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Charcot-Marie-Tooth Disease and Hearing Loss: A Systematic Review With Meta-Analysis.
Mills, John F; Heiland, Luke D; Nguyen, Shaun A; Close, Michaela F; Meyer, Ted A.
Afiliación
  • Heiland LD; Department of Otolaryngology, Medical University of South Carolina, South Carolina.
  • Nguyen SA; Department of Otolaryngology, Medical University of South Carolina, South Carolina.
  • Close MF; Department of Otolaryngology, Medical University of South Carolina, South Carolina.
  • Meyer TA; Department of Otolaryngology, Medical University of South Carolina, South Carolina.
Otol Neurotol ; 45(7): 732-739, 2024 Aug 01.
Article en En | MEDLINE | ID: mdl-38956759
ABSTRACT

OBJECTIVE:

To characterize the pattern of hearing loss in Charcot-Marie-Tooth (CMT) disease to help guide clinical management. DATABASES REVIEWED CINAHL, PubMed, and Scopus.

METHODS:

Two independent investigators selected studies on CMT patients with pure-tone average (PTA) and auditory brainstem response (ABR) data. Case reports, case series <5 patients, and data that overlapped with another study were excluded. Investigators performed data extraction, quality rating, and risk-of-bias assessment using the Newcastle-Ottawa Scale. Meta-analysis of mean difference using fixed/random effects models was used. Also, data were analyzed using a weighted one-way analysis of variance, with post-hoc Tukey's test for comparison.

RESULTS:

Ultimately, 6 prospective studies (N = 197) were included. The most common demyelinating subtype (CMT1A) had significantly prolonged ABR latency values across wave III (0.20 ms, 95% confidence interval [CI] 0.05-0.35), wave V (0.20 ms, 95% CI 0.01-0.39), waves I-III (0.20 ms, 95% CI 0.01-0.39), and waves I-V (0.20 ms, 95% CI 0.01-0.39) when compared to matched controls. The autosomal recessive demyelinating subtype (CMT4C) had significantly worse PTA when compared to the most common subtype (CMT1A) (Δ 28.93 dB, 95% CI 18.34-39.52) and nondemyelinating subtype (CMT2A) (Δ 28.3 dB, 95% CI 15.98-40.62).

CONCLUSIONS:

Patients with CMT can present with a variety of phenotypes depending on the causative mutation. The ABR interpeak latency values for the most common demyelinating form of CMT are delayed when compared to matched controls. Most subtypes have normal hearing thresholds, apart from CMT4C, which presents with mild hearing loss on average.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Charcot-Marie-Tooth / Potenciales Evocados Auditivos del Tronco Encefálico / Pérdida Auditiva Límite: Humans Idioma: En Revista: Otol Neurotol Asunto de la revista: NEUROLOGIA / OTORRINOLARINGOLOGIA Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Charcot-Marie-Tooth / Potenciales Evocados Auditivos del Tronco Encefálico / Pérdida Auditiva Límite: Humans Idioma: En Revista: Otol Neurotol Asunto de la revista: NEUROLOGIA / OTORRINOLARINGOLOGIA Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos