Hearing loss in enlarged vestibular aqueduct and incomplete partition type II

Ahadizadeh, Emily N.; Ascha, Mustafa; Manzoor, Nauman F.; Gupta, Amit; Semaan, Maroun T.; Megerian, Cliff A.; Otteson, Todd D.

doi:10.1016/j.amjoto.2017.06.010

Cited by 19 publications

(6 citation statements)

References 25 publications

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“…They are significant causative agents in congenital SNHL. 1 - 5 Incomplete partition (IP) malformations with normal external dimensions and abnormal internal cochlear structures comprise the most common IEM subgroup. IP malformations can be divided into IP-I, IP-II, and IP-III.…”

Section: Introductionmentioning

confidence: 99%

“… 7 - 8 Considering the etiology of LVA, the anomaly may be caused by either a secondary genetic mutation during embryonic development or environmental factors during early childhood. 5 The etiology of hearing loss (HL) in patients with LVA has not been fully elucidated. However, many mechanisms have been proposed, including cochlear dysplasia, damage to hair cells due to increased endolymphatic fluid pressure, and genetic factors.…”

Section: Introductionmentioning

confidence: 99%

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The Audiological Profile and Rehabilitation of Patients with Incomplete Partition Type II and Large Vestibular Aqueducts

Demirtaş,

Özbal Batuk,

Dinçer D'Alessandro

et al. 2024

JIAO

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The Audiological Profile and Rehabilitation of Patients with Incomplete Partition Type II and Large Vestibular Aqueducts

Demirtaş,

Özbal Batuk,

Dinçer D'Alessandro

et al. 2024

JIAO

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“…Classically, hearing loss (HL) in EVA patients is bilateral, predominantly affects higher frequencies, and ranges from mild to profound in severity [7,8]. Typically, HL is diagnosed between 3.5 and 5 years of age, but approximately 50% of cases may have a later onset and a progressive nature [9]. The progression may be rapid in early childhood [10] and associated with minor head trauma, infection, or delayed secondary hydrops [11].…”

Section: Introductionmentioning

confidence: 99%

Cochlear implantation in children with enlarged vestibular aqueduct (EVA): relationship to Pendred syndrome diagnosis, surgical outcomes, and radiological findings

Remjasz-Jurek¹,

Clarós²,

Clarós-Pujol³

et al. 2023

J Hear Sci

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IntroductionThe cochlear implant (CI) procedure in patients with inner ear malformations is challenging. The aim of this study was to evaluate auditory perception and speech development in children with enlarged vestibular aqueduct (EVA) and to relate the results to the diagnosis of Pendred syndrome (PS), imaging findings, surgical course, and postoperative period.Material and methodsThe study group consisted of 49 children with EVA, aged 11 months to 15 years, with severe to profound hearing loss. The EVA patients included 22 with PS and 27 with nonsyndromic EVA (NSEVA). The control group consisted of 46 children with nonsyndromic deafness. Outcomes after cochlear implantation were evaluated annually for at least 10 years. Auditory performance was assessed by categories of auditory performance (CAP) and Meaningful Auditory Integration Scale (MAIS). Speech outcomes were evaluated by Speech Intelligibility Rating Scale (SIR) and Meaning Use of Speech Scale (MUSS). Genetic counselling, imaging studies, and vestibular testing were also evaluated when available.ResultsAll patients included in the study benefited from cochlear implants, especially when implantation was performed before the age of 3 years. After CI, EVA patients (PS and NSEVA) achieved a steeper rate of increase in auditory perception and speech intelligibility, demonstrating higher scores at each follow-up point compared to nonsyndromic patients (NS). There were no differences in auditory and speech perception between NSEVA and PS patients. In addition to EVA, the most commonly diagnosed malformation was incomplete partition type 2 (IP-2), the presence of which negatively affected postoperative outcomes. During cochleostomy, cerebrospinal fluid (CSF)/perilymph leakage was observed in 50% of implanted ears, but its presence did not affect the final outcomes.ConclusionsEarly cochlear implantation is associated with satisfactory speech and auditory development in children with EVA. Due to the presence of inner ear malformations in patients with Pendred syndrome, detailed imaging of the temporal bone is indicated. Despite the frequent occurrence of CSF/perilymph leakage during cochleostomy, patients with EVA benefit satisfactorily from cochlear implantation.

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“…[2][3][4] It is found together with a dilated vestibule and an enlarged vestibular aqueduct. 3 The exterior dimensions (height and diameter) of a cochlea with IP-II are similar to those in normal cases. 2 Cochlear implantation is a standard treatment modality for children with severe to profound sensorineural HL.…”

mentioning

confidence: 99%

“…2 Incomplete partition type-II (IP-II), also known as the classic Mondini deformity or cochlear dysplasia, is an anomaly in which the apical part of the modiolus and the corresponding interscalar septa are defective, and the middle and apical turns coalesce to form a cystic apex. [2][3][4] It is found together with a dilated vestibule and an enlarged vestibular aqueduct. 3 The exterior dimensions (height and diameter) of a cochlea with IP-II are similar to those in normal cases.…”

mentioning

confidence: 99%

Speech Perception and Sound Localization Skills in Inner Ear Malformations: Children With Incomplete Partition Type‐II

Kocabay

Batuk

Sennaroğlu

et al. 2023

Otolaryngol.--head neck surg.

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Objective The present study aimed to evaluate binaural auditory skills in bimodal and bilateral pediatric cochlear implant (CI) users with incomplete partition type‐II (IP‐II) and to reveal the effect of IP‐II on performance by comparing the results to pediatric CI users with normal cochlear morphology. Study Design Cross‐sectional study. Setting Tertiary referral center. Methods Forty‐one CI users (mean age 8.8 ± 1.9) were grouped as bimodal (BIM‐IP) and bilateral (BIL‐IP) users with IP‐II; bimodal (BIM‐N) and bilateral (BIL‐N) users with normal cochlear anatomy. Speech perception in noise and sound localization skills were compared under 2 conditions; binaural (bilateral or bimodal) and monaural (first CI alone). Results BIM‐IP and BIL‐IP showed no performance difference in binaural tasks. The BIM‐N group showed remarkably poor performance in comparison to the groups of BIL‐IP (p = .007), BIM‐IP (p < .001), and BIL‐N (p = .004) in terms of speech‐in‐noise skills. In sound localization abilities, similar significant differences were found between the group of BIM‐N and the groups of BIL‐IP (p = .001), BIM‐IP (p < .001), and BIL‐N (p = .004). All groups showed statistically significant improvements in binaural condition on both tasks (p < .05). Conclusion We revealed that bilateral and bimodal pediatric CI users with IP‐II benefitted from implantation as much as bilateral users with normal anatomy. Differences in residual hearing between groups may explain the poor performance of bimodal users with normal cochlear morphology. To the best of our knowledge, it is the first study to unveil binaural performance characteristics in children diagnosed with a specific inner ear malformation subgroup.

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Hearing loss in enlarged vestibular aqueduct and incomplete partition type II

Cited by 19 publications

References 25 publications

The Audiological Profile and Rehabilitation of Patients with Incomplete Partition Type II and Large Vestibular Aqueducts

The Audiological Profile and Rehabilitation of Patients with Incomplete Partition Type II and Large Vestibular Aqueducts

Cochlear implantation in children with enlarged vestibular aqueduct (EVA): relationship to Pendred syndrome diagnosis, surgical outcomes, and radiological findings

Speech Perception and Sound Localization Skills in Inner Ear Malformations: Children With Incomplete Partition Type‐II

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