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167 Possible Causes for Profound Congenital Sensorineural Hearing Loss

  • Usher Syndrome Type 1

    Usher syndrome type I (USH1) is characterised by profound congenital sensorineural hearing loss, vestibular dysfunction, and prepubertal onset of retinitis pigmentosa.[ncbi.nlm.nih.gov] Three clinical subtypes have been described, of which the most severe form, USH type 1 (USH1), is characterized by profound congenital sensorineural hearing loss, constant[jamanetwork.com] Subjects III-1 and III-2 from Family 1 have profound congenital sensorineural hearing loss (SNHL). Their status with regard to retinitis pigmentosa (RP) is unknown.[nejm.org]

  • Usher Syndrome Type 1J

    Type 1 is associated with profound congenital sensorineural hearing loss and poor vestibular function, most commonly caused by mutation in the MYO7A gene.[webeye.ophth.uiowa.edu] Subjects III-1 and III-2 from Family 1 have profound congenital sensorineural hearing loss (SNHL). Their status with regard to retinitis pigmentosa (RP) is unknown.[nejm.org] Genes involved in Type I Usher syndrome type I is the most severe form of Usher syndrome and is characterized by congenital, profound, sensorineural hearing loss; vestibular[usher-syndrome.org]

  • 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] Hearing loss is always bilateral, congenital, sensorineural and profound. Psychomotor development is normal.[orpha.net] profound bilateral sensorineural hearing loss and generalized albino-like hypopigmentation of skin, eyes and hair that has material basis in mutation in the MITF gene on[wikidata.org]

  • Congenital Deafness

    Children with congenital or perinatally acquired profound sensorineural hearing loss ( 90 dB) may present by age 6-9 months, whereas those with lesser degrees of hearing loss[patient.info] hearing loss ranges from mild to profound hearing loss.[wvdhhr.org] In syndromic hearing loss, the auditory pathology may be conductive and/or sensorineural, unilateral or bilateral, symmetrical or asymmetrical, and progressive or stable.[wvdhhr.org]

  • 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]

  • Long QT Syndrome

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

  • Microtia

    […] canals, and bilateral profound sensorineural hearing loss (HL).[ncbi.nlm.nih.gov] OBJECTIVES: This article presents the first report of cochlear implantation in a patient with congenital aural atresia, microtia, dysplastic cochlea and internal auditory[ncbi.nlm.nih.gov]

  • 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] 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 predominant sensorineural high-frequency loss for type 2) associated with retinitis pigmentosa (pigment deposits on fundoscopy and a flat or diminished electroretinogram[orpha.net]

  • Deafness

    Children with congenital or perinatally acquired profound sensorineural hearing loss ( 90 dB) may present by age 6-9 months, whereas those with lesser degrees of hearing loss[patient.info] 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] Measles may cause auditory nerve damage but usually gives rise to a chronic middle ear problem giving rise to a mixed hearing loss.[en.wikipedia.org]

  • Middle Ear Cholesteatoma

    A recurrent cholesteatoma was observed in one ear; one patient experienced a postoperative profound sensorineural hearing loss.[ncbi.nlm.nih.gov] Conclusions: ITMC in adults may have both congenital and acquired origin. It may grow silently over many years and develops into a massive size before being detected.[mattioli1885journals.com] CONCLUSIONS: ITMC in adults may have both congenital and acquired origin. It may grow silently over many years and develops into a massive size before being detected.[ncbi.nlm.nih.gov]

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