Desempenho de audição e linguagem pós implante coclear em crianças com síndrome de Waardenburg

Polanski, José Fernando; Kochen, Ana Paula; Oliveira, Carolina Arcoverde de

doi:10.1590/2317-1782/20202018295

Cited by 4 publications

(11 citation statements)

References 12 publications

(15 reference statements)

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“…Five studies reported on intra‐ or post‐operative complications 15,24,28,31,38 . A total of 12 complications were reported in as many patients.…”

Section: Resultsmentioning

confidence: 99%

“…All studies were published between 2000 and 2022. Study characteristics are summarized in Table 1 15,24–38 …”

Section: Resultsmentioning

confidence: 99%

“…33 Thirteen studies reported on the type of implant used. 15,[24][25][26][27][28][29][30][31][32]37,38 Reporting on WS type was variable, with 10 studies specifying the subtype of the condition based on clinical manifestation of the syndrome. 24,25,27,28,30,32,34,37,38 Five studies reported on genetic analysis in 29 patients following identification of hearing loss or clinical diagnosis; range of mutations including SOX10/PAX3/SNA12/ SW2B/SW2C/EDN3/EDNRB/MITF, though the method of identification of these mutations was not reported in any series.…”

Section: Demographicsmentioning

confidence: 99%

“…15,[24][25][26][27][28][29][30][31][32]37,38 Reporting on WS type was variable, with 10 studies specifying the subtype of the condition based on clinical manifestation of the syndrome. 24,25,27,28,30,32,34,37,38 Five studies reported on genetic analysis in 29 patients following identification of hearing loss or clinical diagnosis; range of mutations including SOX10/PAX3/SNA12/ SW2B/SW2C/EDN3/EDNRB/MITF, though the method of identification of these mutations was not reported in any series. 24,25,30,34,37 Radiological assessment with either preoperative computed tomography (CT) or magnetic resonance imaging (MRI) was reported in 100 patients across 10 studies (Table 1) 15,24,25,27,28,[30][31][32]34,37 ; findings varied from normal anatomy, to malformations of the cochlea, vestibule or semicircular canals.…”

Section: Demographicsmentioning

confidence: 99%

“…The Meaningful Auditory Integration Scale (MAIS) or the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used most frequently across six studies to assess postoperative speech perception. 24,26,30,32,37,38 Polanski et al, reported both pre and post intervention IT-MAIS/MAIS demonstrating improvement from average scores of 3%-90%. Categories of Auditory Performance (CAP) were also commonly used to assess postoperative performance in six studies, 24,27,[29][30][31][32] with two also utilizing CAP preoperatively.…”

Section: Audiological Outcomesmentioning

confidence: 99%

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Outcomes of Cochlear implantation in early‐deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis

Lovett

Eastwood

Metcalfe

et al. 2023

Laryngoscope Investig Oto

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ObjectiveThis systematic review aims to establish the expected hearing and speech outcomes following cochlear implantation (CI) in patients with profound congenital deafness secondary to Waardenburg syndrome (WS).MethodsA systematic review of the literature and narrative synthesis was performed in accordance with the PRISMA statement. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on language or year of publication.ResultsSearches identified 186 abstracts and full texts. Of these, 16 studies met inclusion criteria reporting outcomes in 179 patients and at least 194 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 11 peri/post‐operative complications were reported. The methodological quality of included studies was modest, mainly comprising noncontrolled case series with small cohort size. All studies were OCEBM grade III–IV.ConclusionCochlear implantation in congenitally deafened children with Waardenburg Syndrome is a well‐established intervention as a method of auditory rehabilitation. Due to the uncommon nature of the condition, there is a lack of large‐scale high‐quality studies examining the use of cochlear implantation in this patient group. However, overall outcomes following implantation are positive with the majority of patients demonstrating improved audiometry, speech perception and speech intelligibility supporting its use in appropriately selected cases.

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“…Five studies reported on intra‐ or post‐operative complications 15,24,28,31,38 . A total of 12 complications were reported in as many patients.…”

Section: Resultsmentioning

confidence: 99%

“…All studies were published between 2000 and 2022. Study characteristics are summarized in Table 1 15,24–38 …”

Section: Resultsmentioning

confidence: 99%

Section: Demographicsmentioning

confidence: 99%

Section: Demographicsmentioning

confidence: 99%

Section: Audiological Outcomesmentioning

confidence: 99%

See 3 more Smart Citations

Outcomes of Cochlear implantation in early‐deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis

Lovett

Eastwood

Metcalfe

et al. 2023

Laryngoscope Investig Oto

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Ocular Manifestations in Patients with Sensorineural Hearing Loss

Parameswarappa

Zaheer

Chhablani

et al. 2022

JOVR

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(SNHL) can have a large impact on the outcome and treatment of pediatric patients. Due to the common co-incidence of ocular manifestations and SNHL in children, both ophthalmologic and hearing loss screening and routine examinations must be conducted to minimize adverse outcomes and worsening of pathology. Early evaluation and diagnosis is imperative for intervention and further development of the patient. Coincidence requires a thorough evaluation that includes a comprehensive history, examination, and diagnostic testing. In this article, a literature review was conducted to analyze the presentations of various diseases and syndromes, such as Alport Syndrome, Waardenburg Syndrome, Norrie Disease, Usher Disease, Stickler Syndrome, Marfan Syndrome, Congenital Rubella, and Hereditary Optic Neuropathies. We divided the various ocular pathologies into anterior and posterior segment presentations and associated systemic findings for better understanding. Additionally, this review aims to include an update on the management of patients with both ocular and hearing loss manifestations.

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Hearing characteristics and cochlear implant effects in children with Waardenburg syndrome: a case series

Fan¹,

Ni²,

Chen³

et al. 2022

Transl Pediatr

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Background: Waardenburg syndrome (WS) has high clinical and genetic heterogeneity. We aimed to investigate the clinical characteristics of children with WS, and to analyze the effect of cochlear implantation in children with WS who had severe sensorineural hearing loss. Methods:The clinical characteristics of children with WS diagnosed and treated in the past 5 years in the Department of Otolaryngology, Shanghai Children's Hospital were retrospectively analyzed. The 5 WS cases, including 2 males and 3 females, had bilateral severe sensorineural hearing loss. Cochlear implantation was performed between 8 and 21 months old. Audiology tests were conducted, including otoacoustic emissions (OAEs), auditory brainstem response (ABR), and multiple auditory steady-state evoked responses (ASSR). Preoperative computerized tomography (CT) and magnetic resonance imaging (MRI) were performed to evaluate the development of the inner ear and brain. All WS cases were evaluated for hearing and speech abilities before cochlear implantation and at 1 month, 6 months, 12 months, and 24 months after implantation.Results: Among the 5 cases, 3 were WS1, 1 was WS2, and 1 was WS4. All 5 cases received cochlear implantation, and postoperative CT showed that the implant position was good. The infant toddler meaningful auditory integration scale (IT-MAIS) and meaningful use of speech scale (MUSS) scores of all cases increased with hearing age, and IT-MAIS scores were lower than those of normal hearing children of the same age.Conclusions: Children with WS usually have hearing loss. In WS cases with severe sensorineural hearing loss, early cochlear implantation can achieve better hearing and speech development.

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Desempenho de audição e linguagem pós implante coclear em crianças com síndrome de Waardenburg

Cited by 4 publications

References 12 publications

Outcomes of Cochlear implantation in early‐deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis

Outcomes of Cochlear implantation in early‐deafened patients with Waardenburg syndrome: A systematic review and narrative synthesis

Ocular Manifestations in Patients with Sensorineural Hearing Loss

Hearing characteristics and cochlear implant effects in children with Waardenburg syndrome: a case series

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