Malformations of the lateral semicircular canal correlated with data from the audiogram

Venkatasamy, Aïna; Foll, D. Le; Eyermann, C.; Vuong, Hella; Rohmer, Damien; Charpiot, Anne; Veillon, F.

doi:10.1007/s00405-019-05294-y

Cited by 6 publications

(4 citation statements)

References 12 publications

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“…Bilateral and unilateral malformations of the lateral semicircular canal are reported to be one of the most common radiological inner ear malformations and are associated with sensorineural as well as conductive hearing loss (16,17). Inner ear anomalies in 22q11DS patients, concerning the lateral semicircular canal have been reported previously.…”

Section: Discussionmentioning

confidence: 90%

Case Report: Challenging Otologic Surgery in Patients With 22q11.2 Deletion Syndrome

et al. 2020

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Patients with 22q11.2 deletion syndrome frequently have conductive hearing loss and/or chronic otitis media. Otologic surgery is often opted for. We present two patients undergoing otologic surgery. This case report outlines the typical otologic surgical challenges in patients with 22q11.2 deletion syndrome. Case one is a 52 year old male patient with chronic otitis media who underwent a mastoidectomy. The pre-operative CT scan showed a fused lateral semicircular canal and vestibule. Peroperatively, the lateral semicircular canal could not be used as a landmark to identify the facial nerve. Case two is a 10 year old female patient with conductive hearing loss. A middle ear inspection was performed where a bony epitympanic fixation of the malleus was encountered. In addition, the manubrium of the malleus was atrophic and also fixated. The bony fixation was removed, as was the manubrium of the malleus. Otologists should be aware of these typical anatomical variations in patients with 22q11.2 deletion syndrome. We recommend to use CT scanning of the middle and inner ear when preparing for otologic surgery in 22q11.2 deletion syndrome.

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Section: Discussionmentioning

confidence: 90%

Case Report: Challenging Otologic Surgery in Patients With 22q11.2 Deletion Syndrome

et al. 2020

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“…SC malformations were found as the most common inner ear malformations in all types of hearing loss in our study. Venkatasamy et al 29 found an association between SC malformations and different types of hearing loss. They suggested that multiple structures sharing the same embryological origin, such as the footplate of the stapes and labyrinth, derive from ectoderm, and may be affected by interruptions during embryogenesis, leading to various degrees of clinical outcomes.…”

Section: Discussionmentioning

confidence: 99%

“…They suggested that multiple structures sharing the same embryological origin, such as the footplate of the stapes and labyrinth, derive from ectoderm, and may be affected by interruptions during embryogenesis, leading to various degrees of clinical outcomes. 29 …”

Section: Discussionmentioning

confidence: 99%

The Relationship Between the Third Window Abnormalities and Inner Ear Malformations in Children with Hearing Loss

et al. 2021

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OBJECTIVE: To evaluate the relationship between the third window abnormalities and congenital inner ear malformations in pediatric patients with different types of hearing loss. If such a relationship should exist, it would be important to take it into account, in order to diagnose and treat pediatric hearing loss cases more accurately. Methods: Two hundred twenty-one children with hearing loss who had temporal bone computed tomography (CT) examination and were identified from 2013 to 2018 were retrospectively evaluated. The types of hearing loss were grouped as sensorineural hearing loss (SNHL), conductive hearing loss (CHL), and mixed hearing loss (MHL). Third window abnormalities included superior semicircular canal (SC) dehiscence, posterior SC dehiscence, enlarged vestibular aqueduct (EVA), X-linked stapes gusher, perilymph fistula, and bone dyscrasias. Congenital inner ear malformations included cochleovestibular, SC, and internal acoustic canal malformations. The relationships were analyzed with chi-square and Fisher’s exact tests. Results: In the study, 40 patients had unilateral hearing loss and 181 had bilateral hearing loss. In 402 ears, the rates of SNHL, CHL, and MHL were 88.5%, 6.9%, and 4.4%, respectively. EVA was the most common third window abnormality (41/402; 9.7%), and SC malformations were the most common inner ear malformations (53/402; 13.2%). In the SNHL group, superior and posterior SC dehiscence were associated with cochleovestibular malformations ( P = .035 and.020, respectively). In the CHL group, there was a relationship between EVA and SC malformations ( P = .041). No relationships were found in the MHL group. Conclusion: Third window abnormalities and congenital inner ear malformations may be encountered simultaneously in children with SNHL and CHL.

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“…3 Although lateral SCC malformation is itself not causative of hearing loss, its presence is strongly associated with comorbid sensorineural hearing deficits and should therefore be suggestive of possible underlying genetic or acquired embryological insult that globally impairs inner ear development and function. 4 While early embryological insults are associated with complete aplasia of the entire vestibular apparatus, later insults are thought to preferentially cause lateral SCC dysplasia as it is the last of the three canals to ossify. 3 Among patients with imaging-confirmed malformation of the lateral SCC, the most common pathologic finding was lateral SCC dilation, followed by complete aplasia and partial hypoplasia.…”

Section: Discussionmentioning

confidence: 99%

Partial Duplication of the Lateral Semicircular Canal—A Novel Anatomical Malformation in a Child with Barakat Syndrome

Kannan

Tran

2021

Journal of Pediatric Neurology

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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.

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Malformations of the lateral semicircular canal correlated with data from the audiogram

Cited by 6 publications

References 12 publications

Case Report: Challenging Otologic Surgery in Patients With 22q11.2 Deletion Syndrome

Case Report: Challenging Otologic Surgery in Patients With 22q11.2 Deletion Syndrome

The Relationship Between the Third Window Abnormalities and Inner Ear Malformations in Children with Hearing Loss

Partial Duplication of the Lateral Semicircular Canal—A Novel Anatomical Malformation in a Child with Barakat Syndrome

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