Hearing Loss in Patients With Morquio Syndrome: Protocol for a Scoping Review (Preprint)

Abstract

UNSTRUCTURED Objective: This scoping review aimed to understand the extent and type of evidence in relation to physiopathology, classification, epidemiology, clinical management, and effect of therapy for hearing loss in patients with mucopolysaccharidosis (MPS) IVA. Introduction: Mild to moderate hearing loss is common in patients with MPS IVA. The hearing loss can be conductive, sensorineural, or mixed. However, in these patients, the mixed form is frequent, attributed to the combination of conductive and neurosensory elements, with a slowly progressive evolution. Conductive hearing loss may be secondary to recurrent upper respiratory tract infections, serous otitis media, and deformity of the ear ossicles due to accumulation of glycosaminoglycans (GAGs). Meanwhile, the sensorineural form is mainly attributed to the accumulation of GAG in the auditory system.. Inclusion criteria: This scoping review include participants of both sexes, without specific age, who are diagnosed with mucopolysaccharidosis IVA and who develop hearing loss as a comorbidity. None exclusion criteria (country, language or document type) will be applicable. Methods: This scoping review includes participants of both sexes, without specific age, who are diagnosed with MPS IVA and develop hearing loss as comorbidity. No exclusion criteria (country, language, or document type) will be applicable. The information sources will include experimental and quasi-experimental, analytical observational, observational, and qualitative studies. Unpublished literature will not be covered. Gray literature will not be covered. Two independent reviewers will participate in the process of screening the literature, paper selection, and data extraction, and this process will be performed blindly. When all manuscripts have been selected by two reviewers, disagreements that arise between the reviewers at each stage of the selection process will be resolved through discussion or with an additional reviewer. Results will be reported with descriptive statistics and diagrammatic or tabular displayed information as explained in the JBI guidelines.