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69 Possible Causes for Bilateral Congenital Profound Sensorineural Hearing Loss, Congenital Onset

  • 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] […] profound Type II Congenital mild-severe sloping; progressive Progressive later onset Type III Retinitis Pigmentosa Congenital Onset prebalance puberty problems; absent caloric[studyslide.com] 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]

  • 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] USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness[genecards.org] Usher syndrome type I should be suspected in individuals with: Congenital (i.e., prelingual) profound bilateral sensorineural hearing loss No significant vestibular responses[centogene.com]

  • 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] Though the disease is defined by congenital bilateral deafness and a later onset of visual field loss, symptoms vary from person to person and progress at different rates.[institut-vision.org] Usher syndrome type I is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa.[ncbi.nlm.nih.gov]

  • Congenital Deafness

    hearing loss ranges from mild to profound hearing loss.[wvdhhr.org] Homepage Rare diseases Search Search for a rare disease Autosomal dominant optic atrophy and congenital deafness ORPHA:3212 Synonym(s): Konigsmark-Knox-Hussels syndrome Prevalence[orpha.net] Illustrative case histories are presented as well as priorities for future investigations into the pathology of congenital and genetic hearing loss.[ncbi.nlm.nih.gov]

  • Microtia

    […] canals, and bilateral profound sensorineural hearing loss (HL).[ncbi.nlm.nih.gov] Examples would be congenital or early onset of otosclerosis (calcium deposits develop on middle ear bones), middle ear structures damaged due to cholesteatoma , or changes[successforkidswithhearingloss.com] 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]

  • 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] One autosomal recessive subtype of LQTS, Jervell and Lange-Nielsen syndrome ( JLNS ), is characterized by congenital bilateral sensorineural hearing loss as well as early-onset[invitae.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]

  • Deafness

    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] RESULTS: In our cohort of congenital/early-onset SSD (n   210), the most prevalent cause in children was cochlear nerve deficiency (43.7%; 87 of 199 patients undergoing CT[ncbi.nlm.nih.gov] In specific, the present results emphasize that mutations in ACTG1 result in a diverse spectrum of onset ages, including congenital in addition to post‑lingual onset.[ncbi.nlm.nih.gov]

  • Usher Syndrome Type 1

    , profound sensorineural hearing loss, adolescent-onset retinitis pigmentosa and loss of vestibular function.[ltd.aruplab.com] Abstract Usher syndrome type I is an autosomal recessive disease characterized by profound congenital hearing impairment and vestibular dysfunction followed by the onset of[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]

  • Usher Syndrome Type 1D

    Usher syndrome type I is characterized by congenital, bilateral, profound sensorineural hearing loss, vestibular areflexia, and adolescent-onset retinitis pigmentosa.[ncbi.nlm.nih.gov] This subtype is characterized by congenital onset of moderate to severe hearing loss, onset of RP during or after puberty and normal vestibular function.[umd.be] 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]

  • Long QT Syndrome 6

    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] History & ECG: Onset and offset of syncopal episode Siblings, or family members with unexplained syncope or sudden death Family history of “seizures” or congenital deafness[de.slideshare.net] In some cases, LQTS may be associated with congenital profound bilateral sensorineural hearing loss, known as Jervell and Lange-Nielsen syndrome (JLNS).[mayomedicallaboratories.com]

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