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35 Possible Causes for Bilateral Congenital Profound Sensorineural Hearing Loss, Ear Deformity

  • Microtia

    […] canals, and bilateral profound sensorineural hearing loss (HL).[ncbi.nlm.nih.gov] Microtia and Other Ear Deformities Congenital ear deformities occur in many different syndromes or alone.[cranioutah.com] The Microtia – Congenital Ear Deformity Institute has evaluated and performed thousands of surgeries in order to correct children’s ear deformities.[microtia.net]

  • Congenital Deafness

    hearing loss ranges from mild to profound hearing loss.[wvdhhr.org] A case illustrating close resemblance between a genetically induced Scheibe malformation and rubella inner ear deformities is present.[ncbi.nlm.nih.gov] A temporal bone CT scan will be able to detect malformations of the inner ear like Mondini deformities, Michel aplasia, a dilated vestibular aqueduct and a dilation of the[symptoma.com]

  • Deafness

    These included a history of pre-implant meningitis, congenital inner ear deformity, and basilar skull fracture.[web.archive.org] 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] Sensorineural total deafness may occur as a result of congenital deformities, inner ear infections, or head trauma. 3) Mixed hearing loss This is a combination of conductive[medicalnewstoday.com]

  • Autosomal Recessive Deafness 24

    A CT scan of the right ear revealed deformation and fusion of the malleus and incus. The right vestibule was found to be bulbous and mildly dilated.[audiologyonline.com] […] born with profound congenital deafness.[vdocuments.site] Classical mondini inner ear deformity includes a reduce number of turns of cochlea, enlarged vestibule, abnormal semicircular canal and enlarged vestibular aqueduct 37.[ayubmed.edu.pk]

  • Autosomal Recessive Deafness 38

    […] born with profound congenital deafness.[vdocuments.site] A CT scan of the right ear revealed deformation and fusion of the malleus and incus. The right vestibule was found to be bulbous and mildly dilated.[audiologyonline.com] Classical mondini inner ear deformity includes a reduce number of turns of cochlea, enlarged vestibule, abnormal semicircular canal and enlarged vestibular aqueduct 37.[ayubmed.edu.pk]

  • Autosomal Recessive Deafness 23

    […] born with profound congenital deafness.[vdocuments.site] A CT scan of the right ear revealed deformation and fusion of the malleus and incus. The right vestibule was found to be bulbous and mildly dilated.[audiologyonline.com] Classical mondini inner ear deformity includes a reduce number of turns of cochlea, enlarged vestibule, abnormal semicircular canal and enlarged vestibular aqueduct 37.[ayubmed.edu.pk]

  • Hereditary Hyperekplexia

    Tietz syndrome Tietz syndrome is a genetic hypopigmentation and deafness syndrome characterized by congenital profound bilateral sensorineural hearing loss and generalized[amp.pharm.mssm.edu] […] of middle thumb and index finger with deformity, and scoliosis.[pediatricneurosciences.com] He had minor dysmorphic features in the form of high arched palate, prominent ears, flat bridge of nose, small eyes, malformation of both hands in the form of undue separation[pediatricneurosciences.com]

  • Autosomal Recessive Deafness 22

    […] born with profound congenital deafness.[vdocuments.site] Classical mondini inner ear deformity includes a reduce number of turns of cochlea, enlarged vestibule, abnormal semicircular canal and enlarged vestibular aqueduct 37.[ayubmed.edu.pk] Patients with the common Down's syndrome (Trisomy 21), often have inner ear malformations.[dizziness-and-balance.com]

  • Autosomal Recessive Deafness 103

    […] born with profound congenital deafness.[vdocuments.site] A CT scan of the right ear revealed deformation and fusion of the malleus and incus. The right vestibule was found to be bulbous and mildly dilated.[audiologyonline.com] A dedicated thin cut CT or MRI of the temporal bones is useful for detecting malformations of the inner ear (i.e., Mondini deformity, Michel aplasia, enlarged/dilated vestibular[ncbi.nlm.nih.gov]

  • Autosomal Dominant Deafness 33

    […] born with profound congenital deafness.[docslide.net] Classical mondini inner ear deformity includes a reduce number of turns of cochlea, enlarged vestibule, abnormal semicircular canal and enlarged vestibular aqueduct 37.[ayubmed.edu.pk] Patients with the common Down's syndrome (Trisomy 21), often have inner ear malformations.[dizziness-and-balance.com]

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