International Journal of Pediatric Otorhinolaryngology

Editorial Board

2010-08-01

The influence of the auditory prosthesis type on deaf children's voice quality

Jesús Valero Garcia, Josep M. Vila Rovira, Laura González Sanvicens — 2010-08-01

Abstract: Objective: In the last years, technology has made it possible for deaf people, and especially for deaf children, to enter the world of sound and, as a consequence, to facilitate communicative competence in oral language, which used to be an insurmountable obstacle. With this article we are trying to carry out a revision of the descriptions that have traditionally featured deaf children's voice.Methods: We analyse the voice quality obtained out of a research with 62 children (35 girls and 27 boys) with profound deafness of several degrees, aged on average 7 years 4 months, and users of different types of auditory prostheses (analogue/digital hearing aids or cochlear implants) experimental group – Voice quality was evaluated from the production of a sustained vowel /a:/ for four-five seconds, considering F0, jitter, shimmer and NHR values.Results: The results are compared with the ones obtained from a control group of hearing children (n=54). The Experimental Group shows altered voice quality parameters. Particularly in F0 (294.079Hz) and shimmer (0.568dB), there are statistically significant values in comparison with the control group (p<0.001). With regards to jitter (1.474%), differences were smaller. Nevertheless, the results show how the profiles reached by deaf children are nowadays more similar to those reached by the hearing control group. However, the degree of hearing impairment and the type of prosthesis used can determine the parameters of deaf children's voice quality to a great extent. Thus, the digital hearing aid users are the ones who present better voice quality values: F0 (265.50z); jitter (1.009%) and shimmer (0.486dB); whereas implant users: F0 (287.93z); jitter (1.344%) and shimmer (0.526dB), and particularly analogue hearing aid users: F0 (323.80z); jitter (1.999%) and shimmer (0.687dB), did show significant differences in comparison with the control group of hearing children.Conclusions: In the study of voice quality in children with profound hearing loss, it is very important to have information both about the degree of hearing loss and the kind of prosthesis used. Implant users show more altered voice quality than digital hearing aid users. However, the hearing loss they compensate is much more important than the hearing loss compensated by the hearing aids. Therefore, we consider that both prostheses help children with hearing loss to have a more normalized voice quality than what scientific literature has traditionally stated.Finally, we question the validity of using some acoustic parameters as indicators of voice quality in deaf children having no laryngeal problems.

Battery ingestion in children

Tal Marom, Abraham Goldfarb, Eyal Russo, Yehudah Roth — 2010-08-01

Abstract: Introduction: Ingestion of batteries by children became more frequent in recent years, due to the increasing accessibility of electronic toys and devices to children. Due to their electrochemical composition, impacted batteries in the esophagus may cause an extensive damage. Following the removal of a battery, the post-esophagoscopy management is still controversial.Case presentation: An otherwise healthy 8 year-old boy presented to the pediatric emergency room 3h after the unintentional swallowing of a lithium battery. On examination, the patient was diaphoretic and tachypneic. Plain PA chest film revealed a 2.5cm diameter radiopaque round object in the upper esophagus. The patient was scheduled for an urgent rigid esophagoscopy which was performed 2h after admission. Esophagoscopy findings included an impacted lithium battery in an advanced emptying process at a level of 17cm from the incisor teeth, with a 3rd degree ulcerative esophagitis. It was not possible to visualize either the distal esophagus or the stomach. A nasogastric tube was not inserted because of a significant risk for esophageal perforation if bluntly passed. Post-operative medical therapy included fasting, administration of intravenous antibiotic therapy, antacids, and steroids. Flexible esophagoscopy superior to the level of the mucosal injury performed one day later, revealed erosive esophagitis, without evidence of perforation. Upper digestive tract gastrografin swallow test performed 2 days after esophagoscopy did not demonstrate a leak from the esophagus, and oral feeding was carefully re-initiated. Treatment was discontinued the following day. Follow up on days 10 and 14 revealed a healthy child with normal swallowing.Discussion: Battery ingestion-related injury results from direct pressure necrosis, local electrical currents and alkali leakage. Signs and symptoms of ingested battery are related to impaction duration, size of battery, battery content and peristaltic waves of the esophagus. Appropriate imaging studies should be performed to maximize identification of the foreign body before esophagoscopy. Esophageal stenting and adjuvant medical therapy (steroid therapy, antibiotic therapy and anti-reflux therapy) have a low evidence level of clinical benefit following caustic injuries from impacted batteries and spillage of their content to the esophagus. A judicious management should be tailored in each patient. Increased public and health personnel awareness is necessary to diminish the incidence of battery ingestion.

Speech production intelligibility of early implanted pediatric cochlear implant users

Mirette G. Habib, Susan B. Waltzman, Bobby Tajudeen, Mario A. Svirsky — 2010-08-01

Abstract: Objectives: To investigate the influence of age, and age-at-implantation, on speech production intelligibility in prelingually deaf pediatric cochlear implant recipients.Methods: Forty prelingually, profoundly deaf children who received cochlear implants between 8 and 40 months of age. Their age at testing ranged between 2.5 and 18 years. Children were recorded repeating the 10 sentences in the Beginner's Intelligibility Test. These recordings were played back to normal-hearing listeners who were unfamiliar with deaf speech and who were instructed to write down what they heard. They also rated each subject for the intelligibility of their speech production on a 5-point rating-scale. The main outcome measures were the percentage of target words correctly transcribed, and the intelligibility ratings, in both cases averaged across 3 normal-hearing listeners.Results: The data showed a strong effect of age at testing, with older children being more intelligible. This effect was particularly pronounced for children implanted in the first 24 months of life, all of whom had speech production intelligibility scores of 80% or higher when they were tested at age 5.5 years or older. This was true for only 5 out of 9 children implanted at age 25–36 months.Conclusions: Profoundly deaf children who receive cochlear implants in the first 2 years of life produce highly intelligible speech before the age of 6. This is also true for most, but not all children implanted in their third year.

Adenotonsillectomy improves the strength of respiratory muscles in children with upper airway obstruction

2010-08-01

Abstract: Objective: The aim of this paper is to study the respiratory muscle strength by evaluating the maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP) and lung volume before and 3 and 6 months after adenotonsillectomy. This is an interventional, before and after trial. It was set at the Department of Otolaryngology, University of São Paulo, School of Medicine. We included 29 children (6–13 years old), both genders, consecutively recruited from the waiting list for adenotonsillectomy. Children were submitted to maximal inspiratory pressures (MIP), maximal expiratory pressure (MEP) evaluation using an analog manovacuometer, lung volume, using incentive expirotometer and thoracic and abdominal perimeter using a centimeter tape. Children were evaluated in 3 different moments: 1 week before and 3 and 6 months after surgery.Results: MIP improved significantly 3 months (p<0.001) after adenotonsillectomy and MEP did not change (p=1). There were increases in lung volume (p=000), chest (p=0.017) and abdominal perimeter (p=0.05). Six months after surgery, all parameters improved. MIP (p=0), MEP (p=0), lung volume (p=0.02), chest (p=0.034) and abdominal perimeter (p=0.23).Conclusion: This study suggests that there was an improvement in respiratory muscular strength, once there was a significant improvement in maximal inspiratory pressure, lung volume and other parameters after adenotonsillectomy.

Mucin gene expression and mouse middle ear epithelium

Joseph E. Kerschner, Jizhen Li, Katsuyuki Tsushiya, P. Khampang — 2010-08-01

Abstract: Objectives: To investigate the expression of recently identified human mucin genes in an in vitro model of cultured mouse middle ear epithelial cells (MMEEC).Methods: MMEEC were established, RNA was extracted and primers were designed for RT-PCR to assess for expression of mucin genes Muc1, Muc2, Muc3, Muc4, Muc5AC, Muc5B, Muc6, Muc7, Muc8, Muc9, Muc10, Muc11/12, Muc13, Muc15, Muc16, Muc17, Muc18, Muc19 and Muc20 expression.Results: Mucin genes Muc1, Muc2, Muc3, Muc4, Muc5AC, Muc5B, Muc9, Muc10, Muc13, Muc15, Muc16, Muc18, Muc19 and Muc20 were identified and expressed in MMEEC. The genes Muc6, Muc7, Muc8, Muc11/12 and Muc17 were not identified.Conclusion: Many of the mucin genes that have been recently identified in human MEE and chinchilla MEE are also expressed in MMEEC. There are differences in expression, however, which may have implications in utilizing various animal models for study of middle ear physiology and pathogenesis; specifically as it relates to mucin gene expression.

Nasal encephalocele: Endoscopic excision with anesthetic consideration

2010-08-01

Abstract: Objective: Nasal encephalocele may presents as a nasal mass, its treatment is surgical and it should be done early in life. When removal is indicated, there are multiple surgical approaches; including lateral rhinotomy, a transnasal approach and a coronal flap approach. However, the treatment of a basal intranasal encephalocele using transnasal endoscopic approach could obviates the possible morbidity associated with other approaches. The aim of this study was to evaluate the efficacy of endoscopic removal of intranasal encephalocele, also to document the role of anesthetist in the operative and postoperative periods.Methods: Nine cases with nasal encephalocele were included in this study; CT and/or MRI were used in their examination. The lesions were removed via transnasal endoscopic approach. Preoperative evaluation, intervention and postoperative follow-up were presented with discussion of anesthesia used for those children.Results: The lesions of all patients were removed successfully with no recurrence through the follow-up period of at least 21 months. No cases showed morbidity or mortality intra- or post-operatively.Conclusions: Endoscopic excision of intranasal encephalocele is an effective method with high success rate. Anesthetist plays an important role in the operative and postoperative period, even during the endoscopic follow up; sedation of the children is usually needed.

Treatment of persistent middle ear effusion in cleft palate patients

C. Szabo, K. Langevin, S. Schoem, K. Mabry — 2010-08-01

Abstract: Objectives: The goals of the research project are to learn how to individualize otologic care for cleft palate patients and to be able to counsel families of children with cleft palate on the benefit of tympanostomy tubes, hearing issues and risks of multiple sets of tubes.Methods: The study is a retrospective chart review. Patients with a cleft palate with or without a cleft lip born between 1 January 2000 and 31 December 2005 referred to the Connecticut Children's Medical Center Craniofacial Department were included in the study. The patients were offered individualized ear surgery (PE tube placement) only if persistent middle ear fluid was present for over 3 months with a conductive hearing impairment. The primary outcome measures included the newborn hearing screening results, number of ear tube surgeries, and complications of PE tube insertion.Results: There were 86 patients with cleft palate spectrum with or without cleft lip (45 females and 41 males). Twelve had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive. By 5 years old, 84 (98%) patients received at least one set of ear tubes for persistent middle ear fluid with conductive hearing impairment, while 2 received no tubes (2%). Of those who received ear tubes, the range was 1–6 with a mean of 1.7. Twelve patients (14%) had tympanosclerosis. Eight patients (9%) had eardrum perforation. One patient had myringoincudopexy. Of the 86 patients, 12 had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive.Conclusions: (1) The majority of children born with cleft palate do not have middle ear fluid at birth. (2) Most children with cleft palate will likely develop persistent middle ear fluid with conductive hearing loss. Risks of complications from ear tubes in cleft palate patients are few and manageable using standard sized ear tubes.

Mutational analysis of HOXA2 and SIX2 in a Bronx population with isolated microtia

2010-08-01

Abstract: Objective: Microtia is a developmental malformation of the external ear with genetic and environmental causes. The prevalence of microtia varies but several studies suggest increased incidence in Hispanic and African American populations. No causal genetic mutations have been identified in these populations. Mutations in the homeobox gene HOXA2 caused microtia in a single Iranian family. Another homeobox gene, SIX2, acts downstream of HOXA2 during development and provides another possible candidate for mutational analysis.Methods: To determine whether mutations in HOXA2 or SIX2 cause sporadic microtia, DNA sequencing analysis was performed on exons in both genes in 8 patients of Hispanic and African descent in the Bronx. Identified variants were assayed in an additional 4 patients and 100 Hispanic control samples using Sequenom MassArray to rule out causality in heterozygous patients.Results: No mutations were identified in the coding sequence of HOXA2 or SIX2. Four novel single nucleotide variants were identified among the patient samples. These variants lie in the intron and 3′ UTR of HOXA2 and the 5′ and 3′ UTRs of SIX2. One variant in the intron of HOXA2 lies in a conserved predicted transcription factor binding site for SMARCA3. All four variants are also present at >5% frequency in Hispanic control samples, ruling out these novel variations as causal.Conclusions: Lack of mutations in the coding regions of HOXA2 or SIX2 among the sporadic microtia patients studied indicate different etiologies. Identification of four novel single nucleotide polymorphisms in patients and controls of Hispanic descent, but not of Caucasian populations, points to genetic diversity in an understudied population.

Lexical effects on spoken word recognition performance among Mandarin-speaking children with normal hearing and cochlear implants

Nan Mai Wang, Che-Ming Wu, Karen Iler Kirk — 2010-08-01

Abstract: Objective: This investigation aimed to examine the effects of word frequency and lexical neighborhood density on spoken word recognition of monosyllables and disyllables in Mandarin by normal hearing children and children with cochlear implants. The lexical characteristics were incorporated from the Neighborhood Activation Model (NAM), which suggests that words in the mental lexicon are organized into similarity neighborhoods. The difficulty of a listener's task is affected by the frequency of the target word and the density of the lexical neighbors from which that word must be identified. The Monosyllabic Lexical Neighborhood Test and the Disyllabic Lexical Neighborhood Test in Mandarin Chinese (Mandarin LNT and MLNT) were developed to take into account the effects of these linguistic and cognitive demands on speech perception performance.Methods: Three stages were conducted in this investigation. In the first stage, Mandarin words of monosyllables and disyllables were selected and their lexical properties were calculated from the CHILDES database. Four lexically “easy” and four lexically “hard” word lists in Mandarin LNT as well as two word lists across lexical properties among disyllables were determined based on their relative word frequencies and neighborhood densities. In the second stage, word stimuli were verified by 30 children of the NH group and 36 children from the CI group. In the third stage, the inter-list equivalency and test–retest reliability of word lists across lexical properties were determined, and the correlations of Mandarin LNT and MLNT with other measures and inter-rater reliability were also investigated.Results: Word recognition scores were higher among disyllables than among monosyllables. Lexically “easy” disyllabic words were better recognized than their “hard” counterparts and the monosyllables among two groups of children. However, no lexical effects on word recognition of Mandarin monosyllables were observed for either group. No significant differences were found among word lists in each combination of syllable structure and lexical property. Inter-rater reliability, inter-list equivalency, and test–retest reliability were revealed. The Mandarin LNT and MLNT were found to be highly reliable measures of spoken word recognition (r=0.84; p<0.01) with acceptable equivalency between lists (r=0.638–0.876).Conclusion: Lexical effects on Mandarin word recognition were only demonstrated among disyllabic words by NH and the CI children, while Mandarin homophones appearing in monosyllabic words were suggested. Lexical effects on spoken word recognition in Mandarin are not substantially demonstrated as in English, but the Mandarin LNT and MLNT provided reliable information on the spoken word recognition of pediatric CI users in the initial stage after implantation as well as in the rehabilitation progress.

Tragal cartilage augmentation in repair of cleft palate, a new technique and its clinical implications

W.F. Ezzat, H. Rabie, M. Tarabishi — 2010-08-01

Abstract: Background: Cleft palate is one of the most common congenital anomalies of the head and neck worldwide. In addition to the evident feeding and growth problems, patients are involved with and suffer from speech, hearing and dental problems. Many surgical techniques and modifications have been advocated to improve functional outcome and aesthetic results, aiming at normal speech, minimizing growth disturbances, and establishing a competent velopharyngeal sphincter. Despite the variety of techniques described for repair of the clefts, there is still a relatively high incidence of postoperative fistula reaching up to 35%. This is mainly related to type and degree of the defect, and type of surgical repair.Objectives: To evaluate the efficacy of placement of tragal cartilage free graft between the oral and nasal mucosal layers of the neo-palate in improving success rates, and anatomical and functional outcomes in repair of cleft palate with reduction of the extent of dissection.Patients and methods: Fourteen patients were managed by our technique, only in large cases minimal von Langenbeck lateral release incisions were made. In all cases a tragal cartilage graft was interpositioned and fixed to the muscle layer of the neo-palate, 2–3 extramucosal trans-muscle sutures were placed for 3–4 weeks if needed, and the patients were followed up for a minimum of 12 months during which functional and anatomical assessments were done.Results: Results, including both anatomical and functional outcomes, were favorable with no gross failures, permanent significant fistula formations (one case with minor non-significant fistula), nor donor site co morbidities.Conclusion: The use of tragal cartilage free graft to augment the area at the junction between the hard and soft palate appears to be a safe and effective method in repair of cleft palate that reduces the incidence of postoperative palatal fistulae, without donor site comorbidities.

Prevalence of signs and symptoms of temporomandibular dysfunction in male adolescent athletes and non-athletes

2010-08-01

Abstract: Purpose: The aim of the study was to compare the prevalence of signs and symptoms of temporomandibular dysfunction (TMD) in male adolescent athletes—basketball players and non-athletes and to examine the association between signs and symptoms of TMD in male adolescents in different Tanner stages.Methods: The subjects were 46 male basketball players ages 10–13 years and 41 male adolescent non-athletes ages 10–18 years selected from the Department of Pediatrics (School of Medicine, Federal University of São Paulo) as a control group. A questionnaire was used to assess the signs and symptoms of TMD. According to the answers on the questionnaire the adolescents were classified in two categories: no signs or symptoms present (score A—absent), at least one sign or symptom present (score P—present). The ones who got score “P” were submitted to a standardized functional examination of the masticatory system by the same examiner. Pubertal status was assessed based on physical examination by physicians from our Division. The adolescents were classified according to Tanner stages into three subgroups: subgroup 1 (before the growth spurt), subgroup 2 (growth spurt period), subgroup 3 (end of growth spurt). Significant differences between athletes and non-athletes were assessed using non-parametric Wilcoxon test for continuous variables and the Fisher's exact test for categorical variables. The level of significance used was 5%.Results: There was no significant difference between the athletes and non-athletes in exhibiting at least one sign or symptom of TMD (p=0.1148). When comparing the adolescents who presented at least one symptom of TMD to the different subgroups of Tanner Stages no statistically significant differences were found (p=0.8357).Conclusion: The lack of significant differences among male adolescents athletes and non-athletes may be caused by the size of the sample, the age of the athletes or by a probable protective action of testosterone levels in male athletes. The lack of statistical difference comparing the adolescents who presented at least one sign or symptom of TMD to subgroups of Tanner is probably because estrogen is the risk factor hormone and the levels of estrogen in male adolescents are very low.

Clonal spread of β-lactamase-producing amoxicillin–clavulanate-resistant (BLPACR) strains of non-typeable Haemophilus influenzae among young children attending a day care in Japan

2010-08-01

Abstract: Objective: Resistant strains of non-typeable Haemophilus influenzae (NTHi) are one of the principal causes of recurrent acute otitis media (otitis prone), rhinosinusitis, and pneumonia in young children. β-Lactamase-nonproducing ampicillin-resistant (BLNAR) strains are particularly common in Japan, and β-lactamase-producing amoxicillin–clavulanate resistant (BLPACR) strains are now emerging. We investigated the nasopharyngeal carriage status of these resistant strains among children attending a same day care center during a 10-year period.Methods: From 1999 to 2008, we obtained nasopharyngeal swab specimens from young children attending a same day care center and examined the incidence of resistant strains of NTHi. Antimicrobial resistance of NTHi was identified based on PCR analysis of mutation of the penicillin binding protein (PBP) genes. Pulsed-field gel electrophoresis (PFGE) was performed to examine the clonal relationship of each resistant strain.Results: The prevalence of resistant strains of NTHi among the children attending this day care has significantly increased during the past 10 years and most of this day care children recently have resistant strains with PBP gene mutations in their nasopharynx. Genetically BLPACR (gBLPACR) strains have rapidly increased since 2007 and PFGE analysis demonstrated that all gBLPACR were clonally identical. This is the first report of apparent clonal dissemination of gBLPACR strains of NTHi occurring in a certain environment such as day care.Conclusions: The rapidly increasing prevalence of resistant strains, in particular gBLPACR, in this day care center may predict a high incidence of these resistant bacteria from clinical isolates in the near future and potential serious medical problems worldwide.

Vocal tract dimensional development of adolescents: An acoustic reflection study

Steve A. Xue, Regine Wing Chi Cheng, Lawrence Manwa Ng — 2010-08-01

Abstract: Objective: The purpose of the study was to investigate the effects of age and gender on adolescents’ vocal tract dimensional development with acoustic reflection technology (ART).Methods: A total of ninety-five male and female adolescents aged between 10 and 18 divided into three age groups were tested with acoustic reflection technology (ART) and acoustic program to secure their vocal tract dimensional parameters and the vowel formant frequencies.Results: Significant age and gender effects were found not only in vocal tract length, but also segmental volumetric measurements, as well as the vowel formant frequencies.Conclusions: The findings of this study have provided insights on the developmental trend of adolescents’ vocal tracts. The study has also offered a preliminary anatomical database of adolescents’ vocal tract dimensional growth for otolaryngologists, clinical anatomists, speech therapists and other health professionals of swallowing, respiration and communicative disorders.

Cisplatinum ototoxicity in children, long-term follow up

Talal Al-Khatib, Natasha Cohen, Anne-Sophie Carret, Sam Daniel — 2010-08-01

Abstract: Objective: To assess the long-term ototoxicity effect of platinum chemotherapy in a series of pediatric patients.Design: A prospective cohort study.Methods: Patients who received platinum chemotherapy were identified through review of the pharmacy records from 2000 to 2005. Audiograms pre- and post-treatment with cisplatin were noted. The patients were brought back long after treatment for a repeat audiogram and a questionnaire to assess the impact of ototoxicity on their quality of life.Results: Forty-nine patients received platinum chemotherapy. Patients’ exclusion: two had no pre-chemo audiograms, one had retinoblastoma with congenital hearing loss, three were lost to follow up, five deceased, and seven refused participation. The total number of patients included was 31 with long-term follow up total of 21 patients. The follow up period ranged from 1.5 to 6.6 years (median of 3.4 years). Fourty-two percent (13/31) of the patients suffered from otoxicity (3 mild, 3 moderate, 7 severe-profound). Thirty-three (7/21) of audiograms worsened on long-term follow up. Questionnaire revealed 70% subjective hearing loss with 40% requiring hearing aids.Conclusion: Ototoxicity after platinum chemotherapy can present or worsen years after completion of therapy. Therefore, we recommend long-term follow up.

Assessment of Mandarin-speaking pediatric cochlear implant recipients with the Mandarin Early Speech Perception (MESP) test

Yun Zheng, Sigfrid D. Soli, Zhaoli Meng, Yong Tao, Kai Wang, Ke Xu, Hong Zheng — 2010-08-01

Abstract: Objective: The primary purpose of the current study was to evaluate the Mandarin Early Speech Perception (MESP) test for use in objective outcome assessment of pediatric CI recipients in China. Although almost 5000 pediatric CIs have been implanted in China, very little is known about the speech perception abilities of these recipients, in large part because of the previous unavailability of objective outcome assessment tools for Mandarin-speaking children.Method: The Mandarin Early Speech Perception (MESP) test was administered to 25 pediatric Mandarin-speaking cochlear implant (CI) recipients implanted under the age of 10 years at West China Hospital of Sichuan University.Results: Data from 22 children reaching MESP Categories 3 (Spondee Perception), 5 (Consonant Perception), and 6 (Tone Perception) were analyzed. The 5 children reaching Category 6 were significantly younger at implantation than children reaching the other two categories. Comparison of MESP scores for CI recipients and normally hearing children revealed that scores for the majority of CI recipients reaching Categories 3 and 5 fell within the range of normal performance, while scores for all CI recipients reaching Category 6 exceeded the average normal performance. MESP scores for Mandarin-speaking CI recipients and ESP scores for English-speaking CI recipients fell on a common trajectory that, after slightly more than 3 years of age, exceeded the ceiling defined by the first four categories common to both tests. After 4 years of age, most Mandarin-speaking had reached Category 6.Conclusions: These results provide preliminary validation of the MESP test for use in evaluation of Mandarin-speaking pediatric CI recipients. Use of objective outcome assessment tools such as the MESP following early cochlear implantation can be used to document the progress of CI recipients and identify those recipients who may need additional rehabilitative intervention.

Newborn hearing screening and genetic testing in 8974 Brazilian neonates

2010-08-01

Abstract: Objective: An early diagnosis has been a priority in the audiological practice. Identifying hearing loss until 3 months old through Universal Newborn Hearing Screening and intervention before 6 months old, minimize the impact of auditory loss in the health and communication development of these children. However, in the clinical practice, despite the help of the risk indicators in the audiological and etiological diagnosis, the integrated services have come up against the challenge of determining the causes of auditory loss, bearing in mind that approximately 50% of the subjects who have congenital loss do not show risk factors in their clinical history. The current research aims introduce together etiologic and audiological diagnosis of newborns.Methods: We eluted dried blood spots from paper and performed genetic testing for 35delG mutation in 8974 newborns that were also screened for transient otoacoustic emissions (TOAE). In addition, the A1555G and A827G mutations in the MTRNR1 mitochondrial gene were screened in all newborns.Results: We have found 17 individuals who failed in TOAE. Among them, we detected 4 homozygous newborns for 35delG mutation and 3 individuals with A827G mutation in the MTRNR1 mitochondrial gene. The frequency of 35delG carriers was 0.94% [84/8974]. In all 17 individuals who failed in OAE no other mutation besides those mentioned above was found.Conclusions: The results greatly contribute to the public health area indicating the etiologic diagnosis, allowing family counseling as well as the early rehabilitation treatment or surgical intervention. Over time that will help to reduce the costs of rehabilitation considerably.

Acute otitis media: To follow-up or treat?

Tomislav Stevanovic, Zoran Komazec, Slobodanka Lemajic-Komazec, Rajko Jovic — 2010-08-01

Abstract: Acute otitis media (AOM) is the most common diagnosis for which antibiotics are prescribed in children. However, due to their widespread use, we are witnesses to increased development of bacterial resistance to antibiotics. The purpose of this study was to evaluate the necessity of antibiotic treatment in patients with AOM. Our study included 314 children, aged between 2 months and 6 years. Children were divided into two groups: the first group included children with less severe forms of AOM, who received symptomatic therapy and “wait-and-see” approach (237 children); the second group presented with purulent ear infection and received antibiotic treatment from the beginning (77 children). After symptomatic therapy, resolution of the disease, without use of any antibiotics, was established in 61% of patients, compared to the overall sample of children with AOM. None of the children developed complications that would require surgical treatment. In the second group of children, receiving antibiotics, almost the same therapeutic effects (80%) were achieved with the use of amoxicillin, amoxicillin-clavulanate and cefixime, while the worst results were obtained after using azithromycin.The wait-and-see approach is recommended in forms of AOM without serious signs and symptoms, because it significantly reduces the use of antibiotics and their potential adverse effects.

Histopathological morphometric study of cochleosaccular dysplasia in Dalmatian dogs

2010-08-01

Abstract: Objective: To analyze temporal bones of deaf Dalmatian dogs from 5 days after birth to adulthood to better understand the pathogenesis of cochleosaccular dysplasia.Methods: This is an experimental animal histopathological temporal bone study that included two groups of temporal bones. Group I consisted of 41 temporal bones from deaf Dalmatian dogs and group II of 25 temporal bones from 15 “normal” aged-matched, hearing Black Labradors. Morphometric analysis included: stria vascularis and spiral ligament area measurements, and cell counts of spiral ganglion, Scarpa's ganglion, and hair cells of saccular macula.Results: The following findings were significantly less in deaf Dalmatian group compared to hearing Labradors: (1) cellular area of the stria vascularis in all cochlear turns; (2) cellular area of spiral ligament in the inferior part of the basal turn; (3) cellular density of spiral ganglion cells within segments III and IV; (4) number of Scarpa's ganglion cells; and (5) density of saccular hair cells types I and II. A borderline negative correlation was found between average density of spiral ganglion cells of segments III and IV and age in group I. Young deaf animals showed some cochlear hair cells, however in adult dogs all hair cells were replaced by supporting cells.Conclusion: General pattern of cochleosaccular dysplasia is variable, even when only one etiology, the genetic one, is involved. The gradual degeneration of inner ear elements in the cochleosaccular degeneration might indicate that early intervention might be crucial to stop the progression of cochleosaccular dysplasia.

Massive retropharyngeal neurofibroma in a pediatric patient

Elizabeth S. Piazza, Kimberly A. Donnellan, Aimee M. Goodier, Jeffrey D. Carron — 2010-08-01

Abstract: Retropharyngeal tumors in Neurofibromatosis Type I patients have rarely been presented in the literature and none in a child. We present the case of an 11-year-old patient with a huge retropharyngeal plexiform neurofibroma which was successfully removed without sequelae. Radical resection is a viable option for treatment of these patients.

Sialoblastoma of salivary glands in children: Chemotherapy should be discussed as an alternative to mutilating surgery

2010-08-01

Abstract: Sialoblastoma is a very rare congenital salivary gland tumor. No consensus has been reached concerning the treatment of this tumor due to its rarity. The treatment of reference is surgery, which can be mutilating, in the case of a locally invasive tumor. The treatment of metastatic disease is also controversial. The authors report a new case of a 6-year-old girl with a progressively growing left parotid mass since birth. The first cytological diagnosis was that of pleomorphic adenoma. Due to local progression, superficial parotidectomy was performed at the age of 3.5 years and revealed a diagnosis of sialoblastoma. Six months later, local recurrence and lung metastasis were treated by neoadjuvant chemotherapy with a very good partial response on the local recurrence and the lung metastasis, allowing complete parotidectomy with sacrifice of the facial nerve. Bilateral lung biopsies after adjuvant chemotherapy showed total necrosis. No recurrence was observed with a follow-up of 1 year.This case and a review of the literature confirm the very good chemosensitivity of this tumor and argue in favor of neoadjuvant chemotherapy for locally invasive tumors rather than extensive mutilating surgery.

Successful management of a tracheo-innominate fistula in a 7-year-old child

Rodrigo C. Silva, David H. Chi — 2010-08-01

Abstract: The purpose is to describe the presentation and management of a tracheo-innominate artery fistula in a tracheostomy-dependent child. The study design is case report and the method is retrospective chart review. 7-Year-old female with a history of anoxia at birth, seizure disorder, tracheostomy and ventilator dependency since infancy presented to community hospital after one episode of spontaneous bleeding around and through the tracheostomy tube. The patient was transported to a tertiary pediatric teaching hospital and had more profuse bleeding upon arrival. She was taken emergently to the Operating Room (OR) where the cuff of a 5.0 endotracheal tube (ETT) placed through the existing stoma was used to tamponade the bleeding. The airway was secured proximally with a rigid bronchoscope which also provided photodocumentation during the procedure. The cardiothoracic surgery service performed ligation of the innominate artery and repair of the anterior tracheal wall defect through a midline sternotomy approach. The patient was monitored for 7 days in the ICU sedated and paralyzed and returned the OR for a new airway endoscopy and replacement of the ETT with a 5.0 tracheostomy tube. The patient was discharged after 12 days of hospitalization. Subsequent interval endoscopies revealed well-healed fistulous site and no further complications after 12 months of follow-up. Tracheo-innominate artery fistulae can be successfully managed if recognized and treated early. Special attention should be given to “sentinel events” that often precede more catastrophic bleeds.

Laryngeal juvenile xanthogranuloma: Imaging finding

L.M. Wang, Q.H. Chen, Y.X. Zhang, Y.Q. Fang, Z.C. Wang, Q. Zhu — 2010-08-01

Abstract: Laryngeal juvenile xanthogranuloma is rare and there were only four cases have been reported previously in English literature. We report a case of isolated JXG of larynx in the superglottic region. The mass was well-defined and homogeneous hypoechoic in ultrasonography while presented indistinct boundary and soft tissue density in Computed Tomography images. Radiological findings are nonspecific but could help to understand the extent of the lesion. We suggest that JXG should be considered as differential diagnosis for laryngeal stridor. Laryngeal ultrasound is safe and reliable, which could be considered a useful diagnostic tool for supplementing laryngoscopy.

Hypoplastic epiglottis in a non-syndromic child: A rare anomaly with serious consequences

Habib G. Rizk, Michel Nassar, Ziad Rohayem, Simon J. Rassi — 2010-08-01

Abstract: Hypoplasia of the epiglottis is a rarely reported anomaly. These patients may have complex malformations or are totally asymptomatic. We herewith present the case of a 26-month-old infant who was repeatedly treated for severe aspiration pneumonias that didn’t resolve with gastrostomy. The role of epiglottis in protecting the airway is an unsolved question. The long-term evolution of hypoplasic epiglottis cases is also not known. The presentation and the evolution in cases of epiglottic anomalies vary a lot to deduce a trend. Close specialized follow-up is needed to answer these questions and to optimize these patients’ prognosis.

Case report: Treatment failure using propanolol for treatment of focal subglottic hemangioma

Karina T. Canadas, Eric D. Baum, Stella Lee, Samuel T. Ostrower — 2010-08-01

Abstract: Subglottic hemangioma is a rare, potentially life threatening tumor of infancy which poses serious treatment challenges. A number of medical and surgical therapies over the years have met with variable success, and are associated with numerous potential morbidities. A potential windfall in the management of infantile hemangiomas has arisen with the recent identification of propanolol as a highly efficacious and relatively safe new treatment modality. At least five reports in the literature have described the rapid, successful treatment of airway hemangiomas with oral propanolol. We describe the first reported treatment failure with propanolol for subglottic hemangioma in an infant who initially responded dramatically to the medication.

Management of infantile subglottic hemangioma: Acebutolol or propranolol?

2010-08-01

Abstract: The successful management of subglottic hemangioma with propranolol has been reported. We report three cases of subglottic hemangioma treated with the cardioselective beta-blocker acebutolol, 8mg/kg/day. Treatment was efficient in two cases while an open procedure was necessary in the third child. In our experience, acebutolol could be easily administered in oral form twice-a-day only with a dose that was adaptable according to the growth of the child and showed no side effects.We also report a case of rebound growth after beta-mimetic drug use and the efficiency of propranolol treatment in such a recurrence.Considering the lack of side effects and the advantages in terms of administration, we suggest acebutolol as a first-line treatment of subglottic hemangiomas for which intervention is required.

Cochlear implantation in pontine tegmental cap dysplasia

2010-08-01

Abstract: Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are multiple cranial neurophaties and ataxia. Sensorineural hearing loss of varying severity is almost always present. To date, 14 cases of PTCD have been reported in the literature. We present a child with PTCD and profound bilateral sensorineural hearing loss who underwent cochlear implantation. To the best of our knowledge, cochlear implantation in PTCD has not been previously reported. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. At 22 months’ postoperative evaluation, the patient who was placed into speech perception category 0 (no detection of speech) preoperatively progressed to category 3 (beginning word identification). Before implantation, the child had connected speech unintelligible. At the last follow-up, she had connected speech intelligible to a listener who has little experience of a deaf person's speech. Cochlear implantation allowed this child to improve her quality of life, increasing her self-confidence, independence, and social integration.

A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia

Kate Blackmore, David M. Wynne — 2010-08-01

Abstract: Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed.

Comment on “Adenoidectomy for otitis media with effusion in 2–3-year-old children” by Margaretha L. Casselbrant et al. [Int. J. Pediat. Otorhinolaryngol. 73 (2009) 1718–1724]

Giovanni Felisati, Carlotta Pipolo — 2010-08-01

The paper by Margaretha L. Casselbrant et al.: Adenoidectomy for otitis media with effusion in 2–3-year-old children, published on the last number (December 2009) of the International Journal of Pediatric Otorhinolaryngology reports the opinion of “the pediatric group of Pittsburgh”: one of the most appreciated scientific group regarding this field and very much esteemed by us. For this reason and because of the very precise statistical analysis of this report, the manuscript will surely become a very important reference in the field of the management of otitis media with effusion (OME).

Margaretha L. Casselbrant, Ellen M. Mandel, Charles D. Bluestone — 2010-08-01

We appreciate the comments of Drs. Felisati and Pipolo as this gives us the opportunity to clarify any misunderstandings. Their first comment states that our population did not have only OME but also had AOM which therefore invalidates the conclusions of our study regarding the efficacy of adenoidectomy for chronic OME. We made an effort to clearly define our population to make it evident as to the type of child included. All of the subjects in our study had chronic OME with or without episodes of AOM, which is shown in Table 1. In this young age group, most children will have had at least one episode of AOM [Teele et al., Ann. Otol. Rhinol. Laryngol. 89 (Suppl. 68) (1980) 5–6]. As with any study, the conclusions should be applied to the type of population enrolled.

Calendar of Events

2010-08-01

Guide for Authors

2010-08-01