Progressive Hearing Loss: Causes & Reasons

Possible Causes for Progressive Hearing Loss

Five patients had progressive hearing loss and seven patients (32%) experienced sudden deafness. [ncbi.nlm.nih.gov]

Tinnitus and progressive sensorineural hearing loss are frequent complains. A stabilization of hearing function has been reported with enzyme replacement therapy (ERT). [ncbi.nlm.nih.gov]

Tinnitus occurred in the context of sudden deafness for 2 patients, and progressive hearing loss for 2 other patients. [ncbi.nlm.nih.gov]

Polyendocrine - Polyneuropathy Syndrome

DFNA71 is characterized by bilateral mild to moderate hearing loss before age 20 years, which gradually progresses to severe to profound hearing loss. [ghr.nlm.nih.gov]

[…] developmental delay and Progressive hearing impairment that can help you solving undiagnosed cases. [mendelian.co]

Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorinural hearing loss C1832466 OMIM100150HP:0001730 HP:0000408 Progressive sensorineural hearing impairment1 [mseqdr.org]

Ménière Disease

There are some with frequent vertigo attacks, progressive hearing loss and persistent annoying tinnitus even through the continuous standard medical treatments. [ncbi.nlm.nih.gov]

Abstract Ménière's disease is an inner ear disorder characterized by vertigo attacks, fluctuating and progressive hearing loss, tinnitus, and aural fullness in the affected [ncbi.nlm.nih.gov]

At last follow-up, the patient had not experienced any vertiginous attacks but was observed to have progressive hearing loss. [ncbi.nlm.nih.gov]

Presbycusis

When a parent hears the words "progressive hearing loss", there is a lot of uncertainty and anxiety. What is a progressive hearing loss? [deafness.about.com]

It usually involves both a loss of hearing sensitivity and a reduction in the clarity of speech. presbycusis Age-related hearing loss Audiology A progressive loss of hearing [medical-dictionary.thefreedictionary.com]

Definition (CSP) progressive bilateral loss of hearing that occurs in the aged. [66.199.228.237]

Congenital Deafness

Abstract In a family with perceptive, non-progressive hearing loss several of the members suffered from atopic dermatitis. [ncbi.nlm.nih.gov]

Patients with slowly progressing hearing loss should be checked for Alport Syndrome, Stickler Syndrome and Pendred Syndrome with a particular focus on a computed tomography [symptoma.com]

High frequency progressive sensorineural hearing loss. [bmb.oxfordjournals.org]

Acute Sensorineural Hearing Loss by Acute Acoustic Trauma

Progressive hearing loss is the main complication of acoustic trauma. Tinnitus (ear ringing) can also occur. [medlineplus.gov]

Bilateral progressive hearing loss: This is a condition that progresses over several months and is treated with corticosteroids and sometimes with drug therapy. [reverehealth.com]

Bilateral progressive hearing loss over several months, also diagnosed as autoimmune inner ear disease, is managed medically with long-term corticosteroids and sometimes with [hearingloss.org]

Acoustic Trauma

Abstract Progressive hearing loss after single episodes of acute acoustic trauma (Knalltrauma) has been reported in only a few cases. [ncbi.nlm.nih.gov]

Another 20 patients without progressive hearing loss for at least 3 years after acoustic trauma were assigned to Group B. [ncbi.nlm.nih.gov]

Clinical findings Partial, generally slowly progressive hearing loss, especially for high-pitched sounds, accompanied by ringing or other noises. [medical-dictionary.thefreedictionary.com]

Otosclerosis

This far-advanced otosclerosis often presents with a family history of hearing loss or a gradually progressive hearing loss that starts early in adult life. [emedicine.medscape.com]

There was no history of hearing loss (or CED) in her family. She had been suffering from progressive hearing impairment since childhood. [ncbi.nlm.nih.gov]

Symptoms include slowly progressive hearing loss, which is bilateral in 70% of cases. Vertigo is uncommon. [emedicine.medscape.com]

Autosomal Dominant Deafness 28

Individual chapters give coverage to balance disorders, tinnitus, progressive hearing loss, auditory neuropathy, central auditory processing disorders and unilateral hearing [books.google.com]

In general, hearing loss in DFNA6/14 is not progressive, however, some families were reported to have progressive hearing loss that could be attributed to presbycusis [ 28 [molvis.org]

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

Autosomal Dominant Deafness 9

1 Severe to profound hearing loss Abnormal vestibular function Delayed walking and sitting Type 2 mild to severe hearing loss normal vestibular function Type 3 Progressive [en.wikibooks.org]

Family #32 was identified as having early-onset, progressive sensorineural hearing loss, similar to the symptoms in DFNA9 patients with cochlin mutations in the vWFA domain [ncbi.nlm.nih.gov]

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

Autosomal Dominant Deafness 11

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

High frequency progressive sensorineural hearing loss. [academic.oup.com]

Autosomal Dominant Deafness 64

[…] nonsyndromic hearing loss, designated as DFNA64. [humpath.com]

hearing loss. [genecards.org]

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

Autosomal Recessive Deafness 103

Worsening speech or school performance may herald long-standing or progressive mild-to-moderate hearing loss. [emedicine.medscape.com]

(PMID: 10793131) Berryman M … Bretscher A (Molecular biology of the cell 2000) 2 3 4 22 60 Progressive hearing loss and vestibular dysfunction caused by a homozygous nonsense [genecards.org]

hearing loss (hearing loss was progressive within one year of detecting it) versus slowly progressive hearing loss (hearing loss was progressive within years after detecting [ejo.eg.net]

Autosomal Recessive Deafness 24

Other HEAD AND NECK American Journal of Neuroradiology June 2003, 24 (6) 1130-1132; Abstract Summary: A 31-year-old male patient presented with progressive mixed hearing loss [ajnr.org]

Rapidly progressing hearing loss was detected in 62 (62%) while 38 (38%) had slow progressive loss. Bilateral hearing loss was found in all of the cases. [karger.com]

Autosomal Recessive Deafness 44

Geno Mag Summary (A;A) 0 common in clinvar (A;G) 3 Carrier of a recessive deafness mutation [ PMID 16380907 ] GJB2 mutations and degree of hearing loss: a multicenter study [snpedia.com]

hearing loss. [ncbi.nlm.nih.gov]

loss Postoperative profound sensorineural hearing loss Premature canities Primary hypersomnia Profound sensorineural hearing loss Progressive sensorineural hearing loss and [icdlist.com]

Bilateral Sensorineural Hearing Loss

The case was diagnosed to be idiopathic, generalized gingival fibromatosis with progressive hearing loss. [ncbi.nlm.nih.gov]

hearing loss: A nonfamilial variant of Jones syndrome. ( 24963260 ) Gita B....Dembla G. 2014 9 Violaceous papules of the back: a quiz. [malacards.org]

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

Autosomal Recessive Deafness 23

Rapidly progressing hearing loss was detected in 62 (62%) while 38 (38%) had slow progressive loss. Bilateral hearing loss was found in all of the cases. [karger.com]

hearing loss. [ncbi.nlm.nih.gov]

Autosomal Recessive Deafness 22

Hearing loss progresses over three to four months, and an associated autoimmune disorder may be present. [aafp.org]

Autosomal Dominant Deafness 56

In general, hearing loss in DFNA6/14 is not progressive, however, some families were reported to have progressive hearing loss that could be attributed to presbycusis [ 28 [molvis.org]

hearing loss. [genecards.org]

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

Autosomal Recessive Deafness 76

Worsening speech or school performance may herald long-standing or progressive mild-to-moderate hearing loss. [emedicine.medscape.com]

A locus contributing to the early onset, rapidly progressing hearing loss of A/J mice ( ahl4, age-related hearing loss 4) was reported to map to chromosome 10 in a region [ingentaconnect.com]

High frequency progressive sensorineural hearing loss. [academic.oup.com]

Autosomal Recessive Deafness 42

Progression of low-frequency sensorineural hearing loss (DFNA6/14-WFS1). Arch OtoHNS 2003:129;421-42 Lesperance MM and others. [dizziness-and-balance.com]

In Alport's syndrome, there is a combination of progressive loss of kidney function and progressive sensorineural hearing loss, caused by mutations in the collagen α5 (IV) [nejm.org]

loss; autosomal dominant deafness type 3A; Hystrix-like ichthyosis with deafness AD, AR 3 GJB3 603324 erythrokeratoderma variabilis progressive; nonsyndromic hearing loss [centogene.com]

Autosomal Dominant Deafness 33

In general, hearing loss in DFNA6/14 is not progressive, however, some families were reported to have progressive hearing loss that could be attributed to presbycusis [ 28 [molvis.org]

Usher syndrome type III is characterized by progressive hearing loss and progressive deterioration of vestibular function. [consilieregenetica.org]

• We report the clinical and otopathologic findings in three persons from two kindreds affected with adult-onset autosomal dominant progressive sensorineural hearing loss. [jamanetwork.com]

Sensorineural Hearing Impairment

A Korean family presenting progressive hearing loss with migraine was ascertained. [ncbi.nlm.nih.gov]

A 38-year-old male with superficial siderosis presented with bilateral progressive sensorineural hearing loss. [ncbi.nlm.nih.gov]

Progressive hearing loss is a significant finding in patients with MWS. [ncbi.nlm.nih.gov]

Autosomal Recessive Deafness 1B

(PMID: 16804542) Delmaghani S … Petit C (Nature genetics 2006) 2 3 4 60 A p.C343S missense mutation in PJVK causes progressive hearing loss. [genecards.org]

Rapidly progressing hearing loss was detected in 62 (62%) while 38 (38%) had slow progressive loss. Bilateral hearing loss was found in all of the cases. [karger.com]

Autosomal Recessive Deafness 84

hearing loss. [cags.org.ae]

Rapidly progressing hearing loss was detected in 62 (62%) while 38 (38%) had slow progressive loss. Bilateral hearing loss was found in all of the cases. [karger.com]

High Myopia-Sensorineural Deafness Syndrome

Epidemiology 1-3/1000 live births - 1/1000 at birth and 2/1000 at 9-16years (due to delayed diagnosis, acquired hearing loss, late onset or progressive hearing loss). [starship.org.nz]

hearing loss. [nature.com]

Autosomal Recessive Deafness 38

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

Surveillance Sequential audiologic examinations are essential to: Document the stability or progression of the hearing loss; Identify and treat superimposed hearing losses [ncbi.nlm.nih.gov]

Lermoyez Syndrome

Tinnitis and progressive hearing loss followed by an attack of vertigo, after which hearing improves. [yourdictionary.com]

Ler·mo·yez syn·drome ( lār-mwah'yā ), increasing hearing loss and tinnitus preceding an attack of vertigo, after which the hearing improves. Variant of Ménière disease. [medical-dictionary.thefreedictionary.com]

Muckle-Wells Syndrome

The audiograms of members of the same family were compared to describe the family-specific risk of hearing loss progression. [ncbi.nlm.nih.gov]

Hearing impairment starts at high frequencies and can subsequently progress to profound hearing loss. Progression is age dependent. [ncbi.nlm.nih.gov]

The progressive nerve damage leads to sensorineural hearing loss that becomes evident during adolescence. [ivami.com]

Deafness, Autosomal Dominant 23

[…] ability in a zebrafish model of the progressive dominant hearing loss DFNA28. [genome.jp]

hearing loss [3]. [wikigenes.org]

Conclusions Progressive hearing loss is common in individuals with KCNQ4 mutations. [bmcmedgenet.biomedcentral.com]