What’s already known about this topic?
- Ear, nose, and throat (ENT) problems frequently occur in MPS I, II,
and VI and are often the earliest clinical manifestations of these
diseases;
- Upper airway complications and obstructive sleep apnea (OSA) can be
caused by macroglossia, tonsilloadenoid of hypertrophy stiffness in
the upper airway, temporomandibular joints, nasal dysmorphism, flaccid
redundant supraglottic soft tissue, and tracheomalacia.
- Infiltration of the cochlear nerve afferents, cochlear nerves and
stria vascularis with GAGs causes sensorineural hearing loss;
- Other ENT disorders like persistent copious nasal discharge, chronic
recurrent rhinosinusitis, and adenotonsillar hypertrophy often occur
in MPS patients.