Bilateral congenital deafness: What investigations should be performed?

BACKGROUND: The introduction of newborn hearing screening has led to earlier identification of children with congenital sensorineural hearing loss (SNHL). Aetiological clarification offers several benefits. There is currently a lack of agreement on which examinations should be recommended. OBJECTIVE: Descriptive review of the literature reporting investigations performed to establish the aetiology of congenital SNHL and comparison of the management policy in Swiss referral centres. METHODS: PubMed Search from 1985 to March 2016 with specific search terms; study selection according to inclusion/exclusion criteria; narrative analysis by use of defined criteria and question-naire. RESULTS: Ninety-two studies were finally included in this review. Forty studies investigated more than a single aetiology. Overall frequencies of aetiological parameters investigated were: genetic (47 studies), radiological (35), ophthalmic (35), serological (32), cardiac (25), renal (14), endocrine (12), neurological (8). Most of the studies were retrospective and various limitations such as poor population description, incomplete data or deficiencies in methodological quality were frequently detected. The variability detected in the investigative approach chosen by Swiss referral centres reflects the heterogeneous data seen in the literature. CONCLUSIONS: The evidence in the literature regarding an appro-priate evaluation is mostly of low quality and difficult to assess owing to high heterogeneity. Nevertheless, imaging, genetic testing, neuropaediatric and ophthalmological evaluations, electrocardiograms and cytomegalovirus analysis have been identified as examinations to be included in the assessment of children with congenital SNHL. There is a need for international consensus on the various issues of such an evaluation, such as choice of investigations and diagnostic criteria.

Chronic panrhinosinusitis without nasal polyps: long-term outcome after functional endoscopic sinus surgery.

OBJECTIVE: The goal of this study was to evaluate the long-term outcome after functional endoscopic sinus surgery (FESS) for chronic panrhinosinusitis without nasal polyps by using symptom scoring and an endoscopic outcome evaluation. STUDY DESIGN: Seventy-seven patients with chronic panrhinosinusitis without nasal polyps (Kennedy computed tomography [CT] scan stages I to III) were followed up for at least 3 years after FESS. Preoperative evaluation included a CT scan and an immunoallergologic evaluation. Three years after FESS, all patients were interviewed and scored endoscopically. RESULTS: Ninety-two percent of the patients showed a marked global improvement after FESS. The endoscopic control showed normal findings in 54% of all ethmoidal cavities. The postoperative endoscopic score correlated significantly with the subjective satisfaction ratings (P < 0.001). The preoperative CT staging proposed by Kennedy was predictive for necessity of revision surgery in 15% of the patients. CONCLUSIONS: Our data suggest that FESS for chronic panrhinosinusitis without nasal polyps has a good long-term outcome on subjective symptoms and endoscopic findings. SIGNIFICANCE: According to this study, subjective improvement correlates significantly with the postoperative endoscopic findings in the ethmoidal cavities of patients with chronic panrhinosinusitis without polyps at a long-term follow-up.