The cases of cochleovestibular syndromes admitted into the O.R.L. ward during one year are statistically processed. 81 cases of hypoacusis of perception and vertiginous syndromes were admitted between January 1 and December 31, 1987. 42 of them were males and 29 females. 31 were pure cochlear syndromes, 32 vestibular syndromes without affecting the cochlea, and 15 were cochleovestibular syndromes. The paper surveys the available ways of investigation, by which a topographic and etiologic diagnosis can be determined, within certain limits and an adequate treatment can be recommended.
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Eur Arch Otorhinolaryngol
December 2024
King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Objective: Cochlear nerve deficiency (CND) accounts for about one-third of congenital hearing loss cases and presents several challenges during management. Currently, cochlear implant (CI) and auditory brainstem implants (ABI) represent the primary management options for CND. However, robust evidence on the outcomes of different CND management approaches remains limited.
View Article and Find Full Text PDFTher Adv Rare Dis
October 2024
Department of Otorhinolaryngology & HNS, Leiden University Medical Centre, Leiden, The Netherlands.
Here, we describe two congenitally deaf male siblings with the same compound heterozygotic, likely pathogenic mutations in the FGF3 gene, associated with the labyrinthine aplasia, microtia and microdontia (LAMM) syndrome. Both children had bilateral cochleovestibular aplasia, precluding cochlear implantation. The elder brother received an auditory brainstem implant (ABI) with very limited auditory responses.
View Article and Find Full Text PDFRev Med Interne
October 2024
Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France. Electronic address:
Eur Arch Otorhinolaryngol
October 2024
Department of Otorhinolaryngology, Department of Head and Skin, Ghent University Hospital, Ghent, Belgium.
Lancet Rheumatol
January 2025
Department of Internal Medicine, Hôpital Bichat-Claude Bernard, Assistance Publique Hôpitaux de Paris, Université Paris Cité, Paris, France; Centre de Recherche sur l'Inflammation, INSERM UMR1149, CNRS ERL8252, Faculté de Médecine site Bichat, Laboratoire d'Excellence Inflamex, Paris, France. Electronic address:
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