Relevance of cleft palatal width in short-term otologic outcomes.

OBJECTIVE: Children with cleft palate are at increased risk for Eustachian tube dysfunction (ETD) and conductive hearing loss from chronic otitis media. While it has been proposed that the severity of ETD is related to the severity of cleft palate, data are lacking to support this hypothesis. An improved understanding of the relevance of cleft width may have prognostic value that could inform decisions on the timing of tympanostomy tube placement and choice of tympanostomy tube design. The objective of this study was to assess severity of ETD in children with narrow, moderate, and wide cleft palate, with examination of hearing outcomes, number of tympanostomy procedures, and incidence of otologic complications. METHODS: Retrospective chart review was conducted on 58 patients with primary palatoplasty performed at a single academic medical center from January 1, 2016-December 31, 2019. The primary outcome was the number of otologic procedures performed after the initial palatoplasty. Secondary outcomes included audiometric findings, number of tympanostomy tube placements, presence of effusion at the time of myringotomy, and occurrence of any postoperative otologic complication. Outcomes were compared for patients with narrow (<10 mm), moderate (10-15 mm), and wide (>15 mm) cleft palate. Analysis included consideration of cleft palatal morphology (Veau I - IV), presence of Robin sequence or syndromes, and risk factors for otitis media. RESULTS: Patients with moderate and wide cleft palate underwent higher mean numbers of otologic procedures [narrow: 1.3 (95% confidence interval [CI] 0.9, 1.7); moderate: 1.6 (95% CI 1.1, 2.1); wide: 1.8 (95% CI 1.2, 2.4)]. Moderate and wide cleft palate were less likely to have normal hearing after their first tympanostomy (narrow: 50%, 10/20; moderate: 25%, 6/24; wide: 36%, 5/14). Patients with a wide cleft palate had a shorter median time between first and second tympanostomy procedures (median, IQR; narrow: 27.0, 20.8-35.7; moderate 20.4, 16.3-25.9; wide 17.3, 11.5-23.4). CONCLUSION: Our findings suggest that patients with wider cleft palate may be more susceptible to severe ETD. Further large-scale study may help to allow for more informed and personalized clinical decision making for management of cleft palate, incorporating cleft width for prognosis of risks for persistent middle ear dysfunction.

Recurrent inhibition is decreased in patients with amyotrophic lateral sclerosis.

Recurrent inhibitory pathways are powerful modulators of motor neuron excitability. Renshaw cell activation can both inhibit homologous motor neurons and disinhibit antagonists. In spastic spinal-cord-injured patients, recurrent inhibition is consistently increased, and clinical reductions in spasticity are associated with reduced recurrent inhibition. In this study, we evaluated 12 spastic patients with amyotrophic lateral sclerosis (ALS) to see whether a similar mechanism was operating. In contrast to spinal-cord-injured patients, spastic patients with ALS showed strikingly reduced recurrent inhibition, as assessed by a conditioned H-reflex technique, which produces a response (H') whose amplitude is inversely correlated with activity in recurrent inhibitory pathways. The mean ratio of the maximum H' response to the maximum H-reflex response (H'/H ratio) was 0.55, significantly greater than the ratio seen in normal subjects. Amplitude of the H' correlated with amplitude of the Achilles tendon reflex. Thus, in patients with classical ALS, recurrent inhibition appears to be abnormally reduced compared with control subjects, suggesting a different physiology for spasticity in this setting than in spinal cord transection.

Root stimulation studies in the evaluation of patients with motor neuron disease.

Nerve root stimulation may be employed in patients with motor neuron disease (MND) to rule out motor neuropathy with conduction block. The diagnostic utility of these studies is unknown, in part because the range of amplitude changes across nerve root segments in patients with active neuronal degeneration has not been well studied. We reviewed root stimulation studies in 32 patients (59 nerves) with MND and found segmental amplitude reduction from 0 to 45%, a range similar to values reported for normal subjects; there was no suggestion of conduction block based on our usual criteria.

A pilot randomized trial of oxandrolone in inclusion body myositis.

BACKGROUND: Inclusion body myositis (IBM) remains without effective therapy. As anabolic steroids have myotrophic properties, the authors studied whether a synthetic androgen, oxandrolone, would have efficacy in IBM. METHODS: A double-blind, placebo-controlled, crossover design was used. Patients received oxandrolone or placebo for 12 weeks followed by a minimum 2-month washout period, followed by 12 weeks of the alternative treatment. Maximal voluntary isometric contraction testing (MVICT), manual muscle testing (MMT), and functional performance testing were obtained before and after each treatment period, with the whole-body MVICT score as the primary outcome measure. RESULTS: Of 19 patients enrolled, 16 (14 men, 2 women; median age 68.5 years) had complete data for at least the first treatment period, with 13 completing the entire study. Whole-body MVICT improved by a median of 15.5 kg with drug and 4.1 kg with placebo (p = 0.06), whereas MMT demonstrated a median increase of 2.0 Medical Research Council points with drug and 0.9 point with placebo (p = 0.33). Upper-extremity MVICT demonstrated a significant treatment effect, with strength increasing a median 6.3 kg with drug vs 2.5 kg with placebo (p = 0.006). Stair climbing also increased a median of 1 step on average with drug versus no change with placebo (p < 0.001). Minimal adverse effects occurred. CONCLUSIONS: Oxandrolone had a borderline significant effect in improving whole-body strength and a significant effect in improving upper-extremity strength as measured by MVICT. Given these findings, further study of this drug, possibly in combination with an immunomodulating agent, is warranted.

Nonvasculitic, steroid-responsive mononeuritis multiplex.

We report two patients with mononeuritis multiplex, both of whom had focal inflammation of the perineurium and endoneurium on sural nerve biopsy without necrosis of blood vessel walls, histologic evidence of lymphoid malignancy, or mycobacterial infection. The predominant early sensory symptoms were asymmetric pain and paresthesias; subsequently, muscle weakness developed. Electrophysiologic studies showed an asymmetric sensorimotor axon loss radiculoneuropathy with denervation of limb and paraspinal muscles. Spinal fluid protein was elevated in one patient. There was no cause or underlying systemic disease. Marked improvement occurred with steroid therapy.

Sensorineural hearing loss in the mdx mouse: a model of Duchenne muscular dystrophy.

Sensorineural hearing loss has been identified in several types of muscular dystrophy, but few studies have investigated any relationship between Duchenne muscular dystrophy and hearing. An animal model of Duchenne muscular dystrophy, the mdx mouse, exhibits the same genetic defect as humans. We performed brainstem auditory evoked responses on mdx and control mice in order to assess sensorineural hearing loss. The amplitude and latency of wave I for each animal were measured at increasing sound pressure levels. A significant increase in threshold and a decrease in wave I amplitude were found in the mdx mice. These results indicate that significant sensorineural hearing loss is associated with muscular dystrophy in the mdx mouse. Possible cellular mechanisms contributing to the hearing deficit are presented.

Nasally inhaled dornase alfa in the postoperative management of chronic sinusitis due to cystic fibrosis.

OBJECTIVE: To determine the benefit of nasally inhaled dornase alfa in cystic fibrosis (CF) sinusitis. DESIGN: Retrospective chart review comparing postsurgical course, radiographic studies, and pulmonary function test results in patients who were treated with nasally inhaled dornase alfa with those in patients who were not treated with dornase alfa. PATIENTS: Twenty consecutive patients with CF who underwent functional endoscopic sinus surgery from 1993 to 1997 were included in the study. Treatment with nasally inhaled dornase alfa was initiated in 5 of the 20 patients after they underwent functional endoscopic sinus surgery. SETTING: Tertiary care academic center. RESULTS: The dornase alfa-treated patients had less mucosal edema and no polyps at serial endoscopy over 3 years compared with the non-dornase alfa-treated patients. The patients who received nasally inhaled dornase alfa also underwent fewer revision functional endoscopic sinus surgical procedures (1.6 vs 3.2), even though there was essentially no change in pulmonary function test results in these patients. CONCLUSIONS: Sinusitis continues to be a major cause of morbidity in patients with CF. Symptomatic patients frequently require multiple drug regimens, including long-term systemic antibiotic therapy, topical and systemic steroid therapy, and antibiotic nasal irrigations. This preliminary study indicates the potential impact of nasally inhaled dornase alfa in controlling postoperative symptoms in CF sinusitis.

An assessment of cochlear hair-cell loss in insulin-dependent diabetes mellitus diabetic and noise-exposed rats.

OBJECTIVE: The purpose of this study was to investigate if insulin-dependent diabetes mellitus causes degenerative changes in the inner ear and whether these changes are exacerbated by noise exposure. METHODS: Insulin-dependent diabetes mellitus was induced in male rats using streptozotocin (65 mg/kg of body weight, intravenously). Half the animals were exposed to 95 dB of random noise for 12 hours per day over a period of 6 months. The cochleae were removed, fixed, decalcified, dissected, and the hair cells counted. RESULTS: A significant loss of outer hair cells was exhibited in both noise-exposed groups; however, although there was no significant difference between these two groups, the noise-exposed diabetic animals had significant loss in more turns than did the noise-exposed control animals. The diabetic animals were not statistically different from the control animals. CONCLUSION: These results suggest that insulin-dependent diabetes mellitus may increase the hair-cell loss caused by noise overstimulation.

Timing of percutaneous endoscopic gastrostomy tube placement in head and neck cancer patients.

Percutaneous endoscopic gastrostomy (PEG) is an effective method for providing alimentation in patients with upper aerodigestive tract carcinoma. Multiple complications of this procedure have been reported, ranging from leakage around the tube to tumor seeding of the abdominal cavity. This study was undertaken to determine whether the timing of PEG tube placement with respect to primary tumor extirpation led to a difference in the number and severity of observed complications. The medical records of 43 patients with head and neck carcinoma who had PEG tubes placed from 1995 to 1996 were retrospectively reviewed. Comparisons of timing of PEG tube placement, complication, location, and stage of the primary tumor were performed. In addition, the use of adjuvant therapy with respect to the time of PEG tube placement and complications was evaluated. Of these, 23% were done before and 30% during surgery at the time of primary tumor resection (9 of 13 were after primary removal). One patient had an intraabdominal abscess. Minor complications occurred in 15 of 43 patients (35%) and included granulation tissue at the PEG site, leakage, and tube displacement. Eight of the 9 patients who underwent intraoperative PEG after tumor resection had no complications. Patients who underwent PEG during or after surgery had significantly fewer complications than those who underwent preoperative PEG or had unresectable tumors (P = 0.038). The largest number of complications occurred in patients who underwent preoperative PEG (57%) followed by patients whose tumors were unresectable (31%). There was no statistical difference with regard to tumor location or postoperative x-ray therapy in PEG complications. This study demonstrates that PEG tube placement after tumor resection has the lowest incidence of postoperative complications. Performing PEGs intraoperatively after tumor resection can prevent the need for additional anesthesia to provide alimentation in patients with upper aerodigestive tract carcinoma.

The all ulnar motor hand without forearm anastomosis.

Anomalous ulnar innervation of intrinsic hand muscles, inferred in a number of clinical series, has been documented only rarely in electrophysiological studies. Using near-nerve and standard conduction studies we evaluated 2 cases with nearly exclusive ulnar innervation of hand muscles. There was no evidence of anomalous communication in the forearm. Digital sensory fibers were normally distributed in median and ulnar nerves. The anomalous motor innervation most likely results from palmar communication between ulnar and median branches (Riche-Cannieu anastomoses). The normal distribution of digital afferents suggests that such anastomoses are formed primarily by motor axons.

Forearm velocity in carpal tunnel syndrome: when is slow too slow? .

OBJECTIVE: To correlate the frequency of superimposed processes (SPs) such as radiculopathies, polyneuropathies, and plexopathies with median motor forearm conduction velocity (MMFCV) in patients with carpal tunnel syndrome (CTS). DESIGN: All cases of diagnosed CTS were retrospectively analyzed for evidence of SPs. SETTING: Electrophysiology laboratory of a tertiary care center. PARTICIPANTS: One hundred fifty-five patients (44 men, 111 women), ages 19 to 94, who were referred for and met electrophysiologic criteria for CTS, both with and without MMFCV slowing. MAIN OUTCOME MEASURE: The frequency of SPs in patients with no, mild, moderate, and severe MMFCV slowing. RESULTS: A total of 192 arms from 155 patients were studied. Of 14 arms with mild slowing (MMFCV of 47.0 to 49.9 m/sec), 2 (14%) had an SP. Of 15 arms with moderate slowing (MMFCV of 43.0 to 46.9 m/sec), 7 [corrected] (46%) had an SP. Of 9 arms with severe slowing (MMFCV of < 43.0 m/sec), 4 (44%) had an SP. The frequency of SPs for both the moderate and severe groups was significantly higher than that in patients with a normal MMFCV (p < .01); of 154 arms with a normal MMFCV, only 9 (6%) had an SP. CONCLUSION: In cases of CTS, the finding of moderate to severe slowing of MMFCV (< 47.0 m/sec) should prompt a careful electrophysiologic investigation to exclude an SP.

Fibrillations in lumbosacral paraspinal muscles of normal subjects.

Although paraspinal muscle fibrillations and positive sharp waves (PSWs) are used to help identify neuromuscular disorders, the frequency of these abnormalities in normal subjects is uncertain. We performed lumbosacral paraspinal electromyography in 65 normal subjects. Twenty-seven (42%) had fibrillations and/or PSWs, with the prevalence of these findings increasing with age (r = 0.830, P = 0.040). These data suggest isolated fibrillations and PSWs in lumbosacral paraspinal muscles, especially of older subjects, are nonspecific findings.

Maternal Bilateral Radial Neuropathy During Childbirth in Hereditary Neuropathy With a Predisposition to Pressure Palsies (HNPP).

A 30-year-old woman developed severe bilateral radial neuropathies during vaginal delivery of twins, likely secondary to positioning and muscular effort. Subsequent evaluation led to the diagnosis of hereditary neuropathy with predisposition to pressure palsies. Avoidance of prolonged muscular effort in the arms in conjunction with medial intervention to shorten the second stage of labor may help prevent debilitating radial nerve injury in women with this disorder.

Sensory and mixed nerve conduction studies in the evaluation of ulnar neuropathy at the elbow.

The relative sensitivities of sensory, mixed nerve, and motor conduction studies in assessing ulnar neuropathy at the elbow have not yet been established. Using surface electrodes, we performed conduction studies across the elbow segment in 43 patients with symptoms referable to the ulnar nerve and 40 control subjects. Segmental slowing of motor conduction localized the lesion to the elbow in 14 of 21 patients (67%) with clear evidence of ulnar neuropathy on physical examination but only in 2 of 22 (9%) with subtle or no physical examination abnormalities. The diagnostic yield was increased by the finding of segmental slowing of sensory or mixed nerve conduction across the elbow to 86% and 68%, respectively, for each of the groups. We conclude that surface-recorded sensory and mixed nerve conduction studies appear to be more sensitive than motor studies in the electrodiagnosis of ulnar neuropathy at the elbow and are especially valuable in patients with subtle clinical involvement.

Heat sensitivity of sensory fibers in carpal tunnel syndrome.

Elevations in temperature may produce conduction block in demyelinated neurons. A well-described phenomenon in multiple sclerosis, it has also been reported in some patients with inflammatory demyelinating polyneuropathies. We used carpal tunnel syndrome (CTS) as a model to study the effect of heat on nerves with focal demyelination secondary to chronic compression. Compound motor and sensory responses were measured in 12 CTS patients and 12 normal subjects at 32 degrees C and with heating to 42 degrees C. Changes in relative motor response amplitude and area were similar for both normal subjects and CTS patients. In CTS patients, however, sensory response amplitude and area decreased 34.3% and 48.9%, significantly more than the 25.2% and 39.1% reductions in normal subjects (P=0.021 and P=0.018 respectively). We hypothesize that these reductions in response amplitude are secondary to the occurrence of heat-induced conduction block in demyelinated sensory neurons.

Differentiation between axonal and demyelinating neuropathies: identical segments recorded from proximal and distal muscles.

The presence of significant slowing of motor nerve conduction velocity is considered one of the electrodiagnostic hallmarks of demyelinating neuropathies; however, slowing of conduction velocity may also accompany severe axonal loss. When compound muscle action potential (CMAP) amplitudes are markedly reduced, it is frequently difficult to determine if conduction velocity slowing is due to axonal loss with dropout of the fastest conducting fibers or demyelination. To evaluate the relationship between conduction velocity and axonal dropout, we compared conduction velocities through the same segment of nerve recording from distal and proximal peroneal muscles in patients with chronic neuropathies, in patients with motor neuron disease, and in control subjects. In controls and patients with motor neuron disease, conduction velocities were normal with no significant difference between proximal and distal sites. In patients with axonal neuropathies, conduction velocities were preferentially slowed when recording from distal muscles and relatively normal when recording from proximal sites. Patients with demyelinating neuropathies showed marked slowing of conduction at both sites. We conclude that comparing conduction velocity obtained from proximal versus distal muscle recordings provides a simple, reliable aid for differentiating between chronic axonal and demyelinating polyneuropathies, especially in cases with conduction velocity slowing and low CMAP amplitudes.

High-temperature repetitive nerve stimulation in myasthenia gravis.

High temperature enhances the decrement on repetitive nerve stimulation (RNS) in patients with myasthenia gravis (MG). However, the limit of this phenomenon at high temperature is unknown. Three-hertz ulnar RNS was performed in 7 patients with MG at a skin temperature of 32 degrees C and then with the hand in a 44 degrees C water bath. At 32 degrees C, the mean decrement preactivation was 5% (range, 0-24%); after 1 min of exercise, the mean decrement reached a maximum of 11% (range, 1-34%) 2 min postactivation. At a hand temperature of 42 degrees C, the mean decrement preactivation was 17% (range, 0-65%); after exercise, the mean decrement reached a maximum of 29% (range, 5-74%) 1 min postactivation. In 3 subjects, RNS was normal at 32 degrees C, but a definite decrement developed with heating. These findings demonstrate that very high temperature can improve the sensitivity of ulnar RNS for postsynaptic neuromuscular transmission defects.

Electromyography and magnetic resonance imaging in the evaluation of radiculopathy.

Electromyography (EMG) and magnetic resonance imaging (MRI) are commonly used in the diagnosis of cervical and lumbosacral radiculopathy, but the agreement between the two studies is unknown. We retrospectively studied 47 patients with a clinical history compatible with either cervical or lumbosacral radiculopathy who were evaluated with both an EMG and a spine MRI within 2 months of each other. Among these patients, 55% had an EMG abnormality and 57% had an MRI abnormality that correlated with the clinically estimated level of radiculopathy. The two studies agreed in a majority (60%) of patients, with both normal in 11 and both abnormal in 17; however, only one study was abnormal in a significant minority (40%), suggesting that the two studies remain complementary diagnostic modalities. The agreement was higher in patients with abnormal findings on neurologic examination, underscoring the difficulty of confirming the diagnosis in mild radiculopathy.

Chronic motor axonal neuropathy: pathological evidence of inflammatory polyradiculoneuropathy.

Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process.

Sural/radial amplitude ratio in the diagnosis of mild axonal polyneuropathy.

As proximal nerves are relatively spared in length-dependent, axonal polyneuropathy, we theorized that a sural/radial amplitude ratio (SRAR) might be a sensitive indicator of mild polyneuropathy. In this study, sural amplitudes and SRARs in patients with signs of mild axonal polyneuropathy were compared to those of normal, age-matched control subjects. Sural and radial sensory responses were measured in a standard fashion in all subjects. Thirty polyneuropathy patients had an average SRAR of 0.29 as compared to 0.71 for the 30 normal subjects. An SRAR of less than 0.40 was a strong predictor of axonal polyneuropathy, with 90% sensitivity and 90% specificity, as compared to an absolute sural amplitude of less than 6.0 microV, which had sensitivity of only 66%. Additionally, unlike the sural amplitude, the ratio did not vary significantly with age. We conclude that the SRAR is a sensitive, specific, age-independent electrodiagnostic test for mild axonal polyneuropathy.