;

ENT specialist

Common clinical symptoms of Hunter syndrome that ENT specialists will encounter include: frequent hearing loss (due to otitis media or other conductive impairment or sensorineural degeneration); breathing and chewing/swallowing difficulties and obstructive sleep apnoea due to respiratory obstructions (enlarged tonsils and adenoids, nasal obstructions); skeletal deformities; and abdominal distension.1,2

Otological and audiological evaluations performed every 6–12 months can be used to monitor hearing loss, while a diagnosis of the airway obstructions requires a comprehensive evaluation of the medical history of the patient, a current physical examination and imaging procedures. Pulmonary function can be tested using spirometry, and an overnight sleep study, conducted at a hospital or at home, can be used to evaluate the severity of obstructive sleep apnoea. For a more detailed assessment, a bronchoscopy can be used to evaluate respiratory involvement with a rigid bronchoscope and a flexible bronchoscope.1,2

As a paediatric otolaryngologist I think that frequently we’re the first line of seeing patients that have syndromes. Many times, we’re in a position, because we’re examining the head and neck in detail, day-in and day-out in children, we’re able to take in nuances that sometimes the paediatrician may overlook

- Dr David Karas

Managing airway problems in Hunter syndrome patients often involves surgery, but preparation is crucial: patients should be assessed by a multidisciplinary team that includes a cardiologist, an ENT specialist and an anaesthetist with experience in MPS diseases before any surgical procedure. However, due to the progressive nature of airway involvement, surgery may only provide a temporary solution and continual monitoring is important.2

 

Download an information leaflet for ENTs
For further information on the specialist management of Hunter syndrome patients by ENTs

Register for exclusive content

The contact information collected in this form will be used to subscribe you to receive electronic communication via email. *

You have the right to opt out of receiving electronic communications at any time by using the opt out link in the communication or by contacting us at privacyconnect@takeda.com

For more information on how Takeda processes your personal data, please refer to our Privacy Notice

Ok