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52 Possible Causes for Profound Prelingual Sensorineural Deafness

  • Chudley-McCullough Syndrome

    sensorineural deafness.[cags.org.ae] Shahin et al. (2010) and Walsh et al. (2010) reported a large consanguineous Palestinian kindred in which seven individuals had severe to profound prelingual, bilateral, non-syndromic[cags.org.ae]

  • Lethal Occipital Encephalocele - Skeletal Dysplasia Syndrome

    A form of non-syndromic sensorineural deafness characterized by prelingual, profound, non-progressive hearing loss.[nectarmutation.org] Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information[nectarmutation.org]

  • Myopia

    Deafness and myopia syndrome is characterized by moderate-profound, bilateral, congenital or prelingual deafness and high myopia.[ncbi.nlm.nih.gov] Autosomal recessive non-syndromic hearing loss is one of the most prevalent human genetic sensorineural defects.[ncbi.nlm.nih.gov]

  • Deafness

    Deafness and myopia syndrome is characterized by moderate-profound, bilateral, congenital or prelingual deafness and high myopia.[ncbi.nlm.nih.gov] Autosomal recessive non-syndromic hearing loss is one of the most prevalent human genetic sensorineural defects.[ncbi.nlm.nih.gov]

  • Deafness, Autosomal Recessive, Type DFNB1A

    Hearing loss, sensorineural, prelingual profound, vestibular dysfunction (in some patients) Deafness, Autosomal Dominant 3B; DFNA3B Deafness, Autosomal Recessive 1B, DFNB1B[genetics.ouhsc.edu] Type OMIM Gene Description Deafness, Autosomal Dominant 3A; DFNA3A Deafness, Autosomal Recessive 1A; DFNB1A 601544 220290 GJB2/CX26 Nonsyndromic neurosensory deafness, hearing[genetics.ouhsc.edu] 612643 612645 GJB6/CX30 Childhood-onset, progressive, moderate-to-severe high-frequency sensorineural hearing impairment.[genetics.ouhsc.edu]

  • Weissenbacher-Zweymuller Syndrome

    deafness characterized by prelingual, profound, non-progressive hearing loss.[innatedb.com] Deafness, autosomal recessive, 53 (DFNB53) [MIM:609706]: A form of non-syndromic sensorineural deafness characterized by prelingual, profound, non-progressive hearing loss[genecards.org] Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information[innatedb.com]

  • Deafness, Autosomal Dominant 23

    Hearing loss, sensorineural, prelingual profound, vestibular dysfunction (in some patients) Deafness, Autosomal Dominant 3B; DFNA3B Deafness, Autosomal Recessive 1B, DFNB1B[genetics.ouhsc.edu] The 10 affected members of the family, as well as the 11 affected members of the three other families, suffered from a prelingual severe to profound form of sensorineural[cags.org.ae] Type OMIM Gene Description Deafness, Autosomal Dominant 3A; DFNA3A Deafness, Autosomal Recessive 1A; DFNB1A 601544 220290 GJB2/CX26 Nonsyndromic neurosensory deafness, hearing[genetics.ouhsc.edu]

  • Pendred's Syndrome

    Salient Features 2-6 Ears : Deafness : Severe to profound Sensorineural deafness which usually starts early in childhood (prelingual).[genetics4medics.com] Also, deafness in PDS generally profound ( 60 dB) with prelingual onset [ 29 ], and sometimes a fluctuating but worsening course [ 30 – 32 ], consistent with a progressive[ojrd.biomedcentral.com] sensorineural hearing loss, inner ear anomalies such as Mondini’s dysplasia, EVA or vestibular anomalies, and goiter [ 26 – 28 ].[ojrd.biomedcentral.com]

  • Digitorenocerebral Syndrome

    Deafness, autosomal recessive, 86 (DFNB86) [MIM:614617]: A form of non-syndromic deafness characterized by prelingual onset of profound sensorineural hearing loss affecting[genecards.org] Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures syndrome (DOORS) [MIM:220500]: A syndrome characterized by sensorineural deafness, mental retardation[genecards.org]

  • Familial Infantile Myoclonic Epilepsy

    Deafness, autosomal recessive, 86 (DFNB86) [MIM:614617]: A form of non-syndromic deafness characterized by prelingual onset of profound sensorineural hearing loss affecting[genecards.org] Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures syndrome (DOORS) [MIM:220500]: A syndrome characterized by sensorineural deafness, mental retardation[genecards.org]

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