Bilateral Congenital Profound Sensorineural Hearing Loss & Loss of Vision: Causes & Reasons

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Patient File

Sex unknown, Age unknown

Reported Symptoms

Bilateral Congenital Profound Sensorineural Hearing Loss, Loss of Vision,

Negated

None

Unsure

None

Possible Causes

Usher Syndrome Type 1H

Usher syndrome type I is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa. [ncbi.nlm.nih.gov]

[…] and vision loss beginning somewhat later in life. [ncbi.nlm.nih.gov]

Usher syndrome is a condition characterized by partial or total hearing loss and vision loss that worsens over time. [ghr.nlm.nih.gov]

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Usher Syndrome Type 1K

Usher syndrome type I is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa. [ncbi.nlm.nih.gov]

[…] and vision loss beginning somewhat later in life. [ncbi.nlm.nih.gov]

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

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Usher Syndrome Type 1

profound sensorineural hearing loss, adolescent-onset retinitis pigmentosa and loss of vestibular function. [ltd.aruplab.com]

Individuals with Usher Syndrome have hearing loss and vision loss. [evolvegene.com]

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

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Usher Syndrome Type 1D

Usher syndrome type I is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa. [ncbi.nlm.nih.gov]

An autosomal recessive condition characterized by sensorineural hearing loss and progressive loss of vision due to retinitis pigmentosa. [medical-dictionary.thefreedictionary.com]

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

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Usher Syndrome Type 1J

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

Usher syndrome is a relatively rare genetic disorder caused by a mutation in any one of 10 genes resulting in a combination of hearing loss and visual impairment, and is a [disabled-world.com]

Usher syndrome type I should be suspected in individuals with: Congenital (i.e., prelingual) profound bilateral sensorineural hearing loss No significant vestibular responses [centogene.com]

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Usher Syndrome Type 3B

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

Usher syndrome is a relatively rare genetic disorder caused by a mutation in any one of 10 genes resulting in a combination of hearing loss and visual impairment, and is a [disabled-world.com]

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Tietz Syndrome

From Wikidata Jump to navigation Jump to search monogenic disease that is characterized by congenital profound bilateral sensorineural hearing loss and generalized albino-like [wikidata.org]

Vitamins and Minerals VO2 Max Volleyball Walking Warm-Ups Water Polo Water Skiing Weight Belts Weight Gain Weight Training Weight/Fat Loss Wellness Wheelchair Exercise Wrestling [ptonthenet.com]

Hearing loss is always bilateral, congenital, sensorineural and profound. Psychomotor development is normal. [orpha.net]

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Alstrom Syndrome

Hearing All subjects showed a bilateral symmetrical moderate to profound sensorineural hearing loss. The onset of hearing loss was either congenital or early childhood. [journal.frontiersin.org]

[…] symptoms Cone Rod Dystrophy Sensorineural Hearing Loss Dilated Cardiomyopathy Obesity Insulin resistant Child 10-18 Lay terms Clinical terms Total loss of vision Not everyone [geneticdisordersuk.org]

Financial Services Advocacy Deaf/Hard of Hearing Diabetes Family Support Heart Disease/Defects Research Visually Impaired / Blind Counties Serviced Nationwide International [aboutspecialkids.org]

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Retinitis pigmentosa 40

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

A loss of side vision, or tunnel vision, is also common as RP progresses. [bostonlaser.com]

A loss of side vision, or tunnel vision, also is common as RP progresses. [nweyes.com]

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Retinitis Pigmentosa 23

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

Significance and relevance: The progressive loss of neurons from the retina underlies the loss of vision, and eventually blindness, that occurs in prevalent diseases such [brainscape.com]

Loss of vision 67 (69.8%) and night blindness 56 (58.3%) were the leading symptoms. [dovepress.com]

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Usher Syndrome Type 2A

Progressive loss of vision due to retinitis pigmentosa begins in late childhood or adolescence. [sema4.com]

The hearing loss can be partly compensated by providing patients with hearing aids or cochlear implants, but the loss of vision is currently untreatable. [repository.ubn.ru.nl]

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Familial Progressive Vestibulocochlear Dysfunction

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]

Loss of vision [ 4 ] usually starts with night blindness and this is followed by loss of peripheral vision. Some degree of tunnel vision can continue until quite late. [patient.info]

Gradual vision loss in the child occurs due to Retinitis Pigmentosa (RP). [usherkidsaustralia.com]

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Refsum Disease

Retinitis pigmentosa and sensorineural hearing loss Usher syndrome type I is characterized by a congenital, bilateral, profound sensorineural deafness, vestibular areflexia [ncbi.nlm.nih.gov]

Vision loss usually starts with a loss of night vision and over the years it can lead to complete blindness. [wiki.ggc.usg.edu]

The patient was a 4-year-old girl with severe hearing and visual impairment, together with failure to thrive. 5 Subsequently, in 2005, the first case of living-donor liver [pediatrics.aappublications.org]

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Non-Syndromic Ocular Stickler Syndrome Type 1

[…] type 1 patients have congenital bilateral profound hearing loss and absent vestibular function d) type 2 patients have moderate losses and normal vestibular function. e) [juniperpublishers.com]

A disease-causing mutation in any one of these genes can lead to vision loss. [cehjournal.org]

RP causes night blindness and progressive loss of peripheral vision in early stages and loss of central vision later in life. [journals.plos.org]

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Adult-Onset Autosomal Recessive Cerebellar Ataxia

bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa. [refsumdisease.org]

People with SCA7 develop loss of vision. SCA3 is also referred to as, 'Machado-Joseph Disease.' [disabled-world.com]

Vision loss is sometimes associated with SCA7. In adults, this vision loss may come on before the ataxia. [verywell.com]

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Usher Syndrome 3A

profound sensorineural hearing loss, adolescent-onset retinitis pigmentosa and loss of vestibular function. [ltd.aruplab.com]

Invest Ophthalmol Vis Sci 2008;49:2651-2660 Pennings RJ Huygen PL Orten DJ Wagenaar M van Aarem A Kremer H Kimberling WJ Cremers CW Deutman AF: Evaluation of visual impairment [karger.com]

Diagnostic methods Clinical diagnosis is based on findings of bilateral sensorineural hearing loss (symmetric, congenital and profound for type 1, and moderate to severe with [orpha.net]

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Infantile Refsum Disease

loss of central vision late in the course of the disease. [encyclopedia.com]

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Congenital Deafness

hearing loss ranges from mild to profound hearing loss. [wvdhhr.org]

However, not all cases Usher Syndrome lead to both hearing loss and vision loss. [babyhearing.org]

Visual impairment in severe and profound sensorineural deafness. Arch Dis Child. 1995;73(1):53–6. [smw.ch]

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Deafness

Mumps (Epidemic parotitis) may result in profound sensorineural hearing loss (90 dB or more), unilateral (one ear) or bilateral (both ears). congenital rubella (also called [en.wikipedia.org]

We showcase the use of gas-permeable (GP) contact lenses to provide useful, functional VA in a patient with long-standing visual impairment secondary to corneal neovascularization [ncbi.nlm.nih.gov]

[…] without balance and vision deficits. 2017 Institut Pasteur. [ncbi.nlm.nih.gov]

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Tricho-Retino-Dento-Digital Syndrome

Symptoms INHERITANCE: Autosomal recessive HEAD AND NECK: [Ears]; Bilateral congenital profound sensorineural hearing loss; [Mouth]; Diastema; [Teeth]; Oligodontia Ehlers-Danlos [findzebra.com]

Visual impairment is a common finding; amblyopia is the major cause. [emedicine.medscape.com]

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Congenital Cytomegalovirus Infection

One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, [ncbi.nlm.nih.gov]

However, from 80% to 90% will have complications within the first few years of life that may include hearing loss, vision impairment, and varying degrees of learning disability [en.wikipedia.org]

Vision Problems/Loss Vision problems or loss may occur in 10-20% percent of children with symptomatic congenital CMV. [nationalcmv.org]

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Long QT Syndrome 13

The Jervell and Lange-Nielsen syndrome is an autosomal recessive form of LQTS and is characterized by congenital profound bilateral sensorineural hearing loss and long QT [medicinenet.com]

He has 20% vision, memory loss and has been diagnosed with long QT syndrome. He is having physiotherapy, occupational therapy and speech and language therapy. [myheart.org.uk]

In some cases, LQTS may be associated with congenital profound bilateral sensorineural hearing loss, known as Jervell and Lange-Nielsen syndrome (JLNS). [mayomedicallaboratories.com]

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Craniodiaphyseal Dysplasia

Tietz syndrome Tietz syndrome is a genetic hypopigmentation and deafness syndrome characterized by congenital profound bilateral sensorineural hearing loss and generalized [amp.pharm.mssm.edu]

blindness Impaired hearing (progressive) deafness Other ocular problems Strabismus Bulging eyes (exophthalmos) Loss of binocular vision Hypertelorism Seizures Mental retardation [healthh.com]

[…] of vision [ 4 ] Deformation of ossicles due to bony overgrowth Significant brain compression Epilepsy Complete hearing inability Reduced air spaces of mastoid, middle ear [primehealthchannel.com]

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Hereditary Hyperekplexia

Norrie Disease Norrie disease (ND) is a very rare X-linked genetic disorder which primarily causes blindness or significant vision loss in boys from birth. [geneticdisordersuk.org]

impairment. [amp.pharm.mssm.edu]

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Autosomal Dominant Deafness 33

[…] born with profound congenital deafness. [docslide.net]

Symptoms usually manifest in the first decade of life, and, in most cases, visual impairment develops gradually over many years. [molvis.org]

During the second decade, night blindness and loss of peripheral vision become evident and inexorably progress. [consilieregenetica.org]

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Autosomal Recessive Deafness 23

[…] born with profound congenital deafness. [vdocuments.site]

None had visual impairment, also supporting this rule. [journals.plos.org]

This evaluation can provide families with information on etiology of hearing loss, prognosis for progression, associated disorders (e.g., renal, vision, cardiac), and likelihood [bcidaho.com]

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Autosomal Recessive Deafness 24

[…] born with profound congenital deafness. [vdocuments.site]

loss and whether balance or Medical Risk Factors for dual Sensory Impairments of Vision and Hearing … Read Full Source Investigation Of Sensorineural Hearing Loss Period [bighearingzone.com]

This evaluation can provide families with information on etiology of hearing loss, prognosis for progression, associated disorders (e.g., renal, vision, cardiac), and likelihood [bcidaho.com]

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Usher Syndrome Type 2D

[…] and vision impairment due to retinitis pigmentosa. [ncbi.nlm.nih.gov]

Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss that is mild to moderate in low frequencies and severe to profound in higher frequencies, [healio.com]

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Autosomal Recessive Deafness 46

[…] born with profound congenital deafness. [vdocuments.site]

Effects of hearing and vision impairments on the Montreal Cognitive Assessment. [zdoc.site]

This evaluation can provide families with information on etiology of hearing loss, prognosis for progression, associated disorders (e.g., renal, vision, cardiac), and likelihood [bcidaho.com]

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Autosomal Recessive Deafness 38

[…] born with profound congenital deafness. [vdocuments.site]

This evaluation can provide families with information on etiology of hearing loss, prognosis for progression, associated disorders (e.g., renal, vision, cardiac), and likelihood [bcidaho.com]

Apre-lingual bilateral sensorineural hearing impairment poses a substantial problem asit negatively impacts on the subjects ability to conduct a normal social life. [vdocuments.site]

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