Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

Acta Otolaryngol. 2015 Nov 7;:1-5

Authors: Omichi R, Maeda Y, Sugaya A, Kataoka Y, Kariya S, Nagayasu R, Nakagawa A, Yamashita T, Abe K, Nishizaki K

Abstract
CONCLUSION: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA).
OBJECTIVE: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms.
METHODS: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (± SD) age, 61.6 ± 8.9 years), CCA (n = 16; 62.8 ± 9.5 years), and age-matched controls (n = 169; 62.5 ± 10.7 years). To differentiate specific hearing loss for MSA-C and CCA from presbycusis, the shape of audiograms was examined based on previously established audiological criteria.
RESULTS: When audiogram shape was defined according to audiological criteria, the odds ratio for LTHL in SCD compared to controls was 2.492 (95% confidence interval (CI) = 1.208-5.139; p < 0.05, Pearson's Chi-square test) in MSA-C and 2.194 (95% CI = 0.709-6.795) in CCA. When the selection of audiogram shape according to these criteria was verified by three certified audiologists, odds ratios for LTHL in MSA-C and CCA were 3.243 (95% CI = 1.320-7.969) and 3.692 (95% CI = 1.052-12.957), respectively, significantly higher than in controls.

PMID: 26549828 [PubMed - as supplied by publisher]

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Improved method of gastrostomy tube replacement using a small-caliber transnasal endoscope.

Improved method of gastrostomy tube replacement using a small-caliber transnasal endoscope.

Acta Otolaryngol. 2015 Nov 7;:1-4

Authors: Chang H, Huang Y, Yao W, Zhang L, Li Y

Abstract
CONCLUSION: Gastrostomy tube replacement using a new approach through the abdominal-wall stoma with a small-caliber trans-nasal endoscope is feasible, fast, and safe compared with the trans-oral approach.
OBJECTIVES: To evaluate the feasibility of a new technique using a trans-nasal endoscope for gastrostomy tube replacement.
METHODS: Between June 2005 and December 2013 in the Peking University Third Hospital, 69 patients underwent gastrostomy tube replacement using the trans-oral approach (conventional method) or a small-caliber trans-nasal endoscope inserted through the abdominal-wall stoma (new method). A retrospective review was performed of the medical records of those patients, including demography and information about the surgical procedure and any complications. Patients were classified into the conventional group and the new method group. Descriptive statistics for all continuous variables were mean ± standard deviation and for categorical variables were number and percentage.
RESULTS: Gastrostomy tube replacement was achieved in 69 of 69 cases (100%); 23 of these procedures were performed using the new method. The surgery time with the conventional method (8.3 ± 2.0 min) was significantly longer than with the new method (6.0 ± 0.9 min, p < 0.001). With the conventional method, there was one patient (2%) with post-operative fever and skin infection; no complications occurred with the new method.

PMID: 26549728 [PubMed - as supplied by publisher]

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Prognostic value of psychological state in cochlear implantation.

Prognostic value of psychological state in cochlear implantation.

Acta Otolaryngol. 2015 Nov 7;:1-5

Authors: Han JJ, Shin MS, Song JJ, Pai I, Oh S, Kim B, Park JH, Lee JH, Oh SH

Abstract
CONCLUSION: Perioperative Minnesota Multiphasic Personality Inventory (MMPI) scores may be beneficial for predicting prognosis of cochlear implantation (CI). A positive attitude for social interaction in particular correlates with a better speech outcome. Proper perioperative psychological management may, therefore, assist in the auditory rehabilitation of CI patients.
OBJECTIVE: To determine the perioperative psychological state of CI patients and its relationship with patient prognosis after CI.
METHODS: This study prospectively enrolled 29 patients who underwent CI from 2005-2013. The MMPI was administered to assess psychosocial and emotional issues surrounding CI and the Korean version of the Central Institute of Deafness (K-CID) score was used to measure speech perception.
RESULTS: CI resulted in a significant improvement on the MMPI Paranoia scale (p = 0.02). Patients with abnormal pre-operative and post-operative MMPI scores also had an earlier onset of deafness, longer duration of deafness, and lower K-CID scores than patients with normal MMPI scores (all p < 0.05). The post-CI K-CID score had a significant negative correlation with the pre-operative MMPI Schizophrenia score (p < 0.01) and significant negative correlations with the post-operative MMPI Paranoia (p = 0.02), Psychasthenia (p = 0.02), Schizophrenia (p = 0.04), Hypomania (p = 0.02) and Social Introversion (p = 0.03) scores.

PMID: 26548342 [PubMed - as supplied by publisher]

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Comparison of distortion product otoacoustic emissions and pure tone audiometry in occupational screening for auditory deficit due to noise exposure.

Comparison of distortion product otoacoustic emissions and pure tone audiometry in occupational screening for auditory deficit due to noise exposure.

J Laryngol Otol. 2015 Nov 9;:1-8

Authors: Wooles N, Mulheran M, Bray P, Brewster M, Banerjee AR

Abstract
OBJECTIVE: To examine whether distortion product otoacoustic emissions can serve as a replacement for pure tone audiometry in longitudinal screening for occupational noise exposure related auditory deficit.
METHODS: A retrospective review was conducted of pure tone audiometry and distortion product otoacoustic emission data obtained sequentially during mandatory screening of brickyard workers (n = 16). Individual pure tone audiometry thresholds were compared with distortion product otoacoustic emission amplitudes, and a correlation of these measurements was conducted.
RESULTS: Pure tone audiometry threshold elevation was identified in 13 out of 16 workers. When distortion product otoacoustic emission amplitudes were compared with pure tone audiometry thresholds at matched frequencies, no evidence of a robust relationship was apparent. Seven out of 16 workers had substantial distortion product otoacoustic emissions with elevated pure tone audiometry thresholds.
CONCLUSION: No clinically relevant predictive relationship between distortion product otoacoustic emission amplitude and pure tone audiometry threshold was apparent. These results do not support the replacement of pure tone audiometry with distortion product otoacoustic emissions in screening. Distortion product otoacoustic emissions at frequencies associated with elevated pure tone audiometry thresholds are evidence of intact outer hair cell function, suggesting that sites distinct from these contribute to auditory deficit following ototrauma.

PMID: 26549131 [PubMed - as supplied by publisher]

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A novel technique for identifying the fistulous point in a direct carotid-cavernous fistula.

A novel technique for identifying the fistulous point in a direct carotid-cavernous fistula.

J Clin Neurosci. 2015 Nov 5;

Authors: Tsuji K, Nakagawa N, Fukawa N, Nagatsuka K, Nakano N, Kato A

Abstract
The fistulous point in a direct carotid-cavernous fistula (CCF) can often be difficult to identify because of high-flow shunting. A novel technique that is useful for identifying the fistulous point is reported. A 71-year-old woman underwent endovascular therapy for a left direct CCF that presented with sudden diplopia and tinnitus. To identify the fistulous point, vertebral angiography with manual compression of the left carotid artery was attempted, as was slow injection of a contrast agent from a balloon guiding catheter, closing off the left internal carotid artery; however, the shunt flow was very rapid, and identification was not possible. Therefore, three-dimensional digital subtraction angiography of the vertebral artery was performed while also performing manual aspiration from the balloon guiding catheter, closing off the left internal carotid artery. This reduced early visualization of the cavernous sinus and enabled an aneurysm in the cavernous sinus to be clearly visualized. Embolization was performed transarterially and transvenously, and the shunt flow disappeared completely. Vertebral angiography combined with manual aspiration from a balloon guiding catheter closing off the internal carotid artery is useful for identifying the fistulous point in a direct CCF.

PMID: 26549676 [PubMed - as supplied by publisher]

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Cancer treatment in determination of hearing loss.

Cancer treatment in determination of hearing loss.

Braz J Otorhinolaryngol. 2015 Oct 17;

Authors: Oliveira PF, Oliveira CS, Andrade JS, do Carmo Santos TF, de Oliveira-Barreto AC

Abstract
INTRODUCTION: Chemotherapy and radiotherapy in oncology have repercussions in hearing health, and can damage structures of the inner ear. These repercussions usually, result in a bilateral and irreversible hearing loss.
OBJECTIVE: To identify sensorineural hearing loss cases with complaints of tinnitus and difficulty in speech understanding and investigate their relationship with the types of chemotherapy and radiotherapy the patients received.
METHODS: Cross-sectional, clinical, observational, analytical, historical cohort study of 58 subjects treated in a public hospital in the state of Sergipe, diagnosed with neoplasia. The subjects were submitted to anamnesis, conventional pure tone audiometry, and speech recognition threshold.
RESULTS: Of the 116 ears, 25.9% presented sensorioneural hearing loss characterized by changes in high frequencies. There was a positive correlation between hearing loss and the association of chemotherapy and radiotherapy (p=0.035; R=0.196). The auditory complaint analysis shows that most of the subjects had tinnitus and speech understanding difficulty, even with a normal auditory threshold.
CONCLUSIONS: Cancer treatment causes hearing loss, associated with the administration of chemotherapy and radiotherapy. Cyclophosphamide increased the risk of causing hearing loss. Complaints of tinnitus and speech understanding difficulty were observed.

PMID: 26549572 [PubMed - as supplied by publisher]

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High doses of salicylate reduces glycinergic inhibition in the dorsal cochlear nucleus of the rat.

High doses of salicylate reduces glycinergic inhibition in the dorsal cochlear nucleus of the rat.

Hear Res. 2015 Nov 5;

Authors: Zugaib J, Ceballos CC, Leão RM

Abstract
High doses of salicylate induce reversible tinnitus in experimental animals and humans, and is a common tinnitus model. Salicylate probably acts centrally and induces hyperactivity in specific auditory brainstem areas like the dorsal cochlear nucleus (DCN). However, little is known about the effect of high doses of salicylate in synapses and neurons of the DCN. Here we investigated the effects of salicylate on the excitability and evoked and spontaneous neurotransmission in the main neurons (fusiform, cartwheel and tuberculoventral) and synapses of the DCN using whole cell recordings in slices containing the DCN. For this, we incubate the slices for at least 1 h in solution with 1.4 mM salicylate, and recorded action potentials and evoked and spontaneous synaptic currents in fusiform, cartwheel (CW) and putative tuberculoventral (TBV) neurons. We found that incubation with salicylate did not affect the firing of fusiform and TBV neurons, but decreased the spontaneous firing of cartwheel neurons, without affecting AP threshold or complex spikes. Evoked and spontaneous glutamatergic neurotransmission on the fusiform and CW neurons cells was unaffected by salicylate and evoked glycinergic neurotransmission on fusiform neurons was also unchanged by salicylate. On the other hand spontaneous glycinergic transmission on fusiform neurons was reduced in the presence of salicylate. We conclude that high doses of salicylate produces a decreased inhibitor drive on DCN fusiform neurons by reducing the spontaneous firing of cartwheel neurons, but this effect is not able to increase the excitability of fusiform neurons. So, the mechanisms of salicylate-induced tinnitus are probably more complex than simple changes in the neuronal firing and basal synaptic transmission in the DCN.

PMID: 26548740 [PubMed - as supplied by publisher]

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Anterior petrosal approach: The safety of Kawase triangle as an anatomical landmark for anterior petrosectomy in petroclival meningiomas.

Anterior petrosal approach: The safety of Kawase triangle as an anatomical landmark for anterior petrosectomy in petroclival meningiomas.

Clin Neurol Neurosurg. 2015 Oct 28;139:282-287

Authors: Borghei-Razavi H, Tomio R, Fereshtehnejad SM, Shibao S, Schick U, Toda M, Kawase T, Yoshida K

Abstract
OBJECT: Anterior petrosectomy through the middle fossa is a well-described option for addressing cranial base lesions of the petroclival region. To access posterior fossa through middle fossa, we quantitatively evaluate the safety of Kawase triangle as an anatomical landmark.
METHOD: We reviewed pre- and postoperative Multi-Slice CT scan (1mm thickness) of patients with petroclival meningioma between Jan 2009 and Sep 2013 in which anterior petrosectomy was performed to access the posterior fossa part of the tumor. The distances between drilling start and finish edge to the vital anatomical skull base structures such as internal auditory canal (IAC) and superior semicircular canal and petrous apex (petrous part of the carotid artery) were measured and analyzed.
RESULTS: Drilling entrance length is directly related with tumor size. The distances between anatomical structures and drilling points decrease with increasing tumor size, but it always remains a safe margin between drilling points and IAC, internal carotid artery (ICA), and semicircular canals in axial and coronal views.
CONCLUSION: The Kawase triangle is shown to be a safe anatomical landmark for anterior petrosectomy. The described landmarks avoid damage to the vital anatomical structures during access to the posterior fossa through middle fossa, despite temporal bone anatomical variations and different tumor sizes.

PMID: 26552034 [PubMed - as supplied by publisher]

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Temporomandibular joint disorder from skull-base osteomyelitis: a case report.

Temporomandibular joint disorder from skull-base osteomyelitis: a case report.

Maxillofac Plast Reconstr Surg. 2015 Dec;37(1):39

Authors: Lee SC, Kim JH, Kim CH, Kim BJ

Abstract
Skull-base osteomyelitis is a rare disease affecting the medulla of the temporal, sphenoid, and occipital bones. In general, it occurs due to external ear canal infections caused by malignant external otitis. Skull-base osteomyelitis usually affects elderly diabetic patients. The patient, a 58-year-old man, was referred for evaluation and management of the left jaw. Clinical examination of the patient revealed pain in the left jaw and mouth-opening deflection to the left. The maximum active mouth opening was measured to about 27 mm. Panoramic, CT, and CBCT revealed bone resorption patterns in the left condyle. Through control of diabetes, continued pharmacological treatment, arthrocentesis, and occlusal stabilization appliance therapy were carried out. The extent of active mouth opening was increased to 45 mm, and pain in the left jaw joint was alleviated. This was a case wherein complications caused by failure to control diabetes induced skull-base osteomyelitis. There is a need for continued discussion about the advantages and disadvantages of arthrocentesis with lavage for patients with skull-base osteomyelitis and other treatment options.

PMID: 26550559 [PubMed - as supplied by publisher]

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Cost-Effectiveness Analysis of Microscopic and Endoscopic Transsphenoidal Surgery versus Medical Therapy in the Management of Microprolactinoma in the United States.

Cost-Effectiveness Analysis of Microscopic and Endoscopic Transsphenoidal Surgery versus Medical Therapy in the Management of Microprolactinoma in the United States.

World Neurosurg. 2015 Nov 5;

Authors: Jethwa PR, Patel TD, Hajart AF, Eloy JA, Couldwell WT, Liu JK

Abstract
BACKGROUND: Although prolactinomas are effectively treated with dopamine agonists, some have proposed curative surgical resection for select cases of microprolactinomas to avoid life-long medical therapy. We performed a cost-effectiveness analysis comparing transsphenoidal surgery (either microsurgical or endoscopic) and medical therapy (either bromocriptine or cabergoline) using decision analysis modeling.
METHODS: A two-armed decision tree was created using TreeAge Pro Suite 2012 comparing upfront transsphenoidal surgery versus medical therapy. The economic perspective was that of the healthcare third-party payer. Based on a literature review, plausible distributions for costs and utilities were assigned to each potential outcome, taking into account medical and surgical costs and complications. Base case analysis, sensitivity analysis, and Monte Carlo simulations were performed to determine the cost-effectiveness of each strategy at 5-year and 10-year time horizons.
RESULTS: In the base case scenario, microscopic transsphenoidal surgery was the most cost-effective option at 5 years from the time of diagnosis. However, by the 10-year time horizon, endoscopic transsphenoidal surgery became the most cost-effective option. At both time horizons, medical therapy (both bromocriptine and cabergoline) were found to be more costly and less effective than transsphenoidal surgery (i.e. medical arm was dominated by the surgical arm in this model). Two-way sensitivity analysis demonstrated that endoscopic resection would be the most cost-effective strategy if the cure rate from endoscopic surgery was greater than 90% and the complication rate was less than 1%. Monte Carlo simulation was performed for endoscopic surgery vs. microscopic surgery at both time horizons. This analysis produced an incremental cost-effectiveness ratio (ICER) of $80,235/QALY at 5-year and $40,737/QALY at 10-years, implying that with increasing time intervals, endoscopic transsphenoidal surgery is the more cost-effective treatment strategy.
CONCLUSIONS: Based on the results of our model, transsphenoidal surgical resection of microprolactinomas, either microsurgical or endoscopic, appears to be more cost-effective than life-long medical therapy in young patients with life expectancy greater than 10 years. We caution that surgical resection for microprolactinomas be performed only in select cases by experienced pituitary surgeons at high-volume centers with high biochemical cure rates and low complication rates.

PMID: 26548828 [PubMed - as supplied by publisher]

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[Morphology and phenomenology of crossbow injuries with a review of the literature].

[Morphology and phenomenology of crossbow injuries with a review of the literature].

Arch Kriminol. 2014 Nov-Dec;234(5-6):154-65

Authors: Kernbach-Wighton G, Madea B

Abstract
This paper presents two cases of survived and fatal injuries caused by broad-head arrows. Case no. 1 is characterised by repeated assaults resulting in severe injuries to the legs of the victim. The lesions showed characteristic patterns due to the arrow-heads with four cross-shaped blades. Case no. 2 represented a suicide with the crossbow arrow entering the upper mouth region at close range, penetrating the base of the skull, the pons and the inter-hemispheric space, with the tip of the arrow being lodged in the skullcap. Morphology of the wounds, ballistic features and technical characteristics are discussed and the available literature is reviewed.

PMID: 26548017 [PubMed - in process]

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Challenges in Histologic Diagnosis of Nonchordomatous Lesions of the Clivus.

Related Articles

Challenges in Histologic Diagnosis of Nonchordomatous Lesions of the Clivus.

JAMA Otolaryngol Head Neck Surg. 2015 Aug;141(8):745-50

Authors: Kristin J, Schipper JH, Jalkhi M, Zahnert T, Wagenmann M

Abstract
IMPORTANCE: Nonchordomatous clival lesions are rare and represent a wide range of different benign and malignant pathologies. For an accurate and specific final diagnosis, biopsy procedures and/or resections followed by histologic examination are mandatory.
OBJECTIVE: To illustrate the challenges in obtaining a final histologic diagnosis in patients with various types of clival lesions.
DESIGN, SETTING, AND PARTICIPANTS: We performed a retrospective analysis of medical records of 24 patients who underwent endonasal endoscopic biopsy of the clivus between February 1, 2005, and June 1, 2013, in 2 medical university hospitals. Analysis was conducted between January 1 and August 15, 2014.
INTERVENTIONS: All patients underwent endoscopic biopsy of the clivus.
MAIN OUTCOMES AND MEASURES: The number of biopsies performed to establish a diagnosis in clival lesions and the problems encountered when analyzing the radiologic findings and histologic results.
RESULTS: In 14 of 24 patients (58%), a conclusive histologic diagnosis of the nonchordomatous clival lesion could be determined. Despite up to 3 endonasal endoscopic biopsies, the histologic result could not be clearly specified in the remaining 10 patients (42%). No major complications occurred. Treatment based on the testing results included endonasal endoscopic surgery, radiotherapy or radiochemotherapy, and/or follow-up examination.
CONCLUSIONS AND RELEVANCE: Challenges can occur in the radiologic evaluation and pathologic differentiation of diverse bone lesions with overlapping morphologic features as well as in the differentiation between neoplastic, reactive, inflammatory, and metabolic bone lesions and developmental disorders. Despite more than 1 biopsy, histologic classification will not always lead to a definitive diagnosis. In such cases, an interdisciplinary team should decide whether additional biopsies should be performed or whether clinical, endoscopic, and radiologic controls are sufficient.

PMID: 26086528 [PubMed - indexed for MEDLINE]

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Measurement of Voluntary Cough Production and Airway Protection in Parkinson Disease.

Measurement of Voluntary Cough Production and Airway Protection in Parkinson Disease.

Arch Phys Med Rehabil. 2015 Nov 6;

Authors: Silverman EP, Carnaby-Mann G, Singletary F, Hoffman-Ruddy B, Yeager J, Sapienza C

Abstract
OBJECTIVE: To examine relationships between Peak expiratory (cough) airflow rate and swallowing symptom severity in participants with Parkinson Disease DESIGN: Participants were cued to cough into an analog peak flow meter then swallowed three, 20 mL thin liquid barium boluses. Analyses were directed at detecting potential relationships among disease severity, swallowing symptom severity and PEFR.
PARTICIPANTS: Sixty eight male and females with PD.
INTERVENTIONS: Not applicable MAIN OUTCOME MEASURES: PEFR and swallow symptom severity RESULTS: PEFR varied significantly across swallowing severity classifications. Participants with more severe disease displayed a significant, linear decrease in PEFR compared to those participants with earlier stage, less severe disease. Swallowing symptom severity varied significantly across groups when comparing participants with less severe PD to those with more severe PD. Participants with early-stage PD demonstrated little to no swallowing symptoms and had the highest measures of PEFR. In contrast, participants with the most severe swallowing symptoms also displayed the lowest measures of PEFR.
CONCLUSIONS: Relationships existed among PD severity, swallowing symptom severity and PEFR in participants with PD. PEFR may eventually stand as a non-invasive predictor of aspiration risk in those with PD, particularly later-stage disease. Inclusion of PEFRs into existing clinical swallowing assessments may increase the sensitivity and predictive validity of these assessments.

PMID: 26551228 [PubMed - as supplied by publisher]

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Tongue acupuncture in treatment of post-stroke dysphagia.

Tongue acupuncture in treatment of post-stroke dysphagia.

Int J Clin Exp Med. 2015;8(8):14090-4

Authors: Cai H, Ma B, Gao X, Gao H

Abstract
Tongue acupuncture is a technique that treats illness through acupuncture applied to the tongue. This study was designed to assess its therapeutic effects in the treatment of post-stroke dysphagia. A clinical control study was conducted with randomly selected 180 patients with post-stroke dysphagia. The patients were assigned into 2 groups: 90 in the Tongue acupuncture group received tongue acupuncture on the basis of conventional medication, 90 in the conventional acupuncture group received acupuncture on the neck and wrist. Acupoints in the tongue are Juanquan (EX-HN10) (at the midpoint of dorsal raphe of the tongue) and Haiquan (EX-HN11) (Sublingual frenulum midpoint). Acupoits on the body are Fengchi (GB20) and Neiguan (PC6). The effective rate, the national institutes of health stroke scale (NIHSS), TV X-ray fluoroscopy swallowing function (VFSS), the incidence rate of pneumonia were used to evaluate the efficacy after 4 weeks treatment. The NIHSS and VFSS of tongue acupuncture group were improved significantly than that of the conventional group (P < 0.01, respectively). The incidence rate of pneumonia decreased (P < 0. 01). The effective rate of the tongue acupuncture group was higher than that of conventional group (96.67% vs. 66.67%, P < 0. 01). On the basis of the conventional medication, tongue acupuncture would effectively improve the swallow functions, decrease the neurological deficit and reduce the incidence of pneumonia in patients with post-stroke dysphagia.

PMID: 26550374 [PubMed]

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Spinocerebellar ataxia type 36 exists in diverse populations and can be caused by a short hexanucleotide GGCCTG repeat expansion.

Related Articles

Spinocerebellar ataxia type 36 exists in diverse populations and can be caused by a short hexanucleotide GGCCTG repeat expansion.

J Neurol Neurosurg Psychiatry. 2015 Sep;86(9):986-95

Authors: Obayashi M, Stevanin G, Synofzik M, Monin ML, Duyckaerts C, Sato N, Streichenberger N, Vighetto A, Desestret V, Tesson C, Wichmann HE, Illig T, Huttenlocher J, Kita Y, Izumi Y, Mizusawa H, Schöls L, Klopstock T, Brice A, Ishikawa K, Dürr A

Abstract
OBJECTIVE: Spinocerebellar ataxia 36 (SCA36) is an autosomal-dominant neurodegenerative disorder caused by a large (>650) hexanucleotide GGCCTG repeat expansion in the first intron of the NOP56 gene. The aim of this study is to clarify the prevalence, clinical and genetic features of SCA36.
METHODS: The expansion was tested in 676 unrelated SCA index cases and 727 controls from France, Germany and Japan. Clinical and neuropathological features were investigated in available family members.
RESULTS: Normal alleles ranged between 5 and 14 hexanucleotide repeats. Expansions were detected in 12 families in France (prevalence: 1.9% of all French SCAs) including one family each with Spanish, Portuguese or Chinese ancestry, in five families in Japan (1.5% of all Japanese SCAs), but were absent in German patients. All the 17 SCA36 families shared one common haplotype for a 7.5 kb pairs region flanking the expansion. While 27 individuals had typically long expansions, three affected individuals harboured small hexanucleotide expansions of 25, 30 and 31 hexanucleotide repeat-units, demonstrating that such a small expansion could cause the disease. All patients showed slowly progressive cerebellar ataxia frequently accompanied by hearing and cognitive impairments, tremor, ptosis and reduced vibration sense, with the age at onset ranging between 39 and 65 years, and clinical features were indistinguishable between individuals with short and typically long expansions. Neuropathology in a presymptomatic case disclosed that Purkinje cells and hypoglossal neurons are affected.
CONCLUSIONS: SCA36 is rare with a worldwide distribution. It can be caused by a short GGCCTG expansion and associates various extracerebellar symptoms.

PMID: 25476002 [PubMed - indexed for MEDLINE]

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Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

Acta Otolaryngol. 2015 Nov 7;:1-5

Authors: Omichi R, Maeda Y, Sugaya A, Kataoka Y, Kariya S, Nagayasu R, Nakagawa A, Yamashita T, Abe K, Nishizaki K

Abstract
CONCLUSION: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA).
OBJECTIVE: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms.
METHODS: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (± SD) age, 61.6 ± 8.9 years), CCA (n = 16; 62.8 ± 9.5 years), and age-matched controls (n = 169; 62.5 ± 10.7 years). To differentiate specific hearing loss for MSA-C and CCA from presbycusis, the shape of audiograms was examined based on previously established audiological criteria.
RESULTS: When audiogram shape was defined according to audiological criteria, the odds ratio for LTHL in SCD compared to controls was 2.492 (95% confidence interval (CI) = 1.208-5.139; p < 0.05, Pearson's Chi-square test) in MSA-C and 2.194 (95% CI = 0.709-6.795) in CCA. When the selection of audiogram shape according to these criteria was verified by three certified audiologists, odds ratios for LTHL in MSA-C and CCA were 3.243 (95% CI = 1.320-7.969) and 3.692 (95% CI = 1.052-12.957), respectively, significantly higher than in controls.

PMID: 26549828 [PubMed - as supplied by publisher]

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Improved method of gastrostomy tube replacement using a small-caliber transnasal endoscope.

Improved method of gastrostomy tube replacement using a small-caliber transnasal endoscope.

Acta Otolaryngol. 2015 Nov 7;:1-4

Authors: Chang H, Huang Y, Yao W, Zhang L, Li Y

Abstract
CONCLUSION: Gastrostomy tube replacement using a new approach through the abdominal-wall stoma with a small-caliber trans-nasal endoscope is feasible, fast, and safe compared with the trans-oral approach.
OBJECTIVES: To evaluate the feasibility of a new technique using a trans-nasal endoscope for gastrostomy tube replacement.
METHODS: Between June 2005 and December 2013 in the Peking University Third Hospital, 69 patients underwent gastrostomy tube replacement using the trans-oral approach (conventional method) or a small-caliber trans-nasal endoscope inserted through the abdominal-wall stoma (new method). A retrospective review was performed of the medical records of those patients, including demography and information about the surgical procedure and any complications. Patients were classified into the conventional group and the new method group. Descriptive statistics for all continuous variables were mean ± standard deviation and for categorical variables were number and percentage.
RESULTS: Gastrostomy tube replacement was achieved in 69 of 69 cases (100%); 23 of these procedures were performed using the new method. The surgery time with the conventional method (8.3 ± 2.0 min) was significantly longer than with the new method (6.0 ± 0.9 min, p < 0.001). With the conventional method, there was one patient (2%) with post-operative fever and skin infection; no complications occurred with the new method.

PMID: 26549728 [PubMed - as supplied by publisher]

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Prognostic value of psychological state in cochlear implantation.

Prognostic value of psychological state in cochlear implantation.

Acta Otolaryngol. 2015 Nov 7;:1-5

Authors: Han JJ, Shin MS, Song JJ, Pai I, Oh S, Kim B, Park JH, Lee JH, Oh SH

Abstract
CONCLUSION: Perioperative Minnesota Multiphasic Personality Inventory (MMPI) scores may be beneficial for predicting prognosis of cochlear implantation (CI). A positive attitude for social interaction in particular correlates with a better speech outcome. Proper perioperative psychological management may, therefore, assist in the auditory rehabilitation of CI patients.
OBJECTIVE: To determine the perioperative psychological state of CI patients and its relationship with patient prognosis after CI.
METHODS: This study prospectively enrolled 29 patients who underwent CI from 2005-2013. The MMPI was administered to assess psychosocial and emotional issues surrounding CI and the Korean version of the Central Institute of Deafness (K-CID) score was used to measure speech perception.
RESULTS: CI resulted in a significant improvement on the MMPI Paranoia scale (p = 0.02). Patients with abnormal pre-operative and post-operative MMPI scores also had an earlier onset of deafness, longer duration of deafness, and lower K-CID scores than patients with normal MMPI scores (all p < 0.05). The post-CI K-CID score had a significant negative correlation with the pre-operative MMPI Schizophrenia score (p < 0.01) and significant negative correlations with the post-operative MMPI Paranoia (p = 0.02), Psychasthenia (p = 0.02), Schizophrenia (p = 0.04), Hypomania (p = 0.02) and Social Introversion (p = 0.03) scores.

PMID: 26548342 [PubMed - as supplied by publisher]

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[Obstructive Sleep Apnea: review and a case presentation].

[Obstructive Sleep Apnea: review and a case presentation].

Refuat Hapeh Vehashinayim (1993). 2015 Jul;32(3):6-15, 66

Authors: Fleissig Y, Laviv A, Peacock ZS, Casap N

Abstract
Obstructive sleep apnea may be a life threatening situation if does not get proper attention. Risk factors are easy to find, therefore general dental surgeons should be aware of them and refer if needed. This literature review clearly explains how obstructive sleep apnea is diagnosed and treated. We present a case of a patient with severe obstructive sleep apnea (apnea hypopnea index of 87.5/hour), who underwent bi jaw surgery with 2 piece Le Fort 1 maxiilary advancement, mandibular bilateral sagittal split osteotomy (BSSO) advancement, and genial tubercle advancement. Post-surgery sleep study demonstrated near resolution of previously severe obstructive sleep apnea with overall apnea hypopnea index (AHI) of 3.7/hour consistent with a successful surgical outcome.

PMID: 26548146 [PubMed - in process]

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Indeterminate Single Thyroid Nodule: Synergistic Impact of Mutational Markers and Sonographic Features in Triaging Patients to Appropriate Surgery.

Indeterminate Single Thyroid Nodule: Synergistic Impact of Mutational Markers and Sonographic Features in Triaging Patients to Appropriate Surgery.

Thyroid. 2015 Nov 8;

Authors: De Napoli L, Bakkar S, Ambrosini CE, Materazzi G, Proietti A, Macerola E, Basolo F, Miccoli P

Abstract
BACKGROUND: Patients labeled as having indeterminate thyroid nodular disease following fine needle aspiration cytology are at risk of non-optimal initial surgery: an overly radical total thyroidectomy, or an unnecessary two stage operation. The objective of this study was to assess the impact of combining mutational markers and ultrasonographic features preoperatively, on predicting the risk of malignancy in patients with indeterminate nodules, thereby, offering them a tailored initial surgical intervention.
METHODS: The records of 258 patients who underwent conventional open total thyroidectomy for single nodules reported as suspicious for a follicular neoplasm (Bethesda category IV) in a 4 year period were reviewed. Main issues addressed included: certain ultrasonographic findings (individually and in combination), mutational markers (BRAF and NRAS), and combinations of both. Correlation of these with malignancy was assessed, so was their ability to predict malignancy. The usefulness of combining the absence of suspicious sonographic features and the absence of mutational markers was also evaluated.
RESULTS: Among the 258 patients with an indeterminate diagnosis, only 90 lesions were found to be malignant. The sonographic features that correlated significantly with malignancy were irregular margins, microcalcifications and a "taller-than-wide shape". The presence of irregular margins was the feature with the highest positive predictive value. Combinations of two or more features were always associated with a predictivity in excess of 90%, and at times at 100%. NRAS mutation was the most common gene alteration. Both BRAF and NRAS mutations were mutually exclusive and correlated significantly with malignancy. Their predictivity of malignancy was high particularly when combined with suspicious sonographic features (100%). The major limitation of both suspicious sonographic features and/or mutational markers was their low occurrence in malignancy. The absence of both mutational markers and suspicious sonographic features proved extremely useful in tailoring surgical strategy, as it could have ultimately spared 143 out of 258 patients (55%) an overly radical thyroidectomy.
CONCLUSION: The preoperative utility of mutational markers and sonographic features in combination has a synergistic impact. It can predict the risk of malignancy with high accuracy, properly triaging patients to appropriate surgery.

PMID: 26548748 [PubMed - as supplied by publisher]

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Dorsal and Ventral Pathways for Prosody.

Dorsal and Ventral Pathways for Prosody.

Curr Biol. 2015 Nov 4;

Authors: Sammler D, Grosbras MH, Anwander A, Bestelmeyer PE, Belin P

Abstract
Our vocal tone-the prosody-contributes a lot to the meaning of speech beyond the actual words. Indeed, the hesitant tone of a "yes" may be more telling than its affirmative lexical meaning [1]. The human brain contains dorsal and ventral processing streams in the left hemisphere that underlie core linguistic abilities such as phonology, syntax, and semantics [2-4]. Whether or not prosody-a reportedly right-hemispheric faculty [5, 6]-involves analogous processing streams is a matter of debate. Functional connectivity studies on prosody leave no doubt about the existence of such streams [7, 8], but opinions diverge on whether information travels along dorsal [9] or ventral [10, 11] pathways. Here we show, with a novel paradigm using audio morphing combined with multimodal neuroimaging and brain stimulation, that prosody perception takes dual routes along dorsal and ventral pathways in the right hemisphere. In experiment 1, categorization of speech stimuli that gradually varied in their prosodic pitch contour (between statement and question) involved (1) an auditory ventral pathway along the superior temporal lobe and (2) auditory-motor dorsal pathways connecting posterior temporal and inferior frontal/premotor areas. In experiment 2, inhibitory stimulation of right premotor cortex as a key node of the dorsal stream decreased participants' performance in prosody categorization, arguing for a motor involvement in prosody perception. These data draw a dual-stream picture of prosodic processing that parallels the established left-hemispheric multi-stream architecture of language, but with relative rightward asymmetry.

PMID: 26549262 [PubMed - as supplied by publisher]

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Effect of Endotracheal Tube Size on Vocal Outcomes After Thyroidectomy: A Randomized Clinical Trial.

Related Articles

Effect of Endotracheal Tube Size on Vocal Outcomes After Thyroidectomy: A Randomized Clinical Trial.

JAMA Otolaryngol Head Neck Surg. 2015 Aug;141(8):690-5

Authors: Mehanna R, Hennessy A, Mannion S, O'Leary G, Sheahan P

Abstract
IMPORTANCE: The optimum size of endotracheal tube (ETT) for general anesthesia remains unresolved. Choice of ETT size may be of particular relevance to thyroid surgery because of the increased risk of laryngeal trauma and concerns regarding postoperative vocal outcomes.
OBJECTIVE: To test our hypothesis that intubation with a smaller ETT would lead to reduced postoperative vocal impairment and associated reduced laryngoscopic evidence of laryngeal trauma compared with intubation with a standard-size ETT.
DESIGN, SETTING, AND PARTICIPANTS: This double-blind randomized clinical trial studied patients 18 years and older undergoing elective thyroidectomy at an academic teaching hospital from October 15, 2012, through June 13, 2013.
INTERVENTIONS: Patients were randomized to group 1 (standard-size ETT, 8.0 mm for men and 7.5 mm for women; n = 24) or group 2 (small ETT, 7.0 mm for men and 6.5 mm for women; n = 25). Patients were assessed preoperatively and at 24 hours and 3 weeks postoperatively.
MAIN OUTCOMES AND MEASURES: Fiberoptic videolaryngoscopy with modified scoring system, voice assessment using the GRBAS (grade, roughness, breathiness, asthenia, strain) rating scale, vocal self-assessment using the 30-item Voice Handicap Index, and subjective pain score.
RESULTS: At 24 hours, no significant differences were found between patients in groups 1 and 2 in change in GRBAS scores, change in laryngoscopic score (1.71 vs 1.76, P = .90), or postoperative pain score (3.3 vs 3.2, P = .91). At 3 weeks, no significant differences were found in changes in the 30-item Voice Handicap Index score (-2.2 vs -1.3, P = .74), GRBAS scores, or laryngoscopic score (0.25 vs 0.16, P = .67).
CONCLUSIONS AND RELEVANCE: We did not find evidence that smaller ETT size for thyroidectomy has any significant effect on postoperative vocal outcomes, incidence of laryngeal trauma as assessed by laryngoscopy, or pain scores. However, because of the small sample size, our study may have been underpowered to detect small differences.
TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT02136459.

PMID: 26158868 [PubMed - indexed for MEDLINE]

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Voice Outcomes Following Posterior Cordotomy With Medial Arytenoidectomy in Patients With Bilateral Vocal Fold Immobility.

Related Articles

Voice Outcomes Following Posterior Cordotomy With Medial Arytenoidectomy in Patients With Bilateral Vocal Fold Immobility.

JAMA Otolaryngol Head Neck Surg. 2015 Aug;141(8):728-32

Authors: Hillel AT, Giraldez L, Samad I, Gross J, Klein AM, Johns MM

Abstract
IMPORTANCE: Voice quality-of-life (VQOL) and perceptual voice outcomes are presumed to worsen following posterior cordotomy with medial arytenoidectomy for bilateral vocal fold immobility (BVFI); however, subjective and objective voice outcomes are not well studied in this postsurgical patient population.
OBJECTIVE: To evaluate VQOL and perceptual voice outcomes following posterior cordotomy with medial arytenoidectomy for BVFI.
DESIGN, SETTING, AND PARTICIPANTS: Retrospective medical record review of 15 patients with BVFI who underwent posterior cordotomy with medial arytenoidectomy at a tertiary care academic hospital from 2009 to 2012.
INTERVENTIONS: Suspension microlaryngoscopy was performed to expose the posterior glottis. A posterior cordotomy and medial arytenoidectomy was performed anterior to the vocal process of the vocal fold in a medial to lateral fashion.
MAIN OUTCOMES AND MEASURES: Data included age, sex, tracheostomy status, number of cordotomies, and voice outcomes. Voice-Related Quality of Life (VRQOL) and Consensus Auditory Perceptual Evaluation of Voice (CAPE-V) data were collected preoperatively and postoperatively surrounding a single procedure. Comparisons within a single group were performed with a paired t test. Statistical significance was determined at P ≤ .05.
RESULTS: Eight patients (53%) were male, and 7 (47%) were female. Six patients (40%) required a tracheotomy at some point during treatment, 4 were successfully decannulated. For all 15 patients, the mean VRQOL scores improved 12 points from 47.33 to 59.33 after posterior cordotomy (P = .12). Mean CAPE-V overall severity scores in 13 patients increased 26 points after posterior cordotomy with medial arytenoidectomy from 38.12 to 62.77 (P = .01), indicating further deviance from normal.
CONCLUSIONS AND RELEVANCE: To our knowledge, this is the first study to compare VQOL with perceptual voice outcomes following posterior cordotomy with medial arytenoidectomy in a series of patients with BVFI. Patients who underwent posterior cordotomy in this study had significantly reduced perceptual voice outcomes with unchanged VQOL. While postcordotomy patients have a dysphonia that is noticeable to voice professionals, most patients in this study subjectively felt as though their voice improved after surgery. Surgeons should be aware of these factors when counseling patients considering cordotomy for BVFI.

PMID: 26110673 [PubMed - indexed for MEDLINE]

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Predictors of Intensive Care Unit Stay After Pediatric Supraglottoplasty.

Related Articles

Predictors of Intensive Care Unit Stay After Pediatric Supraglottoplasty.

JAMA Otolaryngol Head Neck Surg. 2015 Aug;141(8):704-9

Authors: Albergotti WG, Sturm JJ, Stapleton AS, Simons JP, Mehta DK, Chi DH

Abstract
IMPORTANCE: Supraglottoplasty is a common procedure performed without evidence-based postoperative management plans. Patients are routinely admitted to the intensive care unit (ICU) postoperatively, but this may not be necessary in all cases.
OBJECTIVE: To determine (1) whether routine admission to the ICU after supraglottoplasty is warranted in all patients who undergo this procedure and (2) which factors predict requirement for ICU-level care.
DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series and analysis of immediate postoperative outcomes of all children aged 1 month to 18 years who underwent supraglottoplasty at 1 tertiary-care children's hospital from January 1, 2008, through January 31, 2014. Exclusion criteria included preoperative admission to the ICU, preoperative need for positive-pressure ventilation, history of major airway reconstruction, or any concomitant other major procedure.
EXPOSURE: Supraglottoplasty.
MAIN OUTCOMES AND MEASURES: Need for ICU-level care as defined by need for intubation, positive-pressure ventilation, multiple doses of racemic epinephrine, or oxygen via nasal cannula at greater than 4 L/min within the first 24 hours.
RESULTS: Of 223 patients identified, 25 (11.2%) met our criteria for ICU-level care. Nine patients required intubation. Twenty of the 25 patients met ICU criteria within 4 hours of surgery. Univariate analysis was performed on 38 risk factors. Risk factors for ICU requirement that remained statistically significant on multivariable analysis (P < .05) included surgical duration longer than 30 minutes (odds ratio [OR], 4.48 [95% CI, 1.51-13.19]; P = .007), nonwhite race (OR, 4.42 [95% CI, 1.54-12.66]; P = .006), and a preoperative diagnosis of gastroesophageal reflux disease (OR, 0.10 [95% CI, 0.09-0.36]; P < .001).
CONCLUSIONS AND RELEVANCE: Our study suggests that most children undergoing supraglottoplasty do not require ICU-level care postoperatively. Those who require ICU-level care are likely to be identified within the first 4 hours after surgery. Consideration for routine ICU admission should be given to those with longer surgical duration and those of nonwhite race.

PMID: 26067476 [PubMed - indexed for MEDLINE]

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Cryptococcus neoformans hyperfilamentous strain is hypervirulent in a murine model of cryptococcal meningoencephalitis.

Related Articles

Cryptococcus neoformans hyperfilamentous strain is hypervirulent in a murine model of cryptococcal meningoencephalitis.

PLoS One. 2014;9(8):e104432

Authors: Feretzaki M, Hardison SE, Wormley FL, Heitman J

Abstract
Cryptococcus neoformans is a human fungal pathogen that causes lethal infections of the lung and central nervous system in immunocompromised individuals. C. neoformans has a defined bipolar sexual life cycle with a and α mating types. During the sexual cycle, which can occur between cells of opposite mating types (bisexual reproduction) or cells of one mating type (unisexual reproduction), a dimorphic transition from yeast to hyphal growth occurs. Hyphal development and meiosis generate abundant spores that, following inhalation, penetrate deep into the lung to enter the alveoli, germinate, and establish a pulmonary infection growing as budding yeast cells. Unisexual reproduction has been directly observed only in the Cryptococcus var. neoformans (serotype D) lineage under laboratory conditions. However, hyphal development has been previously associated with reduced virulence and the serotype D lineage exhibits limited pathogenicity in the murine model. In this study we show that the serotype D hyperfilamentous strain XL280α is hypervirulent in an animal model. It can grow inside the lung of the host, establish a pulmonary infection, and then disseminate to the brain to cause cryptococcal meningoencephalitis. Surprisingly, this hyperfilamentous strain triggers an immune response polarized towards Th2-type immunity, which is usually observed in the highly virulent sibling species C. gattii, responsible for the Pacific Northwest outbreak. These studies provide a technological advance that will facilitate analysis of virulence genes and attributes in C. neoformans var. neoformans, and reveal the virulence potential of serotype D as broader and more dynamic than previously appreciated.

PMID: 25093333 [PubMed - indexed for MEDLINE]

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Petrous apex cephalocele presenting with cerebrospinal fluid rhinorrhea in an adult.

Petrous apex cephalocele presenting with cerebrospinal fluid rhinorrhea in an adult.

J Clin Neurosci. 2015 Nov 5;

Authors: Warade AG, Misra BK

Abstract
Petrous apex cephalocele (PAC) is a rare condition with very few case reports in the literature. We report a 26-year-old man with cerebrospinal fluid rhinorrhea that was misdiagnosed elsewhere and operated unsuccessfully via the endonasal route. CT cisternography revealed a 3mm right PAC for which he underwent a right subtemporal extradural approach and successful repair. We present what is to our knowledge the first case report in the literature of an adult presenting with cerebrospinal fluid leak and discuss the diagnostic dilemmas in the diagnosis of PAC, difficulties in management and review the available literature.

PMID: 26549681 [PubMed - as supplied by publisher]

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Do obese patients have worse outcomes after direct lateral interbody fusion compared to non-obese patients?

Do obese patients have worse outcomes after direct lateral interbody fusion compared to non-obese patients?

J Clin Neurosci. 2015 Nov 6;

Authors: Adogwa O, Farber SH, Fatemi P, Desai R, Elsamadicy A, Cheng J, Bagley C, Gottfried O, Isaacs RE

Abstract
Obese patients undergoing lumbar spinal fusion surgery are a challenge to the operating surgeon. Direct lateral interbody fusion (DLIF) has been performed for degenerative disease of the lumbar spine with good outcomes; nevertheless, how obese patients fare compared to non-obese patients after DLIF remains unknown. The primary aim of this study is to compare rates of postoperative complications and long-term outcomes between obese and non-obese patients undergoing DLIF. Sixty-three patients (obese: 29, non-obese: 34) undergoing index DLIF for degenerative disease of the spine between 2010 and 2012 at our institution were retrospectively enrolled. We analyzed data on demographics, postoperative complications, back and leg pain, and functional disability over 2years. Patients completed the Oswestry Disability Index (ODI) and Visual Analog Scale (VAS) back and leg pain numerical rating scores before surgery, then at 12 and 24months after surgery. Outcomes and complication rates were compared between the cohorts. The cohorts were similar at baseline. Postoperative complications rates were similar between obese and non-obese patients. There was no statistically significant difference in the incidence of durotomy (p=0.91), anterior thigh numbness (p=0.60), cerebrospinal fluid leak (p=0.91), postoperative infection (p=0.37), or bleeding requiring transfusion (p=0.16). No patient experienced a nerve injury or psoas hematoma. Both cohorts had similar 2year improvement in VAS for back pain, leg pain, and ODI. Our study demonstrates that obese and non-obese patients undergoing DLIF have similar complication profiles; hence, a patient's weight should not be a contraindication to DLIF.

PMID: 26549673 [PubMed - as supplied by publisher]

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A 7666-bp genomic deletion is frequent in Chinese Han deaf patients with non-syndromic enlarged vestibular aqueduct but without bi-allelic SLC26A4 mutations.

A 7666-bp genomic deletion is frequent in Chinese Han deaf patients with non-syndromic enlarged vestibular aqueduct but without bi-allelic SLC26A4 mutations.

Int J Pediatr Otorhinolaryngol. 2015 Oct 23;

Authors: Pang X, Chai Y, He L, Chen P, Wang X, Li L, Jia H, Wu H, Yang T

Abstract
OBJECTIVES: To investigate the genetic cause of the patients with non-syndromic enlarged vestibular aqueduct (EVA) but without bi-allelic SLC26A4 mutations.
METHODS: Presence of a homozygous genomic deletion was detected in a Chinese Han deaf patient (D1467-1) who failed to amplify the first three exons of SLC26A4. The breakpoints of the deletion were fine-mapped and revealed by PCR amplification and sequencing. This deletion was subsequently screened in 22 Chinese Han EVA probands with mono-allelic SLC26A4 mutations. The possible founder effect of the newly identified genomic deletion was evaluated by haplotype analysis.
RESULTS: A homozygous c.-2071_307+3801del7666 deletion of SLC26A4 was identified in patient D1467-1. This novel genomic deletion was subsequently identified in 18% (4/22) of the Chinese Han EVA probands with mono-allelic SLC26A4 mutations. Haplotype analysis showed that this genomic deletion is likely a founder mutation in Chinese Hans.
CONCLUSION: Our results suggested that the cryptic c.-2071_307+3801del7666 deletion of SLC26A4 is relatively frequent in Chinese Han non-syndromic EVA patients without bi-allelic SLC26A4 mutations. Screening of this genomic deletion should be incorporated into the routine DNA testing of SLC26A4 in Chinese Hans.

PMID: 26549381 [PubMed - as supplied by publisher]

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Airway response to sirolimus therapy for the treatment of complex pediatric lymphatic malformations.

Airway response to sirolimus therapy for the treatment of complex pediatric lymphatic malformations.

Int J Pediatr Otorhinolaryngol. 2015 Oct 30;

Authors: Alemi AS, Rosbe KW, Chan DK, Meyer AK

Abstract
Head and neck lymphatic malformations can create airway management challenges requiring tracheotomy. Sirolimus, an inhibitor of mammalian target of rapamycin (mTOR), may inhibit growth of lymphatic malformations. We describe two patients born with large lymphatic malformations with improved airway symptoms following sirolimus therapy. Patient #1 underwent tracheotomy and multi-modal therapy including sirolimus with reduction in airway involvement but regrowth after discontinuation of sirolimus. Patient #2 also experienced a significant response to sirolimus allowing for extubation and discharge without tracheotomy. Early initiation of sirolimus therapy should be considered as a means to avoid tracheotomy in complex head and neck lymphatic malformations.

PMID: 26549380 [PubMed - as supplied by publisher]

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Hydroxyurea-induced oral ulceration.

Hydroxyurea-induced oral ulceration.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):e232-4

Authors: Badawi M, Almazrooa S, Azher F, Alsayes F

Abstract
Hydroxyurea is an antimetabolite that is widely used in the treatment of many benign and malignant conditions. This drug is usually well tolerated but has a number of side effects that vary in incidence. In cases of clinically significant adverse events, hydroxyurea is usually discontinued either temporarily or permanently, depending on treatment need versus harm caused by side effects. Here, we report a case of oral ulceration associated with hydroxyurea treatment in a patient who had chronic myelogenous leukemia. The patient rapidly developed an oral ulcer 12 days after administration of the drug. Hydroxyurea was discontinued, and the oral lesion appreciably decreased in size and severity. Physicians and dentists should be aware of the association between hydroxyurea and oral lesions.

PMID: 26548732 [PubMed - in process]

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Response to letters to the editor.

Response to letters to the editor.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):778-9

Authors: Greene CS, Obrez A

PMID: 26548731 [PubMed - in process]

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Drs. Greene and Obrez's article inaccurate.

Drs. Greene and Obrez's article inaccurate.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):776

Authors: Cooper BC

PMID: 26548730 [PubMed - in process]

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Cone beam computed tomography (CBCT) sialography-an adjunct to salivary gland ultrasonography in the evaluation of recurrent salivary gland swelling.

Cone beam computed tomography (CBCT) sialography-an adjunct to salivary gland ultrasonography in the evaluation of recurrent salivary gland swelling.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):771-5

Authors: Kroll T, May A, Wittekindt C, Kähling C, Sharma SJ, Howaldt HP, Klussmann JP, Streckbein P

Abstract
OBJECTIVE: Cone beam computed tomography (CBCT) sialography could help improve the visualization of the ductal system of salivary glands. The aim of this retrospective investigation was to monitor the use of CBCT sialography for the diagnosis of pathologies within the intraglandular ductal system when ultrasonography was inconclusive.
STUDY DESIGN: Fourteen consecutive patients suffering from recurrent swelling of a major salivary gland were evaluated. In 12 patients (8 female; 4 male; average age 46 years), a radiopaque contrast agent could be injected into the ductal system, followed by a routine CBCT. Four blinded examiners evaluated the acquired data sets retrospectively.
RESULTS: CBCT revealed seven stenosis, two salivary stones, one complete duct atresia, one intraglandular duct ectasia, and one regular duct system. Three of the detected pathologies were strictly intraglandular.
CONCLUSIONS: CBCT sialography shows promise as a supplementary noninvasive diagnostic tool for the visualization of the intraglandular ductal system of the major human salivary glands. Controlled studies to further validate this method should be undertaken.

PMID: 26548729 [PubMed - in process]

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Three-dimensional evaluation of human jaw bone microarchitecture: correlation between the microarchitectural parameters of cone beam computed tomography and micro-computer tomography.

Three-dimensional evaluation of human jaw bone microarchitecture: correlation between the microarchitectural parameters of cone beam computed tomography and micro-computer tomography.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):762-70

Authors: Kim JE, Yi WJ, Heo MS, Lee SS, Choi SC, Huh KH

Abstract
OBJECTIVE: To evaluate the potential feasibility of cone beam computed tomography (CBCT) in the assessment of trabecular bone microarchitecture.
STUDY DESIGN: Sixty-eight specimens from four pairs of human jaw were scanned using both micro-computed tomography (micro-CT) of 19.37-μm voxel size and CBCT of 100-μm voxel size. The correlation of 3-dimensional parameters between CBCT and micro-CT was evaluated.
RESULTS: All parameters, except bone-specific surface and trabecular thickness, showed linear correlations between the 2 imaging modalities (P < .05). Among the parameters, bone volume, percent bone volume, trabecular separation, and degree of anisotropy (DA) of CBCT images showed strong correlations with those of micro-CT images. DA showed the strongest correlation (r = 0.693).
CONCLUSIONS: Most microarchitectural parameters from CBCT were correlated with those from micro-CT. Some microarchitectural parameters, especially DA, could be used as strong predictors of bone quality in the human jaw.

PMID: 26548728 [PubMed - in process]

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Differences in sequential posttreatment salivary IL-6 levels between patients with and patients without locoregional recurrences of oral squamous cell carcinoma: Part III of a cohort study.

Differences in sequential posttreatment salivary IL-6 levels between patients with and patients without locoregional recurrences of oral squamous cell carcinoma: Part III of a cohort study.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):751-760.e2

Authors: Sato J, Ohuchi M, Wada M, Ohga N, Asaka T, Yoshikawa K, Miyakoshi M, Hata H, Satoh A, Kitagawa Y

Abstract
OBJECTIVE: Sequential postoperative salivary interleukin-6 (IL-6) concentrations were examined in patients with oral squamous cell carcinoma (OSCC) who had early or late locoregional recurrences or those who did not.
STUDY DESIGN: Twenty-seven consecutive patients with OSCC were originally included in the study. All patients underwent radical surgery. Four saliva samples were collected before (periods I and II) and after (periods III and IV) surgery, and IL-6 concentrations were measured.
RESULTS: Although postoperative (period III: at the time of discharge) salivary IL-6 level was significantly higher in patients with early locoregional recurrence (P = .02) than in those without, no such relationships were observed for preoperative IL-6 concentrations (periods I and II). Postoperative (period IV: 24 months after surgery) IL-6 level was significantly higher in patients with late locoregional recurrence (P = .03) than in those without, but no such relationships were observed for IL-6 concentrations in periods I, II, and III.
CONCLUSIONS: Sequential postoperative salivary IL-6 concentration may be a useful marker for diagnosis of early and late locoregional recurrence in OSCC.

PMID: 26548727 [PubMed - in process]

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Topical lipophilic epigallocatechin-3-gallate on herpes labialis: a phase II clinical trial of AverTeaX formula.

Topical lipophilic epigallocatechin-3-gallate on herpes labialis: a phase II clinical trial of AverTeaX formula.

Oral Surg Oral Med Oral Pathol Oral Radiol. 2015 Dec;120(6):717-24

Authors: Zhao M, Zheng R, Jiang J, Dickinson D, Fu B, Chu TC, Lee LH, Pearl H, Hsu S

Abstract
OBJECTIVE: Previous in vitro and in vivo studies indicated that catechins from the tea plant (Camellia sinensis) have a therapeutic effect on herpes simplex virus infections. The aim of this study was to clinically evaluate a topical proprietary formulation containing lipophilic catechins (AverTeaX, Camellix, LLC, Evans, GA, USA) on recurrent herpes labialis.
STUDY DESIGN: A double-blind, placebo-controlled, randomized trial with 40 participants, initially in two groups.
RESULTS: Compared with the vehicle (100% glycerin USP, CVS Pharmacies, Inc., Woonsocket, RI, USA) group, AverTeaX applied topically six to eight times daily resulted in a significant reduction in clinical episode duration (median 4.5 days vs. 9 days; P = .003) and shortened blistering and ulceration stages within an episode from a median of 3 days to 1 day (P = .0003). Median quality-of-life scores, based on a multiquestion survey, showed significant differences between the groups with respect to duration of itching, from a median of 4 days to 1 day (P = .0021), and duration until symptom free, from a median of 8 days to 4 days (P = .0016). Significant differences were not found for median scores for itching, pain, burning, swelling, bleeding, and stress. Adverse effects were not reported.
CONCLUSION: AverTeaX formulation containing lipophilic catechins effectively inhibited herpes simplex labialis infection with clinical significance.

PMID: 26548726 [PubMed - in process]

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