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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
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Hearing Loss in Mucopolysaccharidosis
Cibele Gomes Bicalho1, Emília Katiane Embiruçu de Araújo Leão2,3, Álvaro Muiños de Andrade1
1Department of Otorhinolaryngology, Hospital Universitário Professor Edgard Santos, Salvador, BA, Brazil.
International Archives of Otorhinolaryngology
|August 11, 2021
Summary
Mucopolysaccharidosis (MPS) patients frequently experience hearing loss, primarily conductive, confirmed by audiology tests. Early otorhinolaryngological evaluation and hearing rehabilitation are crucial for managing this progressive condition.
Area of Science:
- Otorhinolaryngology
- Genetics
- Rare Diseases
Background:
- Mucopolysaccharidosis (MPS) comprises rare genetic disorders.
- Lysosomal enzyme deficiencies cause glycosaminoglycan (GAG) accumulation.
- GAG accumulation leads to multisystemic, chronic, and progressive symptoms.
Purpose of the Study:
- To characterize the otorhinolaryngological findings in MPS patients.
- To evaluate audiology test results in individuals with MPS.
- To highlight the importance of audiological assessment in MPS management.
Main Methods:
- A case series study design was employed.
- Initial evaluation included 24 patients with various MPS types (I, II, IIIA, IV, VI).
- Otorhinolaryngological examinations and audiology tests were conducted.
Main Results:
- Hearing loss was the most prevalent complaint.
- Audiology tests confirmed hearing loss in nearly all patients (almost 100%).
- Conductive hearing loss was the predominant type observed.
Conclusions:
- Comprehensive evaluation of complaints, physical exams, and audiology is vital for MPS patients.
- Otorhinolaryngologists should be integral to the multidisciplinary care team for MPS.
- Timely hearing monitoring and early rehabilitation are essential for improving outcomes in MPS.
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