X's indicate examined; arrow, present patient. [doi.org]

In the family, phenotypic variability is present. [ncbi.nlm.nih.gov]

Oscillopsia or blurred vision while reading, in lateral gaze, or with head motion can be a presenting complaint (1012,1013). [neuroophthalmology.ca]

It is present at birth and is non-progressive. [chargesyndrome.org.uk]

These are only used for the purpose of ruling out diseases with similar presentations. Whipple disease, for example, has a similar presentation and can be diagnosed using polymerase chain reaction. [eyewiki.aao.org]

• Asymptomatic

  RESULTS: A 50-year-old man had had progressive sensorineural hearing loss and dysequilibrium for 15 years; he had been asymptomatic at the age of 35 years. [ncbi.nlm.nih.gov]

  Results A 50-year-old man had had progressive sensorineural hearing loss and dysequilibrium for 15 years; he had been asymptomatic at the age of 35 years. [doi.org]

  The risk factors for the tumor include the presence of NF2 and a positive family history of schwannoma Vestibular Schwannomas may be symptomatic or asymptomatic. [dovemed.com]

  Type I ACM which is asymptomatic or with only mild dysfunction can be observed without treatment. Posterior fossa decompression via a number of approaches is used for symptomatic ACM, with shunting when hydrocephalus is present. [neuroophthalmology.ca]

  Vestibular evoked myogenic potentials (VEMP) can detect asymptomatic saccular hydrops. Laryngoscope (2006) 116 (6):987–92. doi:10.1097/01.mlg.0000216815.75512.03 PubMed Abstract | CrossRef Full Text | Google Scholar 39. [frontiersin.org]

• Difficulty Walking

  Symptoms may be chronic or acute onset (as early as one to three days) and may include: Difficulty in low-light areas Difficulty walking over carpet or unleveled surfaces Disequilibrium Dizziness Ear fullness Ear ringing Imbalance Light-headedness Motion [centrahealth.com]

  (MeSH) Gait Apraxia : difficulty in walking. (OxfordMed) Impaired ambulation not attributed to sensory impairment or motor weakness. [richardsonthebrain.com]

• Macula

  It is suggested that the mucopolysaccharide deposit could cause strangulation of nerve endings.The maculas and crista ampullaris are what allow for non-visual sensation of head movements. [en.wikipedia.org]

  Khetarpal et al 3 discovered an acidophyllic mucopolysaccharide-containing substance, especially in cochleas, maculas, and cristae of patients with DFNA9, as well as severe degeneration of vestibular and cochlear sensory axons and dendrites. [doi.org]

  Age-related blood flow and capillary changes in the rat utricular macula ... JARO 03:167-173, 2001 Poretti, A., et al. (2013). "Vestibular impairment in patients with Charcot-Marie-tooth disease." Neurology 80(23): 2099-2105. Rinne T, and others. [tchain.com]

  Their vestibular loss arises from an acidophilic mucopolysaccharide deposit, identified in the cochlea and macula, that causes strangulation of the nerve endings (Huygen et al. 1989, 1991 ; Verhagen et al. 2000 ; Cremers et al., 2005 ; Robertson et al [physreports.physiology.org]

• Progressive Hearing Loss

  Sensorineural hearing loss is usually congenital, severe to profound and non-progressive. However, hearing loss may be later onset and progressive in some patients. [asperbio.com]

  All affected families showed a similar progressive hearing loss and vestibular dysfunction. [nature.com]

  The subtype IIA, however, may demonstrate progressive hearing loss not found in the other Type II expressions. These children may eventually need a cochlear implant as their hearing loss progresses. [audiologyonline.com]

  Hearing loss can be fluctuating and is progressive in 15%–20% of the cases. [asha.org]

  Gazquez I, Moreno A, Aran I et al. (2012) MICA‐STR A.4 is associated with slower hearing loss progression in patients with Ménière's disease. Ontology & Neurotology 33(2): 223–229. [els.net]

• Fluctuating Hearing Loss

  Our patient showed progressive sensorineural hearing loss with at least 1 instance of (partial) threshold fluctuation. Vestibular function showed some fluctuation between the ages of 35 and 38 years, but without complaints. [doi.org]

  Hearing loss can be fluctuating and is progressive in 15%–20% of the cases. [asha.org]

  LVAS is typically bilateral and almost always leads to some degree of progressive or fluctuating hearing loss. Although LVAS is a congenital condition, hearing loss may not be present from birth. [dallashear.com]

• Benign Paroxysmal Positional Vertigo

  Patients who are typically referred for vestibular rehabilitation therapy are those diagnosed with dizziness, imbalance, vertigo, Meniere’s syndrome, benign paroxysmal positional vertigo (BPPV), neck-related dizziness and migraines. [centrahealth.com]

  […] of the vestibular nerve, usually caused by viral infection Benign positional paroxysmal vertigo (benign positional vertigo) Transient episodes of vertigo caused by stimulation of vestibular sense organs by canalith; affects middle-age and older patients [aafp.org]

  In 1995, we saw the patient again because of benign paroxysmal positional vertigo during positioning on the left side, which was confirmed by a positive Dix-Hallpike test. [doi.org]

• Blurred Vision

  Patients report feelings of vague dissiness, blurred vision, dysequilibrium in the dark, and progressive hearing impairment. [en.wikipedia.org]

  SEVERAL YEARS ago, we described a family with autosomal dominant progressive vestibulocochlear dysfunction resulting in sensorineural hearing loss and vestibular areflexia. 1, 2 The patients reported vague dizziness, blurred vision, dysequilibrium in [doi.org]

  (scotoma) - Double vision (diplopia) - Blurred vision - Nystagmus - Ocular pain - Sudden loss of vision with pain in or behind the eye is (caused by optic neuritis) - These early signs may subside after 3-6 weeks without any residual deficit - For others [quizlet.com]

  Oscillopsia or blurred vision while reading, in lateral gaze, or with head motion can be a presenting complaint (1012,1013). [neuroophthalmology.ca]

  Refractive errors cause blurred vision and are the most common reason why a person goes to see an eye professional. [richardsonthebrain.com]

• Oscillopsia

  Early in 1998, at the age of 50 years, the patient had another episode of vertigo, nausea, and vomiting, combined with head movement–dependent oscillopsia. [doi.org]

  He suffered from instability in the dark, head movement-dependent oscillopsia, paroxysmal positional vertigo, and vertigo with and without nausea. Hearing impairment started unilaterally, predominantly in the high frequencies. [ncbi.nlm.nih.gov]

  Reported symptoms include: Sensorineural hearing loss Vestibular areflexia Hearing impairment Vertigo Nausea and vomiting Head movement-dependent oscillopsia The disease is an inherited autosomal dominant disease, but the physiological cause of the dysfunction [en.wikipedia.org]

  […] or acute onset (as early as one to three days) and may include: Difficulty in low-light areas Difficulty walking over carpet or unleveled surfaces Disequilibrium Dizziness Ear fullness Ear ringing Imbalance Light-headedness Motion sensitivity Nausea Oscillopsia [centrahealth.com]

  Oscillopsia or blurred vision while reading, in lateral gaze, or with head motion can be a presenting complaint (1012,1013). [neuroophthalmology.ca]

• Hyporeflexia

  Vestibular testing showed hyporeflexia with unilateral caloric weakness of the left labyrinth (slow-phase velocity at culmination of caloric nystagmus response of 15°/s to 30°C stimulation and 5°/s to 44°C), combined with a hyperactive response to hot-water [doi.org]

[…] patients with PSP show the following abnormalities, although these are not specific for PSP: Diminished total sleep time Increased awakenings Progressive loss of rapid-eye-movement (REM) sleep [7, 8] Decreased REM–to–non-REM (NREM) quotient [9, 10] See Workup [emedicine.medscape.com]

"I came to the Jamerson Family YMCA for treatment of vertigo. My treatment was totally successful! After my treatment with Jessica, I am no longer experiencing dizziness when lying on my back or tilting my head back. [centrahealth.com]

Treatment measures for Vestibular Schwannoma may depend upon the type and location of the tumor. In many cases, if the tumor is small-sized and there are no symptoms, no treatment may be necessary. [dovemed.com]

Removal of the clot is the usual treatment. [britannica.com]

Treatment of acute otitis media typically includes antibiotics and sometimes additional medications prescribed by the physician. [dallashear.com]

[…] muzzy’ head causing poor cognitive function (a most distressing symptom when trying to work or study for an exam) Patients can often suffer anxiety and depression as a result of enduring some or all the preceding symptoms without clear diagnosis and treatment [migraine.ie]

What is the Prognosis of Vestibular Schwannoma? (Outcomes/Resolutions) The prognosis of Vestibular Schwannoma (benign tumor) is excellent, if the tumor can be removed without damaging the underlying nerve. [dovemed.com]

The prognosis for patients with PSP is poor, with the average time from onset of symptoms to death ranging from 5-9 years. [eyewiki.aao.org]

The affected cohort in Golbe’s study consisted entirely of white persons; however, the survey population included only 5.7% black individuals, thus preventing any meaningful analysis regarding race. [22] Prognosis Studies of cohort patients dying under [emedicine.medscape.com]

Prognosis in bilateral vestibular hypofunction. Laryngoscope, 109, 1999, 35-41. 35 patient records were reviewed Hain TC, Cherchi M, and Yacovino DA “ Bilateral vestibular weakness. [tchain.com]

Distribution of certainty of etiology. A definite etiology was found in 47% of the BVH patients, a probable etiology in 22% of the BVH patients and in 31% the etiology of BVH remained idiopathic. Figure 2. [frontiersin.org]

When PS and nonsydromal LVA are considered part of the same disease spectrum, the prevalence of this disorder as an etiology for congenital deafness is 5.5% (Smith & Van Camp, 2006). [asha.org]

Do not use laboratory tests to initially identify the etiology of dizziness. C 10 Laboratory tests identify the etiology of vertigo in less than 1 percent of patients with dizziness. [aafp.org]

viruses, and environmental factors) - Etiology of PNDs - This is suspected because these diseases are more prevalent among families and certain racial groups - Etiology of PNDs - These and their autoimmune response also may be involved as an external [quizlet.com]

(Etiology) The exact cause and mechanism of Vestibular Schwannoma formation, in a majority of cases, is unknown; they are known to be the result of sporadic mutations. This implies that they do not have a preceding family history of the condition. [dovemed.com]

Authored by 60 internationally recognized researchers, the book describes the normal development of the ear, updates the classification and epidemiology of hearing loss, and surveys the usage of audiometric tests and diagnostic medical examinations. [books.google.com]

Epidemiology The incidence is about 1 in 25,000. About 3-6% of hearing impaired children have the condition. Types 1 and 2 are more common than type 3 and together account for 90-95% of Usher's syndrome and about 10% of all children born deaf. [patient.info]

To date, there have been only a few epidemiologic studies investigating PSP associations. [emedicine.medscape.com]

Ruano L, Melo C, Silva MC and Coutinho P (2014) The global epidemiology of hereditary ataxia and spastic paraplegia: a systematic review of prevalence studies. Neuroepidemiology 42(3): 174–183. [els.net]

The role of heredity in the pathophysiology of PSP remains elusive. Although there are anecdotal reports in the literature that describe apparent familial PSP, several larger series have not noted this association. [emedicine.medscape.com]

In addition there must be a low to high density in at least three of the following structures: oculomotor complex, dentate nucleus, striatum, or medulla.[16, 33] Pathophysiology As stated previously, PSP is a known tauopathy. [eyewiki.aao.org]

This may be related to the underlying pathophysiology of demyelination. Presumably there are many other cases like this involving other hereditary as well as acquired neuropathic illnesses. [tchain.com]

Tubes can restore hearing to normal, prevent fluid from recurring, decrease the number of acute ear infections, and prevent damage to the middle ear bones as well as other more serious ear complications. [dallashear.com]

Resources About Genetic Hearing Loss from Around the Web "A Parent's Guide to Genetics of Hearing Loss" - Center for Disease Control and Prevention (CDC) Hereditary Hearing Loss Homepage Medscape Reference Genetic Sensorineural Hearing Loss [babyhearing.org]

[…] devices or cochlear implants [ 9 ] (which appear to enhance the quality of life) [ 10 ] Orientation and mobility training Communication services and independent living training that may include learning Braille, low vision services, or auditory training Prevention [patient.info]

Many ALS patients can live longer and more productive lives because of current research into the cause, prevention and cure for the disease. Improvements in medical management, including nutrition and breathing, regularly increase patient survival. [hopkinsmedicine.org]