Dataset Information

Mitochondrial Disease and Hearing Loss in Children: A Systematic Review.

ABSTRACT:

Objectives

Hearing loss is a clinical symptom, frequently mentioned in the context of mitochondrial disease. With no cure available for mitochondrial disease, supportive treatment of clinical symptoms like hearing loss is of the utmost importance. The aim of this study was to summarize current knowledge on hearing loss in genetically proven mitochondrial disease in children and deduce possible and necessary consequences in patient care.

Methods

Systematic literature review, including Medline, Embase, and Cochrane library. Review protocol was established and registered prior to conduction (International prospective register of systematic reviews-PROSPERO: CRD42020165356). Conduction of this review was done in accordance with MOOSE criteria.

Results

A total of 23 articles, meeting predefined criteria and providing sufficient information on 75 individuals with childhood onset hearing loss was included for analysis. Both cochlear and retro-cochlear origin of hearing loss can be identified among different types of mitochondrial disease. Analysis was hindered by inhomogeneous reporting and methodical limitations.

Conclusion

Overall, the findings do not allow for a general statement on hearing loss in children with mitochondrial disease. Retro-cochlear hearing loss seems to be found more often than expected. A common feature appears to be progression of hearing loss over time. However, hearing loss in these patients shows manifold characteristics. Therefore, awareness of mitochondrial disease as a possible causative background is important for otolaryngologists. Future attempts rely on standardized reporting and long-term follow-up.

Level of evidence

NA Laryngoscope, 132:2459-2472, 2022.

SUBMITTER: Roesch S

PROVIDER: S-EPMC9790539 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Mitochondrial Disease and Hearing Loss in Children: A Systematic Review.

Roesch Sebastian S O'Sullivan Anna A Zimmermann Georg G Mair Alois A Lipuš Cvetka C Mayr Johannes A JA Wortmann Saskia B SB Rasp Gerd G

The Laryngoscope 20220221 12

<h4>Objectives</h4>Hearing loss is a clinical symptom, frequently mentioned in the context of mitochondrial disease. With no cure available for mitochondrial disease, supportive treatment of clinical symptoms like hearing loss is of the utmost importance. The aim of this study was to summarize current knowledge on hearing loss in genetically proven mitochondrial disease in children and deduce possible and necessary consequences in patient care.<h4>Methods</h4>Systematic literature review, includ ...[more]

PMID: 35188226

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Dataset Information

Mitochondrial Disease and Hearing Loss in Children: A Systematic Review.

Objectives

Methods

Results

Conclusion

Level of evidence

Publications

Mitochondrial Disease and Hearing Loss in Children: A Systematic Review.

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