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Related Experiment Videos

Hereditary vestibulo-cochlear dysfunction and vascular disorders.

W I Verhagen1, P L Huygen, E J Theunissen

  • 1Department of Neurology, Canisius-Wilhelmina Hospital, Nijmegen, The Netherlands.

Journal of the Neurological Sciences
|August 1, 1989
PubMed
Summary
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[From gene to disease; non-syndromic, autosomal dominant, low-frequency sensorineural hearing loss (DFNA6/14)].

Nederlands tijdschrift voor geneeskunde·2003
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Hereditary cochleovestibular dysfunction due to a COCH gene mutation (DFNA9): a follow-up study of a family.

Clinical otolaryngology and allied sciences·2002
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Fluctuant, progressive hearing loss associated with Menière like vertigo in three patients with the Pendred syndrome.

International journal of pediatric otorhinolaryngology·2001
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Progressive fluctuant hearing loss, enlarged vestibular aqueduct, and cochlear hypoplasia in branchio-oto-renal syndrome.

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·2001
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The DFNA10 phenotype.

The Annals of otology, rhinology, and laryngology·2001
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A Dutch family with progressive autosomal dominant non-syndromic sensorineural hearing impairment linked to DFNA13.

Clinical otolaryngology and allied sciences·2001

A rare genetic condition causes progressive hearing loss and Dandy syndrome in a family, characterized by Dandy syndrome and oscillopsia. This autosomal dominant disorder appears linked to vascular issues, not typical neurological or ototoxic causes.

Area of Science:

  • Neuroscience
  • Genetics
  • Ophthalmology

Background:

  • Progressive autosomal dominant vestibulocochlear dysfunction can lead to significant neurological and auditory impairment.
  • Dandy syndrome, characterized by oscillopsia (visual disturbance) dependent on head movements, presents a diagnostic challenge.

Observation:

  • A family exhibited a unique progressive vestibulocochlear disorder.
  • Affected individuals presented with Dandy syndrome and sensorineural hearing loss.
  • No history of infectious diseases, neurological disorders, or ototoxic drug use was reported.

Findings:

  • The family's condition followed an autosomal dominant inheritance pattern.
  • A high incidence of vascular disorders, including hypertension, stroke, and myocardial infarction, was noted within the family.

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  • The vestibulocochlear dysfunction was progressive and linked to head movement-induced oscillopsia.
  • Implications:

    • This case highlights a potential genetic link between vestibulocochlear dysfunction, Dandy syndrome, and vascular disorders.
    • Understanding this genetic etiology may inform future diagnostic and therapeutic strategies for similar conditions.
    • Further research is warranted to elucidate the specific genetic mechanisms and their interaction with vascular health.