Variations in the cochlear implant experience in children with enlarged vestibular aqueduct

Pritchett, Cedric V.; Zwolan, Teresa A.; Huq, Farhan; Phillips, Andrew W.; Parmar, Hemant; Ibrahim, Mohannad; Thorne, Marc C.; Telian, Steven A.

doi:10.1002/lary.25187

Cited by 23 publications

(27 citation statements)

References 31 publications

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“…Whether there is a difference in audiometric outcomes between these IEVA and IP-II malformations is unclear at present. This aspect was also studied in a retrospective study of 55 patients who had cochlear implantation (IEVA ¼ 18, IP-II ¼ 33, IP-I ¼ 3) (17). Mean age at implantation for IEVA group was 112.8 months which was significantly different from IP-II group (58.4 mo).…”

Section: Discussionmentioning

confidence: 99%

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome

Manzoor¹,

Wick²,

Wahba³

et al. 2016

Otology &Amp; Neurotology

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Bilateral sequential cochlear implantation can be performed safely in patients with EVA. Audiometric outcomes are excellent and comparable to pediatric cochlear implant patients with no malformations. CSF gusher rates can be minimized by trans-round window approach. Further long-term studies are needed to identify differences within IP-EVA spectrum deformities, audiometric outcomes, and proportions of EVA patients who will need cochlear implantation for hearing rehabilitation.

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Section: Discussionmentioning

confidence: 99%

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome

Manzoor¹,

Wick²,

Wahba³

et al. 2016

Otology &Amp; Neurotology

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“…An examination of the literature studies revealed that the hearing loss associated with IP-II and LVA malformations is heterogeneous, and the conductive component is not associated with middle ear pathologies [2,12,13,17,18,[20][21][22][23][24][25][26] . Roesch et al [25] found that patients with IP-II and isolated LVA malformations were diagnosed not only with severe to profound hearing loss but also with moderate asymmetrical hearing loss.…”

Section: Discussionmentioning

confidence: 99%

Audiological and Radiological Characteristics in Incomplete Partition Malformations

Batuk¹,

Çınar²,

Özgen³

et al. 2017

Int Adv Otol

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OBJECTIVE:To compare the audiological and radiological findings of patients with incomplete partition malformations (IPs) and analyze the relationship between the audiological and radiological findings. MATERIALS and METHODS:The study included 84 patients (168 ears) with IPs as follows: 26 patients with Type I;IP-I (41 ears), 54 patients with Type II;IP-II (108 ears), and 4 patients with Type III;IP-III (8 ears). Remaining 11 ears were diagnosed with other inner ear malformations. Air and bone conduction thresholds were determined with pure tone audiometry, and the air bone gap was recorded in all patients with IPs. Magnetic resonance imaging studies and computerized tomography scans of the temporal bones were analyzed using the PACS system of our university. RESULTS:It was found that all the ears with IP-I were diagnosed with severe to profound hearing loss. The degree of the hearing loss varied from mild to severe/profound in patients with IP-II. Severe to profound mixed hearing loss (MHL) was determined in all ears with IP-III. The air bone gap was larger in the lower frequencies in the IP-II cases diagnosed with MHL. There was not a significant difference between the air bone gap and the size of the vestibular aqueduct in ears with IP-II (p>0.05).CONCLUSION: Each type of IP has different audiological findings. Depending on the type and degree of the hearing loss, it is possible to choose the appropriate audiological intervention. Patients with IP should be evaluated according to the type of malformation.

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“…Although there have been many reports on the outcome of CI in patients with EVA, most of them focused on children and did not include the results of genetic analysis. 5,6,8,9,14,15) There are few reports on the outcome of CI performed in the adult period in patients with EVA. Miyamoto, et al 7) reported the outcome of CI in 14 adults and 9 children with EVA, and concluded that the auditory and speech recognition performance did not differ compared to control subjects.…”

Section: Discussionmentioning

confidence: 99%

“…A recent study suggested that children with isolated EVA demonstrated better speech performance following CI compared to those with concomitant cochlear anomalies, although statistical significance was not reached. 9) When managing hearing loss in patients with EVA, special patient characteristics have to be considered. There is a wide variability concerning the degree of residual hearing in the prelingual period that either progress gradually or suddenly aggravate in the adolescent or adult period.…”

Section: Introductionmentioning

confidence: 99%

“…[10][11][12][13] Despite the fact that EVA can result from various causes, most of the previous studies reporting the outcome of CI in patients with EVA have not included the genetic data. [6][7][8][9]14,15) A study by Mey, et al 16) included the results of genetic analysis but only reported the surgical outcome and complications of CI in patients with EVA. Lai, et al 17) reported the results of SLC26A4 mutations but did not compare the outcome of CI according to mutation types.…”

Section: Introductionmentioning

confidence: 99%

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Auditory Outcome of Cochlear Implantation in Adolescent and Adult Patients with Enlarged Vestibular Aqueduct and Biallelic SLC26A4 Mutations

Song¹,

Yoo²,

Choi³

et al. 2017

Korean J Otorhinolaryngol-Head Neck Surg

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Background and ObjectivesZZMutations of the SLC26A4 gene cause congenital hearing loss and enlarged vestibular aqueduct (EVA). A considerable proportion of patients with SL-C26A4 mutations have significant residual hearing at birth that eventually worsen and become the cause for cochlear implantation (CI) later in their adolescence or adulthood. We analyzed the auditory outcome and prognostic factors of CI in patients with EVA and biallelic SLC26A4 mutations showing progressive early-onset hearing loss, who eventually had implantation in their adolescent or adult periods. Subjects and MethodZZSixteen patients with EVA carrying biallelic SLC26A4 mutations who received CI after 12 years of age were included for analysis. The outcome and prognostic factors of CI were analyzed. The postoperative follow-up period ranged from 3 to 48 months. ResultsZZThe age at CI ranged from 12 to 44 years. The categories of auditory performance score was significantly improved after CI from 3.1 to 4.9 (p＜0.05). The mean sentence scores improved significantly in the auditory-visual and auditory-only conditions (p＜0.05). The significant prognostic factors were measurable bone conduction thresholds, preoperative residual hearing, recent history of sudden aggravation of hearing loss, and preoperative speech intelligibility rating scores. There was a tendency of lower postoperative sentence scores in the group with homozygous H723R mutation, but statistical significance was not reached. ConclusionZZDespite the early-onset of hearing loss, significant improvement in auditory performance can be expected after CI in adolescent and adult patients with EVA and biallelic SLC26A4 mutations. Significant prognostic factors should be considered in selecting candidates and preoperative counseling for CI.

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Variations in the cochlear implant experience in children with enlarged vestibular aqueduct

Abstract: 4.

Cited by 23 publications

References 31 publications

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome

Audiological and Radiological Characteristics in Incomplete Partition Malformations

Auditory Outcome of Cochlear Implantation in Adolescent and Adult Patients with Enlarged Vestibular Aqueduct and Biallelic SLC26A4 Mutations

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