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45 Possible Causes for Moderate to Severe Congenital Sensorineural Hearing Loss

  • Usher Syndrome Type 2A

    Usher syndrome type II (USH2) should be suspected in individuals with: Congenital sensorineural hearing loss that is mild to moderate in the low frequencies and severe to[centogene.com] Note Defects in USH2A has been found in a patient with a form of non-syndromic sensorineural hearing loss. {ECO:0000269 PubMed:25388789}.[genecards.org] […] syndrome presents with congenital, profound sensorineural loss (see Figure 2A) and no vestibular function.[asha.org]

  • Congenital Deafness

    Usher type 2A Autosomal recessive USH2A Extracellular matrix protein Congenital moderate-to-severe sensorineural hearing loss (normal vestibular function) and retinitis pigmentosa[bmb.oxfordjournals.org] Usher type 1E Autosomal recessive PCD15 Protocadherin Profound congenital deafness, retinitis pigmentosa, vestibular arreflexia.[bmb.oxfordjournals.org]

  • Salti-Salem Syndrome

    […] on audiometry, absent vestibular function on formal testing and typical RP; 121 were diagnosed with USH2 on the basis of sloping moderate to severe congenital sensorineural[jmg.bmj.com] Results Clinical evaluation A total of 188 families were recruited for the NCUS study: 47 were diagnosed with USH1 on the basis of having profound congenital hearing loss[jmg.bmj.com] hearing loss on audiometry and normal vestibular function on formal testing with typical RP; four were diagnosed with USH3 on the basis of progressive sensorineural hearing[jmg.bmj.com]

  • Autosomal Recessive Deafness 76

    Usher type 2A Autosomal recessive USH2A Extracellular matrix protein Congenital moderate-to-severe sensorineural hearing loss (normal vestibular function) and retinitis pigmentosa[academic.oup.com] Usher type 1E Autosomal recessive PCD15 Protocadherin Profound congenital deafness, retinitis pigmentosa, vestibular arreflexia.[academic.oup.com]

  • Autosomal Dominant Deafness 64

    Usher type 2A Autosomal recessive USH2A Extracellular matrix protein Congenital moderate-to-severe sensorineural hearing loss (normal vestibular function) and retinitis pigmentosa[academic.oup.com] Usher type 1E Autosomal recessive PCD15 Protocadherin Profound congenital deafness, retinitis pigmentosa, vestibular arreflexia.[academic.oup.com]

  • Autosomal Dominant Deafness 11

    Usher type 2A Autosomal recessive USH2A Extracellular matrix protein Congenital moderate-to-severe sensorineural hearing loss (normal vestibular function) and retinitis pigmentosa[academic.oup.com] Usher type 1E Autosomal recessive PCD15 Protocadherin Profound congenital deafness, retinitis pigmentosa, vestibular arreflexia.[academic.oup.com]

  • Autosomal Recessive Deafness 84B

    Usher type 2A Autosomal recessive USH2A Extracellular matrix protein Congenital moderate-to-severe sensorineural hearing loss (normal vestibular function) and retinitis pigmentosa[academic.oup.com] Usher type 1E Autosomal recessive PCD15 Protocadherin Profound congenital deafness, retinitis pigmentosa, vestibular arreflexia.[academic.oup.com]

  • Autosomal Dominant Deafness 9

    Hearing loss can be classified based on age at onset (pre or post-lingual), type of ear defect (conductive, sensorineural or mixed), degree of hearing loss (mild, moderate[mafiadoc.com] Congenital/pre-lingual forms of deafness are always of sensorineural type, of which half are due to environmental factors (ototoxic drugs like aminoglycosides, cisplatin;[mafiadoc.com] , severe and profound), and can be syndromic/non syndromic3.[mafiadoc.com]

  • Conductive Hearing Loss

    Conductive hearing loss (CHL), far more common than sensorineural hearing loss in children, can be acquired or congenital, can range from mild to moderately severe, and can[ncbi.nlm.nih.gov]

  • 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] Individuals with Type 2 Usher Syndrome typically have a congenital moderate-to-severe sensorineural hearing loss and normal balance.[californiaearinstitute.com] Usher syndrome type II (USH2) should be suspected in individuals with: Congenital sensorineural hearing loss that is mild to moderate in the low frequencies and severe to[centogene.com]

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