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244 Possible Causes for Profound Sensorineural Deafness

  • Autosomal Recessive Deafness 24

    Affiliated tissues include brain, and related phenotype is profound sensorineural hearing impairment.[malacards.org] sensorineural deafness, whichcharacterises the rst form of autosomal recessivedeafness described (DFNB1).[vdocuments.site] Ben-Yosef et al. (2001) identified mutations in 4 families from Pakistan with congenital profound nonsyndromic sensorineural deafness in 159 families studied.[genome.jp]

  • Usher Syndrome Type 1J

    USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness[genecards.org] Born hard of hearing with a sloping sensorineural loss from mild loss in low frequencies to severe-profound loss in high frequencies. Onset of hearing loss 0-40yr.[dbproject.mn.org] Table 1: Types of Usher Syndrome Symptom Type 1 Type 2 Type 3 Hearing Loss Born deaf with profound hearing loss and have a "corner audiogram" with responses only to very loud[dbproject.mn.org]

  • Usher Syndrome Type 1K

    Usher syndrome type I is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa. Unless fitted with a cochlear implant, individuals do not typically develop speech. Retinitis pigmentosa (RP), a progressive, bilateral, symmetric[…][ncbi.nlm.nih.gov]

  • Usher Syndrome Type 1

    A 10-year-old boy whose speech was hardly intelligible had been diagnosed as having profound sensorineural deafness (120-dB loss) at the age of 20 months.[jamanetwork.com] Abstract Usher syndrome type I (USH1), the most severe form of this syndrome, is characterized by profound congenital sensorineural deafness, vestibular dysfunction, and retinitis[ncbi.nlm.nih.gov] It is an autosomal recessive disorder characterized by profound congenital sensorineural deafness, retinitis pigmentosa, and vestibular abnormalities.[ncbi.nlm.nih.gov]

  • Congenital Deafness

    INTRODUCTION: Children with severe to profound sensorineural deafness can acquire vocabulary and syntactic structures to communicate by oral language, after cochlear implant[ncbi.nlm.nih.gov] Deafness may be conductive, sensorineural or mixed.[bmb.oxfordjournals.org] The deafness in LQTS is sensorineural in mechanism, and this is the only type of deafness associated with this genetic disorder.[handsandvoices.org]

  • Growth Failure

    The patient also had profound sensorineural deafness and mental retardation.[nejm.org] Defects of IGF1 are also characterized by microcephaly, developmental delay and, in some cases, sensorineural deafness.[oncohemakey.com] Poor responses to sound were noted, and audiograms showed profound bilateral sensorineural deafness (auditory threshold, 90 dB).[nejm.org]

  • Presbycusis

    Genetics is one cause of sensorineural hearing loss. Half of all cases of profound deafness in children have a genetic source.[bodyandhealth.canada.com] Causes Hearing loss is a spectrum with minor hearing problems at one end and profound, complete deafness at the other.[bodyandhealth.canada.com] Sensorineural hearing loss is caused by damage to the inner ear or to the nerves that send sound to the brain.[bodyandhealth.canada.com]

  • Autosomal Recessive Deafness 38

    Usher syndrome type I caused by mutations in USH1C is an autosomal recessive sensory defect involving congenital profound sensorineural deafness, vestibular dysfunction, and[antibodies-online.com] sensorineural deafness, whichcharacterises the rst form of autosomal recessivedeafness described (DFNB1).[vdocuments.site] USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness[genecards.org]

  • Deafness with Labyrinthine Aplasia - Microtia - Microdontia

    Profound congenital sensorineural deafness is bilateral in all individuals reported to date.[ncbi.nlm.nih.gov] Diagnosis [ edit ] Diagnosis is based on clinical findings. ' Clinical findings' Profound congenital sensorineural deafness is present CT scan or MRI of the inner ear shows[en.wikipedia.org] This condition is characterized by profound bilateral congenital sensorineural deafness associated with inner ear anomalies, microtia type I that is typically bilateral, and[cags.org.ae]

  • Branchio-Oculo-Facial Syndrome

    We present a 4-year-old girl with congenital profound sensorineural deafness associated with inner ear malformation (incomplete partition type II, enlarged vestibule, and[ncbi.nlm.nih.gov] deafness.[ncbi.nlm.nih.gov] Previously reported amino acid substitutions in TFAP2A involved only DNA binding domain in four patients with BOF syndrome who were not reported to have profound sensorineural[ncbi.nlm.nih.gov]

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