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Unilateral Deafness with Delayed Endolymphatic Hydrops


Presentation

  • Kerber's current research focus is using health services research approaches to optimize patient care and health care utilization for dizziness presentations.[books.google.com]
  • This case study describes a 48-year-old male who presented with complaints of episodic vertigo and disequilibrium 15 years following the onset of unilateral sensorineural hearing loss.[mayoclinic.pure.elsevier.com]
  • In accordance with histopathologic data, 10% of patients did not have EH in the affected ear, while EH may be present in clinically silent ears of patients with MD.[ajnr.org]
Surgical Procedure
  • If conservative therapies fail to manage symptoms, a surgical procedure is an option, but it is very invasive and exposes the brain in order to repair the damaged bone.(6) 7-SSHL Sudden sensorineural hearing loss (SSHL) occurs infrequently; only 1 in[tinnitusformula.com]
  • Conclusion Several medical approaches and surgical procedures are available for control of vertigo.[michiganear.com]
  • Including the bilateral case, a total of 68 surgical procedures were performed for ipsilateral DEH. Contralateral DEH.[thefreelibrary.com]
  • In this transmastoid surgical procedure, an incision is made behind the ear, and the mastoid bone is entered.[earcentergreensboro.com]
  • Labyrinthectomy A labyrinthectomy is a surgical procedure which destroys the whole inner ear; that is the hearing and vestibular function.[menieres.org.uk]
Chills
  • […] dizzy, unsteady, lightheaded, or faint (9) derealization (feelings of unreality) or depersonalization (being detached from oneself) (10) fear of losing control or going crazy (11) fear of dying (12) paresthesias (numbness or tingling sensations) (13) chills[books.google.com]
Italian
  • Edited by recognised Italian otolaryngologists, this textbook has been written by authors (many from the US) from multidisciplinary backgrounds including otolaryngology, neurology, anatomy, physiology, epidemiology and surgery.[books.google.com]
Euthyroid
  • Giuseppe Chiarella, Sara Tognini, Andrea Nacci, Roberta Sieli, Giuseppe Costante, Claudio Petrolo, Valentina Mancini, Pietro Hiram Guzzi, Giuseppe Pasqualetti, Ettore Cassandro, Bruno Fattori, Diego Russo and Fabio Monzani, Vestibular disorders in euthyroid[doi.org]
Pathologist
  • Schuknecht was a pathologist. Thus a little hard to be sure by this definition -- unless you are already dead.[dizziness-and-balance.com]
Chest Pain
  • During these attacks there are symptoms such as shortness of breath or smothering sensations; palpitations, pounding heart, or accelerated heart rate; chest pain or discomfort; choking; and fear of going crazy or losing control. ‏[books.google.com]
  • It is the third most common symptom presentation (combined with imbalance and falls) for outpatient medical consultation, behind chest pain and fatigue.[clevelandclinicmeded.com]
Palpitations
  • During these attacks there are symptoms such as shortness of breath or smothering sensations; palpitations, pounding heart, or accelerated heart rate; chest pain or discomfort; choking; and fear of going crazy or losing control. ‏[books.google.com]
  • Tachycardia, palpitations, shortness of breath, and presyncope are not limited to disorders of the cardiovascular system.[clevelandclinicmeded.com]
Vision Disorder
  • It can also be caused by problems in certain parts of the brain or vision disorders. This book is a comprehensive guide to the diagnosis and management of vertigo.[books.google.com]
Flushing
  • , lightheaded, or faint (9) derealization (feelings of unreality) or depersonalization (being detached from oneself) (10) fear of losing control or going crazy (11) fear of dying (12) paresthesias (numbness or tingling sensations) (13) chills or hot flushes[books.google.com]
Suggestibility
  • الصفحة 324 - History, physical and neurological examinations do not suggest one of the disorders listed in groups 5-1 1 (2) History and/or physical and/or neurological examinations do suggest such disorder, but it is ruled out by appropriate investigations[books.google.com]
  • These findings suggest that patients with sudden deafness or juvenile unilateral total deafness should undergo caloric testing and VEMP test to predict the occurrence of delayed endolymphatic hydrops in the future.[ncbi.nlm.nih.gov]
  • It is suggested that endolymphatic hydrops is in the better-hearing ear of contralateral DEH.[unboundmedicine.com]
  • They reported that "DEH patients, however, showed significant EH in either the cochlea or vestibule, suggesting that EH development and DEH onset were strongly correlated." More study is needed here.[dizziness-and-balance.com]
  • Although some analytic epidemiologic studies have suggested an association between migraine and Meniere disease, more recent studies have not found an elevated incidence of migraine headache in patients with Meniere syndrome compared to the general population[medlink.com]
Fear
  • […] of losing control or going crazy (11) fear of dying (12) paresthesias (numbness or tingling sensations) (13) chills or hot flushes Table 3.2. ‏[books.google.com]
  • All this fear boils down to the one fear of not being able to cope. When you know how to handle things, they look different. The anxiety doesn’t go away but you know you have some control and this is the key to dealing with fear.[menieres.org.uk]
  • Depression, anxiety, fear, anger and avoidance behaviour are common. People change their lifestyle, develop avoidance behaviour, stop going out shopping and to restaurants and are afraid of driving.[lmhofmeyr.co.za]
  • Patients have a fear of falling while sitting or standing, and this can provoke unpleasant sensations of body acceleration and acceleration of the environment.[clevelandclinicmeded.com]
Anxiety Disorder
  • disorder (eg, in response to being away from home or close... ‏[books.google.com]
  • Anxiety disorders, panic disorders, and phobias can also interact with disorders of balance and dizziness. Dizziness with anxiety and panic disorders are not related exclusively to hyperventilation.[clevelandclinicmeded.com]
  • Agoraphobia Agoraphobia is usually defined as a fear of open spaces, however, agoraphobia is an anxiety disorder which consists of the fear of experiencing a difficult or embarrassing situation from which the sufferer cannot escape; such as situations[menieres.org.uk]
Panic Attacks
Depersonalization
  • Trembling or shaking (4) Sensations of shortness of breath or smothering (5) Feeling of choking (6) Chest pain or discomfort (7) Nausea or abdominal distress (8) Feeling dizzy, unsteady, lightheaded, or faint (9) Derealization (feelings of unreality) or depersonalization[books.google.com]
  • […] clinical evidence for endolymphatic hydrops9 in Lyme disease: blurry vision, tinnitus, transient unilateral deafness, sensation of head pressure, extreme sound sensitivity, agoraphobia, gastrointestinal problems, dizziness, imbalance, nausea, vomiting, depersonalization[lymeneteurope.org]
Vertigo
  • This book is a comprehensive guide to the diagnosis and management of vertigo. Beginning with basic anatomy, physiology and epidemiology of dizziness, the following chapters examine different causes of vertigo, their diagnosis and treatment.[books.google.com]
  • The symptoms include aural fullness, fluctuating hearing, and/or episodes of vertigo similar to those observed in Meniere disease and may occur ipsilateral or contralateral to the previously deafened ear.[ncbi.nlm.nih.gov]
  • This case study describes a 48-year-old male who presented with complaints of episodic vertigo and disequilibrium 15 years following the onset of unilateral sensorineural hearing loss.[mayoclinic.pure.elsevier.com]
  • […] phenomenon - sound-induced vertigo, disequilibrium or dizziness, nystagmus and oscillopsia hyperacusis low frequency conductive deafness[ozemedicine.com]
Ataxia
  • Baloh led to important discoveries about balance disorders in the elderly, inherited and sporadic ataxia syndromes, and oculomotor physiology. Dr.[books.google.com]
  • Similarly, the rotational vertebral artery occlusion syndrome, consisting of recurrent attacks of vertigo (minutes), nystagmus, and ataxia resulting from head rotations compressing the vertebral artery.[neuroweb.us]
  • Otologic symptoms are similar to Meniere’s disease but usually bilateral resulting in ataxia and oscillopsia. Management Treatment is with high dose steroid (prednisone 2mg/kg/day) this might prevent permanent hearing loss if administered early.[otologytextbook.net]
  • Historical note and terminology In 1861 French otologist Prosper Meniere (1799-1862) described a group of 11 patients, mostly young men in their 30s and 40s, with recurrent episodes of vertigo, deafness, and tinnitus with associated nausea, vomiting, gait ataxia[medlink.com]
  • A number of drugs can produce a characteristic drug-intoxication syndrome with disorientation, memory and cognitive deficits, gaze-evoked nystagmus, and gait and extremity ataxia. This can be confused with more serious disorders.[clevelandclinicmeded.com]
Aura
  • No aura symptom lasts more than 60 minutes. If more than one aura symptom is present, accepted duration is proportionally increased. ‏[books.google.com]
  • Ménière's disease vestibular migraine: episodic vertigo in patients with a history of migraines basilar type migraine (BTM) vertigo plus more than one neurologic symptom of putative brainstem or bilateral cortical origin occur as part of a headache aura[ozemedicine.com]
  • Vestibular epilepsy may be vague dizziness or true vertigo that may precede the seizure (aura) or actually be or accompany the seizure event.[clevelandclinicmeded.com]
  • These aura symptoms usually last between 5 and 60 minutes before the headache starts (although there is also a type of migraine where these symptoms can happen without a headache).[menieres.org.uk]
  • Vestibular migraine : The diagnostic criteria are the following: 1) recurrent vestibular vertigo attacks; 2) migraine according to the IHS; 3) migrainous symptoms during at least two vertiginous attacks (migrainous headache, photophobia, phonophobia, or aura[neuroweb.us]
Tingling
  • […] abdominal distress (8) feeling dizzy, unsteady, lightheaded, or faint (9) derealization (feelings of unreality) or depersonalization (being detached from oneself) (10) fear of losing control or going crazy (11) fear of dying (12) paresthesias (numbness or tingling[books.google.com]
  • Symptoms include gradual hearing loss in one ear, tinnitus, aural fullness, balance issues, and eventually facial tingling or numbness, headaches, and clumsiness or confusion.(9) Because these tumors are slow-growing and benign, often the first line of[tinnitusformula.com]
  • […] retropulsion Autonomic symptoms: Nausea, vomiting, diarrhea, diaphoresis, palpitations, presyncope, or syncope General symptoms: Headache, neck pain, neck stiffness, state of hydration or dehydration Neurologic symptoms: Focal weakness, numbness or tingling[clevelandclinicmeded.com]
Cerebellar Ataxia
  • Paraneoplastic cerebellar ataxia Delayed endolymphatic hydrops : Delayed endolymphatic hydrops (DEH) differs from Ménière disease in that it occurs in pre-existing ear pathology in patients who have a profound unilateral or total deafness that was caused[neuroweb.us]
  • Cervical Vertigo Cervical vertigo was first documented by Claude Bernard in 1858.107 Longet and Shift108,109 described gait disorders resembling cerebellar ataxia after severing cervical muscles in several animal species.108,109 There are several theories[michiganear.com]
Hot Flushes

Workup

  • Overview The author discusses the clinical presentation, pathophysiology, diagnostic workup, and management of Meniere syndrome.[medlink.com]
  • […] during surgery to have an intravestibular lipoma. [6]) As is the procedure for assessing other patients with dizziness and vertigo, the evaluation of these 160 patients included (1) a careful history, (2) an otolaryngologic examination, (3) a blood workup[thefreelibrary.com]

Treatment

  • Beginning with basic anatomy, physiology and epidemiology of dizziness, the following chapters examine different causes of vertigo, their diagnosis and treatment.[books.google.com]
  • Finally, the patient will be seen by an otolaryngologist to determine a diagnosis and discuss treatment options. Treatment Options Much research has been done to determine the efficacy of treatment options for patients with Ménière's disease.[asha.org]
  • The case highlights the diagnostic and treatment challenges associated with this condition and focuses attention on principles that guide the audiologist in collecting evidence that aids in solving these challenges.[mayoclinic.pure.elsevier.com]
  • Surgical treatments for Meniere disease began in the 1900s.[medlink.com]
  • Surgical Treatment: Surgical treatment is indicated when a patient is incapacitated with unilateral Meniere's Disease and quality of life is affected.[audiologyonline.com]

Prognosis

  • The prognosis of patients with Ménière disease varies. Periods of remission punctuated by exacerbations of symptoms are typical. [15] Some patients have minimal symptoms, whereas others have severe attacks.[emedicine.medscape.com]
  • Prognosis of Meniere’s disease by conservative treatment: retrospective study on the time course of the disease. Acta Otolaryngol Suppl (1995) 519:216–8. doi:10.3109/00016489509121908 CrossRef Full Text Google Scholar 15.[journal.frontiersin.org]

Etiology

  • The cause of hearing loss can be viral, head injury, meningitis or any other etiology. Patients develop symptoms of Meniere’s disease. If one ear affected then labyrinthectomy gives excellent result.[otologytextbook.net]
  • The etiology of BPV has been a source of controversy.[michiganear.com]
  • In other words, Ménière syndrome is endolymphatic hydrops caused by a specific condition, and Ménière disease is endolymphatic hydrops of unknown etiology (ie, idiopathic endolymphatic hydrops).[emedicine.medscape.com]
  • The clinical results of this study would seem to support the observations of others that DEH and Meniere's disease are related disorders caused predominantly by cases of viral labyrinthitis with unknown etiology.[thefreelibrary.com]
  • […] vestibular symptoms are identical to those of Ménière's disease secondary endolymphatic hydrops secondary to: endocrine abnormalities trauma electrolyte imbalance autoimmune dysfunction parasitic infections hyperlipidemia hypothesized to be a factor, not an etiology[ozemedicine.com]

Epidemiology

  • Beginning with basic anatomy, physiology and epidemiology of dizziness, the following chapters examine different causes of vertigo, their diagnosis and treatment.[books.google.com]
  • Although some analytic epidemiologic studies have suggested an association between migraine and Meniere disease, more recent studies have not found an elevated incidence of migraine headache in patients with Meniere syndrome compared to the general population[medlink.com]
  • Epidemiology In the United States, a prevalence of 1,000 cases of endolymphatic hydrops per 100,000 population is a reasonable approximation, though it is probably an underestimate.[emedicine.medscape.com]
  • Wuhan, Hubei, China Sulin Zhang, Yangming Leng, Bo Liu & Weijia Kong Key Laboratory of Neurological Disorders of Education Ministry, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China Weijia Kong Department of Epidemiology[nature.com]
  • Several epidemiological studies 2,3 reported SSHL incidence rates of 5-30 cases per 100,000 people per year in industrialized countries.[jove.com]
Sex distribution
Age distribution

Pathophysiology

  • Ispilateral DEH and Meniere's disease may show different pathophysiologies. The incidence of dominant negative summating potential in the better-hearing ear was 20% in the ispilateral DEH cases and 60% in the contralateral DEH cases.[unboundmedicine.com]
  • Pathophysiology The pathophysiology of Ménière's disease is not clearly understood.[asha.org]
  • Pathophysiology, etiology, and differential diagnosis of vertigo. UpToDate [online serial]. Waltham, MA: UpToDate; reviewed March 2018.[aetna.com]
  • Schuknecht HF (1976) Pathophysiology of endolymphatic hydrops. Arch Otorhinolaryngol 212: 253–262. PubMed CrossRef Google Scholar 9. Schuknecht HF (1978) Delayed endolymphatic hydrops. Ann Otol Rhinol Laryngol 87: 743–748. PubMed Google Scholar 10.[link.springer.com]
  • The underlying pathophysiologic mechanism for the development of DEH has been explained as progressive endolymphatic hydrops in the inner ear due to delayed atrophy or fibrous obliteration of the endolymphatic resorption system, resulting from a previous[jstage.jst.go.jp]

Prevention

  • One point is the prevention of acoustic trauma.[jove.com]
  • Preventive Services Task Force. Guide to clinical preventive services: report of the U.S. Preventive Services Task Force. 3d ed. Alexandria, Va.: International Medical Pub., 2002. 14. Salley LH Jr, Grimm M, Sismanis A, Spencer RF, Wise CM.[aafp.org]
  • Management Treatment is with high dose steroid (prednisone 2mg/kg/day) this might prevent permanent hearing loss if administered early. Cyclophosphamide can be used for systemic disease. References 27.[otologytextbook.net]
  • • The aims of management of Meniere disease are to (1) reduce the number and severity of acute vertiginous attacks and their associated symptoms (eg, hearing loss and tinnitus), (2) alleviate chronic symptoms (eg, hearing loss and tinnitus), and (3) prevent[medlink.com]
  • Medication to prevent the attacks. Medication to destroy the inner ear function. Devices Devices like the Menniet device are no better than placebo.[lmhofmeyr.co.za]

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