Calciphylaxis: Is There a Role for Parathyroidectomy?

OBJECTIVE: Calciphylaxis, a rare disorder typically affecting renal failure patients, results in vascular calcification with subsequent skin necrosis, gangrene, and often death from sepsis. Parathyroid hormone is thought to act as a tissue sensitizer leading to these soft tissue changes. As such, parathyroidectomy is often advocated to control this complicated condition. A discussion of calciphylaxis does not exist in the otolaryngology literature, and head and neck surgeons performing parathyroidectomy should be aware of this phenomenon. This study evaluates the success of parathyroidectomy in reversing the ill effects of calciphylaxis in both our patient population and the literature. STUDY DESIGN: Retrospective study and review of the literature. METHODS: Five patients with calciphylaxis treated at our institution were evaluated for mortality, surgical and perioperative complications, wound healing, and predictors of patient outcomes. RESULTS: Two patients died from sepsis and infectious complications of their calciphylaxis shortly after surgery. Of the three survivors, two later died (15 and 18 mo after surgery) from causes not directly related to calciphylaxis. The other long-term survivor required partial amputation of a leg for osteomyelitis. There was one operative complication-- wound infection requiring antibiotic therapy, drainage, and packing. Postoperative hypocalcemia required treatment in two patients. Immediate perioperative survival was more likely in patients with leukocyte counts less than 20,000 cells/mL CONCLUSIONS: Calciphylaxis is a serious disease and patients often succumb to sepsis and infectious complications. Patients with extremely high leukocyte counts from coexistent infections may have a worse prognosis. Although a conclusive effective therapy does not exist, parathyroidectomy can be safely performed and may benefit some patients with what is often an otherwise fatal disease. The literature to date generally confirms our findings. Key Words: Calciphylaxis, parathyroid hormone, parathyroidectomy, skin necrosis, chronic renal failure.     

Is there a functioning vestibulocochlear nerve? Cochlear implantation in a child with symmetrical auditory findings but asymmetric imaging

The finding of an abnormally narrow internal auditory meatus during the assessment of a child for cochlear implantation raises the possibility that the meatus may not contain the normal number of nerves. Even with currently available MRI techniques it may be extremely difficult to decide whether or not to offer cochlear implant in such cases. We present a child of 4 1/2 years, assessed for cochlear implantation. MRI and CT imaging suggested aplasia of one vestibulocochlear nerve and hypoplasia of the other. However, audiological tests showed clear responses to sound in both ears and functional use of sound in her daily life. This child was eventually implanted with encouraging postoperative results.     

Is Word Recognition Correlated With the Number of Surviving Spiral Ganglion Cells and Electrode Insertion Depth in Human Subjects With Cochlear Implants?

OBJECTIVES/HYPOTHESIS: Speech perception scores using cochlear implants have ranged widely in all published series. The underlying determinants of success in word recognition are incompletely defined. Although it has been assumed that residual spiral ganglion cell population in the deaf ear may play a critical role, published data from temporal bone specimens from patients have not supported this hypothesis. The depth of insertion of a multichannel cochlear implant has also been suggested as a clinical variable that may be correlated with word recognition. In the current study these correlations were evaluated in 15 human subjects. STUDY DESIGN: Retrospective review of temporal bone histopathology. METHODS: Temporal bones were fixed and prepared for histological study by standard techniques. Specimens were then serially sectioned and reconstructed by two-dimensional methods. The spiral ganglion cells were counted, and the depth of insertion of the cochlear implant as measured from the round window was determined. Correlation analyses were then performed between the NU6 word scores and spiral ganglion cell counts and the depth of insertion. RESULTS: The segmental and total spiral ganglion cell counts were not significantly correlated (P > .50) with NU6 word scores for the 15 subjects. Statistically significant correlations were not achieved by separate analysis of implant types. Similarly, no significant correlation between the depth of insertion of the electrode array and postoperative NU6 word score was identified for the group. CONCLUSION: Although it is unlikely that the number of residual spiral ganglion cell counts is irrelevant to the determination of word recognition following cochlear implantation, there are, clearly, other clinical variables not yet identified that play an important role in determining success with cochlear implantation.     

Simultaneous Grouping in Cochlear Implant Listeners: Can Abrupt Changes in Level Be Used to Segregate Components from a Complex Tone?

A sudden increase in the amplitude of a component often causes its segregation from a complex tone, and shorter rise times enhance this effect. We explored whether this also occurs in implant listeners (n = 8). Condition 1 used a 3.5-s “complex tone” comprising concurrent stimulation on five electrodes distributed across the array of the Nucleus CI24 implant. For each listener, the baseline stimulus level on each electrode was set at 50% of the dynamic range (DR). Two 1-s increments of 12.5%, 25%, or 50% DR were introduced in succession on adjacent electrodes within the “inner” three of those activated. Both increments had rise and fall times of 30 and 970 ms or vice versa. Listeners reported which increment was higher in pitch. Some listeners performed above chance for all increment sizes, but only for 50% increments did all listeners perform above chance. No significant effect of rise time was found. Condition 2 replaced amplitude increments with decrements. Only three listeners performed above chance even for 50% decrements. One exceptional listener performed well for 50% decrements with fall and rise times of 970 and 30 ms but around chance for fall and rise times of 30 and 970 ms, indicating successful discrimination based on a sudden rise back to baseline stimulation. Overall, the results suggest that implant listeners can use amplitude changes against a constant background to pick out components from a complex, but generally these must be large compared with those required in normal hearing. For increments, performance depended mainly on above-baseline stimulation of the target electrodes, not rise time. With one exception, performance for decrements was typically very poor.     

Noise Enhances Modulation Sensitivity in Cochlear Implant Listeners: Stochastic Resonance in a Prosthetic Sensory System?

Cochlear implants restore auditory sensitivity to the profoundly hearing-impaired by means of electrical stimulation of residual auditory nerve fibers. Sensorineural hearing loss results in a loss of spontaneous activity among the remaining auditory neurons and is accompanied by a reduction in the normal stochastic nature of neural firing in response to electric stimulation. It has been hypothesized that the natural stochasticity of the neural response is important for auditory signal processing and that introducing some optimal amount of noise into the stimulus may improve auditory perception through the implant. In this article we show that, for soft but audible stimuli, an optimal amount of "prosthetic" noise significantly improves sensitivity to envelope modulation in cochlear implant listeners. A nonmonotonic function relates modulation sensitivity and noise level, suggesting the presence of stochastic resonance.     

Cochlear implantation in a patient with sensori-neural deafness secondary to Charcot−Marie−Tooth disease

Abstract

Objective and importance

Charcot?Marie?Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT.

Clinical presentation

A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%.

Intervention

A Med-el Flex^SOFT cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively.

Conclusion

This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT disease. Progress post-implantation is likely to be slower than for the average CI user.     

Is there a better way to do laser-assisted uvulopalatoplasty?

OBJECTIVE: To assess the subjective and objective short- and medium- to long-term results of laser-assisted uvulopalatoplasty (LAUP) for snoring and obstructive sleep apnea. DESIGN: A nonrandomized, prospective, before-after trial. PATIENTS AND INTERVENTIONS: Twenty-five patients underwent a modified procedure of LAUP termed one-stage LAUP, and a matched control group of 24 patients underwent uvulopalatopharyngoplasty. MAIN OUTCOME MEASURES: Subjective analysis of LAUP included a preoperative and 2 postoperative evaluations of the state of snoring (4 weeks and after a mean +/- SD of 12.2 +/- 9.9 months). A score on 5 other sleep-related symptoms was recorded before and after completion of LAUP. The objective polysomnographic outcomes were compared with a control group undergoing uvulopalatopharyngoplasty. RESULTS: In 25 patients, improvement in the state of snoring significantly declined from 76% (n = 19) to 32% (n = 8), and worsening increased from 12% (n = 3) to 32% (n = 8) (P<.001). Evaluation of 5 other sleep-related symptoms showed that 52% of patients (n = 13) improved and 20% (n = 5) worsened. Polysomnography of LAUP patients showed that the mean postoperative respiratory disturbance index worsened significantly (33.1 +/- 23.1) compared with the preoperative one (25.3 +/- 14.3) (P =.05); also, 20% of the procedures were successful and 36% revealed marked worsening. The respiratory disturbance index of uvulopalatopharyngoplasty patients changed from 26.0 +/- 18.0 to 18.7 +/- 21.3, yet improvement did not reach statistical significance (P =.09). Furthermore, 58% (n = 14) of the surgical procedures were successful and only 8% (n = 2) revealed marked worsening. CONCLUSIONS: The favorable, subjective, short-term results of modified LAUP deteriorated over time. The procedure might also lead to aggravation of existing apnea. These findings are probably related to progressive palatal fibrosis and velopharyngeal narrowing originated by the laser beam.     

Is shoulder impingement syndrome a problem in otolaryngologists?

Shoulder problems are well-documented as an occupational illness. The incidence of occupational shoulder problems is increasing. A postal questionnaire survey was conducted to see if otolaryngologists are more susceptible to shoulder impingement syndrome because of their occupation. Endocrinologists were used as the control group. Among 556 questionnaires sent to otolaryngologists, 367 (64.6%) responses were returned compared with 210 questionnaires sent to endocrinologists, of which 138 (65.7%) responses were returned. A total of 88 (24.0%) of the otolaryngologists had suffered from impingement syndrome compared with 15 (10.9%) of the endocrinologists, which was significantly different. Of those with impingement syndrome, more endocrinologists gave a history of injury or overuse compared with otolaryngologists. There must be another factor, leading to increased incidence of impingement syndrome, which may be because of the continuous flexion, and abduction of their shoulders during examination and operating on patients.     

Is shoulder impingement syndrome a problem in otolaryngologists?

Is shoulder impingement syndrome a problem in otolaryngologists? Shoulder problems are well‐documented as an occupational illness. The incidence of occupational shoulder problems is increasing. A postal questionnaire survey was conducted to see if otolaryngologists are more susceptible to shoulder impingement syndrome because of their occupation. Endocrinologists were used as the control group. Among 556 questionnaires sent to otolaryngologists, 367 (64.6%) responses were returned compared with 210 questionnaires sent to endocrinologists, of which 138 (65.7%) responses were returned. A total of 88 (24.0%) of the otolaryngologists had suffered from impingement syndrome compared with 15 (10.9%) of the endocrinologists, which was significantly different. Of those with impingement syndrome, more endocrinologists gave a history of injury or overuse compared with otolaryngologists. There must be another factor, leading to increased incidence of impingement syndrome, which may be because of the continuous flexion, and abduction of their shoulders during examination and operating on patients.     

Is Otalgia be a symptom of anxiety in children?

One of the common causes of ear pain (Otalgia) in children is the pain felt in the temporomandibular region due to bruxism, usually accompanied by stress and anxiety. The diagnosis and treatment of anxiety at an early age will affect the future lives of children.Our aim in the study was to investigate the presence and subtypes of anxiety in children with otalgia due to bruxism. In our study, 86 children aged between 6 and 16 years old who had ear pain and whose pain and tenderness were detected in the temporomandibular joint area and 40 healthy children were included as the control group. The children in both study groups were administered the Spence Children's Anxiety Scale (SCAS) test by the clinical psychologist. There was no statistical correlation between the children with bruxism and normal children (p > 0.05). While anxiety rate was 82% in children with bruxism, this rate was found to be 12.5% in normal children, this result was also statistically significant (p < 0.01). According to the SCAS test, when the subscales of anxiety detected in children with bruxism were evaluated, According to the SCAS test, when the subscales of anxiety detected in children with bruxism were evaluated, it was observed that the performance anxiety was high in the 6–12 and 12–16 age group. However, obsessive symptoms were mostly observed in the 12–16 age group while separation anxiety was detected in children in the 6–12 age group.     

Timing of Endoscopic Sinus Surgery in Children: Is There an Impact on Outcome?

OBJECTIVES/HYPOTHESIS: Endoscopic sinus surgery in children continues to be a controversial issue. Major factors in determining when to operate are the duration of the disease and duration of medical treatment. STUDY DESIGN: Prospective study of children referred to a tertiary center. PATIENTS AND METHODS: Eighty-three patients underwent endoscopic sinus surgery for refractory chronic sinusitis. All patients were followed prospectively to evaluate their response to surgery for a mean follow-up period of at least 1 year. A chi2 analysis and logistic regression analysis were performed to determine statistical difference between duration of symptoms and outcome. RESULTS: The overall success rate of endoscopic sinus surgery was 80%. Children who had surgery between 6 and 12 months of symptoms despite continued medical therapy had an 84% success rate, those who had surgery between 12 and 18 months had a 74% success rate, and those with more than 18 months of treatment for symptoms had an 81% success rate (P > .05). CONCLUSIONS: Duration of the symptoms of chronic sinusitis in children of more than 6 months was not a predictor of success of endoscopic sinus surgery in these children. Although there seemed to be a trend toward having a more successful procedure in children who had surgery between 6 and 12 months of medical treatment versus those who had surgery after more than 12 months of medical treatment, this did not reach statistical significance. A prospective study with more patients may shed more light on this subject.