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Clin. Otolaryngol. 2011, 36, 442-449 Objectives: The influence of an extended preoperative trial of bone-anchored hearing aid (BAHA) and headband on the decision to proceed with surgery in cases of unilateral sensorineural hearing loss is studied. Design: In this prospective study, the headband was introduced after assessment confirmed suitable candidacy. Those who had an initially favourable response to the headband trial were encouraged to complete a 2-week home trial of device and headband. Patients were asked to pay special attention to situations where they perceived high demands on their hearing, e.g. at the dinner table, in the car, walking outside etc. Counselling regarding the BAHA placement, the need for daily care of the implant site and the financial implications of acquiring the device was also given. Setting: Tertiary university hospital. Participants: Patients with unilateral sensorineural hearing loss, fulfilling criteria for BAHA surgery, with favourable response to initial 'office trial' of headband. Main outcome measures: After 2-week home trial, BAHA and headband were returned with a statement of the patient's intentions. Results: Sixteen of 50 (32%) patients who expressed definite interest in BAHA surgery after a positive introduction to the headband in the office did not wish to proceed to surgery having completed a more thorough 2-week trial of device at home. Most patients cited a failure of the device to meet their hearing requirements. Subsequently, we found further attrition with nine more patients (18%) deciding at a still later date, not to proceed with surgery. Ultimately, just 25 of 50 (50%) of our initially enthusiastic group chose not to proceed to surgery. In the group who did proceed, there were no non-users. Conclusions: We propose the importance of an extended preoperative trial where BAHA is considered in the rehabilitation of unilateral sensorineural hearing loss. The trial may foster more realistic expectations of device performance in this challenging group. A 'cooling-off' period before surgery also appears to lead to further attrition with the ultimate reward of no non-users in those proceeding to surgery.

PURPOSE: To evaluate visual impairment in Finnish Usher syndrome type 3 (USH3) and compare this with visual impairment in Usher syndrome types 1b (USH1b) and 2a (USH2a). METHODS: We carried out a retrospective study of 28 Finnish USH3 patients, 24 Dutch USH2a patients and 17 Dutch USH1b patients. Cross-sectional regression analyses of the functional acuity score (FAS), functional field score (FFS*) and functional vision score (FVS*) related to age were performed for all patients. The FFS* and FVS* were calculated using the isoptre V-4 test target instead of the usual III-4 target. Statistical tests relating to regression lines and Student's t-test were used to compare between USH3 patients and the other genetic subtypes of Usher syndrome. RESULTS: Cross-sectional analyses revealed significant deterioration in the FAS (1.3% per year), FFS* (1.4% per year) and FVS* (1.8% per year) with advancing age in the USH3 patient group. At a given age the USH3 patients showed significantly poorer visual field function than the USH2a patients. CONCLUSIONS: The rate of deterioration in visual function in Finnish USH3 patients was fairly similar to that in Dutch USH1b or USH2a patients. At a given age, visual field impairment in USH3 patients was similar to that in USH1b patients but poorer than in USH2a patients.

PURPOSE: To evaluate visual impairment in Usher syndrome 1b (USH1b) and Usher syndrome 2a (USH2a). METHODS: We carried out a retrospective study of 19 USH1b patients and 40 USH2a patients. Cross-sectional regression analyses of the functional acuity score (FAS), functional field score (FFS) and functional vision score (FVS) related to age were performed. Statistical tests relating to regression lines and Student's t-test were used to compare between (sub)groups of patients. Parts of the available individual longitudinal data were used to obtain individual estimates of progressive deterioration and compare these to those obtained with cross-sectional analysis. Results were compared between subgroups of USH2a patients pertaining to combinations of different types of mutations. RESULTS: Cross-sectional analyses revealed significant deterioration of the FAS (0.7% per year), FFS (1.0% per year) and FVS (1.5% per year) with advancing age in both patient groups, without a significant difference between the USH1b and USH2a patients. Individual estimates of the deterioration rates were substantially and significantly higher than the cross-sectional estimates in some USH2a cases, including values of about 5% per year (or even higher) for the FAS (age 35-50 years), 3-4% per year for the FFS and 4-5% per year for the FVS (age > 20 years). There was no difference in functional vision score behaviour detected between subgroups of patients pertaining to different biallelic combinations of specific types of mutations. CONCLUSIONS: The FAS, FFS and FVS deteriorated significantly by 0.7-1.5% per year according to cross-sectional linear regression analysis in both USH1b and USH2a patients. Higher deterioration rates (3-5% per year) in any of these scores were attained, according to longitudinal data collected from individual USH2a patients. Score behaviour was similar across the patient groups and across different biallelic combinations of various types of mutations. However, more elaborate studies, preferably covering longitudinal data, are needed to obtain conclusive evidence.

WFS1 is a novel gene and encodes an 890 amino-acid glycoprotein (wolframin), predominantly localized in the endoplasmic reticulum. Mutations in WFS1 underlie autosomal recessive Wolfram syndrome and autosomal dominant low frequency sensorineural hearing impairment (LFSNHI) DFNA6/14. In addition, several WFS1 sequence variants have been shown to be significantly associated with diabetes mellitus and this gene has also been implicated in psychiatric diseases. Wolfram syndrome is highly variable in its clinical manifestations, which include diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. Wolfram syndrome mutations are spread over the entire coding region, and are typically inactivating, suggesting that a loss of function causes the disease phenotype. In contrast, only non-inactivating mutations have been found in DFNA6/14 families, and these mutations are mainly located in the C-terminal protein domain. In this paper, we provide an overview of the currently known disease-causing and benign allele variants of WFS1 and propose a potential genotype-phenotype correlation for Wolfram syndrome and LFSNHI. (C) 2003 Wiley-Liss, Inc.

UNLABELLED BACKGROUND. An anterior pharyngeal pouch is a common anatomic variation after total laryngectomy. The exact part the pouch has to play in postlaryngectomy swallowing disorders is unknown but might be bigger than expected. METHODS Nine patients with a symptomatic pouch were treated with CO2 laser and questioned about pre- and postoperative complaints. RESULTS Eight out of nine patients noticed an obvious improvement of their swallowing complaints after laser surgery. CONCLUSION Based on the results, it is concluded that CO2-laser treatment with division of the bar of tissue posterior to the pouch is seen as a safe and simple way to treat patients for symptoms that can be wrongly attributed to a total laryngectomy.