a monitor but is mounted on the head on a visor (PT-O1, O1 Products, Westlake Village, CA). This study illustrates the application of these HMD devices to FESS operations. The same surgeon performed the operation in each patient. In one nasal fossa, surgery was performed using conventional video FESS methods. The contralateral side was operated on while wearing the head mounted video display. The device had adequate resolution for the purposes of FESS. No adverse effects were noted intraoperatively. The results on the patients ipsalateral and contralateral sides were similar. The visor did eliminated significant torsion of the surgeons neck during the operation, while at the same time permitted simultaneous viewing of both the patient and the intranasal surgical field.