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9 Possible Causes for Moderate to Severe Prelingual Sensorineural Hearing Loss

  • High Myopia-Sensorineural Deafness Syndrome

    Tackle your toughest challenges and improve the quality of life and long-term outcomes of your patients with authoritative guidance from Fanaroff and Martin s Neonatal-Perinatal Medicine. Drs. Richard J. Martin, Avroy A. Fanaroff, and Michele C. Walsh and a contributing team of leading experts in the field deliver a[…][books.google.de]

  • Usher Syndrome Type 1D

    sensorineural hearing loss, vestibular areflexia, and prepubertal onset of RP.[jmg.bmj.com] USH type II (USH2; OMIM 276901, 276905 and 605472) is characterised by moderate to severe hearing impairment, no vestibular impairment, and onset of RP in the first or second[jmg.bmj.com] […] cases can be clinically classified into three subtypes. 3, 4 USH type I (USH1; OMIM 276900, 276903, 276904, 601067, 602097, 602083 and 606943) is characterised by profound prelingual[jmg.bmj.com]

  • Usher Syndrome Type 3B

    sensorineural hearing loss, vestibular areflexia, and prepubertal onset of RP.[jmg.bmj.com] USH type II (USH2; OMIM 276901, 276905 and 605472) is characterised by moderate to severe hearing impairment, no vestibular impairment, and onset of RP in the first or second[jmg.bmj.com] […] cases can be clinically classified into three subtypes. 3, 4 USH type I (USH1; OMIM 276900, 276903, 276904, 601067, 602097, 602083 and 606943) is characterised by profound prelingual[jmg.bmj.com]

  • Lethal Ataxia with Deafness and Optic Atrophy

    Similar to CMTX5, the patients exhibited a “triad”: prelingual hearing loss, optic atrophy with early teen onset, and severe sensorineural motor neuropathy.[ncbi.nlm.nih.gov] […] with peripheral or optic neuropathy, prelingual progressive sensorineural hearing loss, and central nervous system impairment.[ncbi.nlm.nih.gov] Mild PRS-I deficiency (DFNX-2) results in non-syndromic progressive hearing loss whereas moderate PRS-I deficiency (CMTX5) and severe PRS-I deficiency (Arts syndrome) present[ncbi.nlm.nih.gov]

  • Perrault Syndrome Type 4

    Clinical findings Sensorineural hearing loss (SNHL) in men and women.[ncbi.nlm.nih.gov] […] from moderate with early-childhood onset to profound with prelingual ( congenital ) onset Ovarian dysfunction in women with a 46,XX karyotype Note: Sensorineural hearing[ncbi.nlm.nih.gov] SNHL is bilateral and ranges in severity from moderate with early-childhood onset to profound with prelingual ( congenital ) onset.[ncbi.nlm.nih.gov]

  • Perrault Syndrome Type 2

    Clinical findings Sensorineural hearing loss (SNHL) in men and women.[ncbi.nlm.nih.gov] […] from moderate with early-childhood onset to profound with prelingual ( congenital ) onset Ovarian dysfunction in women with a 46,XX karyotype Note: Sensorineural hearing[ncbi.nlm.nih.gov] SNHL is bilateral and ranges in severity from moderate with early-childhood onset to profound with prelingual ( congenital ) onset.[ncbi.nlm.nih.gov]

  • X-Linked Non-Syndromic Sensorineural Deafness Type DFN

    Clinical classification: HL can be classified on clinical grounds as: • Mild, moderate, severe, or profound • Syndromic or non-syndromic • Prelingual or postlingual • Sensorineural[studyres.com] , moderate if thresholds are between 40-60 dB, severe if thresholds are between 60-80 dB, and profound if thresholds are above 80 dB.[studyres.com] , conductive or mixed • Autosomal recessive, autosomal dominant, sex-linked, or mitochondrial inheritance Hearing loss is considered mild if thresholds are between 20-40 dB[studyres.com]

  • Perrault Syndrome 3

    Clinical findings Sensorineural hearing loss (SNHL) in men and women.[ncbi.nlm.nih.gov] […] from moderate with early-childhood onset to profound with prelingual ( congenital ) onset Ovarian dysfunction in women with a 46,XX karyotype Note: Sensorineural hearing[ncbi.nlm.nih.gov] SNHL is bilateral and ranges in severity from moderate with early-childhood onset to profound with prelingual ( congenital ) onset.[ncbi.nlm.nih.gov]

  • Usher Syndrome 3A

    sensorineural hearing loss, vestibular areflexia, and prepubertal onset of RP.[jmg.bmj.com] USH type II (USH2; OMIM 276901, 276905 and 605472) is characterised by moderate to severe hearing impairment, no vestibular impairment, and onset of RP in the first or second[jmg.bmj.com] […] cases can be clinically classified into three subtypes. 3, 4 USH type I (USH1; OMIM 276900, 276903, 276904, 601067, 602097, 602083 and 606943) is characterised by profound prelingual[jmg.bmj.com]

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