DOCSLIB.ORG

A Case of Dehiscence Syndrome of the Superior Semicircular

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
A Case of Dehiscence Syndrome of the Superior Semicircular Journal of Otolaryngology-ENT Research Dehiscence Syndrome Superior Semicircular Canal: A Case of Dehiscence Syndrome of the Superior Semicircular Abstract Case Report The superior semicircular dehiscence syndrome is a pathology Described in 1998 Volume 6 Issue 6 - 2017 by Minor et al. Which presents several sound induced vertigo symptoms Included, hearing loss and autophony due to bone dehiscense de este semicircular canal. The diagnosis was based on clinical and confirmation is given by temporal bone CT the. Treatment is surgical repair of expectant or continuity if the clinic is disabling. In This paper we present a case of auditory and vestibular DCSS With Medical Technologist Mention Otorhinolaryngology, Chile symptoms in issuing the phoneme “mmm”. Keywords: Superior semicircular canal; Dehiscence; Tullio phenomenon *Corresponding author: Victor Mercado M, Medical Technologist Mention Otorhinolaryngology, Libertad # 1348, Received:6th floor Chile, August Email: 19, 2016 | Published: April 17, 2017 Summary erosion, and none of these patients had a prior history of head trauma. This dehiscence was bilateral in most patients, so it is Dehiscence syndrome superior semicircular canal (DCSS) is suggested that some embryonic alteration might explain these a condition described in 1998 by Minor et al, presents various hallaz-gos, and probably some later event, such as trauma or symptoms including dizziness induced by sound, hearing loss and increased intracranial pressure, could accentuate this condition, autophony by the lack of bone coverage on that channel. Diagnosis tomography crag. The treatment is expectant or surgical repair de-finishingInitial experiments appearance by síntomas Tullio [5] [4]. and subsequently Huizinga ofis continuitybased on clinical if the clinic and isconfirmation incapacitating. is obtained In this article by computed a case of & Euren [6,7] Demostra-rum fenestration semicircular canals in DCSS with hearing and vestibular issuing the phoneme “mmm” pigeons, eye and head movements caused by sound evoked in the symptoms occur. same plane channel, denominating Tullio phenomenon. Initial Introduction demonstratedclinical studies inidentified conditions this faith-phe-such as deafness in patients congé-nita with syphilis [9] DCSS syndrome is the presence of a solution of continuity congenital [8]. However, these findings were subsequently between the apex of the superior semicircular canal (CSS) and encefalocraneano [12] of Lyme [13] disease and chronic otitis mediaof Ménière with cholesteatoma[10] syndrome, semicircular perilinfática [14] [11]channel fistula, erosion. trauma this syndrome in 1998, and is characterized by the presence of vertigocerebral and fossa nystagmus media [1]. frontMinor sounds, et al. [2] pressure were the changesfirst to identify in the In similar pathological conditions that can result Tullio middle ear and / or intracranial pressure. In addition symptoms phenomenon, the pressure in the ear canal (CAE) with the with- such as autophony, instability, oscilloscopy and hyperacusis next movement of the tympanic membrane, produces symptoms they may occur. Minor argued that the failure of bone coverage and signs vestibulares [15]. This phenomenon was demonstrated or dehiscence act as a “third window” moving at the level of in a study carried-do with chinchillas by Hirvonen et al, who the inner ear in addition to the oval and round windows form, investigated the changes of discharge afferents vestibular in allowing the transmission of vibration to the vestibular apparatus, response to changes in the CAE before and after fenestration CSS, producing the sensation of vertigo [2]. The mechanism involved and post reparación [16]. This relationship between the plane in syndrome DCSS would movement blister channel due to and the directional characteristics of the known eye movements increased endolymphatic complacency, dehiscencia [3] generated by the system. the pathophysiological characteristics of patients. Then appears, a vér-tico-rotaryevoked both the nystagmus excitation following or inhibition the plane of CSS of thewill CSS help affected, define A study studying thousand prevalencia4 hue-sos temporary counterclockwise in the case of CSS right and clockwise in the CSS appreciated one DCSS in 0.5% of cases, the bony plate that covered the CSS was less than 0.1 mm2 at 1.4%. No local changes (CT) crag high resolution should rea-Lizar with cuts of at least 0.5 were found in the temporal bone that give explain pu-bone mmizquierdo and Recon-ing [17]. The in con-confirmation the plane channel by to computed minimize tomographythe number Submit Manuscript | http://medcraveonline.com J Otolaryngol ENT Res 2017, 6(6): 00181 Dehiscence Syndrome Superior Semicircular Canal: A Case of Dehiscence Syndrome of Copyright: 2/5 the Superior Semicircular ©2017 Mercado et al. of false positives [18]. Another useful supplementary examination for the diagnosis of this table is vestibular myogenic evoked spontaneous nystagmus. The patient is asked to perform phoneme potentials (VEMP). With an increase in amplitude and a lower “mmm”The observedvideo presentsappearance ausen-ciaof horizonto-rotary oculonistagmografía nystagmus threshold to evoke the potenciales [19]. Halmagyi et al evaluated the OCU-lar movements in response to low-frequency clicks in patients and healthy control group DCSS. The carrier group had a in all positions look, clockwise (Figure 3). A significant caloric ten times greater than that observed in healthy subjects answer, Conclusiontesting left vestibular hyperreflexia seen as parameter. therefore present this technique as a valid alternative to DCSS The DCSS is a relatively new entity. Most publications give research in patients with dizziness and/or disequilibrium [20]. few details of symptoms, mainly mentioning imbalance, vertigo or oscilloscopy noise-induced pressure or maneuvers. In a Clinical Case series reported by Kaski [21], reports that 89% of patients had Male patient 33 years old, administrative mining. Browse box imbalance, oscilloscopy or dizziness in the presence of intense two years of evolution characterized by hearing loss and tinnitus pulsatile autophony left ear. In the past eight months imbalance noise, cry baby, cry or dental strawberry, expanding clinical and vertigo goal by issuing the phoneme “mmm” is added. The presentationexternal sounds of DCSS. of sudden A third onset, of them such had as Tullio telephone, phenomenon traffic review highlights otoscopia standard; Weber test lateralized to the with his own voice when speaking or making buzz. Changes in left ear. Audiometry, hearing loss presents driving left ear upward curve and a gap of 40 dB (125-250-500 Hz), bone conduction symptoms in 25% of subjects. In addition, patients often reported -10dB (Figure 1). With clinical and audiological suspected DCSS hearingintracranial unusual pressure phenomena by coughing, related nose to body blowing, sounds flying, as heard causing his VEMP is requested stimulus Burst 500 Hz, which has a threshold footsteps, his muscles when chewing or eye movements. Pulsatile of p13 waves and n23 to 65 dB nHL and asymmetry in the tinnitus was a frequent complaint. It is important to consider amplitudes of the p13 waves and n23 to 90 dB NHL (Figure 2). diagnosis between benign paroxysmal positional vertigo (BPPV) andthe vertigoDCSS, as that they occurs will bein triggeredthe dental differently. office, and The the intensitydifferential of the sound generated by the turbine reaches the inner ear by bone conduction, it is transmitted by solid media, such as the skull, reaches the ear of a patient with DCSS Tullio phenomenon it occurs becausewith enormous above 95efficiency dB SPL and to trigger maximum a precise speed. rotational However, vertigo.when it All these inconveniences previously mentioned are caused by low frequency sounds (0.5-2 kHz). The patient reported, particularly referring symptoms when issuing the phoneme “mmm” which because the phonatory tube with a frequency of about 110 Hz is partiallygenerates occluded, pressure changesbut includes as well harmonics as a retro reflectedand other resonance, complex frequencies noise. Given the nature of the problem, look for abnormal eye movements against exposure to sound stimuli, inducing positive pressure and/or negative in the CAE during impedance or performing Valsalva maneuver against glottis open and closed glottis. Increased intracranial pressure with the Valsalva maneuver and closed glottis, is transmitted in the CSS, Figure 1: Conductive hearing loss left ear, rising. And GAP 40 dB. , through the meninges and the perilymph of the aqueduct coclear Bone conduction -10 dB. [21], generating eye movements in the same plane but opposite Pathophysiologically the “third window” causes a low-frequency conductivedirection (ampulípeta) hearing loss tois theaccompanied observed byduring thresholds the loud via noise. bone allowing us to exclude the otosclerosis as a differential diagnosis. normal or even lower than 0 dB, with a normal acoustic reflex, however, the presence of a DCSS generate a vibration of the skullBone thatconduction may cause humans the oscillation is less efficient of the durathan overair conduction, the defect, attenuation,leading to the causing creation stimulation of standing of waves hair cells and amplificationin the cochlea sound, at the appropriatewhich is transmitted frequencies. directly This explainsto the inner the symptomsear fluids ofwith tinnitus little and pulsatile [22] autophony. Figure 2: Asymmetry
Load more

Recommended publications
  • Transient Vertigo with Horizontal Nystagmus to Loud Noise and Pressure: Utricular Hydrops Or Vestibular Atelectasis?
    J Int Adv Otol 2020; 16(1): 127-9 • DOI: 10.5152/iao.2019.6283 Case Report Transient Vertigo with Horizontal Nystagmus to Loud Noise and Pressure: Utricular Hydrops or Vestibular Atelectasis? Fatemeh Hassannia , Simon D. Carr , John A. Rutka Department of Otolaryngology, Head and Neck Surgery, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Canada ORCID IDs of the authors: F.R. 0000-0002-4318-8467; S.C. 0000-0001-8863-8022; F.H. 0000-0003-2358-9738. Cite this article as: Hassannia F, Carr SD, Rutka JA. Transient Vertigo with Horizontal Nystagmus to Loud Noise and Pressure: Utricular Hydrops or Vestibular Atelectasis? J Int Adv Otol 2020; 16(1): 127-9. We present an unusual case of a patient with a positive Tullio phenomenon, brief Valsalva-induced transient horizontal nystagmus, reduced left caloric response, and bilateral vestibulo-ocular reflex loss. This study discusses the pathophysiology and differential diagnosis concerning the suspected pathology for the phenomenon of utricular hydrops or vestibular atelectasis and presents a literature review. KEYWORDS: Horizontal nystagmus, hydrops, utricle INTRODUCTION The Tullio phenomenon consistent with sound-induced vertigo and the corresponding evoked eye movement was first described in 1929 by the Italian biologist Pietro Tullio [1]. Since then, clinical studies have identified this phenomenon in several diseases, such as congenital deafness, Meniere’s disease, perilymphatic fistula, head trauma, and superior canal dehiscence syndrome. Different theories have been proposed to explain the mechanism underlying this phenomenon. The close anatomical relationship between stapes footplate and saccule seems to play a great role. This case report presents a patient with a positive Tullio phe- nomenon, valsalva induced horizontal nystagmus, bilateral vestibule-ocular reflex (VOR) loss and a reduced caloric response.
    [Show full text]
  • Headmirror's ENT in a Nutshell Superior Semicircular Canal Dehiscence Expert: Neil Patel, MD Presentation (0:23)
    Headmirror’s ENT in a Nutshell Superior Semicircular Canal Dehiscence Expert: Neil Patel, MD Presentation (0:23) - Symptoms o Autophony – hearing one’s voice in their ear (most common) o Somatosounds – hearing eyes move or feet hit the ground when you walk (second most common) o Pulsatile tinnititus o Vertigo with loud sounds (Tullio phenomenon) or pressure (Hennebert sign) o Nonspecific symptoms: Brain fog, chronic imbalance, mood and learning deficits - Physical examination o Normal otoscopy o Tuning fork (128 or 256 Hz) on lateral malleolus can demonstrate suprathreshold bone conduction o Pneumatic otoscopy can trigger vertigo - Differential diagnosis o Otosclerosis o Meniere’s disease o Syphilis o Spontaneous perilymphatic fistula o Migraine associated vertigo or vestibular migraine o Patulous eustachian tube dysfunction Pathophysiology (2:48) - Bony dehiscence over the superior semicircular canal leading to third window of the inner ear o Can be due to congenital defect or elevated ICP o 3rd window phenomenon . Typically oval window and round window . Sound energy through 3rd window creates conductive hearing loss Workup (5:30) - Imaging o Plain non-contrast temporal bone CT scan . Stenver view – perpendicular cuts to the SSC . Poschl view– parallel cuts to the SSC - Vestibular testing o Determines if the dehiscence is functional o For bilateral SSCD on imaging, it can provide ear specific functional information o Vestibular evoked myogenic potentials (VEMPs) . Cervical VEMPs (cVEMP)– saccule . Occular VEMPs (oVEMP) – utricle (shares
    [Show full text]
  • Redalyc.Síndrome Da Deiscência Do Canal Semicircular Superior
    Revista CEFAC ISSN: 1516-1846 [email protected] Instituto Cefac Brasil Figueiredo de Godoy, Carolina Calsolari; Erhrdt Wiggers Ávila, Kelle Cristine; Neves de Andrade, Adriana; Gil, Daniela Síndrome da deiscência do canal semicircular superior: relato de dois casos Revista CEFAC, vol. 19, núm. 1, enero-febrero, 2017, pp. 119-125 Instituto Cefac São Paulo, Brasil Available in: http://www.redalyc.org/articulo.oa?id=169350110015 How to cite Complete issue Scientific Information System More information about this article Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Journal's homepage in redalyc.org Non-profit academic project, developed under the open access initiative Rev. CEFAC. 2017 Jan-Fev; 19(1):119-125 doi: 10.1590/1982-0216201719112016 Case reports Semicircular superior canal dehiscence: cases reports Síndrome da deiscência do canal semicircular superior: relato de dois casos Carolina Calsolari Figueiredo de Godoy(1) Kelle Cristine Erhrdt Wiggers Ávila(1) Adriana Neves de Andrade(1) Daniela Gil(1) (1) Universidade Federal de São Paulo - São ABSTRACT Paulo/SP Brasil The Superior Semicircular Canal Dehiscence Syndrome (SSCDS) is characterized by bone wear layer overlying the superior semicircular canal. Common symptoms of SSCDS the presence of vertigo asso- Conflict of interest: non-existent ciated with nystagmus induced by intense sound stimuli or changes in intracranial pressure or middle ear. The aim of this study is to describe the audiological and vestibular findings of two patients diagnosed with Superior Semicircular Canal Deiscence Syndrome, with confirmed diagnosis by computed tomo- graphy. Meatoscopy, anamnesis, pure tone audiometry and vocal followed by the acoustic impedance measurements, audiometric Weber, research Tulio phenomenon and Valsalva maneuver, performed by the same researcher in one session were held.
    [Show full text]
  • Click-Evoked Vestibular Activation in the Tullio Phenomenon
    15381Journal ofNeurology, Neurosurgery, and Psychiatry 1994;57:1538-1540 SHORT REPORT Click-evoked vestibular activation in the Tullio phenomenon J G Colebatch, J C Rothwell, A Bronstein, H Ludman Abstract Subjects and methods Click-evoked vestibulocollic reflexes CASE HISTORY were studied in a patient with a unilat- The patient, a 55 year old woman, reported eral Tullio phenomenon (sound induced that she had experienced left retroauricular vestibular symptoms) and the findings pain and impaired balance after a series of were compared with those of a group of forceful sneezes six years previously. Pressure normal subjects. Compared with normal over the mastoid area or loud sounds made subjects, the reflexes elicited from her her feel unsteady and veer to the left, and also symptomatic side were large and had an made her retroauricular pain worse. abnormally low threshold, but retained Associated with these symptoms was the illu- a normal waveform. The click-evoked sion of objects "swimming" in front of her. responses in this patient show changes Clinical examination of her eye movements as consistent with her symptomatology and well as the remainder of the neurological are indicative of a pathological increase examination were normal. The Hallpike in the normal vestibular sensitivity to manoeuvre was negative bilaterally. Loud sound. tones (1 kHz, 110 dB ISO) presented to the left ear through earphones caused visible nys- (7 Neurol Neurosurg Psychiatry 1994;57: 1538-1540) tagmus. This consisted mainly of conjugate torsional left beating and down beating com- ponents.11 Similar loud sounds presented to Patients with the Tullio phenomenon experi- the right ear did not cause nystagmus.
    [Show full text]
  • Vestibular Evoked Potentials: Properties and Clinical Applications of Extraocular Reflexes
    Vestibular evoked potentials: Properties and clinical applications of extraocular reflexes. Sally Marie Rosengren A thesis submitted in fulfilment of the requirements for the degree of Doctor of Philosophy Faculty of Medicine, University of New South Wales, Australia © August 2008 i Originality statement ‘I hereby declare that this submission is my own work and to the best of my knowledge it contains no materials previously published or written by another person, or substantial proportions of material which have been accepted for the award of any other degree or diploma at UNSW or any other educational institution, except where due acknowledgement is made in the thesis. Any contribution made to the research by others, with whom I have worked at UNSW or elsewhere, is explicitly acknowledged in the thesis. I also declare that the intellectual content of this thesis is the product of my own work, except to the extent that assistance from others in the project's design and conception or in style, presentation and linguistic expression is acknowledged.’ Signed …………………………………………….......... Date …………………………………………….............. ii Copyright statement ‘I hereby grant the University of New South Wales or its agents the right to archive and to make available my thesis or dissertation in whole or part in the University libraries in all forms of media, now or here after known, subject to the provisions of the Copyright Act 1968. I retain all proprietary rights, such as patent rights. I also retain the right to use in future works (such as articles or books) all or part of this thesis or dissertation. I also authorise University Microfilms to use the 350 word abstract of my thesis in Dissertation Abstract International (this is applicable to doctoral theses only).
    [Show full text]
  • LETTERS Medications
    1362 J Neurol Neurosurg Psychiatry 2004;75:1362–1366 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2003.029041 on 16 August 2004. Downloaded from more dystonia in his hands while on his have been identified and hydroxygluaric LETTERS medications. aciduria has been proposed to have an His previous diagnostic evaluation autosomal recessive inheritence, occurring Dystonia, tremor, and included serological testing that had revealed in both D and L isoforms. Most of the cases a normal thyroid stimulating hormone con- reported in the literature are identified in parkinsonism in a 54 year old centration, normal liver function tests, nor- early childhood. The D isoform usually man with 2-hydroxyglutaric mal ammonia concentration, and normal presents with neonatal or early infantile aciduria reactive plasma reagent. Head magnetic encephalopathy, seizures, extrapyramidal resonance imaging (MRI) had been per- symptoms, and cardiomyopathy.34 In con- Glutaric aciduria is often considered to be a formed one year before presentation and the trast, the L isoform may present within the rapidly progressing dementing illness with flair images revealed prominent white matter 1st years of life with non-specific motor only occasional extrapyramidal symptoms, 1–7 hyperintensities in the frontal and parietal delay, and later cognitive and motor devel- usually described as dystonia. We present regions, with posterior ex vacuo dilatation of opmental delay. Gait ataxia may occur as a case of late onset 2-hydroxygluaric aciduria his lateral ventricles (fig 1), and mild early as the 2nd year.5 Additional findings and slowly progressive dystonia and parkin- cerebellar atrophy. Urine organic acids were sonism.
    [Show full text]
  • Dizziness in the Outpatient Care Setting
    Review Article Address correspondence to Dr Terry D. Fife, Barrow Dizziness in the Neurological Institute, 240 West Thomas Rd, Suite 301, Phoenix, AZ 85013, Outpatient Care Setting [email protected]. Relationship Disclosure: Terry D. Fife, MD, FAAN Dr Fife serves on the editorial boards of Barrow Quarterly and Neurology. Unlabeled Use of ABSTRACT Products/Investigational Purpose of Review: This article summarizes an approach to evaluating dizziness for Use Disclosure: Dr Fife discusses the the general neurologist and reviews common and important causes of dizziness unlabeled/investigational and vertigo. use of acetazolamide, Recent Findings: Improved methods of diagnosing patients with vertigo and venlafaxine, and zonisamide for the treatment of dizziness have been evolving, including additional diagnostic criteria and charac- vestibular migraine. terization of some common conditions that cause dizziness (eg, vestibular migraine, * 2017 American Academy benign paroxysmal positional vertigo, chronic subjective dizziness). Other uncommon of Neurology. causes of dizziness (eg, superior canal dehiscence syndrome, episodic ataxia type 2) have also been better clarified. Distinguishing between central and peripheral causes of vertigo can be accomplished reliably through history and examination, but imaging techniques have further added to accuracy. What has not changed is the necessity of obtaining a basic history of the patient’s symptoms to narrow the list of possible causes. Summary: Dizziness and vertigo are extremely common symptoms that also affect function at home and at work. Improvements in the diagnosis and management of the syndromes that cause dizziness and vertigo will enhance patient care and cost efficiencies in a health care system with limited resources. Clinicians who evaluate patients with dizziness will serve their patient population well by continuing to manage patients with well-focused workup and attentive care.
    [Show full text]
  • Use of Sound Therapy in Management of Tullio Phenomenon Associated with Unilateral Endolymphatic Hydrops
    International Journal of Otorhinolaryngology and Head and Neck Surgery Mustafa MWM et al. Int J Otorhinolaryngol Head Neck Surg. 2020 Oct;6(10):1765-1767 http://www.ijorl.com pISSN 2454-5929 | eISSN 2454-5937 DOI: http://dx.doi.org/10.18203/issn.2454-5929.ijohns20204176 Original Research Article Use of sound therapy in management of tullio phenomenon associated with unilateral endolymphatic hydrops Mohamed Wael M. Mustafa*, Aida A. Abdelmaksoud Department of Otorhinolaryngology, Qena Faculty of Medicine, South Valley University, Qena, Egypt Received: 14 July 2020 Revised: 19 August 2020 Accepted: 31 August 2020 *Correspondence: Dr. Mohamed Wael M. Mustafa, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Background: The tullio phenomenon consists of the production of vestibular signs or symptoms by an acoustic stimulus. TP was found to be present in many inner ear pathologies such as superior semicircular canal dehiscence, perilymphatic fistula and endolymphatic hydrops. No previous study probed the value of tinnitus maskers in the alleviation of Tullio phenomenon associated with endolymphatic hydrops. Methods: Twenty one patients who had a confirmed unilateral endolymphatic hydrops associated with Tullio phenomenon were selected for the study. Their age ranged from 20 to 52 years old. Tinnitus masker±amplification was used according to the audiological findings of each patient. The device was used in the affected ear for a minimum of 6 months.
    [Show full text]
  • Superior Semicircular Canal Dehiscence Syndrome As Diagnosis of Vestibular Pathology; Diagnosis and Treatment: Review of the Literature
    Journal of Otolaryngology-ENT Research Review Article Open Access Superior semicircular canal dehiscence syndrome as diagnosis of vestibular pathology; diagnosis and treatment: review of the literature Abstract Volume 6 Issue 3 - 2017 Introduction: Superior Semicircular Canal Dehiscence Syndrome is a pathological entity 1 2 that causes incapacitating auditory and vestibular symptoms. It is common that these Valdés-Pons R, Palomino Oquendo JC, 1 1 patients are erroneously diagnosed with simulators or psychiatric diseases, or with any Eiroa Breijo A, Pazo Irazu S, Martínez Egido 1 1 other vestibular pathologies. I, Santamaría Castro ML, González Prado A1 Etiology: It seems to have a genetic predisposition supported in a higher frequency of 1Department of Head and Neck Surgery Otolaryngology Semicircular Canal Dehiscence and bone defects in the tegmen tympani in patients with Hospital Povisa, Spain DFNA 9 (gen COCH). In other cases there is no specific cause justifying communication 2Department of Neurosurgery, Spain between the SSC and cranial cavity. Over the years, and the progressive thinning of the bone, the communication between membranous labyrinthine and cranial cavity, will occur. Correspondence: Valdés-Pons R, Head of Department of Otolaryngology, Head and Neck Surgery, Hospital Povisa, Vigo, Pathophysiology: Superior Semicircular Canal Dehiscence Syndrome is characterized by Spain, Email thinning or loss of bone that covers the SSC and which separates it from of the cranial cavity. A pathologic third window on the vestibular side
    [Show full text]
  • Inner-Ear Disorders Presenting with Air–Bone Gaps: a Review
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Archivio della ricerca- Università di Roma La Sapienza J Int Adv Otol 2020; 16(1): 111-6 • DOI: 10.5152/iao.2020.7764 Review Inner-Ear Disorders Presenting with Air–Bone Gaps: A Review Alfonso Scarpa , Massimo Ralli , Claudia Cassandro , Federico Maria Gioacchini , Antonio Greco , Arianna Di Stadio , Matteo Cavaliere, Donato Troisi, Marco de Vincentiis , Ettore Cassandro Department of Medicine and Surgery, University of Salerno, Salerno, Italy (AS, DT, EC) Department of Sense Organs, Sapienza University of Rome, Rome, Italy (MR, AG, MdV) Department of Surgical Sciences, University of Turin, Turin, Italy (CC) Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy (FMG) Department of Otolaryngology, University of Perugia, Perugia, Italy (ADS) Department of Otorhinolaryngology, University Hospital 'San Giovanni di Dio e Ruggi d'Aragona', Salerno, Italy (MC) ORCID IDs of the authors: A.S. 0000-0001-9219-6175; M.R. 0000-0001-8776-0421; C.C. 0000-0003-4179-9181; F.M.G. 0000-0002-1148-4384; A.G. 0000-0002-4824-9871; A.D.S. 0000-0001-5510-3814; M. d.V. 0000-0002-1990-7820; E.C. 0000-0003-1757-6543. Cite this article as: Scarpa A, Ralli M, Cassandro C, Gioacchini FM, Greco A, Di Stadio A, et al. Inner-Ear Disorders Presenting with Air–Bone Gaps: A Review. J Int Adv Otol 2020; 16(1): 111-6. Air–bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media.
    [Show full text]
  • Vestibular Hypersensitivity to Clicks Is Characteristic of the Tullio Phenomenon
    670 J Neurol Neurosurg Psychiatry 1998;65:670–678 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.5.670 on 1 November 1998. Downloaded from Vestibular hypersensitivity to clicks is characteristic of the Tullio phenomenon J G Colebatch, B L Day, A M Bronstein, R A Davies, M A Gresty, L M Luxon, J C Rothwell Abstract sound and it is only when extremely loud Objectives—The frequency of pathologi- sounds are encountered, such as are achieved cally reduced click thresholds for vestibu- near jet engines or explosions, that there is evi- lar activation was explored in patients dence of vestibular activation.1–3 Various dis- with the Tullio phenomenon (sound in- ease states have been reported to underlie the duced vestibular activation). pathological sound sensitivity that occurs in the Methods—Seven patients (eight aVected Tullio phenomenon, including dislocation of ears) with symptoms of oscillopsia and the stapes footplate, labyrinthine fistulas, en- unsteadiness in response to loud external dolymphatic hydrops, and dehiscence of the sounds or to the patient’s own voice were superior semicircular canal.4–7 examined. In all but one patient, vestibu- We have recently reported a patient with the lar hypersensitivity to sound was con- Tullio phenomenon in whom there was a firmed by the fact that eye movements pathological increase in her sensitivity to a new 8 could be produced by pure tones of 110 dB test of vestibular function using clicks. This intensity or less. Conventional diagnostic finding is of particular importance for various imaging was normal in all cases and three reasons, not least being that conventional tests of the patients had normal middle ears at of vestibular function in these patients show no surgical exploration.
    [Show full text]
  • Superior Semicircular Canal Dehiscence: Transmastoid Obliteration
    HOW I DO IT Superior semicircular canal dehiscence: transmastoid obliteration BY THOMAS MILNER, GEORGIOS KONTORINIS Sometimes patients with dizziness turn out to have slightly more unusual causes of their problem. Thomas Milner and Georgios Kontorinis describe their technique for managing patients who have a diagnosis of superior canal dehiscence as an identified cause of their dizziness. uperior semicircular canal dehiscence (SSCD) is a defect in the integrity of the bony labyrinth Sof the superior semicircular canal resulting in a third window effect. SSCD was first described by Minor et al, who presented a case series of patients with noise or pressure-induced vertigo, associated with a computed tomography (CT) scan demonstrating bony dehiscence overlying the superior semicircular canal [1]. Patients typically present with vertigo induced by loud noises, or manoeuvres that alter middle ear pressure (e.g. sneezing). Occasionally a more chronic vertigo or disequilibrium can develop. During otological examination vertical torsional nystagmus can be elicited by applying tragal pressure (Hennebert’s sign) or exposure to loud noises (Tullio phenomenon). Audiological manifestations include Figure 1. CT scans of a few of the patients with SSCD showing Figure 2. cVEMPS recorded at a level of 70dB (A), six-canal vHIT coronal (A, B, C and D) and sagittal and parasagittal oblique (B and b) and pure-tone audiogram showing low frequency low frequency conductive hearing loss, (b, c and d) sections of the temporal bone. The dehiscence air-bone gap (C) in patients with SSCD. Of note, the six-canal autophony and hypersensitivity to bone is marked with white arrows and its extent can vary; in A the vHIT can show abnormal responses from not necessarily the defect is bilateral.
    [Show full text]