Cochleovestibular manifestations in Fabry disease: Importance of screening and systematic ENT evaluation Publication date: Available online 7 May 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): C. Eyermann, T. Raguin, D. Rohmer, E. Noel, A. Charpiot AbstractObjectivesFabry disease (FD) is an X-linked inherited lysosomal storage disease. It is a multisystem pathology that can include ENT disorders. The aim of the present study was to investigate the cochleovestibular manifestations of FD, in order to show the importance of screening and systematic ENT evaluation. Material and methodsA single-center retrospective study included 14 male and 23 female FD patients. Hearing impairment was defined as hearing loss greater than the 90th percentile for at least one frequency. Vestibular impairment was defined by lateral semicircular canal dysfunction. Age, ongoing enzyme replacement therapy (ERT) and organic (renal, cardiac and cerebrovascular) complications were used as severity markers. ResultsHearing impairment was found in 62.6% of cases, mostly at high frequencies, and was associated with age, ERT, and cardiac and cerebrovascular disorder. It affected 46.7% of asymptomatic adult patients. Vestibular impairment was found in 56% of cases, associated with age; it affected two-thirds of ERT patients, more than 60% of patients with organic complications, and 50% of asymptomatic adult patients. ConclusionsMore than half of patients had ENT involvement. All FD patients should undergo early ENT screening for diagnostic, prognostic and therapeutic purposes. Systematic complete ENT follow-up with auditory and vestibular evaluation should be performed regularly, even for heterozygous women. |
Plan S! Publication date: Available online 26 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): O. Laccourreye, H. Maisonneuve |
Skull vibration induced nystagmus in patients with superior semicircular canal dehiscence Publication date: Available online 25 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): G. Dumas, H. Tan, L. Dumas, P. Perrin, A. Lion, S. Schmerber AbstractObjectiveTo establish optimum stimulus frequency and location of bone conducted vibration provoking a skull vibration induced nystagmus (SVIN) in superior semi-circular canal dehiscences. MethodsSVIN 3D components in 40 patients with semi-circular canal dehiscence (27 unilateral and 13 bilateral) were compared with a group of 18 patients with severe unilateral vestibular loss and a control group of 11 volunteers. ResultsIn unilateral semi-circular canal dehiscences, SVIN torsional and horizontal components observed on vertex location in 88% beat toward the lesion side in 95%, and can be obtained up to 800 Hz (around 500 Hz being optimal). SVIN slow-phase-velocity was significantly higher on vertex stimulation at 100 and 300 Hz (P = 0.04) than on mastoids. SVIN vertical component is more often upbeating than downbeating. A SVIN was significantly more often observed in unilateral than bilateral semi-circular-canal dehiscences (P = 0.009) and with a higher slow phase velocity (P = 0.008). In severe unilateral vestibular lesions the optimal frequency was 100 Hz and SVIN beat toward the intact side. The mastoid stimulation was significantly more efficient than vertex stimulation at 60 and 100 Hz (P < 0.01). ConclusionSVIN reveals instantaneously in unilateral semi-circular canal dehiscences a characteristic nystagmus beating, for the torsional and horizontal components, toward the lesion side and with a greater sensitivity toward high frequencies on vertex stimulation. SVIN three components analysis suggests a stimulation of both superior semi-circular canal and utricle. SVIN acts as a vestibular Weber test, assessing a vestibular asymmetrical function and is a useful indicator for unilateral semi-circular canal dehiscence. |
Less alcohol, more fruit and veg!–Spread the word Publication date: Available online 23 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): F. Rubin, O. Laccourreye |
Transnasal frontal intersinus septum takedown for frontal sinus pyocele Publication date: Available online 22 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): A.J.K. Chua, D.C.W. Chin, X.Y. Huang AbstractIntroductionThe open frontal intersinus septum takedown (FISST) technique was first described in 1976. We describe our experience with an endoscopic transnasal approach to manage a frontal sinus pyocele arising from an obstructed frontal sinus outflow tract due to anterolateral thigh flap reconstruction of a maxillectomy defect. Case reportA 40-year-old lady experienced upper eyelid swelling and purulent nasal discharge 3 weeks after undergoing a left extended medial maxillectomy with free anterolateral thigh flap reconstruction. A computed tomography (CT) scan revealed total opacification of the left frontal sinus. There was no improvement with intravenous antibiotics and she underwent a surgery, whenshe was found intraoperatively to have a frontal sinus pyocele, which was then drained. She then underwent an endoscopic transnasal FISST to ventilate the left frontal sinus via the contralateral frontal recess with good results. A CT scan performed 3 months postoperatively showed a widely patent interfrontal sinus septal window and right frontal outflow tract with no disease recurrence. DiscussionThe FISST is a useful technique to manage unilateral frontal sinus disease by taking advantage of the contralateral outflow tract when the ipsilateral frontal recess is obstructed. |
Temporomandibular joint herniation through the foramen of Huschke with clicking tinnitus Publication date: Available online 20 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): K.H. Lim, J.Y. Jung, J. Rhee, J. Choi AbstractIntroductionThe foramen of Huschke (FH) is an uncommon anatomical variation of the tympanic portion of the temporal bone. It is located on the anteroinferior aspect of the external auditory canal; extremely rarely, the soft tissues around the temporomandibular joint (TMJ) can herniate through it. Case summaryWe report two cases of TMJ herniation through the FH presenting with clicking tinnitus that were treated differently. DiscussionThe treatment of TMJ herniation depends on the presenting symptoms and the patient's willingness to undergo surgical correction. If surgical management is chosen, the bone can be obliterated using tragal cartilage and temporalis fascia. |
Voice outcome indicators for unilateral vocal fold paralysis surgery: A survey among surgeons Publication date: Available online 19 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): G. Desuter, M. Dedry, B. Schaar, J.T. van Lith-Bijl, P.P. van Benthem, E.V. Sjögren AbstractIntroductionStandardization of voice outcomes indicators (VOIs) is an important issue when it comes to evaluating and comparing surgical treatments for Unilateral Vocal Fold Paralysis (UVFP). In a recent review, 11 VOIs were found to represent 80% of the VOIs cited in the literature. A survey was launched among the European laryngologists to acquire surgeons' opinions on the above mentioned preselected VOIs. Material and methodThe electronic survey took place between November and December 2016. Three general questions were asked about surgeon's practice setting(s) and experience. The eleven next questions concerned (a) surgeon's VOIs preference and (b) their estimates of post-operative target values, they would consider being satisfactory. ResultsThe response rate was 16% (50 surveys). The majority of responders worked in tertiary hospitals (50%), had 15 years of experience with UVFP and performed on average 20 UVFP related procedures a year. The VOIs that were favored by the responding surgeons were, in decreasing order of importance, Voice handicap Index (VHI-30), Maximum Phonation Time (MPT), GRBAS-I, Mean Airflow Rate (MeAF), Jitter and Shimmer. There was an excellent consensus on post-operative VOI target values between survey's results and the literature data, except for three VOIs that showed somewhat divergent tendencies (absolute VHI-30, Jitter and Shimmer). ConclusionsThree VOIs are favored by surgeons: VHI-30, MPT and GRBAS-I. Jitter and Shimmer, although very frequently reported and statistically valid in the literature, come last concerning surgeon's choice as VOI for UVFP treatment assessment. |
Rare cause of vertigo in a child Publication date: Available online 19 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): S. Achard, M. Parodi, F. Denoyelle |
Anaplastic lymphoma and silicone in cochlear implants: Let's reassure Publication date: Available online 18 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): M. Risoud, N.-X. Bonne, C. Vincent |
Burnout! Publication date: Available online 18 April 2019 Source: European Annals of Otorhinolaryngology, Head and Neck Diseases Author(s): O. Laccourreye, Q. Lisan |
ENT-MD Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00306932607174,00302841026182,alsfakia@gmail.com
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