Neuro-otologic manifestations of sarcoidosis are rare. Dizziness in particular is a rare presenting complaint of the patient afflicted with this systemic granulomatous disorder. Head and neck and sinonasal presentations of this disease are more common. We reviewed our experience with six such patients who presented for management of their otolaryngologic (ORL) manifestations in order to delineate the involvement of the otolaryngologist in the treatment and diagnosis of these patients, with a focus upon the relevant tests and procedures in the otolaryngologist's de novo diagnosis of sarcoidosis. Studies ordered in the course of otolaryngologic evaluation and their utility in the diagnosis of sarcoidosis by the otolaryngologist are reviewed and classified into useful, supportive, and ancillary. The otolaryngologist played an important role in diagnosis, with four of six patients diagnosed to have sarcoidosis on the basis of their otolaryngologic presentations. Biopsy was performed by the otolaryngologist for diagnosis of sarcoidosis in all four of these cases. Steroids were central to treatment. Oral steroid therapy was the principal treatment: both patients with neuro-otologic sarcoid were successfully managed with oral steroids. Intralesional steroids were necessary to treat the skin lesion. One of six patients patients experienced complications related to steroid use.
The authors have investigated whether genetic abnormalities in two genes, loss of heterozygosity (LOH) of p53 and amplification of the cyclin D1 gene, correlate with clinical outcome in 56 matched pairs of blood and tumor from patients with squamous cell carcinoma of the head and neck (SCCHN). Frequency of p53 LOH was 47.4%, of cyclin D1 amplification 33.9%, and of both abnormalities together 23.7%. p53 LOH was associated with T4 (P = 0.003) and stage IV (P = 0.015) tumors. Cyclin D1 amplification was associated with recurrences and/or metachronous tumors (P = 0.007). The total number of p53 and cyclin D1 abnormalities (scored as zero, one, and two) show a pattern that seems to be additive; the increase in the number of these abnormalities is associated with a proportional increase in the frequency of T4, stage IV, presence of recurrences and/or metachronous tumors, and possibly a proportional decrease in the disease‐free interval in the sample. The association of the markers with recurrences and/or metachronous tumors persists if the tumor stage effect is mathematically removed. The combined analysis of the p53 and cyclin D1 abnormalities seems to be more informative than either of them individually and may have predictive value in SCCHN.
Hands-free triggering and pitch control would improve electrolarynx devices, which are inconvenient to use and sound unnatural. The present study tested the strategy of salvaging voice-related neural signals for hands-free electrolarynx control either by transferring cut recurrent laryngeal nerves (RLNs) to denervated neck strap muscles or by preserving strap muscles with their normal innervation. An RLN nerve transfer was performed at the time of total laryngectomy in 8 individuals, and in 5 of these subjects, strap muscles with intact ansa cervicalis innervation were also preserved for comparison. Neck surface electromyography performed over the strap muscles was used periodically for more than 1 year on phonatory and nonphonatory tasks. Signals were eventually obtained in all subjects from both RLN-innervated and ansa-innervated strap muscles that correlated with speech production. After 1 year, RLN-driven signals were larger than ansa-driven signals in magnitude, and their timing appeared better correlated with intended phonation. The results show that neck surface electromyography is an effective control source for hands-free electrolarynx activation, and that RLN transfer may provide the best approach for obtaining phonation-related activity.
Three individuals with total laryngectomy were studied for their ability to control a hands-free electrolarynx (EL) using neck surface electromyography (EMG) for on/off and pitch modulation. The laryngectomy surgery of participants was modified to preserve neck strap musculature for EMG-based EL control (EMG-EL), with muscles on one side maintaining natural innervation and those on the other side receiving a transferred recurrent laryngeal nerve (RLN). EMG from each side of the neck controlled the EMG-EL across a day of unstructured practice followed by a day of formal training, including EMG biofeedback. Using either control source, participants spoke intelligibly and fluently with the EMG-EL before formal training. This good initial performance did not consistently improve across testing for either control source in terms of voice timing, speech intelligibility, fluency, and intonation of interrogative versus declarative sentences. Neck strap muscles have activation patterns capable of simple alaryngeal voice control without requiring RLN transfer.
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