Subscribe to RSS
Partial Duplication of the Lateral Semicircular Canal—A Novel Anatomical Malformation in a Child with Barakat Syndrome
Several known genetic causes of sensorineural deafness are associated with dysplasia of inner ear structures, including the cochlea and labyrinth. Here, we present a child with Barakat syndrome and sensorineural hearing loss, found to have multiple inner ear anomalies including partial duplication of the posterior limb of the left lateral semicircular canal. To our knowledge, duplication of the semicircular canal has not previously been reported. This finding expands our understanding of the range of anatomical variations observed in congenital inner ear malformations, and further characterizes the phenotypic manifestations of Barakat syndrome.
The local institutional review board approved this retrospective HIPAA-compliant study.
Received: 07 September 2021
Accepted: 03 November 2021
Article published online:
13 December 2021
© 2021. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
- 1 Barakat AJ, Raygada M, Rennert OM. Barakat syndrome revisited. Am J Med Genet A 2018; 176 (06) 1341-1348
- 2 Duncan JS, Lim KC, Engel JD, Fritzsch B. Limited inner ear morphogenesis and neurosensory development are possible in the absence of GATA3. Int J Dev Biol 2011; 55 (03) 297-303
- 3 Johnson J, Lalwani AK. Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope 2000; 110 (10 Pt 1): 1673-1679
- 4 Venkatasamy A, Foll DL, Eyermann C. et al. Malformations of the lateral semicircular canal correlated with data from the audiogram. Eur Arch Otorhinolaryngol 2019; 276 (04) 1029-1034
- 5 Zainol Abidin Z, Mohd Zaki F, Kew TY, Goh BS, Abdullah A. Cochlear nerve canal stenosis and associated semicircular canal abnormalities in paediatric sensorineural hearing loss: a single centre study. J Laryngol Otol 2020; 134 (07) 603-609
- 6 Kwak SH, Kim MK, Kim SH, Jung J. Audiological and vestibular functions in patients with lateral semicircular canal dysplasia and aplasia. Clin Exp Otorhinolaryngol 2020; 13 (03) 255-260