Stridor due to a bridge-like subglottic stenosis in a 10-week-old male infant.

We present an infant with post-intubation stridor caused by a bridge-like subglottic stenosis. At the age of 6 weeks he suffered from a RSV infection with the need for endotracheal intubation. At week 10 acute respiratory distress required a re-intubation. Flexible endoscopy was suggestive for laryngomalacia. Rigid endoscopy revealed a subglottic laterolateral mucosal bridge resulting in a doubling of the airway lumen. Histopathological examination showed a fibrinoid pseudomembrane. Follow up endoscopy showed a grade 1 posterior subglottic stenosis without respiratory compromise. This is the first case in the literature of an infant with a post-intubation bridge-like fibrinoid pseudomembranous subglottic lesion.

Case report: "auditory neuropathy" in a newborn caused by a cerebellopontine angle arachnoid cyst.

We present a 6-week-old girl, referred because of failed newborn hearing screening in the right ear. Click-evoked oto-acoustic emissions were present in both ears, auditory brainstem responses (ABR) were present in the left but totally absent in the right ear. A magnetic resonance imaging (MRI) study revealed a large arachnoid cyst in the right cerebellopontine angle (CPA) and a diagnosis of "auditory neuropathy/auditory dyssynchrony" was established. A microsurgical resection of the cyst wall and fenestration was performed by a retro sigmoid approach. This is the first case in the literature of auditory neuropathy (AN) in an infant caused by a cerebellopontine angle arachnoid cyst.

Organisation of a universal newborn hearing screening programme in Flanders.

OBJECTIVE: Since 1998 an integrated universal newborn hearing screening programme (UNHSP) based on automated auditory brainstem response (AABR) has been implemented in Flanders. The protocol of the UNHSP is based on guidelines defined by the American Academy of Paediatrics (AAP). The aim of this paper is to report on the screening protocol and to assess its feasibility. METHODOLOGY: Descriptive study based upon an analysis of the screening results in the neonatal non-NICU population of Flanders between 1999 and 2004. The UNHSP, organized by Kind en Gezin (K&G), uses a 2-stage protocol: children with a refer at the first screening test are retested, and those with a refer at the retest are referred to a certified centre. Screening and referral centres communicate their data to a central database at K&G. RESULTS: From the beginning of 1999 until the end of 2004 a screening was offered to 97.91% of all eligible babies in Flanders; 91.5% of these babies were screened by K&G using the Algo Portable Newborn Screener. Three-quarters of the referred babies had a confirmed hearing loss. In 57.6% of these babies, hearing loss was bilateral. Some babies had a temporary hearing problem. The false positive rate after two tests was 0.53 per thousand. All ascertained babies started early intervention, most of them before the age of 4 months. CONCLUSIONS: K&G has succeeded in organizing a new, well-structured community-based UNHSP according to the guidelines of the AAP on Neonatal Hearing Screening.

Meniett therapy: rescue treatment in severe drug-resistant Ménière's disease?

CONCLUSION: Our data indicate that Meniett therapy is unlikely to be helpful in the long-term treatment of patients with severe, drug-resistant Ménière's disease (MD) in whom injection of intratympanic gentamicin (ITG) or another destructive procedure would otherwise be performed. OBJECTIVE: To investigate the value of Meniett therapy in patients with drug-resistant MD referred for injection of ITG. MATERIAL AND METHODS: Twelve patients referred for ITG treatment were followed during a 2-month period of Meniett therapy. Symptoms, functional level and hearing status were evaluated using a standardized staging system. Disease-specific quality-of-life measures were obtained before and after Meniett therapy. At the end of the study period, patients were followed for a mean of 37 months, thus providing long-term outcome data. RESULTS: In two patients, Meniett treatment was interrupted after 1 month because of persistent severe vertigo. In the remaining 10 subjects, we found a significant decrease in the median number of vertigo spells from 10.0/month (25th-75th percentile 4.0-19.0) prior to treatment to 3.0/month (25th-75th percentile 1.5-4.5) after treatment (p = 0.02). There was, however, no improvement in hearing status, tinnitus, functional level or self-perceived dizziness handicap. Long-term (>1 year) follow-up data revealed that only 2 subjects preferred to continue Meniett therapy and that ablative surgery had to be performed in 6/12 study patients.

Auditory neuropathy/dyssynchrony as a cause of failed neonatal hearing screening.

The prevalence of auditory neuropathy/dyssynchrony (AN/AD) is not exactly known. We retrospectively analysed the prevalence of this condition among 135 infants who failed a neonatal screening. Hearing screening was performed by automated auditory brainstem responses (AABR). Unilateral presence of click-evoked oto-acoustic emissions with absent auditory brainstem responses was found in 4 infants. Magnetic resonance imaging of the posterior fossa showed an aplasia/hypoplasia of the ipsilateral cochlear nerve in these 4 cases. The prevalence of AN/AD was 19% in infants with confirmed hearing loss. Our findings underscore the role of AABR in neonatal hearing screening.

Sinonasal fibro-osseous hamartoma: case presentation and differential diagnosis with other fibro-osseous lesions involving the paranasal sinuses.

We present the case of a 2-year-old girl who was referred to the ENT Department of the Antwerp University with a bony obstruction of the left side of the nose. The lesion originated from the ethmoid roof and the middle turbinate and extended into the nasopharynx. A partial functional resection was performed using an endoscopic approach. A combination of clinical, radiographic and histological information permitted a diagnosis of a benign fibro-osseous lesion: sinonasal hamartoma. The benign nature of this tumor justified a conservative follow-up.

Obstructive sleep apnea syndrome in children: an overview.

All children are at risk for the development of an obstructive sleep apnea syndrome (OSAS) since adenotonsillar hypertrophy is widespread and the most common cause in children. The clinical picture, etiology and diagnostic approach of OSAS in children is discussed. Attention is given to the indications for polysomnography and the differences between adult and pediatric OSAS. An overview is presented of the different treatment modalities and the possible link between sudden infant death syndrome, pediatric OSAS and adult OSAS.

Endoscopic endonasal ligation in treatment of severe posterior epistaxis.

The technique and indications of an endonasal endoscopic technique for treatment of posterior epistaxis are presented. Three cases were successfully treated in our department in 1999. The posterior vascularisation of the nose is reviewed with emphasis on surgical anatomy. Endoscopic endonasal ligation of posterior nasal arterial vascularisation is compared with other treatment choices in posterior epistaxis. The technique is save and efficient. Since it is also a minimally invasive technique, it has become our first choice surgical procedure for posterior epistaxis.

Site of upper airway obstruction in obstructive apnoea and influence of sleep stage.

Various sites along the upper airway (UA) are prone to narrow and collapse in patients affected by obstructive sleep apnoea. Sleep stages may eventually alter these sites. The present study was designed to determine the site(s) of UA obstruction and the influence of sleep stage on the pattern of obstruction. Twenty eight obstructive sleep apnoea patients underwent UA pressure measurements during polysomnography. Solid-state pressure sensors were located at the nasopharynx, oropharynx, tongue base, hypopharynx and oesophagus and the lower limit of UA obstruction was determined relying on the observed pressure pattern. The site of UA obstruction varied among consecutive apnoeas in all but two patients. The lower limit of UA obstruction was predominantly located at the nasoand oropharynx. Rapid eye movement (REM) sleep was associated with a tendency for obstruction to extend towards lower levels of the UA and nasopharyngeal occlusion was significantly less observed during REM compared to oropharyngeal obstruction. Upper airway obstruction involves more than one specific site of the upper airway in the majority of sleep apnoea patients. Obstruction at lower levels of the upper airway is more likely to be observed during rapid eye movement sleep.

Pattern of upper airway obstruction during sleep before and after uvulopalatopharyngoplasty in patients with obstructive sleep apnea.

Objective: To investigate the pre-and postoperative pattern of upper airway obstruction in obstructive sleep apnea (OSA) patients treated by uvulopalatopharyngoplasty (UPPP).Background: The response rate to UPPP in unselected OSA patients is generally about 50%.Methods: Intraluminal pressure measurements during sleep were employed to analyze the pattern of upper airway obstruction before and after surgery.Results: Ten patients with mild to moderate OSA (respiratory disturbance index 19.7 (16.9-27.5) events/hr underwent a full night polysomnography before and 114 (6 1-138) days after UPPP. UPPP resulted in a significant improvement in subjective snoring and daytime sleepiness, but did not significantly alter the severity of sleep-disordered breathing. Preoperatively, the major site of obstruction was located at the oropharynx in nine patients, seven of them had additional minor obstruction sites outside the oropharynx. Complete relief of upper airway obstruction was only observed in those two patients with collapse confined to the oropharynx.Conclusions: In unselected OSA patients, UPPP improved subjective snoring and daytime sleepiness but did not result in a significant improvement in RDI or sleep architecture. Our results emphasize the need for a pre-operative investigation of the upper airway during sleep to select patients with collapse confined to the oropharynx.