Newborn hearing screening methodology impacts the timing of diagnosis for auditory neuropathy spectrum disorder.

INTRODUCTION: Auditory Neuropathy Spectrum Disorder (ANSD) accounts for 10 % to 15 % of pediatric hearing loss. In most cases, otoacoustic emissions (OAE) are present as the outer hair cell function is normal, and the auditory brainstem response (ABR) is abnormal. Newborn hearing screen (NBHS) is completed using OAE or ABR depending on the institution. Because OAEs are often present in ANSD, NBHS done solely with OAE can miss and delay diagnosis of patients with ANSD. OBJECTIVES: To assess whether NBHS methodology impacts the age of diagnosis of ANSD. METHODS: This is a retrospective study of patients, 0-18 years of age, diagnosed with ANSD at two tertiary pediatric hospitals from 1/01/2010 to 12/31/2018 after referral from NBHS performed in the community. Data recorded included patient demographics, method of NBHS, NICU stay, and age at ANSD diagnosis. RESULTS: 264 patients were diagnosed with ANSD. Of those, 123 (46.6 %) were female, and 141 (53.4 %) were male. Ninety-seven (36.8 %) were admitted to NICU and the mean stay was 6.98 weeks (STD = 10.7; CI = 4.8-9.1). The majority (244, 92.4 %) of patients had NBHS with ABR, and 20 (7.5 %) had NBHS with OAE. Patients screened with ABR were diagnosed with ANSD earlier than those who screened with OAE, with a mean age of 14.1 versus 27.3 weeks (p = 0.0397, CI = 15.2-39.3). Among those screened with ABR, median age at diagnosis was 4 months for NICU infants and 2.5 months for infants with no history of NICU stay over 5 days. In comparison, median diagnosis age was 8 months for non-NICU infants screened with OAEs. CONCLUSION: Patients with ANSD who had NBHS with ABR were diagnosed earlier than those with OAE. Our data suggest that universal screening with ABR may facilitate earlier diagnosis of ANSD and earlier evaluation for aural rehabilitation, especially in high-risk cohorts such as NICU patients. Further research is needed into factors that contribute to earlier diagnosis among patients screened with ABR.

Preoperative cerebral magnetic resonance imaging and white matter changes in pediatric cochlear implant recipients.

INTRODUCTION: Preoperative magnetic resonance imaging (MRI) can yield valuable information regarding the status of the inner ear in pediatric cochlear implant (CI) candidates. Incidentally, cerebral white matter changes are at times noted on these MRI scans. They may indicate post-infectious/postinflammatory changes or progressive neurodegenerative processes. OBJECTIVE: To evaluate the clinical significance of cerebral white matter changes in pediatric CI patients. METHODS: Retrospective chart review of pediatric CI recipients with preoperative MRI between March 2004 and January 2009 was performed. MRI reports along with postoperative audiology and speech and language outcomes were assessed. RESULTS: Fifty-seven patients were included in the study. There were 29 females and 28 males. Mean age was 58 months (range of 12-194 months). Abnormal MRI with varying degrees of white matter changes was noted in 10 (18%) children. Neurological assessments did not reveal any serious central nervous system diseases. Two of the 10 patients demonstrated significant delays and difficulties with postoperative CI performance. CONCLUSIONS: Cerebral white matter abnormalities are not uncommon incidental findings in preoperative MRI scans in pediatric CI candidates. These changes may reflect remote or active pathology which may require neurological assessment. The significance of these findings is still uncertain and should be better clarified as we study more patients.

Manifestações bucais em crianças submetidas a tratamento antineoplásico no Centro Oncológico do Hospital Universitário Oswaldo Cruz / Oral manifestations in children submitted to antineoplasic treatment at the Oncology Center of Oswaldo Cruz University Hospital

Através deste estudo, buscou-se verificar as manifestações bucais do tratamento antineoplásico nas crianças atendidas no Centro Oncológico do Hospital Universitário Oswaldo Cruz (CEON-HUOC), no ano de 1999. A população alvo constou de 30 crianças na faixa etária de 3 a 12 anos recebendo quimio e/ou radioterapia. Na realização do estudo, foram utilizados questionários dirigidos aos pais das crianças, fichas clínicas para levantamento dos índices ceo-d, CPOD e gengivais e testes salivares, com objetivo de verificar as alterações mais freqüentes nos tecidos moles, as alterações salivares, a experiência de cárie e os índices de higiene oral. Os dados obtidos permitiram concluir: a alteração mais freqüente nos tecidos moles foi a mucosite; o fluxo e o pH salivares mostraram-se reduzidos; os índices de cárie no grupo foram elevados, em relação aos índices preconizados pela OMS; as crianças menores de sete anos de idade apresentaram índices de higiene oral mais elevados; as crianças que receberam orientação preventiva antes do tratamento antineoplásico apresentaram índices de higiene oral mais baixos

Unilateral choanal atresia in identical twins: case report and literature review.

Choanal atresia is postulated to be secondary to an abnormality of the rupture of the buccopharyngeal membrane during the embryological period. This condition usually occurs sporadically, but has been described in siblings and successive generations. The genetics remain unclear. We present monozygotic twins with identical findings of unilateral choanal atresia and no other associated anomalies. To our knowledge, this is the first report of such an occurrence.

Imaging and surgical approach of nasal dermoids.

OBJECTIVE: Determine the most accurate and cost effective radiographic evaluation for nasal dermoids. Determine the best surgical approach for excision of nasal dermoids. DESIGN: Retrospective chart review. SETTING: Division of Pediatric Otolaryngology, Children's Hospital and Health Center, San Diego, California. PARTICIPANTS: All patients with nasal dermoids evaluated and treated from 1990 to 2000. INTERVENTION: Preoperative radiographic evaluation and surgical excision. OUTCOME MEASURES: Accuracy of CT and MRI correlated with surgical findings and results. RESULTS: Ten patients were identified with the diagnosis of nasal dermoid. The age at diagnosis ranged from 0 to 24 months, with a mean of 3 months. Six children presented with masses located at the glabella, three patients presented with masses located at the nasal dorsum and one presented with a mass at the nasal tip. Six children underwent a computed tomogram with contrast of the head. Seven children underwent a MRI study of the head. Three children underwent an initial CT followed by MRI. Twenty percent of children were found to have intracranial extension. CT scan accurately diagnosed intracranial extension in one case, was indeterminate in a second case and falsely positive in a third case. MRI correctly diagnosed intracranial extension in two cases and had no false positive or false negative results. No children were found to have associated intracranial anomalies. In the early years of the review, a simple excision was made over the mass with blunt and sharp dissection for removal. (An external rhinoplasty incision is now used with better exposure and improved cosmetic results.) In cases with intracranial communication, a combined approach of external rhinoplasty and craniotomy was used. CONCLUSIONS: MRI alone is the most cost effective and accurate means of evaluating nasal dermoids and is essential for preoperative planning. The surgical approach of choice is external rhinoplasty for both cosmetic reasons and exposure of nasal dermoids with and without intracranial extension.