Auditory and speech performance in children after cochlear implantation with clarionTM

Introduction: Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. Results: Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. Conclusion: Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

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Cochlear Implantation Outcomes in Observed Vestibular Schwannoma: A Preliminary Report

Otolaryngology ◽

10.1177/01945998211045903 ◽

2021 ◽

pp. 019459982110459

Author(s):

Elizabeth S. Longino ◽

Nauman F. Manzoor ◽

Nathan D. Cass ◽

Kareem O. Tawfik ◽

Marc L. Bennett ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Tumor Growth ◽

Vestibular Schwannoma ◽

Cochlear Implantation ◽

Volumetric Analysis ◽

Performance Outcomes ◽

Resonance Imaging ◽

Tertiary Referral Center ◽

Speech Performance

Objective Over the last decade there has been a trend toward observation for small nongrowing vestibular schwannoma (VS). Even without tumor growth, patients commonly experience ipsilateral hearing decline, and hearing rehabilitation remains challenging. This study analyzes hearing and speech performance outcomes after cochlear implantation (CI) in observed VS. Study Design Retrospective review. Setting Tertiary referral center. Methods Chart review was used to include patients with observed VS who had undergone ipsilateral CI, pre- and postimplantation audiometry, and speech performance. Tumor size pre- and postimplantation was measured with volumetric analysis. Results Seven patients with ipsilateral VS and CI were identified. Preimplantation tumor volume was 0.11 to 1.02 cm3. Five subjects were implanted with a straight electrode and two with a perimodiolar electrode. The average preimplant pure tone average was 91.3 dB (range, 80-117 dB) and 61.2 dB (range, 12-118 dB) for the implanted and nonimplanted ears, respectively. In all subjects with at least 1 year of listening experience (n = 6), consonant-nucleus-consonant word scores improved at 6 months and 1 year in the CI-alone and bimodal listening conditions. AzBio scores in quiet also improved at 6 months and 1 year. Of subjects with serial pre- and postoperative magnetic resonance imaging, volumetric analysis demonstrated no tumor growth. Conclusion Our results demonstrate that CI is a successful option for subjects with small nongrowing VS. All subjects had improved performance postimplantation. VS may continue to be observed with serial magnetic resonance imaging given increasing conditionality among CI manufacturers and ability to assess cerebellopontine angle extension despite implant artifact.

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The Impact of Postmeningitic Labyrinthitis Ossificans on Speech Performance After Pediatric Cochlear Implantation

Otology & Neurotology ◽

10.1097/mao.0000000000000877 ◽

2015 ◽

Vol 36 (10) ◽

pp. 1633-1637 ◽

Cited By ~ 8

Author(s):

Christopher C. Liu ◽

Melissa Sweeney ◽

Timothy N. Booth ◽

Kenneth H. Lee ◽

Joe W. Kutz ◽

...

Keyword(s):

Cochlear Implantation ◽

Labyrinthitis Ossificans ◽

Speech Performance ◽

The Impact

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Auditory Outcome after Cochlear Implantation in Children with DFNB7/11 Caused by Pathogenic Variants in TMC1 Gene

Audiology and Neurotology ◽

10.1159/000510156 ◽

2020 ◽

pp. 1-7

Author(s):

Samanta Gallo ◽

Patrizia Trevisi ◽

Chiara Rigon ◽

Ezio Caserta ◽

Dario Seif Ali ◽

...

Keyword(s):

Hearing Loss ◽

Functional Outcome ◽

Cochlear Implantation ◽

Autosomal Recessive ◽

Pediatric Patients ◽

Panel Testing ◽

Pathogenic Variants ◽

Excellent Functional Outcome ◽

Speech Performance ◽

Syndromic Hearing Loss

Introduction: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogeneity. So far, more than 100 pathogenic or likely pathogenic variants in TMC1 gene have been reported in patients with autosomal recessive hearing loss (HL) DFNB7/11. The prevailing auditory phenotype of individuals with DFNB7/11 is congenital, profound, bilateral HL, but the functional outcome after cochlear implantation (CI) described in the literature is variable. The objective of this work is to evaluate the auditory outcome after CI in pediatric patients with DFNB7/11, born to non-consanguineous parents. Methods: A retrospective analysis of genetic and audiological data of DFNB7/11 patients followed up in a single Italian otolaryngology clinic was performed. Cases with biallelic pathogenic variants in TMC1 were selected from the cohort of children with non-syndromic hearing loss who had undergone CI and had been molecularly characterized by multigene panel testing. All patients underwent extensive audiological assessment, and the auditory outcome after CI was evaluated. Results: DFNB7/11 was diagnosed in a total of 3 patients from 2 non-consanguineous families; a novel disease-causing variant in TMC1 was detected [c.962G>A p.(Trp321*)]. All the affected children showed the typical DFNB7/11 phenotype characterized by prelingual, severe-to-profound HL. The patients showed an excellent functional outcome after CI; speech perception, nonverbal cognition, and speech performance were comparable to those of patients with DFNB1 deafness. Discussion/Conclusion: Our results do not support the variable auditory outcome reported in the literature, which may be affected by several social and environmental factors and by the genetic background.

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Systematic Review of Outcomes After Cochlear Implantation in Children With X-Linked Deafness-2

Otolaryngology ◽

10.1177/0194599820932138 ◽

2020 ◽

pp. 019459982093213

Author(s):

Joshua D. Smith ◽

Nour El-Kashlan ◽

Owen A. F. Darr ◽

Marc C. Thorne

Keyword(s):

Systematic Review ◽

Speech Perception ◽

Cochlear Implantation ◽

Auditory Perception ◽

Pure Tone Audiometry ◽

Cochrane Library ◽

Speech Performance ◽

The Mean ◽

Age Range

Objective Outcomes following cochlear implantation in children with X-linked deafness-2 are variable, resulting in challenges in appropriate preoperative counseling. To address this uncertainty, we performed a systematic review and synthesis of the literature on audiologic and speech outcomes after cochlear implantation in these patients to inform prognostic counseling. Data Sources PubMed, Embase, and Cochrane Library were queried for articles published between January 2000 and July 2019. Review Methods We performed a systematic review of all studies published between 2000 and 2019 that reported on (1) children with confirmed X-linked deafness-2 undergoing cochlear implantation and (2) formal assessment of hearing and/or speech capabilities postimplantation. Results Our initial database search yielded 313 articles. Fourteen articles met inclusion criteria. These studies reported on 61 children with X-linked deafness-2 who underwent implantation at a wide age range (1-29 years) for severe-profound sensorineural hearing loss of prelingual onset. The mean follow-up duration after implant activation was 32 months (range, 12-61). Outcome domains assessed at follow-up were heterogeneous, though each study employed at least 1 assessment of hearing (eg, pure tone audiometry), speech perception (eg, Early Speech Perception Test), or auditory perception (eg, Categories of Auditory Perception scores). In 10 of 14 studies, cochlear implantation afforded significant improvement in hearing and speech capabilities relative to preoperative performance or as compared with age-matched, normal-hearing controls. Conclusion The majority of studies demonstrate that cochlear implantation provides improvements in hearing and speech performance in patients with X-linked deafness-2. This information is valuable for decision making regarding cochlear implantation in these patients.

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The Effects of Gusher-Related Intracochlear Pressure Changes on Hearing Preservation in Cochlear Implantation: A Comparative Series

Audiology and Neurotology ◽

10.1159/000489599 ◽

2018 ◽

Vol 23 (3) ◽

pp. 181-186 ◽

Cited By ~ 1

Author(s):

William Crohan ◽

Jay Krishnaswamy ◽

Gunesh Rajan

Keyword(s):

Cochlear Implantation ◽

Large Change ◽

Lateral Wall ◽

Hearing Preservation ◽

Residual Hearing ◽

Electrode Arrays ◽

Significant Drop ◽

Speech Performance ◽

Intracochlear Pressure ◽

Pressure Changes

Aim: To investigate and compare residual hearing preservation between patients based on the presence of intraoperative gusher. Methodology: We retrospectively compared 2 cohorts of cochlear implant recipients significantly distinguished by whether or not they experienced gusher intraoperatively. Patients underwent cochlear implantation using 24-mm lateral wall electrode arrays as well pharmacologic steroid protection. All patients were assessed by a hearing implant MDT. Hearing preservation rates and speech perception outcomes were assessed at 1, 6, 12, 24, 36, 48, and 60 months. Results: The patients with no gusher demonstrated complete hearing preservation. The patients with gusher demonstrated significant postoperative reduction of hearing thresholds, which declined at a significantly higher pace during follow-up. All patients demonstrated significantly better speech performance after cochlear implantation. Conclusion: The present study suggests that intraoperative gusher is associated with a significant drop in residual hearing, both immediately and over time, which may be related to the large change in intracochlear pressure intraoperatively.

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Three-Year Postimplantation Auditory Outcomes in Children with Sequential Bilateral Cochlear Implantation

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348940911800504 ◽

2009 ◽

Vol 118 (5) ◽

pp. 336-344 ◽

Cited By ~ 30

Author(s):

Fanny Scherf ◽

Lieselot Van Deun ◽

Astrid van Wieringen ◽

Jan Wouters ◽

Christian Desloovere ◽

...

Keyword(s):

Cochlear Implantation ◽

Linear Regression Analysis ◽

Older Children ◽

Time Intervals ◽

Speech In Noise ◽

Younger Age ◽

Bilateral Condition ◽

Speech Performance ◽

Almost All ◽

Better Than

Objectives: We report on the auditory abilities and speech performance in quiet and noise of 35 children with sequential bilateral cochlear implantation after 3 years of bilateral implant use. Methods: Testing was done in bilateral and both unilateral listening conditions. The assessments took place before the second implantation and at several time intervals after fitting. As different auditory tests were used, the children were categorized by their age at the second implantation: Younger or older than 6 years. Results: The pure tone averages for the bilateral condition were significantly better than those for either unilateral condition after 12 months of bilateral implant use and remained so from that test interval onward. The speech recognition outcomes in quiet and noise also improved significantly for almost all children after 36 months, although a linear regression analysis showed a beneficial effect of younger age at first implantation on the speech-in-noise results. Conclusions: Bilateral cochlear implantation offered advantages to all children in comparison with the first implant — Even the children who received the second implant after the age of 6 years. Compared to the younger children, the older children needed a longer adjustment period to gain bilateral benefit. However, they obtained similar results after 2 years of bilateral implant use.

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Audiological and Speech Performance After Cochlear Implantation in Cochlear Aplasia Deformity

Cureus ◽

10.7759/cureus.16654 ◽

2021 ◽

Author(s):

Salman F Alhabib

Keyword(s):

Cochlear Implantation ◽

Speech Performance

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Auditory and speech performance after unilateral cochlear implantation for cochlear nerve canal stenosis

Ear Nose & Throat Journal ◽

10.1177/01455613211045563 ◽

2021 ◽

pp. 014556132110455

Author(s):

Si Zhang ◽

Line Wang ◽

Shusheng Gong ◽

Fenqi Gao ◽

Pan Liu ◽

...

Keyword(s):

Cochlear Implantation ◽

Speech Intelligibility ◽

Cochlear Nerve ◽

Profound Hearing Loss ◽

Positive Correlation ◽

Canal Stenosis ◽

Auditory Rehabilitation ◽

Speech Performance ◽

Auditory Integration

To explore the correlation between the width of the bony cochlear nerve canal (CNC) and long-term auditory rehabilitation after unilateral cochlear implantation (CI) in pediatric patients with congenital deafness and bilateral cochlear nerve canal stenosis (CNCS). A retrospective review was performed on 10 patients with bilateral CNCS and bilateral congenital profound hearing loss who each underwent unilateral cochlear implantation. The width of the CNC was determined on computed tomography (CT) imaging and following CI, auditory and speech performance following CI were graded using categories of auditory performance (CAP), speech intelligibility rating (SIR), and the meaningful auditory integration scale (MAIS) at 24 months following implantation. No correlation was noted between CAP score and CNCS at 24 months post CI ( P > .05). A positive correlation was noted between SIR score and CNC width (ρ = .81, P < .05). Similarly, a positive correlation was noted between MAIS and CNC width (ρ = .71, P < .05). The width of the CNC in patients with CNCS is positively correlated with some long-term auditory and speech outcomes after CI.

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Cochlear Implantation in Patients with Chronic Suppurative Otitis Media: Surgical Outcomes and a Management Algorithm

Audiology and Neurotology ◽

10.1159/000505509 ◽

2020 ◽

Vol 25 (3) ◽

pp. 151-157 ◽

Cited By ~ 2

Author(s):

Young Hoon Yoon ◽

Jong Bin Lee ◽

Jee-Hye Chung ◽

Ki Wan Park ◽

Bong Jik Kim ◽

...

Keyword(s):

Otitis Media ◽

Cochlear Implantation ◽

Surgical Outcomes ◽

Open Cavity ◽

Single Stage ◽

Suppurative Otitis Media ◽

Mastoid Pneumatization ◽

Management Algorithm ◽

Speech Performance ◽

Surgical Options

Background: Chronic suppurative otitis media (CSOM) was considered as a contraindication of cochlear implantation (CI) in the past. Recently, various surgical options have been adopted for CI in CSOM patients with showing a low complication rate. Objectives: To evaluate surgical outcomes of CI in patients with CSOM and to propose a management algorithm for those patients. Methods: Thirty-six consecutive patients with CSOM who underwent single stage or staged CI were enrolled. Speech performance, including Categories of Auditory Performance (CAP) test and sentence score, and complications were retrospectively analyzed. Results: The average follow-up was 3.1 years (range 0.5–9.2 years). Postoperative median CAP and sentence scores were 6 and 78%, respectively. Three (8.3%) of the 36 patients had postoperative complications. One experienced breakdown of the ear canal closure. Recurrence of the pars tensa retraction was observed in another patient with adhesive otitis media who underwent CI and cartilage tympanoplasty as a single stage operation. Electrode extrusion occurred in another patient who underwent staged CI with maintenance of a previous open cavity. Subtotal petrosectomy and cavity obliteration were used to manage the latter 2 complications. All implant patients with good mastoid pneumatization exhibited no complications. There were no significant differences in postoperative speech performance and complication rates between single stage CI and staged CI. Based on these current findings, a management algorithm was proposed according to type of CSOM, presence of open cavity, and mastoid pneumatization. Conclusions: Patients with CSOM show good postoperative speech performance after CI. Proper surgical options according to type of CSOM, presence of open cavity, and mastoid pneumatization may help in reducing complications.

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