Progressive Sensorineural Deafness: Causes & Reasons

Possible Causes for Progressive Sensorineural Deafness

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]

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

sensorineural deafness and amyloidosis. [dermnetnz.org]

CINCA Syndrome

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

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]

Richards-Rundle Syndrome

sensorineural hearing loss. [cags.org.ae]

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

[…] spinocerebellar ataxia, sensorineural hearing loss... 245100 - richards-rundle syndrome; rrns - ataxia-deafness-mental retardation syndrome Richards-Rundle syndrome, A very [acronymattic.com]

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]

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]

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

Cogan Syndrome

Clinical Characteristics Ocular Features: This is a disorder of impaired smooth ocular pursuit movements. Voluntary horizontal eye movements are absent or defective while vertical gaze and random eye movements are usually retained. Patients learn early to compensate by sharply turning the head in a jerky,[…] [disorders.eyes.arizona.edu]

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]

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]

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]

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]

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). Orphanet J Rare Dis 2008;3:28. 19. [reviewofophthalmology.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]

Distal Renal Tubular Acidosis

Molecular investigation and long-term clinical progress in Greek Cypriot families with recessive distal renal tubular acidosis and sensorineural deafness due to mutations [scielo.org.co]

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]

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]

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 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]

Usher Syndrome Type 1D

Disease Overview Usher syndrome is a genetic disorder characterized by sensorineural hearing loss or deafness and progressive vision loss due to retinitis pigmentosa. [rarediseases.org]

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]

Usher Syndrome Type 1B

Overview Usher syndrome is a genetic disorder characterized by sensorineural hearing loss or deafness and progressive vision loss due to retinitis pigmentosa. [rarediseases.org]

Sensorineural hearing means it is caused by abnormalities of the inner ear. [rarediseases.org]

Congenital Cataract with Deafness and Hypogonadism

[…] spinocerebellar ataxia, sensorineural deafness, and signs of peripheral neuropathy. [semanticscholar.org]

Progressive hearing loss, ataxia, and polyneuropathy became evident in the third decade. [semanticscholar.org]

Journal of Medical Genetics}, year={1991}, volume={28}, pages={884 - 885} } Two sisters are described with a disorder characterised by mental retardation, congenital cataract, progressive [semanticscholar.org]

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]

Fitzsimmons-Watson-Mellor Syndrome

Search GEO for disease gene expression data for Spastic Paraplegia, Sensorineural Deafness, Mental Retardation, and Progressive Nephropathy. [malacards.org]

[…] paraplegia associated with bilateral sensorineural deafness, intellectual disability, and progressive nephropathy. [rareguru.com]

Familiar spastic paraplegia, bilateral sensorineural deafness, and intellectual retardation associated with a progressive nephropathy. [clinicalsurgeryjournal.com]

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]

Abstract Brown-Vialetto-Van Laere (BVVL) syndrome is a rare disorder with clinical features that include progressive bilateral sensorineural deafness and a variety of cranial [ncbi.nlm.nih.gov]

Mitochondrial Non-Syndromic Sensorineural Deafness

We report here a large family of Balinese Indonesian origin with progressive/congenital sensorineural deafness who carry the A1555G mutation. [link.springer.com]

Moral Familial progressive sensorineural deafness is mainly due to the mtDNA A1555G mutation and is enhanced by treatment with aminoglycosides Am J Hum Genet, 62 (1995), pp [elsevier.es]

Barcelo Familial progressive sensorineural deafness is mainly due to the mtDNA A1555G mutation and is enhanced by treatment of aminoglycosides Am J Hum Genet, 62 (1998), pp [elsevier.es]

Usher Syndrome Type 2C

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

Overview Usher syndrome is a genetic disorder characterized by sensorineural hearing loss or deafness and progressive vision loss due to retinitis pigmentosa. [rarediseases.org]

Sensorineural nerve deafness may be profound or mild, and may be progressive. [rarediseases.org]

Usher Syndrome Type 1E

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

CAPOS Syndrome

[…] atrophy, sensorineural deafness, a pes cavus deformity, and abnormal eye movements. [orpha.net]

We report three family members who presented with a relapsing, early onset cerebellar ataxia, associated with progressive optic atrophy and sensorineural deafness. [ncbi.nlm.nih.gov]

[…] hearing loss (CAPOS syndrome) is a rare autosomal dominant neurological disorder characterized by early onset cerebellar ataxia, associated with areflexia, progressive optic [orpha.net]