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Poor eustachian tube function and prevalence of infectious ear disease are thought to be the reasons for less successful outcome associated with tympanoplasty in children. Since both of these factors are related to age, identification of those patients who may benefit by delaying the surgery has been a concern to otolaryngologists. In an effort to investigate the role of eustachian tube function on the outcome, we tested the tubal function in 63 ears (56 children) undergoing tympanoplasty for central perforations. In 49 ears (78%), the graft took; of these, 33 had good middle-ear function, 8 developed persistent or recurrent otitis media, and 8 had severe retraction or atelectasis. There was a significant association (p less than 0.01) between outcome and preoperative tubal function as determined by combining the active and passive function parameters. However, the prognostic value of tubal function testing was low; predictive values for success and lack of success being 68% and 56%, respectively. Other factors, such as graft placement (medial or lateral) contralateral ear status, and child's age, were not associated with outcome. Consistent with other studies, good eustachian tube function was shown to predict good outcome, but poor tubal function was not helpful in predicting poor outcome. [ABSTRACT FROM AUTHOR]

Tympanomastoid surgery is considered standard management for chronic suppurative otitis media (CSOM) without cholesteatoma, which is unresponsive to ototopical/oral antimicrobial therapy. The following makes this sequence of management less attractive today: 1. potential ototoxicity of ototopical agents; 2. lack of oral antimicrobial agents effective against most common pathogens (e.g., Pseudomonas aeruginosa); 3. frequent occurrence in children who have tympanostomy tubes; and 4. failure of tympanomastoid surgery to eradicate the disease in all cases. We conducted a study in 36 pediatric patients with chronic suppurative otitis media, in which all received parenteral antimicrobial therapy and daily aural toilet (mean duration of treatment = 9.7 days). Thirty-two patients (89%) had resolution of their infection with medical therapy alone; four children required tympanomastoidectomy. Further investigation is needed to understand the etiology, pathogenesis, and most effective methods of management/prevention of CSOM in children. [ABSTRACT FROM AUTHOR]

Objective: To determine the spectrum of connexin 26 (Cx26) mutations and their phenotypes in children with sensorineural hearing loss (SNHL) or mixed hearing loss (MHL)., Design: Children with SNHL or MHL were prospectively tested for mutations in the entire coding region of the Cx26 gene., Patients: Children with SNHL or MHL with no obvious etiology for the hearing loss., Results: Between December 1, 1998, and July 1, 2000, 107 patients with SNHL or MHL from 99 families underwent Cx26 testing. Most patients were aged 1 week to 16 years (61 boys and 46 girls). Thirty (30%) of 99 probands had Cx26 mutations: biallelic mutations were detected in 18 (9 homozygous and 9 compound heterozygous) and single mutations were detected in 12. Twelve previously reported mutations (35delG, 167delT, E47X, L90P, M34T, G12V, V37I, R143W, V84L, V153I, V27I, and 310del14) and 3 novel mutations (E129K, T8M, and N206S) were found. Hearing loss in patients with biallelic Cx26 mutations ranged from unilateral high frequency to bilateral profound. Four children, 2 with biallelic mutations, had temporal bone abnormalities., Conclusions: Connexin 26 mutations are common in children with SNHL, and it is likely that the homozygous and compound heterozygous mutations cause the SNHL. However, pathogenicity is less certain when only a single Cx26 mutation is present. Patients with biallelic Cx26 mutations had a slightly higher incidence of milder hearing loss than in previous studies. Children with SNHL or MHL should be tested for Cx26 mutations early in their evaluation.

Objectives: To evaluate the clinical, audiologic, and temporal bone computed tomograpic findings in patients with hemifacial microsomia and to use the OMENS (each letter of the acronym indicates 1 of the following 5 dysmorphic manifestations: O, orbital asymmetry; M, mandibular hypoplasia; E, auricular deformity; N, nerve involvement; and S, soft tissue deficiency) grading system to assess possible correlations between the severity of dysmorphic features with the type of abnormalities in the temporal bone and with degree of hearing deficit., Design: Retrospective study., Setting: Tertiary care children's hospital., Patient: Forty patients with hemifacial microsomia., Result: Mandibular hypoplasia and auricular abnormalities were the most common clinical manifestations, present in 39 patients (97%) and 38 patients (95%), respectively. Conductive hearing loss was noted in 35 patients (86%) and sensorineural hearing loss in 4 patients (10%). Facial nerve weakness was present in 20 patients (50%). Twenty patients had unilateral aural atresia, 12 patients had unilateral aural stenosis, and 7 patients had bilateral anomalies. Moderate hypoplasia or atresia of the middle ear was noted in 36 patients (90%) and ossicles were malformed in 30 patients (75%). Hypoplasia of the oval window was the most common inner ear abnormality., Conclusions: Severity of craniofacial features (total OMENS score) significantly correlated with the degree of temporal bone abnormality, but no correlation was noted with the degree or type of hearing loss. We recommend the following: (1) use of the OMENS classification system for documentation and analysis of dysmorphic finding in hemifacial microsomia; (2) complete audiologic evaluation in all patients with hemifacial microsomia regardless of the type of craniofacial abnormalities; and (3) temporal bone computed tomography for further evaluation of hearing deficit.

Objective: The purpose of this study was to investigate the association between deep dental overbite and eustachian tube dysfunction (ETD)., Design: Case-control study., Setting: Tertiary care pediatric otolaryngology outpatient clinic at the Children's Hospital, Boston, Massachusetts., Patients: 105 patients between the ages of 2 and 6 years. STUDY MEASUREMENTS: Dental overbite, overjet, and occlusal relationships were measured by an observer who was unaware of ETD status. ETD was defined as having ventilation tubes in place or having the recommendation for ventilation tube placement by an attending pediatric otolaryngologist. In addition, demographic information and medical and social histories were prospectively recorded., Results: In a multivariate logistic regression model, children with deep bites were 2.8 times more likely to have ETD than those without deep bites (P = .03). Other independent risk factors for ETD identified in this model were family history of otitis media (OM) and age less than 3 years., Conclusions: Children with deep dental overbites are at a significantly increased risk for developing ETD.

Objective: To develop a technique to identify and localize the recurrent laryngeal nerve (RLN) during video-assisted thoracoscopic surgery (VATS) for patent ductus arteriosus., Design: Prospective clinical study., Setting: Children's hospital., Participants: Sixty infants and children scheduled for elective closure of patent ductus arteriosus., Interventions: With parental informed consent, 60 infants and children undergoing elective VATS for patent ductus arteriosus were studied. A thin, pencil-point, Teflon-coated, stimulating probe allowed direct stimulation (<2 mA, 100-msec pulse width) of the left RLN inside the thorax. A commercially available 4-channel neurologic monitor recorded compound evoked electromyograms (EMGs) from the left RLN and right RLN (as control) by needle electrodes placed percutaneously in the neck. Hoarseness, stridor, feeding difficulties, and voice changes were assessed postoperatively., Measurements and Main Results: Left RLN EMGs were easily obtained in 59 of the 60 patients. The surgeon correctly identified the RLN visually once in the first 7 patients; this ability subsequently improved. EMG localization of the location or course of the RLN altered dissection, clip size, or clip position in 37 of 59 patients., Conclusion: Intraoperative EMG to identify location and route of the RLN was easy to perform, was effective in identifying RLN position, and appeared to facilitate dissection and clipping of the ductus.

The yeast Sir2 protein, required for transcriptional silencing, has an NAD(+)-dependent histone deacetylase (HDA) activity. Yeast extracts contain a NAD(+)-dependent HDA activity that is eliminated in a yeast strain from which SIR2 and its four homologs have been deleted. This HDA activity is also displayed by purified yeast Sir2p and homologous Archaeal, eubacterial, and human proteins, and depends completely on NAD(+) in all species tested. The yeast NPT1 gene, encoding an important NAD(+) synthesis enzyme, is required for rDNA and telomeric silencing and contributes to silencing of the HM loci. Null mutants in this gene have significantly reduced intracellular NAD(+) concentrations and have phenotypes similar to sir2 null mutants. Surprisingly, yeast from which all five SIR2 homologs have been deleted have relatively normal bulk histone acetylation levels. The evolutionary conservation of this regulated activity suggests that the Sir2 protein family represents a set of effector proteins in an evolutionarily conserved signal transduction pathway that monitors cellular energy and redox states.

Objective: To ascertain the present common causes of sensorineural hearing loss (SNHL) in children and compare them with those of previous reports., Design: A retrospective review of the medical records for all children with a diagnosis of SNHL seen from January 1, 1993, through September 30, 1996, at our institution., Setting: A tertiary care children's hospital., Patients: Three hundred one children, aged 1 week through 18 years, who presented for evaluation of SNHL., Results: Of the 301 children, 68.1% had a definite or probable cause of their SNHL identified; 18.9%, 1 or more possible causes; and 31.9%, no obvious cause. A family history of SNHL or prematurity and/or complicated perinatal course was found in 28.6% of patients. Named syndromes, multiple congenital anomalies, meningitis, or prenatal maternal factors, including maternal prenatal substance abuse, were present in another 38.5%. However, syndromes commonly reported to be associated with SNHL, such as Waardenburg syndrome, were seen in less than 1% of patients. The average age at diagnosis was 3.02 years for the bilateral moderate or worse SNHL; for unilateral SNHL, the average age was 3.97 years. The most useful diagnostic study was computed tomographic scanning., Conclusions: Sensorineural hearing loss is fairly common in children. Extensive workups, often without clear direction, should be reconsidered based on the children with SNHL who otolaryngologists are now seeing. Infant screening programs, although identifying many children earlier, will also provide the opportunity to fine-tune the evaluation (ie, cytomegalovirus titers and/or cultures at birth), increasing the diagnostic yield.

Background: Acupuncture or acupressure at the Nei-Guan (P.6) point on the wrist produces antiemetic effects in awake but not anesthetized patients. The authors studied whether a combined approach using preoperative acupressure and intra- and postoperative acupuncture can prevent emesis following tonsillectomy in children., Methods: Patients 2-12 yr of age were randomly assigned to study or placebo groups. Two Acubands with (study) and two without (placebo) spherical beads were applied bilaterally on the P.6 points; non-bead- and bead-containing Acubands, respectively, were applied on the sham points. All Acubands were applied before any drug administration. After anesthetic induction, acupuncture needles were substituted for the beads and remained in situ until the next day. All points were covered with opaque tape to prevent study group identification. A uniform anesthetic technique was used; postoperative pain was managed initially with morphine and later with acetaminophen and codeine. Emesis, defined as retching or vomiting, was assessed postoperatively. Ondansetron was administered only after two emetic episodes at least 2 min apart. Droperidol was added if emesis persisted., Results: One hundred patients were enrolled in the study. There were no differences in age, weight, follow-up duration, or perioperative opioid administration between groups. Retching occurred in 26% of the study patients and in 28% of the placebo patients; 51 and 55%, respectively, vomited; and 60 and 59%, respectively, did either. There were no significant differences between the groups. Redness occurred in 8.5% of acupuncture sites., Conclusion: Perioperative acupressure and acupuncture did not diminish emesis in children following tonsillectomy.

To date, only ofloxacin has been approved by the US Food and Drug Administration for treatment of ears with a nonintact tympanic membrane. The purpose of this study was to determine the safety and efficacy of topical ciprofloxacin hydrochloride in the treatment of experimental chronic suppurative otitis media caused by Pseudomonas aeruginosa infection in cynomolgus monkeys. Forty adult cynomolgus monkeys were divided into 4 equal groups, and their ears were challenged with P aeruginosa, drained for 3 weeks, then treated twice daily for 4 weeks with 1 of 4 randomly assigned agents: 1) ciprofloxacin, 2) saline, 3) Cortisporin, or 4) vehicle. The animals were followed up with auditory brain stem response testing, culture, otoscopy, and histopathology. Both ciprofloxacin and Cortisporin treatment resulted in a significantly more rapid rate of clearance of P aeruginosa as compared to treatment with saline (100% versus 20%). Eradication was not associated with resolution of otorrhea after a 4-week period of treatment. There were no significant changes in auditory brain stem response wave latencies for any of the treatment groups. Histopathologic data revealed that there was no statistically significant difference in the amount of outer hair cell loss for the ciprofloxacin group as compared to the control ear and other treatment groups. We conclude, therefore, that topical ciprofloxacin is not ototoxic and is effective in sterilizing the otorrhea, but does not promote resolution of the drainage, in this animal model.

Retrotransposon Ty1 faces a formidable cell barrier during transposition--the yeast nuclear membrane which remains intact throughout the cell cycle. We investigated the mechanism by which transposition intermediates are transported from the cytoplasm (the presumed site of Ty1 DNA synthesis) to the nucleus, where they are integrated into the genome. Ty1 integrase has a nuclear localization signal (NLS) at its C terminus. Both full-length integrase and a C-terminal fragment localize to the nucleus. C-terminal deletion mutants in Ty1 integrase were used to map the putative NLS to the last 74 amino acid residues of integrase. Mutations in basic segments within this region decreased retrotransposition at least 50-fold in vivo. Furthermore, these mutant integrase proteins failed to localize to the nucleus. Production of virus-like particles, reverse transcriptase activity, and complete in vitro Ty1 integration resembled wild-type levels, consistent with failure of the mutant integrases to enter the nucleus.