To date, no instruments exist to quantify the psychosocial consequences of voice disorders. The aim of the present investigation was the development of a statistically robust Voice Handicap Index (VHI). An 85-item version of this instrument was administered to 65 consecutive patients seen in the Voice Clinic at Henry Ford Hospital. The data were subjected to measures of internal consistency reliability and the initial 85-item version was reduced to a 30-item final version. This final version was administered to 63 consecutive patients on two occasions in an attempt to assess test-retest stability, which proved to be strong. The findings of the latter analysis demonstrated that a change between two administrations of 18 points represents a significant shift in psychosocial function.

Quality-of-life indicators for dysphagia provide invaluable information to the treating clinician regarding the success or failure of swallowing therapy. The purpose of this study was to develop a clinically efficient, statistically robust patient-reported outcomes tool that measures the handicapping effect of dysphagia on emotional, functional, and physical aspects of individual's lives. 60 statements describing the handicapping effect of dysphagia were collected from patient reports and divided into subscales of physical, emotional, and functional problems. The statements were presented to 77 individuals with dysphagia. Respondents replied never, sometimes, or always to each statement and rated their self-perceived dysphagia severity on a 7-point equal-appearing interval scale. Cronbach's α was performed to assess the internal consistency validation of the items within the questionnaire. The final questionnaire was reduced to 25 items and administered to 214 individuals with dysphagia and 74 controls. Test-retest was performed on 63 individuals with dysphagia. Cronbach's α for the initial and final versions was strong at r = 0.96 and r = 0.94, respectively. Significant differences occurred between the dysphagia and control groups. Test-retest reliability was strong. We present a new, easy-to-complete, statistically robust, patient-reported outcomes measure for assessing the handicapping effect of dysphagia.

Distal myopathy refers to a heterogeneous group of disorders in which the initial manifestations are weakness and atrophy of the hands and feet. We report a family segregating an autosomal dominant distal myopathy, with multiple affected individuals in whom vocal cord and pharyngeal weakness may accompany the distal myopathy, without involvement of the ocular muscles. To our knowledge, this pedigree displays a distinct distal myopathy with the added features of pharyngeal and vocal cord dysfunction (VCPDM) that has not been previously reported. We mapped the MPD2 gene for VCPDM to chromosome 5q within a 12-cM linkage interval between markers D5S458 and D5S1972 in a large pedigree (a maximum LOD score of 12.94 at a recombination fraction of 0 for D5S393) and combined genome screening and DNA pooling successfully adapted to fluorescent markers. This technique provides for the possibility of fully automated genome scans.

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Although dysphagia is a common problem for many Parkinson's disease (PD) patients, the effect of deep brain stimulation (DBS) on swallowing is unclear. Fourteen subjects with advanced PD underwent videofluorographic swallowing studies prior to bilateral DBS of the subthalamic nucleus (STN) and at 3 and 12 months postprocedure. They were tested under several stimulation and medication conditions. Subjects completed the Dysphagia Handicap Index at each time. There was a strong trend toward improved swallowing response for solid intake in the medication-free condition with the stimulator on compared with the stimulator off (P = .0107). Also, there was a trend toward improved oral preparation of thin liquids (P = .0368) in the medication-free condition when the stimulator was on versus off 12 months later. The remaining swallowing parameters showed no change or worsening of swallowing function regardless of stimulator or medication status. Results of the Dysphagia Handicap Index revealed significant improvement in subject self-perception of swallowing 3 and 12 months following the procedure compared with baseline on the functional subscale (P = .020 and P = .010, respectively), the emotional subscale (P = .013 and P = .003, respectively), and the total score (P = .025 and P = .003, respectively). These data suggest that bilateral STN-DBS does not substantively impair swallowing in PD. In addition, it may improve motor sequencing of the oropharyngeal swallow for solid consistencies (which are known to provide increased sensory feedback to assist motor planning of the oropharyngeal swallow). Subjects with advanced PD who are undergoing DBS may perceive significant improvement in swallowing ability despite the lack of objective improvements in swallowing function.

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