Progressive Sensorineural Deafness: Causes & Reasons

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Possible Causes for Progressive Sensorineural Deafness

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

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]

This type is characterized by retinitis pigmentosa and progressive sensorineural deafness. ( Arch Otolaryngol 105:353-354, 1979) Full Text Download PDF Full Text Cite This [jamanetwork.com]

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]

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]

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]

Cogan Syndrome

The electro-oculographic (EOG) features of both horizontal and vertical eye movements in congenital oculomotor apraxia (COMA) were not previously reported. A girl referred to the ophthalmologic department for abnormal eye movements was diagnosed as COMA. The same abnormal ocular movements were observed in her[…] [ncbi.nlm.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]

Autosomal Recessive Deafness 22

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]

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]

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]

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]

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]

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]

Bilateral Sensorineural Hearing Loss

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]

Hereditary Corneal Dystrophy

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]

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

Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome). Orphanet J Rare Dis 2008;3:28. 19. [reviewofophthalmology.com]

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 Dominant Deafness 11

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

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

Fazio-Londe Disease

The disease is characterized by progressive sensorineural deafness, bulbar palsy, and respiratory compromise. 1 The age of onset varies from 0.3–17 years, with a mean age [nature.com]

The BVVL syndrome is a rare neurological disorder of unknown etiology, characterized by progressive pontobulbar palsy associated with sensorineural deafness. [1] The sensorineural [pediatricneurosciences.com]

[…] ponto-bulbar palsy and bilateral sensorineural deafness that usually precedes neurological symptoms. [1], [2], [3] It was first described in 1894 and since then almost 65 [pediatricneurosciences.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]

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

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

Autosomal Dominant Deafness 28

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

Familial Isolated Hypoparathyroidism

Yumita S et al. (1986) Familial idiopathic hypoparathyroidism and progressive sensorineural deafness. [^] 5. [moldiag.com]

Familial idiopathic hypoparathyroidism and progressive sensorineural deafness. Tohoku J Exp Med 1986 ;148: 135 –41. 25. [nejm.org]

12 Deafness, Autosomal Dominant 12 Deafness, Nonsyndromic Sensorineural with Dentinogenesis Imperfecta, Type I Deafness, Progressive Deafness, Sensorineural, with Migraine [sequencing.com]

Usher Syndrome Type 1D

Overview A rare inherited disorder characterized by sensorineural deafness and progressive vision loss which starts during the first decade of life and blindness usually occurs [checkorphan.org]

USH1 is characterised by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness [medical-dictionary.thefreedictionary.com]

USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness [uniprot.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]

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]

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

Autosomal Recessive Deafness 44

[…] loss Renal tubular acidosis with progressive nerve deafness Retinitis pigmentosa, intellectual disability, deafness, hypogenitalism syndrome Seizure, sensorineural deafness [icdlist.com]

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

[…] children: pitfalls for genetic counseling. [ PMID 12189487 ] Progressive hearing loss, and recurrent sudden sensorineural hearing loss associated with GJB2 mutations--phenotypic [snpedia.com]

Cochleosaccular Degeneration - Cataract Syndrome

Silengo M.C., 1992 : A syndrome of progressive sensorineural deafness and cataract inherited as an autosomal dominant trait. [wwe.geoscience.net]

Sebastian syndrome, Fechtner syndrome and macrothrombocytopenia with progressive sensorineural deafness. [genecards.org]

DISEASE: Defects in MYH9 are the cause of macrothrombocytopenia with progressive sensorineural deafness (MPSD) [MIM:600208]. [pathwaycommons.org]

Brown-Vialetto-Van Laere Syndrome

Riboflavin transporter deficiency (RTD) is a progressive motor neuron disorder characterized by respiratory insufficiency, sensorineural deafness and progressive ponto-bulbar [monarchinitiative.org]

It is characterised by progressive sensorineural deafness, followed or accompanied by motor cranial nerve palsies. [dizziness-and-balance.com]

Bulbar Palsy, Progressive, with Sensorineural Deafness. The Catalogue for Transmission Genetics in Arabs, CTGA Database. [ipfs.io]

Usher Syndrome Type 2C

This type is characterized by retinitis pigmentosa and progressive sensorineural deafness. [jamanetwork.com]