Dutch consensus on diagnosis and treatment of hearing loss in patients with intellectual disability

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Richtlijnen voor diagnostiek en behandeling van slechthorendheid bij mensen met een verstandelijke handicap

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Nasal mucociliary transport: new evidence for a key role of ciliary beat frequency.

OBJECTIVES/HYPOTHESIS: Mucociliary transport is an important defense mechanism of the respiratory tract. Nonetheless, the factors determining mucociliary transport are only partially understood. Ciliary beat frequency is assumed to be one of the main parameters, although the experimental evidence remains inconclusive. STUDY DESIGN: Comparing influences on mucociliary transport to influences on ciliary beat frequency. METHODS: The present study measures the effects on mucociliary transport of two ciliary beat frequency-inhibiting compounds (0.1% xylometazoline and 0.9% NaCl) and a ciliary beat frequency enhancer (0.1% salbutamol). The measurements were performed by a technetium-99m nebulizing scintigraphic method. The experiments were carried out in 15 healthy young volunteers. RESULTS: The 0.1% xylometazoline appeared to slow ciliary transport, although the decrease was not significant (P = .44). The 0.9% NaCl did reduce mucociliary transport significantly (P = .033). The 0.1% salbutamol resulted in a highly significant increase of mucociliary transport (P = .009). Xylometazoline brings about drastic changes in the nasal cavity, both anatomically and physiologically. Any comparison of mucociliary transport before and after using this vasoconstrictive agent must take this effect into account. CONCLUSIONS: The present study demonstrates a significant similarity in the effects of NaCl and salbutamol on ciliary beat frequency in vitro and on mucociliary transport in vivo. The evidence from our experiments suggests that ciliary beat frequency is a determining factor in the mucociliary transport rate in the nose

Semilongitudinal and axial CT planes in assessing cochlear patency in cochlear implant candidates.

Item does not contain fulltextOBJECTIVE: To investigate how cochlear patency as seen on computed tomography (CT), using axial plus semilongitudinal planes, is correlated with findings at surgery in cochlear implant patients. METHODS: Pre-operative CT scans of 45 patients were reviewed by three, independent observers. They classified the cochlear patency and recorded the location of any suspected decreased patency. The results were compared with the findings noted during surgery. RESULTS: In nine patients a decreased cochlear patency was found at surgery. The sensitivity and specificity of CT assessment were, respectively, 56-33-11% and 100-86-94%. The interobserver reproducibility is reflected in a mean kappa of 0.46. The sensitivity increased when only patients suffering from post-meningitic deafness were considered. CONCLUSION: Our study suggests that CT scans can be useful in assessing cochlear patency, especially in patients with post-meningitic deafness. This good performance might be explained by the combined use of scans in semilongitudinal and axial planes

Further delineation of the DFNA5 phenotype: results of speech recognition tests.

Speech recognition scores were analyzed in 34 carriers of a DFNA5 mutation. Cross-sectional linear regression analysis (last visit, maximum recognition score in %Correct on age or PTA1,2,4 kHz) established onset age (score 90%) at 16 years and onset PTA1,2,4 kHz level (score 90%) at 41 dB hearing level. The deterioration rate was 0.7%/y in the plot of maximum score against age, whereas the deterioration gradient was 0.4%/dB in the plot of maximum score against PTA1,2,4 kHz. Given the previously demonstrated rapid progression of hearing impairment, speech recognition was relatively good: at age 70, the score was still >50%. De Leenheer, Els M

Clinical features of DFNA5.

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