Progressive-sensorineural-deafness: Causes & Reasons

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Possible Causes for progressive sensorineural deafness

Ménière Disease

[39] In 1972, the Academy defined criteria for diagnosing Ménière's disease as:[39] Fluctuating, progressive, sensorineural deafness. [en.wikipedia.org]

With progress of time, permanent sensorineural deafness and tinnitus sets in. Initially, it begins in one ear but over a period of time, both ears are involved. [symptoma.com]

Equilibrium (AAO HNS CHE) set criteria for diagnosing Ménière's, as well as defining two sub-categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness [en.wikipedia.org]

Muckle-Wells Syndrome

Progressive sensorineural deafness, and, in approximately one third of the patients, amyloidosis of the kidneys as well as of other organs may occur. [ncbi.nlm.nih.gov]

Definition A hereditary periodic fever syndrome characterized by fever, chronic recurrent urticaria, arthralgias, progressive sensorineural deafness, and reactive renal amyloidosis [uniprot.org]

CINCA Syndrome

He developed progressive sensorineural deafness and required bilateral prosthesis at the age of 15. He also suffered from mild mental retardation and left school early. [academic.oup.com]

Audiogram – high-frequency hearing loss initially, progressing to bilateral sensorineural deafness. [dermnetnz.org]

Audiogram – high frequency hearing loss initially, progressing to bilateral sensorineural deafness. [dermnetnz.org]

Oculomotor Apraxia

Cerebellum. 2013 Aug;12(4):557-67. doi: 10.1007/s12311-013-0463-1. Author information 1 INSERM U1028; CNRS UMR5292; Lyon Neuroscience Research Center, ImpAct Team, Bron, 69676, France. muriel.panouilleres@inserm.fr Abstract Ataxia with oculomotor apraxia type 2 (AOA2) is one of the most frequent autosomal recessive[…] [ncbi.nlm.nih.gov]

Richards-Rundle Syndrome

sensorineural hearing loss. [cags.org.ae]

progressive sensorineural hearing loss. [cags.org.ae]

Sensorineural deafness with vestibular hypofunction and peripheral sensory impairment were also present, and intellect was normal. [scinapse.io]

Refsum Disease

The cardinal neurological manifestations of the disease include a demyelinating neuropathy, pes cavus, cerebellar ataxia, sensorineural deafness, anosmia and cranial nerve [path.upmc.edu]

The disease usually follows a progressive course, but acute and sub‐acute presentations have been described. [qjmed.oxfordjournals.org]

The disease usually follows a progressive course, but acute and sub-acute presentations have been described. [path.upmc.edu]

Deafness, Autosomal Dominant 23

Symptoms - Deafness- autosomal dominant nonsyndromic sensorineural 23 Progressive deafness Causes - Deafness- autosomal dominant nonsyndromic sensorineural 23 Not supplied [checkorphan.org]

sensorineural deafness, 600208 COL11A1 Marshall syndrome, 154780 MYH9 May-Hegglin anomaly, 155100 MITF Melanoma, cutaneous malignant, susceptibility to, 8, 614456 ATP1A2 [gsdseq.ir]

[…] susceptibility to, 192500 KCNQ1 Long QT syndrome-1, 192500 KCNE1 Long QT syndrome-5, 613695 COL11A1 Lumbar disc herniation, susceptibility to, 603932 MYH9 Macrothrombocytopenia and progressive [gsdseq.ir]

Gingival Fibromatosis-Progressive Deafness Syndrome

Title Other Names: Gingival fibromatosis with progressive deafness; GFD; Gingival fibromatosis with sensorineural hearing loss; Gingival fibromatosis with progressive deafness [rarediseases.info.nih.gov]

sensorineural deafness Rutherford syndrome[ 22 ] Corneal opacity, mental retardation and aggressive behavior, failure of tooth eruption Cross syndrome[ 23 ] Hypopigmentation [wjgnet.com]

GFD; Gingival fibromatosis with sensorineural hearing loss; Familial gingival fibromatosis associated with progressive deafness See More Categories: Jones syndrome is a [rarediseases.info.nih.gov]

Autosomal Recessive Deafness 42

UniProtKB/Swiss-Prot : 76 Deafness, autosomal recessive, 42: A prelingual, non-progressive form of non-syndromic sensorineural hearing loss. [malacards.org]

2, 3 : Congenital non-progressive sensorineural deafness or deficiency that is mild (26-40 dB) to profound (90 dB) Absence of systemic findings identified by clinical/physical [centogene.com]

sensorineural deafness (De Leenheer et al, 2001). [dizziness-and-balance.com]

Hereditary Corneal Dystrophy

OMIM 217400), Corneal dystrophy with progressive sensorineural deafness, Corneal dystrophy and sensorineural prelingual deafness, Harboyan syndrome. [ojrd.biomedcentral.com]

Congenital corneal dystrophy: progressive sensorineural deafness in a family. Arch Ophthalmol 1971 ; 85 : 27 –32. Magli A, Capasso L, Foa T, Maurino V, Ventruto V. [jmg.bmj.com]

Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome). [eyewiki.aao.org]

Autosomal Dominant Deafness 28

progressive sensorineural deafness, Corneal dystrophy and sensorineural prelingual deafness, Harboyan syndrome. [link.springer.com]

Macrothrombocytopenia and progressive sensorineural deafness, Deafness, autosomal dominant 17 AD 25 117 MYO15A Deafness AR 97 235 MYO3A Deafness AR 9 22 MYO6 Deafness, Deafness [blueprintgenetics.com]

Therefore, whenever a child presents with episodic or progressive ataxia and progressive sensorineural deafness, with or without neurologic or cutaneous symptoms, biotinidase [consilieregenetica.org]

High Myopia-Sensorineural Deafness Syndrome

Therefore, whenever a child presents with episodic or progressive ataxia and progressive sensorineural deafness, with or without neurologic or cutaneous symptoms, biotinidase [quizlet.com]

sensorineural deafness (De Leenheer et al, 2001). [dizziness-and-balance.com]

Autosomal Recessive Deafness 22

2, 3 : Congenital non-progressive sensorineural deafness or deficiency that is mild (26-40 dB) to profound (90 dB) Absence of systemic findings identified by clinical/physical [centogene.com]

Deafness is non-syndromic, sensorineural, and progressive. [academic.oup.com]

Congenital Deafness

Four members of a family had the heriditary syndrome of dominantly inherited progressive optic atrophy and congenital sensorineural deafness. [ncbi.nlm.nih.gov]

sensorineural deafness (De Leenheer et al, 2001). [dizziness-and-balance.com]

The classic phenotype is renal failure and progressive sensorineural deafness. The hearing loss is bilateral and correlated with age (Moon et al, 2009). [dizziness-and-balance.com]

Other Acquired Abnormality of Ear Ossicles

sensorineural deafness. [genecards.org]

Patients with surgically confirmed otosclerosis who show progressive sensorineural deafness disproportionate to age. 2. [drtbalu.co.in]

Deafness is non-syndromic, sensorineural, and progressive. [academic.oup.com]

Autosomal Recessive Deafness 103

Silengo, A syndrome of progressive sensorineural deafness and cataract inherited as an autosomal dominant trait. Clin.Genet. 41 (1992) 293–295. [link.springer.com]

[…] ataxia & progressive sensorineural deafness neurologic or cutaneous symptoms, consider biotinidase deficiency. [ncbi.nlm.nih.gov]

Superficial Siderosis of the Central Nervous System

Progressive sensorineural deafness, cerebellar ataxia, and pyramidal signs comprise the typical clinical presentation. [ncbi.nlm.nih.gov]

Clinically, the cardinal features are progressive sensorineural deafness and cerebellar ataxia. [imj.ie]

Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable [ncbi.nlm.nih.gov]

Autosomal Recessive Deafness 23

2, 3 : Congenital non-progressive sensorineural deafness or deficiency that is mild (26-40 dB) to profound (90 dB) Absence of systemic findings identified by clinical/physical [centogene.com]

Silengo, A syndrome of progressive sensorineural deafness and cataract inherited as an autosomal dominant trait. Clin.Genet. 41 (1992) 293–295. [link.springer.com]

Autosomal Dominant Deafness 9

Familial progressive sensorineural deafness is mainly due to the mtDNA A1555G mutation and is enhanced by treatment with aminoglycosides. [mafiadoc.com]

sensorineural deafness (De Leenheer et al, 2001). [dizziness-and-balance.com]

Isolated Congenital Sclerocornea

OMIM 217400), Corneal dystrophy with progressive sensorineural deafness, Corneal dystrophy and sensorineural prelingual deafness, Harboyan syndrome. [ojrd.biomedcentral.com]

PubMed Central View Article PubMed Google Scholar Harboyan G, Mamo J, Kaloustian V, der Karam F: Congenital corneal dystrophy, progressive sensorineural deafness in a family [ojrd.biomedcentral.com]

List of abbreviations CHED: Congenital hereditary endothelial dystrophy of the cornea CDPD: Corneal dystrophy with progressive sensorineural deafness PPCD: Posterior polymorphous [ojrd.biomedcentral.com]

Thrombocytopenia

sensorineural deafness, Deafness, autosomal dominant 17 AD 25 117 NBEAL2 Gray platelet syndrome AR 10 51 PRKACG Bleeding disorder, platelet-type, 19 AR 1 1 RBM8A *,# Thrombocytopenia [blueprintgenetics.com]

Thrombocythemia, Amegakaryocytic thrombocytopenia AD/AR 23 55 MYH9 Sebastian syndrome, May-Hegglin anomaly, Epstein syndrome, Fechtner syndrome, Macrothrombocytopenia and progressive [blueprintgenetics.com]

Autosomal Dominant Deafness 11

sensorineural deafness (De Leenheer et al, 2001). [dizziness-and-balance.com]

[…] postlingual sensorineural deafness maps to chromosome 14q12-13. [karger.com]

Cryopyrin-Associated Periodic Syndrome

Additionally, 70% of patients experience progressive sensorineural deafness, and 25% experience renal (amyloid A [AA]) amyloidosis that develops into renal dysfunction. [visualdx.com]

MWS is characterized by progressive sensorineural deafness as well as recurrent episodes of skin rash, fever and arthralgia (1). [medicaljournals.se]

Presbycusis

Loss-of-Function Mutations in the PRPS1 Gene Cause a Type of Nonsyndromic X-linked Sensorineural Deafness, DFN2. [deafness.about.com]

A Fork in the Road: Progressive Hearing LossHands and Voices. [deafness.about.com]

Polycystic Ovary Syndrome

Sensorineural deafness and diabetes mellitus were recognized features. 2006 Wiley‐Liss, Inc. [onlinelibrary.wiley.com]

We also demonstrate that the progressive extra‐pyramidal disorder, white matter disease, and abnormal signals of the basal ganglia are common features of this syndrome. [onlinelibrary.wiley.com]

Wells Syndrome

DEFINITION Muckle Wells Syndrome is a dominantly inherited autoinflammatory disease characterized by rashes, fever, arthralgia, progressive sensorineural deafness, and the [flipper.diff.org]

MWS is characterized by recurrent fever and urticarial rash, progressive sensorineural deafness and the development of secondary amyloidosis. [jpgmonline.com]

Hearing loss caused by progressive nerve damage (sensorineural deafness) typically becomes apparent during the teenage years. [coldallergy.org]

Autosomal Dominant Deafness 64

2, 3 : Congenital non-progressive sensorineural deafness or deficiency that is mild (26-40 dB) to profound (90 dB) Absence of systemic findings identified by clinical/physical [centogene.com]

Familial progressive sensorineural deafness is mainly due to the mtDNA A1555G mutation and is enhanced by treatment of aminoglycosides. [academic.oup.com]

Autosomal Recessive Deafness 1B

2, 3 : Congenital non-progressive sensorineural deafness or deficiency that is mild (26-40 dB) to profound (90 dB) Absence of systemic findings identified by clinical/physical [centogene.com]

In this study we performed a genome search to localize a gene for nonsyndromic deafness in a large Indonesian family with nonsyndromic, autosomal dominant progressive sensorineural [nejm.org]

Endocrine Dysfunction

Mutation in mitochondrial ribosomal protein S7 (MRPS7) causes congenital sensorineural deafness, progressive hepatic and renal failure and lactic acidemia. Hum. Mol. [nature.com]

Liver Cirrhosis

Hearing Loss Progressive sensorineural deafness (often related to chronic pressure in the brain from constant inflammation) can occur in many children with NOMID/CINCA, so [autoinflammatory.org]

[…] bilateral perceptive sensorineural deafness 20% Eye problems: affected in at least 80% incidence of eye problems increases with age average age of onset 4.5 years uveitis [dermnetnz.org]

[…] developmental retardation (50%) seizures (25%) spasticity of the legs – rare hypotonia – rare transient hemiplegia – rare Hearing loss: affects 75% begins in late childhood progressive [dermnetnz.org]