Basic characteristics, diagnosis and symptoms of the four cases
Case 1 | Case 2 | Case 3 | Case 4 | |
CF or PCD | PCD | CF | CF | CF |
CF mutation | Carrying one CF mutation dF508 | dF508/R117H | dF508/Y1092X | dF508/dF508 |
Gender | F | F | F | F |
Age (years) | 35 | 57 | 35 | 44 |
Weight (kg) | 117 | 50 | 50 | 58 |
eGFR (mL/min) | >60 | >60 | >60 | >60 |
Mean tobramycin dose (mg/kg/24 hours) | 4.9 | 11.8 | 10.4 | 8.8 |
Treatment duration (days) | 27 | 21 | 15 | 21 |
Clinical/outpatient administration | Outpatient | Clinical | Outpatient | Both |
Time to vestibulotoxicity (days after first treatment day) | 21 | 25 | 13 | 30 |
Unilateral/bilateral vestibulotoxicity | Bilateral | Bilateral | Unilateral | Unilateral |
Reversible/irreversible vestibulotoxicity | Irreversible | Irreversible | Reversible | Reversible |
Diagnosis of vestibular hypofunction (electro-nystagmography (ENG)) | Bilateral vestibulopathy | No central vestibular hypofunction, bilateral non-irritable labyrinths | No central vestibular hypofunction, non-irritable right labyrinth | No central vestibular hypofunction, less irritable right labyrinth |
Description of symptoms | Dizziness, imbalance, reduced memory and concentration, increased imbalance with head movements | Dizziness, nausea, vomiting, increased dizziness with head movements, unable to walk, pressing headache, slurred speech | Dizziness, imbalance, increased dizziness with head movements, nausea and vomiting in the morning | Dizziness, cannot climb stairs, drive, cycle, walk alone |
Follow-up of symptoms | Rehabilitation process, questioning symptoms, symptoms partly disappear and are stable, dependent on mobility scooter | Rehabilitation process, questioning symptoms, symptoms remain, dependent on mobility scooter | Questioning symptoms, symptoms partly disappear | Questioning symptoms, physiotherapy, disappear over time |
Diagnosis hearing loss (audiogram) | Conductive hearing loss indicated by PCD | Bilateral high frequency perceptual loss | Unilateral left high frequency perceptual loss | Normal hearing |
CF, cystic fibrosis; PCD, primary ciliary dyskinesia.